Beruflich Dokumente
Kultur Dokumente
The
5-Minute
Urology
Consult
Editor-in-Chief
3RD EDITION Leonard G. Gomella, MD, FACS
The Bernard W. Godwin Professor of Prostate Cancer
Chairman
Department of Urology
Sidney Kimmel Medical College
Associate Director, Jefferson Sidney Kimmel Cancer Center
Clinical Director, Jefferson Sidney Kimmel Cancer Network
Thomas Jefferson University
Philadelphia, Pennsylvania
Associate Editors
Gerald L. Andriole, MD, FACS
Arthur L. Burnett, II, MD, MBA, FACS
Robert C. Flanigan, MD, FACS
Thomas E. Keane, MB, ChB, FRCSI, FACS
Harry P. Koo, MD, FAAP, FACS
Judd W. Moul, MD, FACS
Raju Thomas, MD, MHA, FACS
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Third Edition
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PREFACE
I
am very pleased to present the third edition of The 5-Minute ally any topic can be searched for on the Internet, the ability to sort
Urology Consult. The first edition was released almost 15 years through the information presented, confirm the validity, and rapidly
ago, with the second edition published in 2010. The continuing find the specific information needed is often very time-consuming
advances in urology lead to this much-needed 2015 update. The and can be prone to error. Multiple studies have shown that many
goal of this book is to provide the reader with useful information in websites can contain erroneous, misleading, or out-of-date infor-
a quick reference format to help with the everyday care of patients mation. Readers of this book can be assured that the information
with urologic problems. This third edition has undergone extensive presented is held to the highest standards possible, as it is writ-
editing and updating to reflect the most current data possible at ten, reviewed, and further edited primarily by academic urologists
the time of publication. and other academic specialists. Every effort is made to present the
Urologic diseases and conditions are common problems seen by most up-to-date standards of care at the time of publication.
all health care providers. Almost one-third of all congenital disorders This book, a member of the popular 5-Minute Consult series
involve the genitourinary system, and the urinary tract accounts for published by Wolters Kluwer Health, generally follows the organi-
almost 25% of all solid tumors in adults. While this book is written zational formatting of the other books in the series. However, there
primarily for urologists, any health care practitioner who deals with are notable exceptions, as this book is focused on a primarily surgi-
urologic complaints and conditions should find the book a useful cal subspecialty. Section I: Urologic Diseases and Conditions
resource. Students of urology, residents and fellows preparing for provides information on more than 300 major topics in the field of
oral and written in-service examination, and practicing urologists urology. The style of this section, while similar to the other books
preparing for certification examinations will find the book a use- in the series, focuses more attention on the surgical management,
ful study aid. While primarily written for practitioners in the United where appropriate. Furthermore, evidence-based medicine refer-
States, the table of contents has been reviewed by our international ences, standard fare in the 5-Minute Consult series, are included
editorial board, which represent more than three dozen countries, in in this urology edition. This is representative of the trend in the field
an attempt to capture as many diseases and conditions as possible of medicine to assign levels of evidence to treatment recommen-
for international readers. dations (see page ix for a further discussion). A challenge with any
The broad array of topics addressed in this book is based on surgical discipline is that, when reviewing published literature, this
reviews of published literature, major textbooks, grand rounds case type of information is not as well represented as in other medi-
presentations, validated Internet resources, and actual patient con- cal disciplines. The reader will note that in this edition, the use of
sultations. Topics are meant to represent real-world clinical ques- evidence-based medicine is identified in chapters as appropriate.
tions from very broad to very specific topics. Some of the topics Many topics are further supported by algorithms and the enhnaced
may appear redundant, such as Section I topics Scrotum and tes- image library available in the ebook version provided along with the
ticle, mass and Testis, tumor and mass, adult, general consid- print version. Both ICD-9 and preliminary high level ICD-10 codes
erations. There is a deliberate reason for this, namely, to frame have been incorporated in preparation for the rollout of ICD-10 in
the thought process to differentiate scrotal masses from testicular late 2015.
masses when the presenting problem is not clear. If it is clearly Section II: Short Topics: A to Z consists of more than 1,300
a testicular mass, then the one topic deals effectively with that diseases, conditions, presenting complaints, or key concepts in the
setting. If it is not a clear mass in the testicle, the reader can ap- field that the practitioner must be aware of but may not be worthy
proach the problem more broadly in terms of a mass within the of a complete 2-page chapter. Section III has been greatly ex-
scrotum that may or may not involve the testicle. Coverage in- panded and now features nearly 90 visual algorithms to enhance
cludes adult and pediatric urology, as well as subspecialty areas many more clinically relevant topics. Section IV is dedicated ex-
of urology such as urologic oncology, endourology, female urology, clusively to a core discipline in our field, Urinalysis and Urine
neurourology, andrology, infectious diseases, and renal transplan- Studies. Section V: Alternative and Complementary Uro-
tation. It represents a core of essential must-know and practical logic Therapies is a focused review that is of interest to both
information specifically written for the field of urology. While some patients and caregivers alike. Section VI: Urologic Drug Ref-
surgical techniques are discussed, this is not meant to be a compre- erence is a very unique collection of information on hundreds of
hensive urologic surgical text. Numerous high-quality publications drugs used in urologic practice in the United States as well as some
address the finer points of urologic surgery. This book addresses traditionally nonurologic medications that are clinically significant
pre and post operative care as well as some intra-operative tech- to the urologic practitioner. Additional urologic applications not of-
niques; however the focus is on more global patient management ten found on the package insert for off-label use in daily care are
issues. included for many medications. These off-label applications are
I am often surprised when asked why medical books such as noted on the basis of published literature with additional input and
this are even necessary as a reference in the modern world be- the personal observations of the authors and editors. Finally, Sec-
cause there is so much information readily available on devices tion VII: Reference Tables is a collection of useful reference
such as smartphones via the Internet. While the reality is that virtu- information and forms. A media and image collection is available in
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vi r r r Preface
the ebook version of this book. Please see information inside front opportunity to work with. My personal interactions with the company
cover on how to access this content. and their willingness to discuss any and all issues relating to the
In any project of this magnitude, there are numerous individuals book are testimony to their corporate philosophy in respecting the
responsible for its success. I would like to thank the following in- authors opinions to develop the best educational products possible
dividuals who provided the initial guidance in 1996 to develop the in the field of medicine. Brian Brown, Keith Donnellan, and Bren-
first urology version of the 5-Minute Consult: Lippincott Williams & dan Huffman are the best partners a medical author could hope
Wilkins editors Carroll Cann and Craig Percy, and an early pioneer to work with. In the final production stages, David Saltzberg and
of the 5-Minute Consult concept, Dr. Mark Dambro. Thanks to my Harish Kumar kept everything moving to stay on schedule. Spe-
administrative assistants Denise Tropea and Barbara Devine, who cial thanks to Philadelphia-based friend and professional photog-
provided key support to keep the contributors and this edition or- rapher Robert Neroni, who captured the spirit of urology in our cover
ganized. A special thanks to the more than 370 authors and editors photo.
who took the time to contribute to this edition and the numerous Our children, Leonard, Patrick, Andrew, and Michael, deserve
contributors to the previous editions that laid a strong foundation credit for their encouragement and patience over the many years
for this third update. To my colleagues who served as Associate, of my time spent working on this project. In this edition, i am very
Consulting, Specialty and International Editors, my most sincere proud that a few of the boys were actually able to make tangible
gratitude and appreciation for the time you took to recruit authors, professional contributions.
create and review content. It is also with great sadness that one of Most importantly, I would like to thank my wife, Tricia, with
our international editors and an icon in the field of Urology, Pro- the usual and customary accolades that authors share about their
fessor John Fitzpatrick passed away during the completion of this spouses in acknowledging the love and support provided. However,
book. He will be missed by all but his numerous contributions to her attention to detail as a behind-the-scenes editorial partner and
our field will live on. skilled reviewer for final content of this book added a degree of
Residents from the Department of Urology of Thomas Jefferson accuracy that I could never have accomplished alone.
University and from the University of West Virginia deserve special Please contact me if you have corrections or suggestions on
acknowledgement. They supported the content of both Section II ways to improve future editions of the book. I hope that The 5-
Short Topics and Section III Algorithms. Their names appear in Minute Urology Consult will provide useful information to allow all
the contributor listing as having been authors but are not specifically of us to care for our urology patients in the best way possible.
recognized for their work in these sections. Now however, they are. LEONARD G. GOMELLA, MD
The editorial and production staff at Wolters Kluwer Health have leonard.gomella@jefferson.edu
distinguished themselves as the best publishing team I have had the www.urologyquestion.com
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EVIDENCE-BASED MEDICINE
E
vidence-based medicine (EBM) is generally defined as the ommendations can be found. However, we recognize that in a pri-
use of current best medical evidence to aid in making deci- marily surgical-based specialty such as urology, this area is not
sions about the care of an individual patient. While the ultimate yet as well defined as in more general areas of medical practice.
decision-making process for or against a given treatment must be As an illustrative example in a chapter on hypertension, the EBM
made between the patient and the health care provider, EBM seeks recommendation might read:
to assess the quality of evidence that a specific course of action
is based on. The underlying principle is the evaluation of medical Use thiazide diuretics as a first-line agent for the
interventions and the literature that supports these interventions in treatment of essential hypertension, as it has the great-
a systematic and organized fashion. Since its introduction as a con- est efficacy in preventing the vascular complications
cept in the modern medicine over 30 years ago, there has been in- of hypertension (5)[A].
creased emphasis on this concept in daily patient care. While there
are currently many different systems of EBM, we have adopted the The A designation, as noted in the algorithm later, implies this
5-Minute Clinical Consult standard of the SORT Taxonomy from recommendation is based on the highest-quality, patient-oriented
the American Academy of Family Physicians. The key components evidence, and should be followed. The number 5 refers to the
are summarized later. A full review of this article can be viewed source, which would be listed under the References heading
at http://www.aafp.org/afp/20040201/548.html. Throughout this as reference #5. Recommendations that are level A evidence are
edition of The 5-Minute Urology Consult, these evidence-based rec- shaded blue in the text.
C Recommendation based on consensus, usual practice, opinion, disease-oriented evidence, or case series
for studies of diagnosis, treatment, prevention, or screening.
r This implies that the reference used does not meet het A or B requirements; these are often treatments recommended
by consensus groups (such as the American Cancer Society). In some cases, they may be the standard of care. But
implicit in a groups recommendations is the bias of the group or author that supports the reference.
Modified from Domino FJ, ed. The 5-Minute Clinical Consult 2010. Philadelphia: Lippincott Williams & Wilkins; 2009.
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EDITORS
ix
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x r r r Editors
Editors r r r xi
xii r r r Editors
Editors r r r xiii
xiv r r r Editors
Editors r r r xv
xvi r r r Editors
CONTRIBUTORS
xviii r r r Contributors
Contributors r r r xix
xx r r r Contributors
Nilay M. Gandhi, MD Tomas L. Griebling, MD, MPH, FACS Jennifer E. Heckman, MD, MPH
Resident John P. Wolf 33-Degree Masonic Resident in Urology
James Buchanan Brady Urologic Distinguished Professor and Department of Urology
Institute Vice Chair of Urology and Faculty University of Wisconsin
Johns Hopkins Medical Institutions Associate Madison, Wisconsin
Baltimore, Maryland The Landon Center on Aging
Jason C. Hedges, MD, PhD
Department of Urology
Francisco Gelpi-Hammerschmidt, MD Assistant Professor
The University of Kansas
Chief Resident Department of Urology
Kansas City, Kansas
Department of Urology Oregon Health & Science
Thomas Jefferson University Jennifer A. Hagerty, DO University
Philadelphia, Pennsylvania Assistant Professor Portland, Oregon
Kristin A. Greco, MD Departments of Urology and Pediatrics Lauren N. Hendrix, MD
Resident Sidney Kimmel Medical College Resident
Department of Urology Division of Urology Division of Urology
Loyola University Medical Center Nemours/Alfred I. DuPont Hospital for Department of Surgery
Maywood, Illinois Children University of Kentucky
Wilmington, Delaware Lexington, Kentucky
Bradley C. Gill, MD, MS
Resident in Urology Sang Won Han, MD Amin S. Herati, MD
Department of Urology Professor of Urology Resident
Cleveland Clinic Department of Urology Department of Urology
Cleveland, Ohio Yonsei University College of Smith Institute for Urology Hofstra North
Medicine Schore LIJ School of Medicine
Leonard G. Gomella, MD, FACS
Seoul, Korea Briarwood, New York
The Bernard W. Godwin Professor of
Prostate Cancer Won K. Han, MD Duane R. Hickling, MD
Chairman Associate Professor Female Pelvic Medicine and
Department of Urology Department of Medicine Reconstructive Surgery Fellow
Sidney Kimmel Medical College Division of Nephrology NYU School of Medicine
Associate Director, Jefferson Sidney Sidney Kimmel Medical College New York, New York
Kimmel Cancer Center Thomas Jefferson University
Clinical Director, Jefferson Sidney Irvin H. Hirsch, MD
Philadelphia, Pennsylvania Clinical Professor
Kimmel Cancer Network
Misop Han, MD Department of Urology
Thomas Jefferson University
Associate Professor of Urology and Thomas Jefferson University
Philadelphia, Pennsylvania
Oncology Philadelphia, Pennsylvania
Tricia Lacy Gomella, MD Department of Urology
Part Time Assistant Professor Steve J. Hodges, MD
Brady Institute of Urology Associate Professor
Department of Pediatrics Johns Hopkins Medical Institutions
The Johns Hopkins University School of Department of Urology
Baltimore, Maryland Wake Forest School of Medicine
Medicine
Baltimore, Maryland Jessica H. Hannick, MD Winston-Salem, North Carolina
Resident Jean Hoffman-Censits, MD
Patrick T. Gomella, MD, MPH
Loyola University Medical Center and Assistant Professor
Resident
Stritch School of Medicine Department of Medical Oncology
Department of Urology
Maywood, Illinois Sidney Kimmel Medical College
George Washington University
Washington, DC Thomas Jefferson University
Samuel Haywood, MD
Philadelphia, Pennsylvania
Michael A. Gorin, MD Resident
Resident Glickman Urologic and Kidney James M. Hotaling, MD, MS
James Buchanan Brady Urologic Institute Andrology Fellow
Institute Cleveland Clinic Foundation Department of Urology
Johns Hopkins Medical Institutions Cleveland, Ohio University of Illinois at Chicago
Towson, Maryland Chicago, Illinois
Kelly A. Healy, MD
Shaun G.S. Grewal, MD Assistant Professor Wayland Hsiao, MD
Chief Resident Department of Urology Assistant Professor
Division of Urologic Surgery Sidney Kimmel Medical College Department of Urology
Washington University Thomas Jefferson University Emory University School of Medicine
Tulsa, Oklahoma Philadelphia, Pennsylvania Atlanta, Georgia
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Contributors r r r xxi
xxii r r r Contributors
Contributors r r r xxiii
Surena F. Matin, MD, FACS Allen F. Morey, MD, FACS Samuel Walker Nickles, MD
Associate Professor Paul C. Peters Chair in Urology Resident
Director Professor Medical University of South Carolina
Minimally Invasive New Technology in Department of Urology Charleston, South Carolina
Oncologic Surgery UT Southwestern Medical Center
Craig S. Niederberger, MD, FACS
Department of Urology Dallas, Texas
Professor and Department Head
The University of Texas MD Anderson Judd W. Moul, MD, FACS Department of Urology
Cancer Center James H. Semans, MD Professor of University of Illinois at Chicago
Houston, Texas Surgery Chicago, Illinois
Derek Matoka, MD Division of Urologic Surgery
Assistant Professor Duke University Medical Center Dmitriy Nikolavsky, MD
Department of Urology Durham, North Carolina Assistant Professor
Loyola University Medical Center Department of Urology
John Patrick Mulhall, MBBCh, FACS,
Stritch School of Medicine SUNY Upstate Medical University
FECSM
Maywood, Illinois Syracuse, New York
Director
Kurt A. McCammon, MD, FACS Sexual and Reproductive Medicine Victor W. Nitti, MD, FACS
Associate Professor Program Professor of Urology and Obstetrics &
Urology Division of Urology Gynecology
Eastern Virginia Medical School Memorial Sloan Kettering Cancer Center Vice Chair
Virginia Beach, Virginia New York, New York Department of Urology
Alana M. Murphy, MD Director
Monica M. Metzdorf, MD
Assistant Professor Female Pelvic Medicine and
Pediatric Urologist
Department of Urology Reconstructive Surgery
Kaiser Permanente
Sidney Kimmel Medical College Department of Urology
Los Angeles, California
Thomas Jefferson University New York University Langone Medical
Reza Mehrazin, MD Philadelphia, Pennsylvania Center
Fellow New York, New York
Fellow, Urologic Oncology Katie S. Murray, DO
Fox Chase Cancer Center Chief Resident Paul H. Noh, MD, FACS, FAAP
Philadelphia, Pennsylvania Department of Urology Director of Minimally Invasive Surgery
The University of Kansas Associate Professor
Matthew A. Meissner, MD Kansas City, Kansas Division of Urology
Resident Cincinnati Childrens Hospital Medical
Jack H. Mydlo, MD
Department of Urology Center
Professor and Chair
UT Southwestern Medical Cincinnati, Ohio
Department of Urology
Center
Temple University School of
Dallas, Texas Samuel Ohlander, MD
Medicine
Urology Resident
Vani S. Menon, MD Philadelphia, Pennsylvania
Department of Urology
Resident Stephen Y. Nakada, MD, FACS University of Illinois at Chicago
Department of Urology The David T. Uehling Professor and Chicago, Illinois
Loyola University Medical Center Chairman
Maywood, Illinois Department of Urology Tara K. Ortiz, MD
University of Wisconsin Urology Resident
Megan M. Merrill, DO
Madison, Wisconsin Department of Surgery
Urologic Oncology Fellow
Division of Urology
Department of Urology Michael J. Naslund, MD
Duke University Medical Center
The University of Texas MD Anderson Professor and Chief
Durham, North Carolina
Cancer Center Division of Urology
Houston, Texas Department of Surgery John J. Pahira, MD
Director Professor of Urology
Robert M. Moldwin, MD, FACS
Maryland Prostate Center Department of Urology
Professor of Urology
University of Maryland School of Georgetown University Hospital
North Shore-LIJ Hofstra University
Medicine Washington DC
School of Medicine
Baltimore, Maryland
Director, Pelvic Pain Center, North Daniel C. Parker, MD
Shore-LIJ Healthcare System Frank M. Nezu, MD Resident in Urology
Arthur Smith Institute for Urology Urology Division Chief Department of Urology
Long Island Jewish Medical Center Howard County General Hospital Temple University
New Hyde Park, New York Clarksville, Maryland Philadelphia, Pennsylvania
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xxiv r r r Contributors
Neal Patel, MD Sandip M. Prasad, MD, MPhil W. Stuart Reynolds, MD, MPH
Resident Assistant Professor of Urology and Assistant Professor
Division of Urology Associate Director of the Urology Department of Urologic Surgery
Department of Surgery Residency Program Vanderbilt University Medical Center
Rutgers-Robert Wood Johnson Medical Medical University of South Carolina Nashville, Tennessee
School Charleston, South Carolina
Kyle A. Richards, MD
New Brunswick, New Jersey Raj S. Pruthi, MD, FACS Urologic Oncology Fellow
Elizabeth K. Peacock, MD Professor and Chief of Urology Surgery
Chief Resident Department of Surgery The University of Chicago Medical Center
Medical University of South Carolina University of North Carolina School of Chicago, Illinois
Charleston, South Carolina Medicine
Julie M. Riley, MD
Chapel Hill, North Carolina
Margaret S. Pearle, MD, PhD, FACS Assistant Professor
Professor of Urology Marcus L. Quek, MD, FACS Director of Endourology
Professor of Internal Medicine Associate Professor Division of Urology
Department of Urology Department of Urology University of New Mexico
UT Southwestern Medical Center Loyola University Medical Center and Albuquerque, New Mexico
Dallas, Texas Stritch School of Medicine
Chad R. Ritch, MD, MBA
Maywood, Illinois
David F. Penson, MD, MPH Clinical Instructor
Hamilton and Howd Chair in Urologic Ganesh V. Raj, MD, FACS Urologic Surgery
Oncology Associate Professor of Urology Vanderbilt University Medical Center
Chair, Department of Urologic Surgery Department of Urology Nashville, Tennessee
Professor of Urologic Surgery, Medicine UT Southwestern Medical Center
Nathan R. Roberts, MD
and Health Policy Dallas, Texas
Resident
Vanderbilt University Medical Center Pravin Rao, MD Department of Urology
Nashville, Tennessee Assistant Professor of Urology Thomas Jefferson University
Michael Perrotti, MD Director of Reproductive Medicine and Philadelphia, Pennsylvania
Albany Urologic Oncology Surgery James S. Rosoff, MD
Albany, New York James Buchanan Brady Urological
Assistant Professor
Institute Department of Urology
John L. Phillips, MD, FACS Johns Hopkins University
Urology Program Director Yale School of Medicine
Baltimore, Maryland New Haven, Connecticut
Department of Urology
New York Medical College Amar J. Raval, MD Sherry S. Ross, MD
Sleepy Hollow, New York Resident
Assistant Professor of Surgery and
Department of Urology
Michael A. Poch, MD Pediatrics
Thomas Jefferson University Department of Surgery
Assistant Professor Philadelphia, Pennsylvania
Genitourinary Oncology Division of Urology
Moffitt Cancer Center Mathew C. Raynor, MD Duke University Medical Center
University of South Florida Assistant Professor Durham, North Carolina
Tampa, Florida Division of Urologic Surgery
Joshua D. Roth, MD
The University of North Carolina School
Dana Point, MD Resident
of Medicine
Resident Department of Urology
Chapel Hill, North Carolina
Division of Urology Indiana University School of Medicine
West Virginia University Nathaniel Readal, MD Indianapolis, Indiana
Morgantown, West Virginia Urology Resident
Eric S. Rovner, MD, FACS
James Buchanan Brady Urology Institute
Michael A. Pontari, MD Professor of Urology
Baltimore, Maryland
Professor and Vice-Chairperson Medical University of South Carolina
Department of Urology Jeremy N. Reese, MD, MPH Charleston, South Carolina
Temple University School of Medicine Resident
Edmund S. Sabanegh, Jr., MD
Philadelphia, Pennsylvania Department of Urology
Chairman
University of Pittsburgh Medical Center
Mary K. Powers, MD Department of Urology
Pittsburgh, Pennsylvania
Resident Professor of Surgery (Urology)
Department of Urology Matthew J. Resnick, MD Cleveland Clinic
Tulane University School of Assistant Professor of Urologic Surgery Lerner College of Medicine at Case
Medicine Vanderbilt University Medical Center Western Reserve University
New Orleans, Louisiana Nashville, Tennessee Cleveland, Ohio
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Contributors r r r xxv
xxvi r r r Contributors
Raju Thomas, MD, MHA, FACS Evalynn Vasquez, MD, MBA Michael E. Woods, MD
Professor and Chairman Resident Associate Professor of Urology
Department of Urology Department of Urology Department of Urology
Tulane University School of Loyola University Medical Center University of North Carolina School of
Medicine Maywood, Illinois Medicine
New Orleans, Louisiana Taylor B. Vaughan, MD Chapel Hill, North Carolina
Adeep B. Thumar, MD Department of Urology
Christopher Wright, MD
Chief Resident Medical University of South Carolina
Urology Resident
Department of Urology Charleston, South Carolina
Rutgers-New Jersey Medical School
Thomas Jefferson University Bryan Voelzke, MD, MS Totowa, New Jersey
Philadelphia, Pennsylvania Assistant Professor
Department of Urology Blake A. Wynia, MD, MPH
Jeffrey J. Tomaszewski, MD Resident
Harborview Medical Center at the
Fellow Department of Urology
University of Washington
Urologic Oncology New York University
Seattle, Washington
Fox Chase Cancer Center New York, New York
Philadelphia, Pennsylvania Srinivas Vourganti, MD
Clinical Fellow National Institutes of Rafael E. Yanes, MD
Edouard J. Trabulsi, MD, FACS Health Resident
Associate Professor National Cancer Institute Department of Urology
Department of Urology Urologic Oncology Branch Mount Sinai Medical Center
Sidney Kimmel Medical College Washington, DC Bay Harbor Island, Florida
Thomas Jefferson University Nikhil Waingankar, MD
Philadelphia, Pennsylvania Resident Shilo Yaniv, MD
Anthony J. Tracey, MD, MPH The Arthur Smith Institute for Urology Clinical Instructor in Urology
Resident North Shore-Long Island Jewish Health Department of Urology
Department of Urology System University of Pittsburgh Medical
Tulane University School of Long Island City, New York Center
Medicine Pittsburgh, Pennsylvania
Dana A. Weiss, MD
New Orleans, Louisiana Pediatric Urology Fellow Matthew A. Young, MD
Matthew A. Uhlman, MD, MBA Surgery, Division of Urology Resident
Resident The Childrens Hospital of Medical University of South Carolina
Department of Urology Philadelphia Charleston, South Carolina
University of Iowa Hospitals and Philadelphia, Pennsylvania
Sven Wenske, MD Austin R. Younger, MD
Clinics
Assistant Professor Resident
Iowa City, Iowa
Department of Urology Medical University of South Carolina
Robert G. Uzzo, MD, FACS Columbia University College of Charleston, South Carolina
Chairman Physicians & Surgeons
Department of Surgical Oncology Lee C. Zhao, MD, MS
New York, New York Assistant Instructor
Fox Chase Cancer Center
Hunter Wessells, MD, FACS UT Southwestern Medical Center
Philadelphia, Pennsylvania
Professor and Nelson Chair in Urology Dallas, Texas
Vladimir A. Valera, MD, PhD Department of Urology
Resident University of Washington Philip T. Zhao, MD
Department of Urology Seattle, Washington Chief Resident
New York Medical College Division of Urology, Department of
Jessica Wetterlin, MD
Valhalla, New York Surgery
Resident
Rutgers-Robert Wood Johnson Medical
Sandip P. Vasavada, MD, FACS Department of Urology
School
Urologic Director Loyola University Medical Center
New Brunswick, New Jersey
Center for Female Pelvic Medicine & Maywood, Illinois
Reconstructive Surgery Daniel A. Wollin, MD Jack Matthew Zuckerman, MD
Cleveland Clinic Lerner College of Resident Resident
Medicine Department of Urology Department of Urology
Glickman Urological Institute New York University Eastern Virginia Medical School
Cleveland, Ohio New York, New York Norfolk, Virginia
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
LWBK1391-FM LWBK1391-Gomella September 19, 2014 21:36
CONTENTS
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Acute Kidney Injury, Adult (Renal Failure, Acute) 6 Ammonium Chloride Loading Test 647
Acute Kidney Injury, Pediatric (Renal Failure, Acute) 8 Ammonium Urate Urolithiasis 647
Acute Scrotum 10 Amsterdam and Bethesda Criteria for Lynch Syndrome 647
Acute Tubular Necrosis 12 Amyloidosis, Genitourinary 26
Addison Disease 14 Anal Sphincter Tone and Sensation, Urologic
Adenofibroma, Metanephric, Pediatric 643 Considerations 647
Adenomatoid Tumors, Testicular and Paratesticular 16 Anderson-Hynes Pyeloplasty 647
Adrenal Adenoma 18 Andrews Procedure (Hydrocele) 648
Adrenal Angiomyolipoma 643 Androgen Deficiency in the Aging Male (ADAM)
and ADAM Questionnaire 648
Adrenal Calcifications 643
Androgen Deprivation Syndrome (ADS)/Metabolic
Adrenal Cortical Carcinoma 20
Syndrome 648
Adrenal Cysts and Pseudocysts 644
Androgen Insensitivity Syndrome (AIS; or Androgen
Adrenal Cytomegaly 644 Resistance Syndrome), Complete (CAIS) and
Adrenal Hemorrhage 644 Partial (PAIS) 648
Adrenal Hypoplasia 644 Androgen/Anabolic Steroid Abuse 649
Adrenal Incidentalomas 644 Andropause (Late-Onset Hypogonadism) 28
Adrenal Insufficiency, Acute (Adrenal Crisis) 22 Angiokeratoma of Fordyce (Penile and Scrotal
Adrenal Mass 24 Angiokeratomas) 649
Adrenal Metastases 644 Angiolymphoid Hyperplasia, Penile 649
Adrenal Myelolipoma (Adrenal Myolipoma) 644 Angiomyxoma, Perineal 649
Adrenal Oncocytoma 644 Angiosarcoma, Genitourinary 649
Adrenalitis 645 Anogenital Intraepithelial Neoplasia 649
Adrenocortical Disease, Primary Pigmented Anorectal Malformations: Imperforate Anus, Cloaca,
Nodular 645 and Urogenital Sinus Anomalies 30
Adrenogenital Syndrome 645 Anorgasmia, Female 649
Adrenoleukodystrophy 645 Anorgasmia, Male 32
Aging Male Survey 645 Anterior Urethral Valves 649
Al Ghorab Corporal Shunt 645 Antiandrogen Withdrawal Syndrome (Flutamide
Withdrawal Syndrome) 649
Al Ghorab Corporal Shunt with Burnett Snake
Maneuver 645 Antisperm Antibodies 650
Alagille Syndrome 646 Anuria and Oliguria, Adult 34
Alkaline Phosphatase, Urologic Considerations 646 Anuria and Oliguria, Pediatric 36
Alkaptonuria 646 Aphthous Ulcer, External Genitalia 650
Allopurinol Hypersensitivity Syndrome (AHS) 646 Appendix Testis and Appendix Epididymis, Torsion 650
Alopecia Genitalium 646 Aristolochic Acid (Fang Chi) 650
-(Alpha) Fetoprotein 646 Arteriovenous Fistula (AVF), Renal (or Arteriovenous
Malformation [AVM]) 650
Alport Disease/Syndrome 646
Artificial Insemination (AI) 651
AlstromEdwards Syndrome 646
Ask-Upmark Kidney 651
Alzheimer Disease, Urologic Considerations 646
Asopa Hypospadias Repair 651
Ambiguous Genitalia 646
Aspergillosis, Genitourinary 651
American Association for the Surgery of Trauma
(AAST) Organ Severity Scales: Genitourinary Aspermia 651
Injuries 647 Assisted Reproductive Technology (ART) 651
Aminoaciduria 647 Asthenospermia 651
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Chemotherapy Toxicity, Urologic Considerations 668 Contact Dermatitis, Urologic Considerations 673
Chlamydia Sexually Transmitted Disease 669 Contrast Allergy and Reactions 98
Chordee 82 Contrast-Induced Nephropathy (CIN) 674
Christmas Tree Bladder 669 Cordonnier and Nesbit Ureteral Anastomosis 674
Chronic Kidney Disease (CKD) 669 Corpora Amylacea (CA) 674
Chronic Kidney Disease, Adult (Renal Failure, Cortical Necrosis, Acute (Renal Cortical Necrosis) 674
Chronic) 84 Costovertebral Angle Tenderness 674
Chronic Kidney Disease, Pediatric (Renal Failure, Cough Stress Test 674
Chronic) 86 Cowper Duct Cyst 674
Chronic Pelvic Pain Syndrome/Chronic Prostatitis
Cowper Gland Carcinoma 675
(CPPS/CP) in Males 669
Cowperitis (Inflammation of Bulbourethral Gland) 675
Chronic Pelvic Pain Syndrome (CPPS) in Females 669
Creatinine, Serum, Increased/Decreased 675
Chronic Prostatitis Symptom Index (CPSI)/NIH-CPSI
(National Institutes of Health CPSI) 670 Crede Maneuver 675
Churg-Strauss Syndrome 670 Cremasteric Reflex 675
Chylocele 670 Cribriform Clear Cell Hyperplasia of the Prostate 675
Chylous Ascites 88 Cryptococcus, Genitourinary 675
Chyluria Crystal-Induced Acute Kidney Injury
90
(Acute Renal Failure) 675
Circumcision, Adult Considerations 92
CT Scan, Urologic Considerations 675
Circumcision, Female 670
Culp-Deweerd Pyeloplasty 676
Circumcision, Pediatric Considerations 94
Cunningham Clamp 676
Cisplatin Toxicity 670
Cushing Disease and Syndrome 100
Clitoral Length 671
Cyclophosphamide (Cytoxan) Toxicity 676
Clitoral Priapism 671
Cystadenocarcinoma, Genitourinary 676
Clitoromegaly 671
Cystadenoma, Genitourinary 676
Clonidine Suppression Test 671
Cystadenoma/Cystadenocarcinoma, Retroperitoneal 676
Clostridium Difficile Colitis, Urologic Considerations 671
Cystatin C 676
Clot Retention 671
Cystic Fibrosis, Urologic Considerations 676
Cobb Collar 671
Cystinosis 677
Cobra Head Sign 671
Cystitis Cystica 677
Coccidiomycosis, Genitourinary 671
Cystitis, Emphysematous 677
Cohen (Cross-Trigonal) Ureteral Reimplantation 672
Cystitis, Eosinophilic 677
Coital Incontinence (Coital leakage/Intercourse
Cystitis Follicularis 677
Incontinence) 672
Cystitis Glandularis and Cystitis Glandularis of
Collecting System Duplication, Complete 672
Intestinal Type 677
Colon and Rectal Cancer, Urologic Considerations 672 Cystitis, General Considerations 102
Column of Bertin, Hypertrophied 672 Cystitis, Granulomatous 677
Compartment Syndrome, Urologic Considerations 672 Cystitis, Hemorrhagic (Infectious, Noninfectious,
Compulsive Masterbation 672 Radiation) 104
Condyloma Acuminata (Veneral Warts) 96 Cystitis, Polypoid and Papillary 677
Condylomata Lata 672 Cystitis, Radiation 677
Congenital Adrenal Hyperplasia (CAH) 672 Cystitis, Viral 678
Congenital Nephrosis/Nephrotic Syndrome 673 Cystocele Grading: BadenWalker, Pelvic Organ
Constipation, Urologic Considerations 673 Prolapse Quantification (POP-Q) 678
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Hodgson Types I, II, III Hypospadias Repair 702 Hypokalemia, Urologic Considerations 707
Honeymoon Cystitis 702 Hypomagnesemia, Urologic Considerations 707
Horseshoe Kidney 702 Hyponatremia, Urologic Considerations 707
Horton-Devine Flip-Flap Hypospadias Repair 702 Hypophosphatemia, Urologic Considerations 707
Hot Flushes/Vasomotor Instability in Males 188 Hypospadias, Free Graft Repair 707
Hounsfield Units 702 Hypospadias, Tubularized Incised Plate (TIP) Repair 707
HPC-1 (Hereditary Prostate Cancer 1 Locus) 703 Hypospadias, 2-Stage Repair 708
HPV (Human Papilloma Virus), Urologic Hypospadias, Urethral Advancement for Subglanular
Considerations 703 and Midshaft Defects 708
Human Growth Hormone (hGH), Urologic Hypospadias 204
Considerations 703 Hysterectomy, Urologic Considerations 708
Hunner Ulcer 703 IC (Interstitial Cystitis) Symptom Index 708
Hutch Diverticulum 703 Ice Water Test 708
Hydatid Cyst (Hydatid Disease) 703 ICIQ-MLUTS (International Consultation on
Hydrocalycosis 703 Incontinence Questionnaire-Male Lower Urinary
Tract Symptoms) 708
Hydrocele of the Spermatic Cord 703
IIEF (International Index of Erectile Function) 708, 987
Hydrocele, Adult & Pediatric 190
Immunocompromised Patients, Urologic
Hydrocolpos and Hydrometrocolpos, Pediatric 192 Considerations 206
Hydronephrosis/Hydroureteronephrosis Immunohistochemical Staining, Urologic
(Dilated Ureter/Renal Pelvis), Adult 194 Considerations 709
Hydronephrosis/Hydroureteronephrosis Imperforate Hymen 709
(Dilated Ureter/Renal Pelvis), Pediatric 196
In Vitro Fertilization (IVF) and Embryo Transfer 709
Hydronephrosis/hydroureteronephrosis
Incontinence Clamps 709
(Dilated Ureter/Renal Pelvis), Prenatal 198
Incontinence Impact Questionnaire (IIQ-7) 709
Hymenal Skin Tags 703
Incontinence (Urinary) with Orgasm (Climacturia) 710
Hyperaldosteronism, Primary (Aldosteronism, Conn
Syndrome) 200 Incontinence, Urinary, Adult Female 210
Hyperbaric Oxygen, Urologic Considerations 703 Incontinence, Urinary, Adult Male 212
Hypercalcemia, Urologic Considerations 704 Incontinence, Urinary, Following Radical
Prostatectomy 214
Hypercalciuria (Absorptive, Renal, and Resorptive) 704
Incontinence, Urinary, Pediatric 216
Hypercarbia During Laparoscopy 704
Indevus Urgency Severity Scale (IUSS) 710
Hypercontinence 705
Indiana Pouch 710
Hyperkalemia, Urologic Considerations 705
Infertile Male Syndrome 710
Hypermagnesemia, Urologic Considerations 705
Infertility, Urologic Considerations 218
Hypernatremia, Urologic Considerations 705
Inflammatory Bowel Disease, Urologic
Hyperoxaluria, Urologic Considerations 705 Considerations 710
Hyperparathyroidism, Urologic Considerations 706 Inflammatory Pseudotumor (Pseudosarcomatous
Hyperphosphatemia, Urologic Considerations 706 Fibromyxoid Tumor) 710
Hyperprolactinemia, Urologic Considerations 202 Infundibular Stenosis 710
Hyperspermia and Hypospermia 706 Infundibulopelvic Dysgenesis 710
Hypertension, Urologic Considerations 706 Inguinal Hernia, Adult, Urologic Considerations 710
Hyperuricosuria 706 Inguinal Hernia, Pediatric, Urologic Considerations 710
Hypocitraturia 706 Injection Therapy for Vesicoureteral Reflux 711
Hypogonadism, Society Definitions 706 Insect Bite, Penis and Scrotum 711
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Penis, Hirsute Papilloma (Pearly Penile Papules, Phosphate Nephropathy, Acute 750
Coronal Papillae) 746 Pinworms, Urologic Considerations 750
Penis, Hypoplasia 746 Pipe Stem Urethra 750
Penis, Kaposi Sarcoma 746 PI-RADS Prostate MRI Scoring System 750
Penis, Leiomyoma 746 PLAP (Placental Alkaline Phosphatase) 751
Penis, Leiomyosarcoma 746 Plasmacytoid Urothelial Carcinoma 751
Penis, Length, Normal 746 Plasmacytoma, Bladder 751
Penis, Leukoplakia 747 Plasmacytoma, Testicular 751
Penis, Malignant Fibrous Histiocytoma (MFH) 747 Ploidy Analysis, Bladder Cancer 751
Penis, Melanoma 747 Ploidy Analysis, Prostate Cancer 751
Penis, Metastasis To 747 Pneumaturia (Gas in Urine) 300
Penis, Neurilemoma (Schwannoma) 747 Pneumoretroperitoneum 751
Penis, Neurofibrosarcoma Pneumoscrotum 751
(Malignant Schwannoma) 747
Polyarteritis Nodosa (PAN), Urologic
Penis, Sclerosing Lipogranuloma (Paraffinoma) 747 Considerations 751
Penis, Sclerosing Nonvenereal Lymphangitis 747 Polycystic Kidney Disease, Autosomal Dominant 302
Penis, Squamous Cell Carcinoma 290 Polycystic Kidney Disease, Autosomal Recessive 304
Penis, Strangulation 747 Polyembryoma 752
Penis, Syringoma 748 Polyhydramnios/Oligohydramnios 306
Penis, Thrombosis of Dorsal Vein 748 Polyoma Virus (BK, JC), Urologic
Penis, Torsion 748 Considerations 308, 752
Penis, Trauma 292 Polyorchidism 752
Penis, Verrucous Carcinoma 748 Polythelia, Urologic Considerations 752
Penis, Webbed 748 Polyuria 752
Penn Pouch 748 Positron Emission Tomography (PET) Imaging,
Pereyra Urethropexy 748 Choline C 11 752
Perineal Grooves 748 Positron Emission Tomography (PET) Imaging,
Perineal Mass Urologic Considerations 752
748
Perineal Pain, Differential Diagnosis Post Micturition Symptoms 752
749
Perineal Trauma (Straddle Injury) Postorgasmic Illness Syndrome (POIS) 752
749
Perinephric Mass Postatrophic Hyperplasia of the Prostate 753
749
Perinephric Stranding 749 Postcoital Prophylactic Antibiotics 753
Perineural Invasion, Urologic Considerations 749 Postcoital Test 753
Peripheral Neuropathy, Urologic Considerations 749 Posterior Tibial Nerve Stimulation (PTNS) 753
Periureteritis 750 Posterior Urethral Valves 310
Periurethral Abscess 750 Postobstructive Diuresis 312
Perlman Syndrome 750 Postoperative Spindle Cell Nodule, Bladder 753
Peyronie Disease 294 Posttransplant Lymphoproliferative Disorder 753
Pfannenstiel Incision 750 Postvoid Dribbling 753
Pheochromocytoma 296 Potassium Sensitivity Testing 753
phi (Prostate Health Index) (See Section II: Potter Syndrome/Potter Sequence 754
Prostate Health Index (phi) and [-2] proPSA) 750 Pouchitis 754
Phimosis and Paraphimosis 298 PraderWilli Syndrome 754
Phimosis, Clitoral 750 Precocious Puberty 754
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Pregnancy, Bacteriuria, Pyuria, and Urinary Tract Prostate Cancer, Locally Advanced
Infection 754 (Pathologic T3, T4) 332
Pregnancy, Hematuria 754 Prostate Cancer, Metastatic (Clinical and
Pregnancy, Radiologic Considerations 754 Pathologic N+, M+) 334
Pregnancy, Renal Transplantation 754 Prostate Cancer, Mucinous Adenocarcinoma 758
Pregnancy, Urinary Diversion 755 Prostate Cancer, Positive Margin Following Radical
Prostatectomy 336
Pregnancy, Urinary Tract Obstruction 755
Prostate Cancer, Prevention (Chemoprevention) 758
Pregnancy, Urolithiasis 314
Prostate Cancer, Rising PSA Following Androgen
Pregnancy, Urologic Malignancy 755
Ablation (Castration-Resistant Prostate Cancer,
Pregnancy, Urologic Medications 755 CRPC and mCRPC) 338
Prehn Sign 755 Prostate Cancer Risk Calculators 758
Prentiss Maneuver 755 Prostate Cancer, Risk Stratification (DAmico
Preputial Stones 755 Classification) 759
PressureFlow Studies 755 Prostate Cancer, Secondary Hormonal Therapy 759
Priapism, Stuttering (Intermittent Priapism) 755 Prostate Cancer, Small Cell (Neuroendocrine) 759
Priapism 316 Prostate Cancer, Squamous and Adenosquamous 759
Primitive Neuroectodermal Tumors (PNET) Prostate Cancer, Urothelial 340
(Extraskeletal Ewing Sarcoma) 756 Prostate Cancer, Very Low Risk and Active
Princeton III Consensus Recommendations: Surveillance 342
Erectile Dysfunction (ED) and Cardiovascular Prostate Health Index (PHI) and [2] proPSA 759
Disease 756
Prostate Urethral Angle 760
Prolactin, Serum Level 756
Prostate, Abscess 344
Prolapse, Staging Systems 756
Prostate, Basal Cell Hyperplasia 760
Propantheline Stimulation Test 756
Prostate, Benign Enlargement (Benign Prostate
Prophylactic Antibiotics, AUA Guidelines 756, 979 Enlargement [BPE]) 760
Prostascint Scan 756 Prostate, Benign Hyperplasia/Hypertrophy (BPH) 346
Prostate Biopsy, Infections and Complications 318 Prostate, Benign Obstruction (Benign Prostatic
Prostate Cancer Screening Guidelines 756, 991 Obstruction [BPO]) 760
Prostate Cancer, Active Surveillance and Prostate, Calculi 348, 760
Watchful Waiting 757 Prostate, Female 760
Prostate Cancer, Basal Cell Carcinoma 757 Prostate, Hematuria 760
Prostate Cancer, Biochemical Recurrence
Prostate, Infarction 760
(Elevated PSA) Following Cryotherapy 320
Prostate, Massage 760
Prostate Cancer, Biochemical Recurrence
(Elevated PSA) Following Radiation Therapy 322 Prostate, Nodule 350
Prostate Cancer, Biochemical Recurrence Prostate Stents (Urolume and Spanner) 761
(Elevated PSA) Following Radical Prostatectomy 324 Prostatic Acid Phosphatase (PAP) 761
Prostate Cancer, Circulating Tumor Cells (CTCs) 757 Prostatic Intraepithelial Neoplasia (PIN) 352
Prostate Cancer, Ductal Adenocarcinoma 757 Prostatic Urethral Polyps 761
Prostate Cancer, Familial 758 Prostatic Utricle Anomalies 761
Prostate Cancer, General 326 Prostatic Utricle Calcification 761
Prostate Cancer, Leiomyosarcoma, and Other Prostatitis, Acute, Bacterial (NIH I) 354
Uncommon Sarcomas 758 Prostatitis, Asymptomatic Inflammatory (NIH IV) 761
Prostate Cancer, Localized (T1, T2) 328 Prostatitis, Chronic Nonbacterial, Inflammatory and
Prostate Cancer, Locally Advanced (Clinical T3, T4) 330 Noninflammatory (NIH CP/CPPS III A and B) 356
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Spinal Cord Compression, Urologic Considerations 786 Testis Cancer, Embryonal Carcinoma 490
Spinal Cord Injury, Urologic Considerations 474 Testis Cancer, Endodermal Sinus Tumors
Spinal Shock 786 (Yolk Sac Tumors) 492
Spindle Cell Neoplasm, Urologic Considerations 786 Testis Cancer, Nonseminomatous Germ Cell
Tumors, General 494
Spinning Top Urethra 787
Testis Cancer, Pediatric, General Considerations 496
Splenic Injury During Radical Nephrectomy 787
Testis Cancer, Seminoma 498
Splenogonadal Fusion 787
Testis, Carcinoid 790
Splenule/Splenosis, Urologic Considerations 787
Testis, Carcinoma In Situ (CIS)/Intratubular Germ
Sports Hernia (Athletic Pubalgia, Sportsmans
Cell Neoplasia (ITGCN) 791
Hernia) 787
Testis, Cystic Lymphangiomas 791
Squamous Metaplasia, Genitourinary 787
Testis, Cysts 791
Stamey Procedure (Urethropexy) 787
Testis, Dermoid Cyst 791
Stamey Test (3-Glass Test, 4-Glass Test,
MearesStamey Test) 788 Testis, Hemangioma 791
Stauffer Syndrome 788 Testis, Leukemia 791
Steinstrasse 788 Testis, Leydig Cell Tumor 500
STING Procedure 788 Testis, Lymphoma 792
Stranguria 788 Testis, Metastasis To 792
Streak Gonad 788 Testis, Microlithiasis 792
Stress Urinary Incontinence, Female 476 Testis, Normal Size 792
Stress Urinary Incontinence, Male 478 Testis, Pain (Orchalgia) 502
Strickler Ureteral Anastomosis 788 Testis, Retractile 792
Stroke (CVA), Urologic Considerations 480 Testis, Sertoli Cell Tumor 504
Struvite 788 Testis, Sex Cord Stromal Tumors 792
Studer Pouch 789 Testis, Teratoma, Extragonadal 792
Superficial Inguinal Pouch of Denis-Browne 789 Testis, Teratoma, Mature and Immature 506
Supernumerary Kidney 789 Testis, Tumor and Mass, Adult, General
Supine Stress Test 789 Considerations 508
Suprapubic Pain, General Considerations 482 Testis, Tumor and Mass, Pediatric, General
Considerations 510
Swyer Syndrome (XY Sex Reversal) 789
Testis, Vasocongestion From Sexual Arousal
Syndrome of Inappropriate Antidiuretic Hormone
Without Ejaculation (Blue Balls) 792
(SIADH) 789
Testosterone (Free and Total) Serum 792
Syphilis 484
Testosterone Replacement Following Localized
Systemic Lupus, Urologic Considerations 789 Prostate Cancer Therapy 793
Tabes Dorsalis 789 Testosterone Replacement Therapy, General
Taghaandan 790 Principles 512
Takayasu Arteritis, Urologic Considerations 790 Testosterone Replacement Therapy, Prostate
Tanner Stages/Classification of Sexual Maturity 790 Cancer Risk 793
Teratoma, Sacrococcygeal, Urologic Considerations 790 Testosterone, Decreased (Hypogonadism) 514
Testicular Feminization Syndrome 790 Tethered Cord 793
Testicular Prosthesis 790 Tethered Cord Syndrome 793
Testis Biopsy, Indications 790 Thiersch-Duplay Hypospadias Repair 793
Testis Cancer, Adult General Considerations 486 Thompson Pyeloplasty 793
Testis Cancer, Choriocarcinoma 488 Thoracic Kidney 794
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Tinea Cruris (Jock Itch) 794 Umbilical Abnormalities, Urologic Considerations 532
TMPRSS2-ERG Gene Fusion, Prostate Cancer 794 Underactive Bladder (Detrusor Underactivity) 534
Toileting Programs 794 Undervirilized Male Syndrome (Mild Androgen
Torsion, Testis or Testicular/Epididymal Appendages 518 Insensitivity) 797
Transesophageal Echocardiogram (TEE), Urologic Undescended Testes (Cryptorchidism) 536
Considerations 794 Uninhibited Detrusor Contraction 797
Transureteroureterostomy, Technique and Urachal Abnormalities 797
Indications 794 Urachal Carcinoma 538
Transplant Rejection, Renal 520 Urachal Carcinoma Staging Systems 798
Transsexualism, Urologic Considerations 794 Urate, Dietary 798
Transurethral Resection (TUR) Syndrome 522 Ureaplasma Urealyticum 798
Tri-Mix 794 Ureter and Renal Pelvic Tumors, General
Trichomoniasis 795 Considerations 540
Trichotemnomania, Pubic 795 Ureter and Renal Pelvis, Squamous Cell
Trichotillomania, Pubic Carcinoma 542
795
Trigonitis Ureter and Renal Pelvis, Urothelial Carcinoma 544
795
Ureter, Agenesis/Atresia 798
Trisomy 4 P 795
Ureter, Deviation 798
Trisomy 8 795
Ureter, Diverticulum 798
Trisomy 9 795
Ureter, Duplicated and Bifid 798
Trisomy 9 P 795
Ureter, Ectopic (Ureteral Ectopia) 799
Trisomy 10 Q 795
Ureter, Fibroepithelial Polyps 799
Trisomy 11 Q 795
Ureter, Fish Hook (Reverse J) 799
Trisomy 13 795
Ureter, Hemangioma 799
Trisomy 18 (Edwards Syndrome) 795
Ureter, Intraoperative Injury 546
Trisomy 20 P 796
Ureter, J Hooking 799
Trisomy 21 796
Ureter, Leiomyoma 799
Trisomy 22 796
Ureter, Leiomyosarcoma 799
Trisomy Syndrome 796
Ureter, Metastasis To 799
Trocar Injury During Laparoscopy 524
Ureter, Nephrogenic Adenoma (NA) 799
True Hermaphroditism (OVO-Testicular Disorder of
Sexual Differentiation [OVO-DSD]) 796 Ureter, Neurofibroma 799
Tuberculosis, Bladder and Urethra 796 Ureter, Obstruction 548
Tuberculosis, Genitourinary, General Considerations 526 Ureter, Pipe-Stem 800
Tuberculosis, Kidney and Ureter 528 Ureter, Radiation Injury To 800
Tuberculosis, Male External Genitalia 796 Ureter, Retrocaval (Circumcaval, Postcaval) 800
Tuberculosis, Prostate and Epididymis 796 Ureter, Shepherds Crook 800
Tuberous Sclerosis 796 Ureter, Spiral (Corkscrew) 800
Tumor Lysis Syndrome (TLS) 796 Ureter, Stone Passage Statistics 800
Tunica Albuginea/Paratesticular Tumors and Cysts 530 Ureter, Stricture 800
Tunica Vaginalis Tumors 797 Ureter, Trauma 550
Turner Syndrome (XO Syndrome) 797 Ureter, Valves 800
TurnerWarwick Inlay Urethroplasty 797 Ureteral Jets 801
UISS-UCLA International Kidney Cancer Staging Ureteral Stricture Following Urinary Diversion 801
System 797 Ureteritis 801
P1: OSO/OVY P2: OSO/OVY QC: OSO/OVY T1: OSO
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Urolithiasis, Cystine, and Cystinuria (Hypercystinuria) 604 Vasography, Technique and Indications 812
Urolithiasis, Drug Induced 809 Venous Leak Syndrome 812
Urolithiasis, Indinavir and Other Protease Inhibitors 809 Vesicoureteral Reflux, Adult 632
Urolithiasis, Infectious (Struvite) 809 Vesicoureteral Reflux, Pediatric 634
Urolithiasis, Matrix 809 Vesiculobullous Lesions, External Genitalia 813
Urolithiasis, Melamine 809 Videourodynamics 813
Urolithiasis, Methotrexate 809 Villous Adenoma, Bladder/Urethra 813
Urolithiasis, Pediatric, General Considerations 606 Vimentin, Staining 813
Urolithiasis, Renal 608 Vincent Curtsy 813
Urolithiasis, Staghorn 610 Vitiligo, Urologic Considerations 813
Urolithiasis, Triamterene 810 Voiding Diary Frequency Volume Chart (FVC) 814
Urolithiasis Ureteral 612 Voiding Symptoms, Definitions (ICS Definitions) 814
Urolithiasis, Uric Acid 614 Von HippelLindau Disease/Syndrome 636
Urolithiasis, Xanthine 810 Vulvar Malignancy, Urologic Considerations 814
Uroradiology Signs 810 Vulvodynia 814
Urosepsis 616 VURD Syndrome 814
Urostomy Problems 618 WAGR Syndrome (Wilms Tumor-Aniridia-Genital
Anomaly Retardation) 814
Urothelial Dysplasia 810
Wallace Ureteral Anastomosis 814
Vacterl/Vater Association 810
Walter Reed Staging System, Testis Cancer 814
Vaginal Agenesis 810
Waterhouse Urethral Stricture Repair 814
Vaginal Atrophy/Vulvovaginal Atrophy, Urologic
Considerations 810 WaterhouseFriderichsen Syndrome 815
Vaginal Discharge, Urologic Considerations 810 Weddellite 815
Vaginal Duplication 810 Wegener Granulomatosis, Urologic Considerations 815
Vaginal Fusion Weiss Criterion 815
811
Vaginal Mass, Newborn Whewellite 815
811
Vaginal Mesh Erosion Whitaker Test 815
620
Vaginal Pessaries, Urologic Considerations Whitlockite 815
811
Vaginal Prolapse WHO 2004 Histologic Classification of Tumors of
811
the Urinary Tract 815
Vaginitis/Vulvovaginitis 622
WHO/ISUP Consensus Classification of Urothelial
Vaginosis 811 Neoplasms (1998, 2004, and 2010) 815
Valsalva Maneuver 811 Wilms Tumor (Nephroblastoma) 638
Vanderbilt Cystectomy Index (VCI) 811 Wilms Tumor Staging System, International Society
Vanishing Testis Syndrome 811 of Pediatric Oncology (SIOP) 816
Varicocele, Adult 624 Wilms Tumor Staging System, National (NWTS) 816
Varicocele, Pediatric 626 Winter Corporal Shunt 816
Vas Deferens, Calcification (CVD) 812 Wolffian Duct Remnants 816
Vas Deferens, Congenital Absence 628 Wound Dehiscence, Urologic Considerations 817
Vas Deferens, Obstruction 812 Wound Infection, Postoperative, Urologic
Vasculitis, Urologic Considerations 812 Considerations 817
Vasectomy and Postvasectomy Pain Syndrome 630 Wunderlich Syndrome 817
Vasectomy Reversal, General Considerations Xanthogranulomatosis (ErdheimChester Disease) 817
(Vasovasostomy) 812 Xanthoma, Bladder 817
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l r r r Contents
X-linked Spinal and Bulbar Atrophy Syndrome Yolk Sac Tumor, Prostate 818
(Kennedy Syndrome) 817 Young Classification of Posterior Urethral Valves 818
XX Gonadal Dysgenesis (46, XX) 817 Young-Dees-Leadbetter Bladder Reconstruction 818
XX Male Reversal Syndrome (XX Male) 817 Young Syndrome 818
XXX Syndrome (Triple X Syndrome, Triplo-X) 817 Zellweger Syndrome (Cerebrohepatorenal Syndrome) 818
XXXY Syndrome 817 Zinner Syndrome 818
XXY Syndrome (Klinefelter Syndrome) 818 Zipper Entrapment 818
Yolk Sac Tumor, Bladder 818 Zona Pellucida Binding Assay 819
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SECTION I
Urologic Diseases and Conditions
Section Editor: Leonard G. Gomella, MD, FACS
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r CT:
BASICS DIAGNOSIS Used to enhance findings on US or solid mass on
US
DESCRIPTION HISTORY Good anatomic detail
r Traditional presentation was palpable mass in the r Prenatal ultrasound (1)
Useful in older children and suspected
newborn/child abdomen Oligohydramnios: Associated with PUV, bilateral malignancies
r Current presentation is usually by prenatal UPJ, urethral atresia, polycystic or multicystic Limitation: High sensitivity of pediatric patients to
ultrasound dysplastic kidneys, renal agenesis radiation exposure, may require sedation
r Most masses are nonsurgical; 87% of surgical Polyhydramnios: Associated with high GI r MRI:
lesions are benign obstructions Good for vascular involvement, adrenal origin
r Almost 2/3 of infantile abdominal masses arise from r Postnatal history
Good anatomic detail
kidneys, followed by GI tract (12%), female genital Initial discovery May gather functional and quantitative
system (10%), retroperitoneum (9%) Duration from detection of mass information
Rapidity of growth Limitation: May require sedation/anesthesia
EPIDEMIOLOGY Constitutional symptoms: Fever, pain, weight loss, r Radionuclide scans:
Incidence UTI, dysuria, hematuria, melena, anorexia, bilious Renal scans: Used to determine renal function,
Abdominal mass in 1 per 1,000 live births vomiting scarring, infection, and obstruction
Prevalence PHYSICAL EXAM Biliary scans: Evaluate for choledochal cysts
Varies with disease type r Perform thorough abdominal exam (2): Liverspleen scans: Used for diagnosis of liver
Size and location tumors or splenic enlargement
RISK FACTORS r VCUG:
Solid or cystic
Genetics Tender or nontender Used to rule out lower urinary tract pathology
r Disease specific
Smooth, irregular, indurated, or soft
r Neuroblastoma Diagnostic Procedures/Surgery
Fixed or mobile N/A
Chromosome 1p deletion Auscultation, percussion, and transillumination
Allelic loss of 11q r Additional exam: Pathologic Findings
Gain of copies of 17q correlate with more Nasogastric tube for intestinal decompression Disease specific
aggressive tumor Foley catheter for urinary decompression
N-MYC oncogene amplification
DIFFERENTIAL DIAGNOSIS
Rectal/introital exam r Hydronephrosis: Most common cause of neonatal
r Polycystic kidney disease, autosomal recessive
DIAGNOSTIC TESTS & INTERPRETATION abdominal mass (1):
(ARPKD) UPJ obstruction: Most common cause of
Gene locus at chromosome 6p21 Lab
r Rhabdomyosarcoma (RMS) r Labs should be tailored to clinical suspicion hydronephrotic abdominal mass
r CBC: Other causes: UVJ obstruction, PUV, VUR,
Mutation of TP53 gene found in tumors of megaureter, and ureteroceles
patients with LiFraumeni syndrome Anemia, neutropenia, thrombocytopenia may <15% of neonates present with mass
Alveolar RMS is associated with translocation suggest bone marrow involvement Later presentation: UTI, flank pain, hematuria
between chromosomes 1 or 2 and 13 Leukocytosis suggests possible infection/ after trauma
Embryonal RMS demonstrates LOH on obstruction r Multicystic dysplastic kidney:
chromosome 11p15.5 r BUN/creatinine/electrolytes
r Wilms tumor 2nd most common cause; together with UPJ
Elevated BUN/creatinine suggests renal constitute 40% of all neonatal abdominal masses
WT1 (11p13): DenysDrash and WAGR (Wilms compromise, dehydration Unilateral flank mass; more common on left, and
tumor, aniridia, genitourinary problems, r Urinalysis:
in boys
retardation) Hematuria seen in Wilms tumor, renal vein US shows multiple noncommunicating cysts of
WT2 (11p15): BeckwithWiedemann thrombosis, UPJ obstruction after trauma various sizes; nuclear scan shows nonfunction on
WTX (Xq11.1): Inactivated in up to 1/3 of Wilms r 24-hr urine
affected side
tumors Elevated homovanillic acid and vanillylmandelic r Multilocular cystic nephroma:
FWT1 (17q), FWT2 (19q): Familial acid seen in neuroblastoma or pheochromocytoma Spectrum from benign cyst to cystic Wilms tumor
LOH at 1p and 16q is associated with an r Serum -hCG and -fetoprotein:
Present in males <5 yr and females >30 yr
increased risk of tumor relapse and death Used in tumors such as teratoma, liver, and Diagnosis by surgical excision
PATHOPHYSIOLOGY germ-cell tumors r Renal vein thrombosis:
Disease specific, related to organ of origin Imaging Most common cause of neonatal hematuria; 65%
r Plain abdominal x-rays: occur in neonatal period, 30% after age 1; male
ASSOCIATED CONDITIONS
Check for obstruction/ileus; airfluid levels on predominance
Disease specific
upright and lateral; absence of air in rectum Classic features: Flank mass, hematuria,
GENERAL PREVENTION Calcifications can suggest neuroblastoma, thrombocytopenia
N/A teratoma, hepatoblastoma, meconium peritonitis, Occurs in conditions associated with dehydration,
urinary, or biliary stones maternal diabetes, sepsis, diarrhea, or sickle cell
r Abdominal US: disease
Primarily evaluation modality r Polycystic kidney disease:
Used to establish location, size, organ of origin, Autosomal recessive; diagnosed in neonatal
internal architecture, and vascular supply period; 50% die in 1st few hours or days usually
Can determine cystic vs. solid from respiratory failure; of survivors, 86% alive at
Inexpensive and noninvasive; rarely requires 1 yr and 67% at 15 yr
sedation
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PATHOPHYSIOLOGY
BASICS r Prerenal and intrinsic renal injury disrupt the DIAGNOSIS
regional perfusion of, and subsequent oxygen
DESCRIPTION delivery to, the kidney (3) HISTORY
r A sudden or recently acquired functional impairment r Evaluate for shock, sepsis, bleeding, dehydration,
The natural arterial gradient of oxygen tension
of the kidney relative to physiologic demands with from cortex to medulla makes the kidney highly gastrointestinal losses
or without actual kidney injury (1) r Assess recent medication exposure, including
susceptible to hypoxic and oxidative injury during
r Causes mild to potentially life-threatening ischemia and reperfusion natural products
alterations in fluid, electrolyte, acidbase and Poorly perfused glomerular endothelial cells r History of urologic surgery, anatomic problems, or
hormonal homeostasis release vasoactive substances, proteases, reactive kidney transplantation
r Terminology evolving from acute tubular necrosis oxygen species, and nitric oxide and activate the r Family history of ESRD, HUS
(ATN) to acute renal failure (ARF) to acute kidney coagulation cascade and complement pathways r Recent trauma, crush injury
injury (AKI) of varying grades to standardize Even well-perfused kidneys can develop AKI r Seek signs of CKD, such as growth delay
reporting, clinical care, and research during sepsis from circulating cytokines, r Review recent blood tests to determine baseline
r Diagnosed by the Pediatric Modified Risk Injury lymphocytes, T cells, and other factors serum creatinine (SCr) and onset of AKI
Failure Loss End Stage Renal Disease (pRIFLE) When found in the presence of cardiac,
criteria based on the estimated creatinine clearance pulmonary, or hepatic failure it is likely due to PHYSICAL EXAM
endothelial activation and circulatory aberrations r Assess hydration status, blood pressure, heart rate,
(eCCl, based on Schwartz formula) and urine output
(UOP) (2) Nephrotoxic agents each have their own and temperature
mechanism of damage, eg, by forming crystals in r Lungs for rales
Risk: eCCl decrease by 25% and/or UOP
the microstructures of the kidney r Abdomen for masses
<0.5 mL/kg/h for 8 hr
Injury: eCCl decrease by 50% and/or UOP Rhabdomyolysis causes intrarenal
vasoconstriction, direct ischemic tubule injury, and DIAGNOSTIC TESTS & INTERPRETATION
<0.5 mL/kg/h for 16 hr
tubular obstruction in acidic urine Lab
Failure: eCCl decrease by 75% or eCCl r SCr and blood urea nitrogen will be elevated
r Postrenal injury is due to antegrade urine flow
<35 mL/min/1.73 m2 and/or UOP <0.3 mL/kg/h r May have hyperkalemia, acidosis
for 24 hr or anuric for 12 hr disruption from the kidney
Must be bilateral to cause pRIFLE findings but can r Hemoglobin will be low if bleeding
Loss: Persistent failure >4 wk
be unilateral if only one kidney is present due to r HUS causes thrombocytopenia, increased lactate
End stage renal disease (ESRD): Persistent failure
>3 mo congenital absence, prior nephrectomy, or kidney dehydrogenase
r No standardized definition for neonatal AKI transplant r When muscular damage is the cause creatinine
r Etiology may be multifactorial, especially in the ICU kinase will be elevated and urinary myoglobin will
EPIDEMIOLOGY setting be positive
Incidence r Urinalysis to detect red blood cells, proteinuria
r A difficult assessment since, until recently, there was ASSOCIATED CONDITIONS r Urine eosinophils indicate interstitial nephritis
no unifying criteria to make the diagnosis CKD increases the risk for AKI
r Strict UOP monitoring to assess AKI stage and
Recent meta-analysis showed reported incidences GENERAL PREVENTION
r Prerenal progression
in hospitalized pediatric population from 182% r Atypical-AKI
(3) Prevent volume depletion (1)[B] Clinical AKI fails to meet the definition
Prevalence Maintain cardiac output and oxygenation with SCr falsely lowered by dilution due to large
Unknown vasopressors and blood transfusions as needed volume fluid resuscitation or transfusions,
RISK FACTORS (1)[C] thereby reflecting the blood donors kidney
r Chronic kidney disease (CKD) increases the risk for r Intrinsic renal function
AKI Careful dosing and therapeutic drug level SCr falsely lowered by muscle wasting or
r Hospitalization, especially in the ICU monitoring of aminoglycosides or avoidance reduced muscle mass due to quadriplegia,
r Exposure to potentially nephrotoxic agents, such as altogether cerebral palsy, or other neuromuscular disorders
Use lipid formulation of amphotericin B or another SCr falsely lowered during sepsis by decreased
nonsteroidal anti-inflammatories (NSAIDs), contrast,
antifungal alternative muscle perfusion
aminoglycosides r Real-time markers of AKI are being sought
r Recent surgery, solid organ or marrow transplant, Contrast-induced AKI
Avoid the use of contrast Candidates include serum cystatin C, kidney injury
cardiopulmonary bypass
Use either iso- or low-osmolar iodinated molecule-1, interleukin-18, liver fatty acidbinding
Genetics contrast media protein, neutrophil gelatinase-associated lipocalin
r If recurrent rhabdomyolysis, consider an underlying
Intravenous fluid expansion for those patients at Imaging
defect in muscle metabolism risk r Renal ultrasound with Doppler analysis of the renal
r If recurrent hemolytic uremic syndrome (HUS)
Avoid NSAIDs artery and veins
consider a defect in the complement cascade Animal models have shown the potential benefit Hydronephrosis indicates obstruction
r If AKI in the presence of CKD then consider inherited of vasodilators, growth factors, antioxidants, and Increased echogenicity consistent with medical
forms of ESRD, such as autosomal recessive anti-inflammatory drugs in preventing AKI renal disease
polycystic kidney disease r A single dose of theophylline given to neonates with
Thrombosis of renal artery or vein
severe perinatal asphyxia has been shown to Small echogenic kidneys, cystic kidneys indicate
significantly reduce the risk of AKI CKD
r Bladder ultrasound
Trabeculated, thick-walled bladder may indicate
lower urinary tract abnormality such as a
neurogenic bladder or obstruction
r Technetium-99m MAG3 renal scan can be used in
prolonged AKI to differentiate prolonged ATN from
permanent cortical necrosis
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ACUTE SCROTUM
Patrick T. Gomella, MD, MPH
Leonard G. Gomella, MD, FACS
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ACUTE SCROTUM
A
Diagnostic Procedures/Surgery r Epididymitis: Chronic FOLLOW-UP
In cases of testicular torsion, surgical exploration is Scrotal elevation, avoid sexual and athletic Patient Monitoring
usually diagnostic and therapeutic activity, warm baths, and NSAIDs Epididymitis due to culture-proven C. trachomatis or
r Appendix torsion: Ibuprofen to reduce inflammation N. gonorrhoeae: refer sex partners for evaluation and
Pathologic Findings
N/A and discomfort treatment disease
r Testis torsion: Pain control may require opioids
DIFFERENTIAL DIAGNOSIS Patient Resources
r Abscess or other infection such as Fournier gangrene Second Line MedlinePlus: Testicular torsion http://www.nlm.
r Appendix torsion (appendix testis or epididymis N/A nih.gov/medlineplus/ency/article/000517.htm
testis) SURGERY/OTHER PROCEDURES
Most commonly seen in prepubertal boys r Urgent scrotal exploration, bilateral fixation for REFERENCES
Most common cause of acute scrotum in this age extravaginal testicular torsion to avoid asynchronous
group contralateral torsion 1. Cummings JM, Boullier JA, Sekhon D, et al. Adult
r Epididymitis due to UTI or STD: Rare or uncommon r Manual detorsion: Use only if surgery is delayed testicular torsion. J Urol. 2002;167(5):21092110.
in pediatric age group; more likely in adult >2 hr 2. Rabinowitz R. The importance of the cremasteric
r Fat necrosis of scrotal wall Testicle most often rotates medially during torsion reflex in acute scrotal swelling in children. J Urol.
r HenochSchonlein purpura Manual detorsion is accomplished by attempting 1984;132(1):8990.
Rash usually present to rotate the testicle laterally toward the thigh
r Incarcerated inguinal hernia The twisting can range from 180720 degrees
r Orchitis: With the exception of mumps orchitis, such that multiple detorsion twists may be ADDITIONAL READING
isolated orchitis without epididymitis in adults is rare required
Yu KJ, Wang TM, Chen HW, et al. The dilemma in the
r Referred pain: Urolithiasis or intra-abdominal However in up to 1/3 of cases, the torsion rotation
diagnosis of acute scrotum: Clinical clues for
process such as appendicitis can be lateral
differentiating between testicular torsion and
r Testicular infarction due to spermatic cord injury or Successful detorsion still requires operative
epididymo-orchitis. Chang Gung Med J. 2012;
intervention and orchidopexy
thrombosis 35(1):3845.
r Testicular torsion: Most common in peripubertal Hallmarks of successful manual detorsion include
pain relief, testicle assuming a lower position in See Also (Topic, Algorithm, Media)
boys but can occur at any age; less common than the scrotum, reorientation of the testicle from r Acute Scrotum Algorithm
appendix torsion transverse lie to vertical positioning, restoration of r Acute Scrotum Image
r Testicular tumor: Usually painless but may have
Doppler blood flow r Appendix Testis and Appendix Epididymis, Torsion
tenderness with trauma r Epididymitis
r Trauma and possible testicular rupture: History ADDITIONAL TREATMENT
r Torsion, Testis, or Testicular/Epididymal Appendages
suggestive; hematocele usually present Radiation Therapy
r Orchalgia; consider voiding dysfunction N/A
Additional Therapies
N/A CODES
TREATMENT
Complementary & Alternative ICD9
Therapies r 604.90 Orchitis and epididymitis, unspecified
ALERT N/A r 608.9 Unspecified disorder of male genital organs
Testicular torsion is a surgical emergency because r 608.20 Torsion of testis, unspecified
the likelihood of testicular salvage diminishes with
the duration of torsion. ONGOING CARE
ICD10
PROGNOSIS r N44.00 Torsion of testis, unspecified
GENERAL MEASURES r In cases of testicular torsion 12 hr is considered the r N45.3 Epididymo-orchitis
r Clinical history, exam, and diagnostic studies
point at which the testis suffers irreversible damage r N50.9 Disorder of male genital organs, unspecified
(urinalysis, Color Doppler Ultrasound) have a high r Torsion surgery outcomes appear better in children
degree of accuracy in making the diagnosis
r Emergent exploration indicated if evaluation than in adults
Salvage rates in males <21 yr was 70% vs. those CLINICAL/SURGICAL
suggests intravaginal testicular torsion or diagnosis
>21 yr who had a salvage rate of 41% (3) PEARLS
is equivocal Potential explanations: Time to presentation
r If torsion is present and surgery cannot be r Color Doppler ultrasonography is the preferred
impacted salvage and patients over 21 yr of age
performed in a reasonable amount of time, manual had a greater degree of cord twisting than the imaging technique for evaluating the acute scrotum.
detorsion should be considered younger patients r Cremasteric reflex is usually absent in testicular
r Most cases of epididymitis can be treated on an
COMPLICATIONS torsion.
outpatient basis
r Testicular torsion
MEDICATION Testicular loss and or atrophy
First Line Infertility
r Epididymitis: Acute r Appendix testis/epididymis torsion
Ice, scrotal elevation, and NSAIDs with antipyretic Usually none long term
for high temperature r Epididymitis
Younger male: Ceftriaxone (250 mg IM) with Scrotal abscess
doxycycline (100 mg PO BID 10 days). Urosepsis
Older males: Ceftriaxone (250 mg IM) along with Chronic orchalgia
a 10-day course of fluoroquinolone for enteric
organisms (ofloxacin 300 mg PO BID or
levofloxacin (500 mg PO BID)
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ADDISON DISEASE
Shaun G.S. Grewal, MD
Gerald L. Andriole, MD, FACS
r APS type 2
BASICS Adrenal insufficiency, Thyroid disease, Type I DM DIAGNOSIS
HLA-DR3, CTLA-4
DESCRIPTION r APS type 4 HISTORY
r Primary adrenal insufficiency r Vague symptoms; requires high index of suspicion
Other autoimmune diseases
r Inadequate production of glucocorticoid and r Congenital adrenal hyperplasia Fatigue, weight loss, anorexia, vomiting, GI
mineralocorticoid complaints, abdominal pain, diarrhea, muscle
21-hydroxylase (CYP21 mutation)
Differentiated from secondary (pituitary) and aches, salt craving, hypotension, behavioral
11-hydroxylase (CYP 11B1 mutation)
tertiary (hypothalamic) causes of adrenal causes of changes, headaches, sweating, depression,
17-hydroxylase (CYP17 mutation)
adrenocorticoid insufficiency in which r Adrenoleukodystrophy (ALD) decreased libido, lethargy
mineralocorticoids are normally spared Acute adrenal insufficiency: Life-threatening
Demyelination of CNS hypotension, acute abdominal pain, vomiting,
r Triple A syndrome (Allgrove)
ALERT fevers
Alacrima, achalasia, neurologic impairment
Acute adrenal insufficiency (Addisonian crisis): PHYSICAL EXAM
r Life-threatening hypotensive shock. PATHOPHYSIOLOGY r Vitals: Orthostatic hypotension
r Autoimmune disorders are the most common cause r Weight loss
r Most common cause is acute withdrawal of
in developed nations (8090%) r Hyperpigmentation
chronic steroid. r Partial or complete T-cell mediated destruction of
r Acute stress (ie, surgery) without an adequate r Pigmented buccal mucosa and nail beds
adrenal cells r Loss of axillary and pubic hair
stress dose of steroids. 90% of adrenal gland must be destroyed to cause r Vitiligo
insufficiency
r Goiter
EPIDEMIOLOGY Decreased production of cortisol, aldosterone, and
Incidence adrenal androgens DIAGNOSTIC TESTS & INTERPRETATION
r 4.76.2 per million in Western populations (1) Hypovolemia and prerenal azotemia cause
Lab
r Females more frequently affected than males orthostatic hypotension, dizziness, and lethargy r Electrolyte disturbances
TB most common cause in underdeveloped Adrenal crisis mostly attributable to
Classic triad: Hyponatremia, hyperkalemia,
nations mineralocorticoid deficiency
azotemia
Autoimmune disorders most common cause in Pituitary compensation with increased ACTH
Hypercalcemia
developed nations (90%) ACTH and proopiomelanocortin-related peptides
Lymphocytosis
stimulate melanocytes causing hyperpigmentation
Prevalence r Adrenal dysgenesis or hypoplasia Hypoglycemia
r 93140 per million (1) Metabolic acidosis
AHC or Triple A syndrome r Screening test
Mortality 0.3 per 100,000 r Adrenal destruction
Measure cortisol, ACTH
RISK FACTORS APS1, APS2, APS4, ALD Low cortisol (<165 nmol/L)
r Tuberculosis Infectious Elevated ACTH (>45 pmol/L)
r Autoimmune disease TB, HIV, CMV, histoplasmosis, cryptococcus, r Confirmation of abnormal screening test
r AIDS coccidioidomycosis
Short corticotropin test
r Immunosuppression Adrenal hemorrhage 250 g ACTH
r Bilateral adrenal hemorrhage Sepsis
Serum cortisol at 0, 30, and 60 min
r Bilateral adrenalectomy Disseminated intravascular coagulation
Peak cortisol <550 nmol/L diagnostic (2)
Anticoagulant therapy
r Drug induced
Bilateral adrenalectomy Imaging
Mitotane, aminoglutethimide, etomidate, r Adrenal infiltration r Routine imaging not recommended in cases of
ketoconazole, suramin, mifepristone definite autoimmune adrenalitis
Adrenal metastasis, primary adrenallymphoma,
Genetics sarcoidosis, amyloidosis, hemochromatosis r CT or MRI in cases of suspected infection,
r 40% of patients with a 1st-/2nd-degree relative r CAH (see Genetics) malignancy, infiltration, hemorrhage
with an associated disorder r Calcifications present in up to 50% with TB
r Isolated autoimmune adrenalitis ASSOCIATED CONDITIONS
r Autoimmune endocrine disorders
HLA-DR3, CTLA 4 r Thyroid disorder (17%)
r APS type 1
r Diabetes mellitus (12%)
Adrenal insufficiency, hypoparathyroidism, chronic
r Gonadal dysfunction (12%)
mucocutaneous candidiasis
AIRE gene (21q22) GENERAL PREVENTION
No general prevention guidelines exist for prevention
of primary hypoaldosteronism.
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ADDISON DISEASE
A
Diagnostic Procedures/Surgery SURGERY/OTHER PROCEDURES FOLLOW-UP
No specific diagnostic procedures Stress dose steroids: 25150 mg hydrocortisone or Patient Monitoring
530 mg methylprednisolone IV day of the procedure r Medic-alert bracelet to be worn at all times
Pathologic Findings
Atrophic adrenals in autoimmune adrenalitis in addition to maintenance therapy; taper to the usual r Instruct patients on proper use of emergency
dose over 12 days. hydrocortisone injections
DIFFERENTIAL DIAGNOSIS r Monitor for signs of appropriate glucocorticoid and
r Primary adrenal insufficiency (Addison disease) ADDITIONAL TREATMENT
r Secondary adrenal insufficiency (pituitary failure) Radiation Therapy mineralocorticoid replacement
No hyperpigmentation (lack of ACTH elevation) N/A Patient Resources
r www.addisonsdisease.net
Etiologies include chronic steroids, Additional Therapies
panhypopituitarism, Sheehan syndrome r Salt loading prior to major stress recommended by r www.addisonssupport.com
(postpartum necrosis), brain trauma, pituitary some
apoplexy, pituitary surgery r Future advances using long-acting hydrocortisone
r Tertiary adrenocortical insufficiency preparations to better mimic physiologic state
REFERENCES
Complementary & Alternative 1. Arlt W, Allolio B. Adrenal Insufficiency. Lancet.
Therapies 2003;361:18811893.
TREATMENT
No established alternative therapies 2. Lvas K, Husebye E. Addisons disease. Lancet.
GENERAL MEASURES 2005;365:20582061.
r Acute adrenal insufficiency (addisonian crisis) 3. Reisch N. Fine tuning for quality of life: 21st
5 Ss: ONGOING CARE century approach to treatment of Addisons
Salt disease. Endocrinol Metab Clin North Am. 2009;
PROGNOSIS
Sugar r Adrenal crisis may be lethal. 38:407418.
Steroids r Recommended dosages for glucocorticoid and
Support
mineralocorticoid replacement rarely cause
Search for precipitating cause
significant side effects; close monitoring is essential
ADDITIONAL READING
MEDICATION to prevent excess treatment. Chakera AJ. Addison disease in adults: Diagnosis and
First Line COMPLICATIONS management. Am J Med. 2010;123:409413.
r Corticosteroid replacement: r Side effects of excess steroid replacement:
Hydrocortisone 1525 mg/d See Also (Topic, Algorithm, Media)
Weight gain, high BP, hyperglycemia, growth r Addison Disease (Adrenocortical Insufficiency)
BID dosing: 20 mg, 10 mg
TID dosing: 10 mg, 5 mg, 5 mg
retardation, bruising, cardiovascular risks, gastric Algorithm
ulcers, poor wound healing, skin striae, r WaterhouseFriderichsen Syndrome
Monitor body weight and signs/symptoms of osteoporosis
over/under replacement r Side effects of excess mineralocorticoid:
r Mineralocorticoid replacement:
Hypertension, bradycardia, hypernatremia, CODES
Fludrocortisone 0.050.20 mg/d congestive heart failure, suppressed renin levels,
Monitor blood pressure, peripheral edema, serum growth retardation
sodium, and potassium r Acute withdrawal of chronic steroid replacement ICD9
r Major stress: Surgery, trauma, sepsis: 255.41 Glucocorticoid deficiency
may precipitate acute adrenal crisis
IV hydrocortisone 100300 mg/d (TID dosing) r Must rule out or treat glucocorticoid deficiency prior ICD10
then taper to initiation of thyroxine for hypothyroidism, as this E27.1 Primary adrenocortical insufficiency
r Minor stress
may precipitate adrenal crisis.
Increase steroid dose 23-fold then taper over
several days CLINICAL/SURGICAL
Second Line PEARLS
r Dehydroepiandrosterone (DHEA) replacement
r Addisonian crisis: Is acute, life-threatening shock.
2550 mg/d r 5 Ss for treatment of addisonian crisis
Impacts mood/feeling of well-being (3)
Salt; Sugar; Steroids; Support; Search for
precipitating cause.
r Classic triad: Hyponatremia, Hyperkalemia,
Azotemia.
r Use stress dose steroids for patients with Addison
disease undergoing surgical procedures.
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Diagnostic Procedures/Surgery
BASICS DIAGNOSIS r Excision via inguinal approach
r Frozen section for pathology proceed to
DESCRIPTION HISTORY orchiectomy with high cord ligation if malignant
r Adenomatoid tumors are benign lesions of the male r Duration of lesion and size
testicular adnexa. r Interval growth Pathologic Findings
r Associated pain, dysuria, tenderness epididymitis r Gross
r Usually 1 cm in size (range 0.57.5 cm) (1)[C]
r Most often asymptomatic r Prior malignancy or scrotal pathology Small (1 cm), well circumscribed without fibrous
r Mesenchymal origin (2)[C] r Exposure to tuberculosis (TB) capsule
Tan-white, homogeneous
r History of sarcoidosis, histoplasmosis r Microscopic
EPIDEMIOLOGY r History of urinary tract infection or sexually
Incidence Adenomatoid cells within fibrous stroma
r The majority of patients present within the 3rd5th transmitted infection Occasional cystic dilation
r Recent GU manipulation bacillus CalmetteGuerin Irregular, somewhat branched-appearing tubular
decades of life (3)[C].
r Adenomatoid tumors are the most common (BCG) instillation structures appear within the tumor, a coalescence
neoplastic processes involving the testicular adnexal PHYSICAL EXAM of the cellular vacuoles, which form a false lumen
and spermatic cord structures (3)[C]. r Scrotal exam (4)[C]
r Adenomatoid tumors in females occur in uterus > Identify location of mass single or multiple DIFFERENTIAL DIAGNOSIS
fallopian tubes > ovary (1)[C]. Evaluate for varicocele or hydrocele r Benign tumors of epididymis:
Compare with contralateral scrotal contents Leiomyoma
Prevalence Evaluate if fixed, mobile, indurated, or
Not well defined Papillary cystadenoma (associated with von
encroaching on other structures HippelLindau syndrome)
RISK FACTORS Evaluate for spermatic cord involvement Lipomas
None described Transillumination to identify if fluid filled Hamartomas
Genetics (spermatocele, hydrocele) Adrenal cortical adenomas
r Inguinal exam r Malignant tumors of the epididymis:
N/A
Evaluate for lymphadenopathy Sarcoma (rhabdomyosarcoma, leiomyosarcoma,
PATHOPHYSIOLOGY Hernia fibrosarcoma, liposarcoma)
r Mesothelial origin is most accepted theory (3)[C]
DIAGNOSTIC TESTS & INTERPRETATION Melanotic neuroectodermal tumor of the
r Benign with no reported cases of metastasis
epididymis
r Capable of local invasion Lab r Extension of primary testicular tumor
r Tumor markers if concern for testicular mass
r Epididymis, tunica vaginalis, spermatic cord are most r Metastatic tumor to epididymis:
-fetoprotein (AFP),
common sites Urologic (prostate, kidney)
-human chorionic gonadotropin (-HCG)
ASSOCIATED CONDITIONS Lactate dehydrogenase (LDH) GI (stomach, colon, carcinoid, pancreas)
Purified protein derivative (PPD) if TB suspected r Other lesions of the epididymis
N/A
No specific labs to diagnose adenomatoid Granuloma (sperm, TB, sarcoidosis)
GENERAL PREVENTION Spermatocele
r Routine self-exam for identification of scrotal tumors
Epididymitis
content masses Imaging Epidermoid inclusion cyst
r Routine genital exam by physician r Scrotal ultrasound
Epididymal abscess
Solid vs. cystic
Location testicular or paratesticular
If located in the tunica albuginea can grow into
the testicular parenchyma and resemble a
testicular malignancy
Vascular or avascular
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ADRENAL ADENOMA
Aaron G. Boonjindasup, MD, MPH
Raju Thomas, MD, MHA, FACS
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ADRENAL ADENOMA
A
DIFFERENTIAL DIAGNOSIS r Steroid supplementation will be needed after COMPLICATIONS
r Adrenal cortical carcinoma (up to 80% functional) adrenalectomy for cortisol-producing tumors until r Hypertension
r Adrenal hemorrhage suppressed HPA recovers (median of 15 mo) r Diabetes mellitus
Bilateral lesions Postoperatively (POD 2): Hydrocortisone 20 mg PO r Atherosclerosis
r Adrenal hyperplasia (pituitary hypersecretion of qAM, 10 mg qPM r Poor wound healing
ACTH) Hydrocortisone slowly tapered over 3 mo to r Nephrolithiasis
r Adrenal myelolipoma 10 mg daily
15% of patients with Cushing syndrome due to
r Lymphoma AM cortisol should be measured and repeated
hypercalciuria
r Metastatic lesion until >10 ng/dL r Adrenal insufficiency (Addison disease)
Confirm recovery of HPA with cosyntropin test
Melanoma, lung, breast, kidney r Monitor for electrolyte disturbances with BMP and
r Neuroblastoma FOLLOW-UP
r Nonfunctioning adenoma postoperative AI in patients with hormonally active Patient Monitoring
tumors and/or SCS r Nonfunctioning benign adrenal mass can be
r Pheochromocytoma r Acute AI (addisonian state) followed with physical and radiologic examinations
r TB, or other infectious cause
520% show enlargement >1 cm
ALERT No guideline on growth velocity on surgical
This is a life-threating condition often preceded by treatment
TREATMENT hypotension unresponsive to fluid resuscitation.
GENERAL MEASURES r May occur in the postoperative state in the setting
r Based on functional status and size of lesion of cortisol-secreting lesion with downregulated
REFERENCES
r Correct hypertension and electrolyte abnormalities. contralateral adrenal function, and in patients 1. Kapoor A, Morris T, Rebello R. Guidelines for the
MEDICATION with previous contralateral adrenal resection or management of the incidentally discovered adrenal
due to concurrent illness or infection. mass. Can Urol Assoc J. 2011;5(4):241247.
First Line r Other nonspecific symptoms may include
r For hormonally active adenomas in patients who 2. Gill IS. The case for laparoscopic adrenalectomy.
refuse surgery or have contraindications to surgery abdominal pain, salt craving, nausea, vomiting, J Urol. 2001;166:429436.
r Conn syndrome fatigue, and fever. 3. Bittner JG, Brunt LM. Evaluation and management
r Electrolyte abnormalities such as hypernatremia or of adrenal incidentaloma. J Surg Onc. 2012;106:
Spironolactone, eplerenone
Aldosterone receptor antagonists in the distal hyperkalemia and other laboratory anomalies such 557564.
convoluted tubule (DCT). as anemia, lymphocytosis, or eosinophilia may 4. Mandevillle J, Moinzadeh A. Adrenal
2nd line Amiloride, triamterene also be found. incidentalomas: AUA Update Series. 2010;29:
Inhibitors of DCT aldosterone sensitive sodium r Prolonged use of etomidate may increase risk of 3339.
channels postoperative adrenal insufficiency. See Also (Topic, Algorithm, Media)
r Cushing syndrome r May begin steroid replacement if high clinical r Adrenal Adenoma Image
Aminoglutethimide index of suspicion. r Adrenal Cortical Carcinoma
Blocks the 1st step in cortisol synthesis r Diagnosis Obtain AM serum cortisol and ACTH r Adrenal Cysts and Pseudocysts
(cholesterol to pregnenolone) r Adrenal Hemorrhage
level:
Metapyrone r Adrenal Incidentaloma
Blocks the final step in cortisol synthesis Normal >10 ng/dL, lownormal
(3.410 ng/dL), AI <3.4 ng/dL r Adrenal Mass
(11-deoxycortisol to cortisol)
Confirmatory testing with evaluation of r Adrenal Mass, Algorithm
Ketoconazole
Inhibits 1st step and to a lesser extent the last response to ACTH stimulation (cosyntropin test) r Adrenal Mass Image
step in cortisol synthesis Measure serum cortisol at baseline r Adrenal Metastasis
Give cosyntropin 0.25 mg IV 1 r Adrenal Myelolipoma
Second Line Measure serum cortisol 60 min after dose r Adrenal Myelolipoma (Adrenal Myolipoma)
N/A
Adequate response: Cortisol >18 g/dL
SURGERY/OTHER PROCEDURES r If acute AI is highly suspected, dont wait for
r Surgical indications
Hormonally active masses
result before treating: CODES
Give 23 L D5 NS quickly and 4 mg
Any masses 5 cm (25% of masses >6 cm are dexamethasone IV
assumed to be adrenal cortical carcinomas) ICD9
Use dexamethasone because IV cortisol will r 227.0 Benign neoplasm of adrenal gland
Masses with suspicious imaging characteristics of
interfere with the diagnosis later during r 255.0 Cushings syndrome
carcinoma
Homogeneous, irregular borders, HU >20 hospitalization r 255.12 Conns syndrome
r Laparoscopic and robotic approaches described, but r Maintenance therapy:
may have limitations with larger lesions Hydrocortisone 30 mg/d ICD10
r D35.00 Benign neoplasm of unspecified adrenal
r Retroperitoneal approach possible for both open Fluorohydrocortisone 0.050.1 g/d
gland
and laparoscopic surgery; may reduce ileus r E24.0 Pituitary-dependent Cushings disease
r Perioperative stress dose steroids indicated during
ONGOING CARE r E26.01 Conns syndrome
unilateral adrenalectomy for cortisol-producing
adenomas and may be indicated for patients with PROGNOSIS
SCS r Untreated Cushing syndrome can be fatal due to CLINICAL/SURGICAL
r Preoperatively: 50 mg hydrocortisone IV q8h postop
day 1
cardiovascular, thromboembolic, or hypertensive PEARLS
complications or infection
r Surgical removal of hormonally active adenomas is r Adrenal lesions should be surgically treated if
usually curative 5 cm or if functional/active.
r No guideline on normal growth velocity for adrenal
lesions.
r Melanoma, lung, breast, colon, and renal cell
cancers have metastatic predilection to adrenal
gland.
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Imaging
BASICS DIAGNOSIS r CT of abdomen is preferred initial study in patients
adrenal lesion:
DESCRIPTION HISTORY Benign tumors
r Most common symptoms are related to excess
Adrenal cortical carcinoma is a primary malignancy Homogeneous appearance with well-delineated
arising in the adrenal cortex cortisol production (Cushings syndrome) in margins
5060%, then virilization (20%), or mixed Generally <46 cm, smooth and round or oval
EPIDEMIOLOGY syndromes (2030%) contour
Incidence r History of onset of symptoms <12 mo is suspicious <10 HFU or rapid washout of contrast <15 min
r Rare: 0.52 cases per million people per year
for ACC Primary ACCs:
r Bimodal occurrence: r Constitutional symptoms: Nonhomogeneous internal architecture
Initial peak in children <5 yr old; Weight loss, malaise, weakness, nausea, or Irregular contour, invasion of surrounding
2nd peak in adults in 4th and 5th decades of life vomiting usually associated with poor prognosis structures
r Female:male ratio 1.5:1 r In children, suggested by generalized weight gain >10 HFU or delayed washout of contrast
r 80130 cases in USA annually and delayed linear growth >15 min
r <5% of all adrenal incidentalomas, with correlation r Nonfunctional tumors may be larger and present r MRI not proven to be more sensitive in
between size of tumor and likelihood of ACC with mass effect differentiating malignant from benign tumors:
2% of lesions <4 cm Painful or palpable mass Preferred imaging modality for evaluation of vena
6% of lesions 46 cm Lower extremity edema caval involvement
25% of lesions >6 cm Urinary obstruction ACCs generally isodense to the liver on
Prevalence BuddChiari syndrome T1-weighted images; intermediate to high signal
Mirrors incidence, as prognosis is poor GI symptoms intensity (brighter white) on T2-weighted images
r Hyperaldosteronism (rare): (less bright than pheochromocytoma).
RISK FACTORS Hypertension r FDG-PET potentially useful in radiologically
Genetic associations (see below) Hypokalemic alkalosis indeterminate lesions.
Genetics r Feminization (rare) r Bone scan if suspicious of skeletal metastases.
r Sporadic cases r Incidental finding during imaging workup for other
Diagnostic Procedures/Surgery
Inactivation of p53 on 17q13 morbidities r Role of percutaneous biopsy limited
Alterations at 11p15 locus, site of IGF-2 Difficult to distinguish between benign and
Activation of -catenin gene PHYSICAL EXAM
r Familial syndromes r Palpable abdominal mass malignant tissue
r Signs of Cushings syndrome (functional ACCs): Concern for seeding biopsy tract
LiFraumeni syndrome
BeckwithWiedemann syndrome Violaceous striae, moon facies, truncal obesity, Pathologic Findings
buffalo hump, glucose intolerance, r Macroscopic:
Multiple endocrine neoplasia (MEN) 1
Congenital adrenal hyperplasia hyperpigmentation Lobulated, orange tumor with necrotic areas,
r Signs of virilization (oligomenorrhea, hirsutism, calcifications, intratumoral hemorrhages
Adenomatous polyposis coli
cystic acne, excessive muscle mass, voice deepening, r Microscopic:
PATHOPHYSIOLOGY temporal balding, clitoromegaly) Weiss criteria for malignancy includes 3 of the
r Difficult to distinguish benign from malignant r Gynecomastia following:
adrenal tumors in absence of metastatic disease. High nuclear grade
r Pathologic features such as mitotic activity, grade, DIAGNOSTIC TESTS & INTERPRETATION Mitotic rate > 5/50/hpf
vascular invasion, various architectural features, and Lab Atypical mitotic fevers
tumor size have not consistently correlated with r Tests for glucocorticoid excess (minimum 3 out of Eosinophilic tumor cell cytoplasm
prognosis. 4 tests) Diffuse architecture in >33% of tumor
r Most (6070%) ACCs are functioning, although this Dexamethasone suppression test Necrosis
is related to the extent of workup. 24-hr urinary free cortisol Vascular invasion
Basal cortisol (serum) Sinusoidal invasion
ASSOCIATED CONDITIONS Basal ACTH (plasma)
r Cushings syndrome secondary to functional tumors Capsular invasion
r Sexual steroids and steroid precursors r Antigen Ki-67 is a promising new
r Familial syndromes (see above)
DHEA-S (serum) immunohistochemical marker
GENERAL PREVENTION 17-OH-progesterone (serum) Marker of proliferative activity
No recommendations Testosterone (serum) Low-risk ACC expressed in <10% of cells
17--estradiol High-risk ACC expressed in >10% of cells
24-hr urine steroid metabolite exam
r Mineralocorticoid excess DIFFERENTIAL DIAGNOSIS
r Functioning adrenal masses:
Potassium (serum)
Aldosterone/renin ratio Adenoma, aldosteronoma, pheochromocytoma
r Nonfunctioning adrenal masses:
Only used in patients with arterial hypertension
and/or hypokalemia Hemorrhage, cyst, metastatic tumor,
r Catecholamine excess to exclude neuroblastoma
r Other: Renal cell carcinoma
pheochromocytoma
Meta- and normetanephrines (plasma)
Catecholamines or metanephrine excretion
(24-hr urine)
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ADRENAL MASS
Kyle A. Richards, MD
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ADRENAL MASS
A
r Adrenocortical carcinoma (3)[A] REFERENCES
Most commonly cortisol secreting TREATMENT
Serum dehydroepiandrosterone (DHEA) 1. Barzon L, Sonino N, Fallo F, et al. Prevalence and
r Sex steroid-secreting tumor GENERAL MEASURES natural history of adrenal incidentalomas. Eur J
r Observation, resection, or medical therapy Endocrinol. 2003;149:273285.
Serum testosterone and 17-estradiol in women r Depends on size of lesion, functionality, malignant 2. Young WF. The incidentally discovered adrenal
with virilization or men with feminization mass. N Engl J Med. 2007;356:601610.
potential, and overall health of patient
Imaging 3. Ng L, Libertino JM. Adrenocortical carcinoma:
MEDICATION
r CT scan (2)[A] Diagnosis, evaluation, and treatment. J Urol.
First Line 2003;169:511.
Benign adrenal adenoma r Cushings syndrome (5)[C]
Usually <3 cm and homogeneous 4. Aron D, Terzolo M, Cawood TJ. Adrenal
Aminoglutethimide, metyrapone, ketoconazole
Density <10 HU with >50% washout of r Pheochromocytoma incidentalomas. Best Pract Res Clin Endocrinol
contrast at 10 min Metab. 2012;26:6982.
Phenoxybenzamine, propranolol 5. Mandeville J, Moinzadeh A. Adrenal incidentaloma.
Adrenocortical carcinoma or adrenal mets r Primary hyperaldosteronism: Spironolactone
Usually >4 cm, heterogeneous, calcifications, AUA Update Series. 2010;29:3439.
r Adrenocortical carcinoma: Mitotane
necrosis
Density >25 HU and <50% washout of Second Line
contrast at 10 min r Adrenocortical carcinoma ADDITIONAL READING
r MRI Cisplatin, etoposide, 5-flurouracil, doxorubicin,
vincristine Taffel M, Haji-Momenian S, Nikolaidis P, et al. Adrenal
Benign adrenal adenoma imaging: A comprehensive review. Radiol Clin N Am.
Rapid contrast washout and high lipid content;
SURGERY/OTHER PROCEDURES 2012;50:219243.
isointense with liver on T2 r Should remove all functional adrenal masses
Adrenocortical carcinoma or adrenal mets r Open surgery if large or locally advanced; evaluate See Also (Topic, Algorithm, Media)
Hyperintense with liver on T2 imaging r Addison Disease
for vein thrombus or adjacent organ invasion r Adrenal Adenoma and Cortical Carcinoma
Pheochromocytoma r Minimally invasive surgery is now accepted
Light bulb sign: see very high signal intensity r Adrenal Angiomyelolipoma
r Remove masses >5 cm; high malignancy risk
on T2-weighted imaging r Adrenal Calcifications
r Metaiodobenzylguanidine (MIBG) scan r Consider partial adrenalectomy for solitary adrenals,
r Adrenal Cysts and Pseudocysts
Useful for extra-adrenal pheochromocytoma bilateral disease, familial syndromes r Adrenal Hemorrhage
Diagnostic Procedures/Surgery ADDITIONAL TREATMENT r Adrenal Incidentaloma
r Primary hyperaldosteronism Radiation Therapy r Adrenal Mass Algorithm
Adrenal vein sampling for lateralization of Only for palliation of bone metastases from adrenal r Adrenal Mass Image
aldosterone production, if unclear by imaging cortical carcinoma r Adrenal Metastasis
r Biopsy r Adrenal Myelolipoma
Additional Therapies
Rule out pheochromocytoma prior to biopsy N/A r Adrenal Oncocytoma
Helpful if concern for metastases or infection r Cushings Disease and Syndrome
Complementary & Alternative
Pathologic Findings Therapies r Pheochromocytoma
See Differential Diagnosis N/A
DIFFERENTIAL DIAGNOSIS
r Adrenal cortical tumors (4)[A] ONGOING CARE CODES
Adenoma
Carcinoma PROGNOSIS ICD9
r Adrenalectomy cures hypertension in 3372% of r 194.0 Malignant neoplasm of adrenal gland
Nodular hyperplasia
r Adrenal medullary tumors r 227.0 Benign neoplasm of adrenal gland
patients with primary hyperaldosteronism (5)[B]
r 1015% recurrence rate after resection of r 255.9 Unspecified disorder of adrenal glands
Pheochromocytoma
Ganglioneuroma/neuroblastoma pheochromocytoma ICD10
r Other adrenal tumors r Adrenocortical carcinoma has poor prognosis r C74.90 Malignant neoplasm of unsp part of
Myelolipoma, lipoma, hemangioma Mean survival 18 mo (4)[B] unspecified adrenal gland
Metastases r D35.00 Benign neoplasm of unspecified adrenal
Hamartoma, teratoma 5-yr survival 1547%
r Infectious or inflammatory gland
COMPLICATIONS r E27.9 Disorder of adrenal gland, unspecified
Abscess or fungal infection r Adrenal insufficiency post adrenalectomy
Amyloidosis, sarcoidosis r Unrecognized malignancy/pheochromocytoma
Cytomegalovirus
FOLLOW-UP
CLINICAL/SURGICAL
Cysts (pseudocysts, parasitic, epithelial- and
endothelial-lined cysts) Patient Monitoring PEARLS
r Congenital adrenal hyperplasia r Conservative management principles (2)[B] r Assess all for biochemical function.
r Hemorrhage r Remove all adrenal masses >5 cm.
Repeat imaging at 6, 12, and 24 mo
r Pseudoadrenal masses Repeat hormonal testing annually for 4 yr r Do not biopsy pheochromocytoma.
Splenic, pancreatic, renal lesions If growth 1 cm or autonomous hormonal
Vascular lesions or technical artifacts secretion, consider surgery
Patient Resources
r www.pheochromocytoma.org
r www.cancer.gov/cancertopics/types/adrenocortical
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AMYLOIDOSIS, GENITOURINARY
Christopher Wright, MD
Mark L. Jordan, MD, FACS
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AMYLOIDOSIS, GENITOURINARY
A
ADDITIONAL READING
TREATMENT ONGOING CARE r Javed A, Canales BK, Maclennan GT. Bladder
GENERAL MEASURES PROGNOSIS amyloidosis. J Urol. 2010;183:23882389.
r In AL amyloidosis treatment is aimed at reducing the r Single institution experience of 421 patients who r Mangera A, Linton KD, Fernando M, et al. What is
production of monoclonal light chain precursor with received high-dose melphalan with stem cell the evidence for the management of urethral
chemotherapy or, occasional, radiotherapy or transplant shows event-free survival and overall amyloidosis? A systematic review of the literature.
surgery of a localized amyloidogenic plasmacytoma survival of 2.6 and 6.3 yr, respectively (4) BJU Int. 2012;109:18581861.
r In AA amyloidosis treatment is generally supportive r Long-term survival in those who develop renal
See Also (Topic, Algorithm, Media)
with therapy directed at primary cause if identified failure remains poor r Amyloidosis Image
Ranges from 1224 mo r Bladder Tumors, Benign and Malignant, General
MEDICATION r AA amyloidosis has better prognosis
First Line Considerations
r High-dose melphalan chemotherapy followed by COMPLICATIONS r Bladder Mass, Differential Diagnosis
autologous blood stem cell transplantation (2) See above r Filling Defect, Upper Urinary Tract (Renal Pelvis and
2567% complete hematologic response seen in FOLLOW-UP Ureter)
single and multicenter trials Patient Monitoring
Response far exceeds cyclic oral melphalan and r Bladder or urethra
prednisone (see 2nd line) Repeat periodic surveillance cystoscopies CODES
r Colchicine can be used in FMF to prevent proteinuria
Recurrence rates >50%
Second Line r Ureters ICD9
r Low-dose oral melphalan with prednisone in a r 277.30 Amyloidosis, unspecified
US or CT to monitor hydronephrosis
cyclical fashion r 277.39 Other amyloidosis
Patient Resources r 583.81 Nephritis and nephropathy, not specified as
Rarely results in complete hematologic response Amyloidosis foundation (www.amyloidosis.org)
or reversal of amyloid-related organ dysfunction acute or chronic, in diseases classified elsewhere
SURGERY/OTHER PROCEDURES ICD10
r Renal transplant REFERENCES r E85.3 Secondary systemic amyloidosis
Graft survival similar to matched controls without 1. Kyle RA, Linos A, Beard CM, et al. Incidence and r E85.8 Other amyloidosis
amyloidosis natural history of primary systemic amyloidosis in r N08 Glomerular disorders in diseases classified
Recurs in graft in 2033% due to continued Olmsted County, Minnesota, 1950 through 1989 elsewhere
activity of underlying disease [see comments]. Blood. 1992;79:18171822.
r TUR of bladder lesion with fulguration
2. Sanchorawala V, Jacobson DR, Seldin JC, Buxbaum
Adjuvant intravesical DMSO has shown success in JN. The Amyloidoses. In: Lichtman MA, Kipps TJ, CLINICAL/SURGICAL
preventing recurrence (3) Seligsohn U, Kaushansky K, Prchal JT, eds. Williams PEARLS
ADDITIONAL TREATMENT Hematology, 8th ed. New York, NY: McGraw-Hill; r Both surgically and radiologically, genitourinary
Radiation Therapy 2010.
3. McCammon KA, Lentzner AN, Moriarty RP, et al. amyloidosis may mimic TCC in GU tract, therefore
Only rarely used for localized amyloidogenic biopsy is needed.
plasmacytoma Intravesical dimethyl sulfoxide for primary r Abdominal fat pad aspirate is preferred location to
amyloidosis of the bladder. Urology. 1998;52:
Additional Therapies 11361138. obtain biopsy, followed by bone marrow and finally
r Supportive care kidney.
4. Cibeira MT, Sanchorawala V, Seldin DC, et al.
Management of heart failure
Outcome of AL amyloidosis after high-dose
Salt restriction, diuretics, and treatment of
melphalan and autologous stem cell
secondary hyperlipidemia for nephrotic syndrome
transplantation: Long-term results in a series of
Analgesics for neuropathic pain
421 patients. Blood. 2011;118:43464352.
Complementary & Alternative
Therapies
N/A
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GENERAL PREVENTION
None
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ANORGASMIA, MALE
Robert L. Segal, MD, FRCS(C)
Arthur L. Burnett, II, MD, MBA, FACS
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ANORGASMIA, MALE
A
See Also (Topic, Algorithm, Media)
ONGOING CARE r Ejaculatory Disturbances (Delayed, Decreased, or
TREATMENT
Absent)
GENERAL MEASURES PROGNOSIS r Erectile Dysfunction, Following Pelvic Surgery or
r Treatment should be etiology specific r Continued support/psychotherapy may be required
Radiation
r May include patient/couple psychoeducation and/or r Anorgasmia related to trauma/surgery, radiation
psychosexual therapy [C] therapy, and neuropathies may not be reversible
r Pharmacologic treatment has met limited success (2)
COMPLICATIONS CODES
MEDICATION None
ICD9
First Line FOLLOW-UP r 302.74 Male orgasmic disorder
None currently FDA approved Patient Monitoring r 608.89 Other specified disorders of male genital
Second Line N/A organs
r No drugs are specifically approved for treatment of
Patient Resources
anorgasmia, so any treatment is off-label (2) N/A ICD10
r F52.32 Male orgasmic disorder
Cyproheptadine (increases cerebral serotonin
levels) r N53.11 Retarded ejaculation
Amantadine (stimulant of dopaminergic nerves) REFERENCES
Bupropion, buspirone, and yohimbine have been
anecdotally employed to reverse SSRI-induced 1. Mulhall JP, Nelson CJ. Male orgasmic disorder: CLINICAL/SURGICAL
What do we know? Contemp Urol. 2007;Feb 1.
anorgasmia PEARLS
2. McMahon CG, Jannini E, Waldinger M, et al.
SURGERY/OTHER PROCEDURES Standard operating procedures in the disorders of r Anorgasmia is often associated with ejaculatory
None orgasm and ejaculation. J Sex Med. 2013;10: disorders.
ADDITIONAL TREATMENT 204229. r Unless a specific organic cause is noted, anorgasmia
Radiation Therapy 3. Rowland D, McMahon CG, Abdo C, et al. Disorders is associated with underlying psychological factors.
of orgasm and ejaculation in men. J Sex Med. r Anorgasmia related to trauma/surgery, radiation
N/A
2010;7(4 Pt 2):16681686. therapy, and neuropathies may not be reversible.
Additional Therapies 4. McMahon CG, Abdo C, Incrocci L, et al. Disorders r There are no approved pharmacologic treatments
r Psychotherapy
of orgasm and ejaculation in men. J Sex Med. for anorgasmia.
r Masturbation retraining
2004;1:5865.
r Education on revised sexual techniques which
maximize arousal
Complementary & Alternative ADDITIONAL READING
Therapies r Perloff MD, Thaler DE, Otis JA. Anorgasmia with
Yohimbine has utility in anecdotal reports gabapentinxs may be common in older patients. Am
J Geriatr Pharmacother. 2011;9(3):199203.
r Segraves RT. Considerations for a better definition of
male orgasmic disorder in DSM V. J Sex Med.
2010;7(2 Pt 1):690695.
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DIFFERENTIAL DIAGNOSIS
BASICS DIAGNOSIS r Prerenal
Burns
DESCRIPTION HISTORY Dehydration
r Typically 1st sign of impaired renal function r Age, sex
Drugs
r Anuria: No urine output r Duration of symptoms
GI losses
r Oliguria: Significantly reduced urine volume r Pre-existing renal disease Heart disease
<1 mL/kg/h in infants r Medications Hemorrhage
<0.5 mL/kg/h in children r Symptoms of urinary tract obstruction Respiratory distress syndrome
r Antenatal history Shock/sepsis
EPIDEMIOLOGY r Family history r Intrinsic renal disease
Incidence Acute tubular necrosis
r 10% of newborns in the NICU (1)[C] PHYSICAL EXAM Exposure to nephrotoxins (drugs, myoglobin, uric
r 25% of children in the ICU (1)[C] r Signs of hypovolemia
acid)
r 1030% of children undergoing cardiac surgery Tachycardia Congenital kidney disease
(1)[C] Hypotension Renal vascular abnormalities
Decreased skin turgor Glomerulonephritis
Prevalence Dry mucous membranes r Urinary tract obstruction
N/A r Signs of hypervolemia
Neurogenic bladder
RISK FACTORS Edema Posterior urethral valves
r Hypovolemia r Signs of obstructive uropathy Meatal stenosis
r Intrinsic renal disease Palpable bladder or kidney Bilateral UPJ or ureteral obstruction or unilateral
r Urinary tract obstruction Meatal stenosis in a solitary kidney
r Glomerulonephritis Bilateral obstructing calculi
r Nephrotoxic medications
DIAGNOSTIC TESTS & INTERPRETATION
Lab
Genetics r Urinalysis
TREATMENT
Dependent on diagnosis Protein, red cells, casts: Possible
PATHOPHYSIOLOGY
glomerulonephritis GENERAL MEASURES
r Prerenal failure Low specific gravity: Possible acute interstitial r Treatment of the underlying cause
nephritis or intrinsic renal disease r Appropriate medical managements for acidbase
Most common cause of oliguria
High specific gravity: Possible prerenal cause disorder, fluid imbalance, electrolyte imbalance
Hypoperfusion in otherwise normal kidneys
Nitrate: Suggests infection (such as hyperkalemia, hyperphosphatemia,
Administration of nephrotoxic agents can r Basic metabolic panel
precipitate oliguria when reduced renal perfusion hypocalcemia)
BUN/Cr ratio: >20 suggests prerenal cause r Strict volume monitoring of input and output
is present
r Intrinsic renal failure Evaluate renal function and electrolyte balance r Avoidance of nephrotoxic agents (NSAIDs, ARBs
Associated with structural kidney damage Imaging (angiotensin receptor blockers), ACEIs
including acute tubular necrosis (ischemia, drugs, r Renal and bladder ultrasound for hydronephrosis (angiotensin-converting-enzyme inhibitors))
or toxins), primary glomerular diseases, or vascular and bladder distention and thickening of the wall MEDICATION
lesions r VCUG for suspected bladder outlet obstruction
r Nuclear renal scan for function, dysplasia, and First Line
Altered tubule cell metabolism leads to ischemia,
Hydration to optimize cardiac output and volume
then altered metabolism and subsequently cell drainage status
death
r Postrenal failure Diagnostic Procedures/Surgery Second Line
Placement of a urethral catheter r Diuretics considered if adequate intravascular
Obstructive uropathy
Usually reversible with relief of the obstruction Pathologic Findings volume status and patient remains oliguric
Dependent on diagnosis r Hemodialysis or peritoneal dialysis to be considered
ASSOCIATED CONDITIONS if severe electrolyte abnormalities or volume
r Pre-existing renal disease
overload
r Obstructive uropathy
GENERAL PREVENTION
r Maintain adequate hydration
r Avoid nephrotoxic agents in children with
underlying renal disease
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AUTONOMIC DYSREFLEXIA
Michael J. Amirian, MD
Patrick J. Shenot, MD, FACS
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AUTONOMIC DYSREFLEXIA
A
SURGERY/OTHER PROCEDURES Patient Resources See Also (Topic, Algorithm, Media)
r Sphincterotomy or sphincter stent prosthesis Christopher & Dana Reeve Foundation Paralysis r Autonomic Dysreflexia Image
Resource Center. Autonomic Dysreflexia.http://www. r Detrusor-Sphincter Dyssynergia
Allows reflex voiding with low-pressure bladder
emptying into a condom catheter (2)[A]. paralysis.org/site/c.erJMJUOxFmH/b.1338071/ r Spinal Cord Injury
r Bladder augmentation k.5E45/Autonomic Dysreflexia.htm
Only in patients with ability to catheterize
r Sacral rhizotomy REFERENCES CODES
For severe cases (3)[B] 1. Vaidyanathan S, Soni BM, Sett P, et al. ICD9
ADDITIONAL TREATMENT Pathophysiology of autonomic dysreflexia: r 337.3 Autonomic dysreflexia
Radiation Therapy Long-term treatment with terazosin in adult and r 596.89 Other specified disorders of bladder
N/A pediatric spinal cord injury patients manifesting r 599.0 Urinary tract infection, site not specified
recurrent dysreflexic episodes. Spinal Cord.
Additional Therapies 1998;36:761770. ICD10
N/A 2. Chancellor M, Gajewski J, Ackmain CF, et al. r G90.4 Autonomic dysreflexia
Complementary & Alternative Long-term follow-up of the North American r N32.89 Other specified disorders of bladder
Therapies Multicenter UroLume Trial for the treatment of r N39.0 Urinary tract infection, site not specified
N/A external detrusor-sphincter dyssynergia. J Urol.
1999;161:15451550.
3. Hohenfellner M, Pannek J, Botel U, et al. Sacral CLINICAL/SURGICAL
ONGOING CARE bladder denervation for treatment of detrusor PEARLS
PROGNOSIS hyperreflexia and autonomic dysreflexia. Urology.
2001;58:2832. r Most common triggers are from the genitourinary
Managed effectively will have little impact on patient
system such as bladder distention or
COMPLICATIONS instrumentation.
Intracerebral and subarachnoid hemorrhage ADDITIONAL READING r AD occurs at and above level of T6.
FOLLOW-UP r Chronic treatment with -blockers may improve
Consortium for Spinal Cord Medicine. Acute
Patient Monitoring some symptoms of AD.
r Clean intermittent catheterization management of autonomic dysreflexia: Individuals r Sphincterotomy or sphincter stent prosthesis allows
with spinal cord injury presenting to health-care
Frequent (at least 4 times daily) facilities, 2nd ed. Available at http://www.pva. reflex voiding with low-pressure bladder emptying
r Regular bowel program into a condom catheter.
org/site/c.ajIRK9NJLcJ2E/b.6305831/k.986B/
r Assess AD symptoms and BP at every appointment Guidelines and Publications.htm, Accessed April 8,
r Teach SCI patients significance of AD 2013.
Symptoms should prompt patients to empty
bladder and bowel
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RISK FACTORS
BASICS Age, diabetes mellitus, sexual intercourse, use of DIAGNOSIS
diaphragm or spermatocide, delayed postcoital
DESCRIPTION micturition, history of recent infection, HISTORY
r Urinary tract infection (UTI) is an inflammatory r Dysuria, frequency, urgency, malaise, rarely
immunosuppression, long-term indwelling catheters,
response of urothelium to bacterial invasion that is pregnancy, neurologic disorders, foreign bodies, low-grade fever, malodorous urine
usually associated with bacteria and pyuria. r Occasionally hematuria (gross): Especially in the
stones, obstructive uropathy, vesicoureteral reflux.
r Bacteriuria: Presence of bacteria in the urine, which female patient; uncommon in children and men
Genetics r Fever and flank pain with upper tract origin:
is normally bacteria free Certain populations may be more susceptible to
r Bacteriuria = valid indicator of bacterial infection or Pyelonephritis
bacteriuria and recurrent UTIs due to distinct r Asymptomatic or atypical symptoms: Young and old
colonization molecular defects causing impaired host responses.
Can be either symptomatic or asymptomatic Certain receptor sites on epithelial cells may patients
Significant bacteriuria: Quantitative count r Young patients: Abdominal discomfort, failure to
predispose some women to UTIs.
>1 105 colony forming units (CFL/mL) in 2 thrive, fever, vomiting, jaundice
consecutive specimens PATHOPHYSIOLOGY r Older patients: May be asymptomatic or have
r Urinary tract is normally sterile.
Majority of individuals with significant bacteria incontinence, fevers, frequency, and urgency
r Bacteriuria usually ascends up the urinary tract from r Varied symptoms with sterile pyuria associated with
have significant pyuria
Usually 1 organism colonizing flora of the gut, vagina, or distal urethra. differeing conditions
>1 organism: Either contamination or r Bacteriuria can also invade the urinary tract r History of childhood fevers: May imply UTIs and
polymicrobial infection hematogenously or through direct transfer after associated congenital abnormalities
r Pyuria: Presence of WBC in the urine: instrumentation. r Problems with toilet training, urgency, incontinence
Generally implies an inflammatory response or r Bacteria colonize the urinary tract and then multiply,
r UTI family history: Mothers, daughters, sisters
infection causing inflammation with pyuria. r History of a risk factor for bacteriuria
Significant pyuria: >10 WBCs/HPF centrifuged r Bacterial factors:
Close association between pyuria and bacteriuria; Certain bacteria are more efficient at adhering to PHYSICAL EXAM
96% of patients who are symptomatic and mucosal cells than others due to fimbria. r Suprapubic tenderness: Cystitis
bacteriuric have >10 WBCs/HPF Virulence factors: Hemolysis, adhesions, colicin, r Flank tenderness: Pyelonephritis
r Sterile pyuria: Presence of WBCs in the urine in the metabolic properties, etc. r Fever: Usually with upper tract infection
absence of bacteriuria: r Host factors: r Children may have abdominal discomfort,
Contamination: Vaginal or prepuce secretions Cystitis prone: Certain patients are more prone to tenderness, or distention.
Infections: Treated UTI, mycobacterial, TB, bacteriuria (transitional cell bacterial receptor
chlamydial, gonococcal, fungal (GU or systemic), sites). DIAGNOSTIC TESTS & INTERPRETATION
viral, haemophilus, bilharzia Menstrual cycle: Bacteriuria may be influenced by Lab
Other infections: Appendicitis, diverticulitis, hormones. r Indications for screening:
prostatitis Postmenopausal: Increasing incidence of Symptomatic patients
Noninfectious: Nephritis, stones, foreign bodies, bacteriuria Pregnant women
transplant rejection, trauma, malignancy, Vaginal pH: Normally acidic pH; colonization with Prior to genitourinary procedures
chemotherapy, nephrotoxic substances, uropathogens may occur as vaginal pH rises r Urine dipstick: Best for screening:
drug-induced interstitial nephritis Competitive organisms: Normal vaginal flora Leukocyte esterase test:
r Cystitis: Clinical syndrome of dysuria, frequency, discourages uropathogenic colonization Detects enzyme release by WBCs
urgency occasionally with suprapubic pain Buccal and vaginal cells: More receptive to Sensitivity 90%, specificity 95% for UTI
Usually indicative of bacterial cystitis but can be uropathogens adherence in cystitis-prone patients Conversion of nitrate to nitrite (Griess test):
associated with infections of the urethra or vagina Local production of IgA, IgG may play defense role. 7080% sensitivity for UTI
or noninfections process such as interstitial Production of mucous protective layer as a local Catalase test: Cannot differentiate infection from
cystitis, bladder carcinoma, or calculi bladder defense inflammation
Blood group antigen (secretors) saturate or block r Microscopy:
EPIDEMIOLOGY
bacterial adherence. Rapid in-office test: 80% accurate; usually fresh
Incidence (1) unspun
r 0.30.5 episodes of bacteriuria per person per year ASSOCIATED CONDITIONS
Diabetes mellitus, pregnancy, immunosuppression, Centrifugation: Increases finding 10-fold
among asymptomatic females aged 1840 Difficult to see bacteria if <1 105 CFU/mL
r Newborns: structural urinary tract abnormalities, indwelling
catheters Vaginal organisms may be misread as
Males: 1.53.6%; females: 0.41.0% uropathogens: Lactobacilli and Corynebacterium
r 15 yr: GENERAL PREVENTION r Gram stain: Increases identification of bacteria with
Males: 0.00.4%; females: 0.72.7% r Screening and treatment of asymptomatic
sensitivity and specificity of 96.2% and 93.0%,
r School-age: bacteriuria in at-risk populations such as pregnant respectively
Males: 0.040.2%; females 0.72.3% patients or prior to urologic intervention can prevent
r Adult (middle-age): subsequent morbidity of UTIs.
Males <1%; females 46% r Screening of asymptomatic spinal cord injury
r Older adults: patients or those with indwelling Foley catheter is
Males 1113%; females 633% not recommended.
r Almost 100% prevalence of bacteriuria in r Bacteriuria and pyuria from an incompletely treated
individuals with long-term, indwelling catheters UTI may be avoided with the appropriate use of
antibiotic class with sufficient duration; patient
Prevalence
r Pregnancy: 27% of all pregnant females (2) compliance should be encouraged.
r Elderly: 20% of females, 10% of males
24% of nursing home residents vs. 12% of
healthy domiciliary elderly (3)
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r Balanitis xerotica obliterans (BXO) is a specific form DIAGNOSTIC TESTS & INTERPRETATION
BASICS of balanitis: Lab
Chronic, progressive, fibrotic disease (a form of Swab of glans/foreskin for viral, bacterial, and fungal
DESCRIPTION lichen sclerosis isolated to the penis) culture
r Balanitis: Inflammation of the glans penis. Elastin is replaced by collagen
r Balanoposthitis: Inflammation of the foreskin and The skin around the meatus becomes white, Imaging
featureless, contracted, causing meatal stricture N/A
glans penis (affects uncircumcised men).
BXO may spread to the foreskin and coronal Diagnostic Procedures/Surgery
EPIDEMIOLOGY r Potassium hydroxide and Tzanck preparation for
sulcus. In extreme cases, the entire end of the
Incidence penis is replaced by fibrotic tissue, becomes men
r Can occur at any age.
thickened and nonretractile, causing sexual and Potassium hydroxide smear evaluates for fungus
r No incidence studies of balanoposthitis have been
voiding issues (eg, weak stream, obstruction) Tzanck preparation for herpes virus
reported in US.
1.5% of uncircumcised boys ages 015 were ASSOCIATED CONDITIONS Pathologic Findings
r Biopsy is indicated for:
affected in a Japanese cohort. Diabetes mellitus
Balanitis that persists and in which the cause
Prevalence GENERAL PREVENTION remains unclear warrants biopsy to rule out
r Common, the exact prevalence is unknown. r Maintain good genital hygiene
coexisting neoplasm or premalignant lesions
Balanitis affects 11% of adult men and 3% of r Retraction of foreskin to clean the glans
For definitive diagnosis of BXO
boys seen in urology clinics. r Keep the glans and foreskin dry
r Circumcision DIFFERENTIAL DIAGNOSIS
RISK FACTORS (1) r Fixed drug eruption (allergy)
r Presence of a foreskin (uncircumcised) r Safe sexual contact
r Contact dermatitis
r Tight foreskin (phimosis) r Manage risk factors (eg, glycemic control)
r Squamous cell carcinoma of the penis
r Poor genital hygiene r Carcinoma in situ of the penis
r Intertrigo (see below) r Zoon (plasma cell) balanitis
r Sexual contact (with or without infection)
DIAGNOSIS
r Psoriasis
r Poorly controlled diabetes mellitus HISTORY r Reiter syndrome (Reactive arthritis/reactive arthritis
r Immunocompromised host r Symptoms may include: Pain, discharge, irritation,
triad) (with circinate balanitis)
r Coexisting penile cancer voiding symptom (dysuria, weak stream) r Human papilloma virus
r Prior episodes and treatment
Genetics r Uncircumcised
N/A r Foreskin retractability TREATMENT
PATHOPHYSIOLOGY (2) r Genital hygiene habits
r The pathophysiology is usually different in young r Sexual contacts, sexually transmitted diseases GENERAL MEASURES
r Meticulous genital hygiene
boys compared to adult men: r Other systemic risk factors (eg, diabetes)
Boys: From bacterial invasion of tissue r Keep the glans and foreskin clean and dry
Men: Combination of poor genital hygiene, PHYSICAL EXAM r Expose the glans to air as often as possible
r Inspection (ulcers, mass, genital pus, edema) r Avoid excessive dampness in the genitals
intertrigo, irritant dermatitis, maceration injury,
and bacterial, or candidal overgrowth r Palpation (tenderness, induration, mass) r Avoid soaps while inflammation is present
Candida is the most common infectious cause r Inguinal lymph nodes should be nonpalpable r Cleaning with soap and water routinely
r Intertrigo refers to a condition in which damp, moist r Manage risk factors (eg, glycemic control)
body areas are predisposed to inflammation:
Involves genitals, inner thighs, underbelly MEDICATION
Risk factors: Grossly overweight, diabetes, bed Treatment depends on the underlying cause (infectious
rest, diaper use, poor personal hygiene vs. inflammatory) and organisms
Skin dampness predisposes to secondary
opportunistic bacterial or fungal overgrowth
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First Line
r Candidal infection: The most common cause of
ADDITIONAL TREATMENT ADDITIONAL READING
Radiation Therapy
infectious balanitis N/A Wikstrom A, Hedblad MA, Syrjanen S. Human
Clotrimazole cream 1%
Miconazole cream 2% Additional Therapies
papillomavirus-associated balanoposthitisa marker
for penile intraepithelial neoplasia? Int J STD AIDS.
B
Apply BID until symptoms resolve N/A 2013;24(12):938943.
Oral fluconazole if symptoms are severe Complementary & Alternative See Also (Topic, Algorithm, Media)
Nystatin cream if allergic to imidazole Therapies r Balanitis and balanoposthitis Image
Imidazole with hydrocortisone if inflammation N/A r Balanitis Xerotica Obliterans
r Anaerobic infection:
r Balanitis, Zoon (Plasma Cell Balanitis)
Metronidazole 400 BID for 1 wk ONGOING CARE r Lichen Sclerosis Et Atrophicus
Optimal dosage schedule is unknown r Penis, Lesion
Alternatively, amoxicillin/clavulanic acid PO or PROGNOSIS
clindamycin topically r Can be recurrent or persistent
r Aerobic infection: r 10% recurrence rate
Group A streptococci, Staphylococcus aureus, r Some patients may require circumcision to prevent CODES
Gardnerella vaginalis are all reported cases of recurrence and ensure resolution.
balanitis. ICD9
Treatment based on sensitivity of the culture COMPLICATIONS r 605 Redundant prepuce and phimosis
r Abscess formation r 607.1 Balanoposthitis
(topical antibiotics, occasionally oral antibiotics)
r BXO: r Penile cellulitis r 607.81 Balanitis xerotica obliterans
Topical steroids (clobetasol propionate or r Progression to Fournier gangrene
betamethasone valerate) offers limited efficacy r Scarring and subsequent phimosis ICD10
r Zoon (plasma cell) balanitis: r N47.1 Phimosis
FOLLOW-UP r N47.6 Balanoposthitis
Topical steroids with or without antibacterial
cream
Patient Monitoring r N48.1 Balanitis
r After an acute episode and treatment is
r Circinate balanitis (Reiter syndrome):
implemented, patients should be seen again to
Hydrocortisone cream 1% apply BID ensure resolution of symptoms and infection. CLINICAL/SURGICAL
Treatment of associated infection
r Irritant, allergic balanitis: Progression to cellulitis or gangrene may occur in PEARLS
diabetic patients with genital infection.
Avoid exposure to irritants especially soaps r Follow closely with genital dysplasia among those r Maintaining good genital hygiene is a key preventive
Emollients aqueous cream: Apply PRN and used men with condyloma with a history of strategy (keep the foreskin and glans clean and dry).
as a soap substitute while inflammation is present balanoposthitis than those with no such history. r Underlying risk factors should also be managed (eg,
Hydrocortisone 1% apply QD or BID until glycemic control in diabetes).
symptoms resolve Patient Resources r Treatment depends on the underlying cause
N/A
Second Line (infectious vs. inflammatory) and organisms.
N/A r Circumcision is reserved for recurrent balanitis,
SURGERY/OTHER PROCEDURES
REFERENCES balanoposthitis, or phimosis that have failed
r Circumcision is reserved for recurrent balanitis, conservative treatments.
1. Vohra S, Badlani G. Balanitis and balanoposthitis. r Balanitis that persists and if the cause remains
balanoposthitis, or phimosis that have failed Urol Clin North Am. 1992;19(1):143147.
conservative treatments. unclear warrants biopsy to rule out coexisting
r Occasionally dorsal slit may be performed. 2. Edwards S. Balanitis and balanoposthitis: A review. neoplasm or premalignant lesions.
Genitourin Med. 1996;72(3):155159.
r For BXO that does not respond to steroid:
Periodical self-dilation with tapered dilators
Dilation by urologists
Formal surgical reconstructive repair
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BCG SEPSIS/BCG-OSIS
John B. Eifler, MD
Michael S. Cookson, MD
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BCG SEPSIS/BCG-OSIS
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PATHOPHYSIOLOGY
BASICS r Bladder calculi are primarily formed in the bladder, DIAGNOSIS
rarely can be a secondary renal stone that has
DESCRIPTION formed in the kidney and passed into the bladder HISTORY
r Bladder calculi (also called bladder stones) are r Patients with SCI, neurogenic bladder may be at
Foreign bodies, retained catheter balloon
calcified material that are present in the bladder. fragments increased risk
r It can originate primarily in the bladder. r Bladder calculi may be asymptomatic and may be
Patients on chronic intermittent catheterization
r It can be a secondary renal stone that formed in the may force pubic hair into the bladder that can incidental finding on imaging (plain x-ray, renal
kidney and passed into the bladder. become calcified over time ultrasound, CT, or flexible cystoscopy)
r Often associated with bladder outlet obstruction in r Stone analysis frequently reveals uric acid stone in r Patients commonly presents with
the US. 50% of the cases Suprapubic or perineal pain
r Historically the removal of bladder calculus was r Other constituents are ammonium urate, calcium Irritative urinary symptoms
performed via an incision in the perineum with the oxalate, and calcium phosphate Intermittent urinary stream
patient in a supine position and the legs elevated r In infected urine, struvite stones are the most Hematuria, gross, and microscopic
(the origin of the term lithotomy position). common Recurrent urinary tract infection
r In patients with spinal cord injuries (SCIs), bladder PHYSICAL EXAM
EPIDEMIOLOGY r Examine the abdomen for palpable bladder or
stones are often composed of struvite or calcium
Incidence phosphate suprapubic tenderness
r The incidence of bladder calculi in the Western
r In endemic areas, low phosphate diet results in r Examine the external genitalia for any abnormalities
world has significantly dropped as a result of
increased ammonium excretion in the urine (meatal stenosis) that may contribute to outlet
improved diet, nutrition, and infection control r Low intake of animal protein contributes to high
r Bladder calculi are endemic in Thailand, Burma, obstruction
urinary oxalate and low urinary citrate levels with r Digital rectal exam to assess for BPH and prostate
Indonesia, Middle east, and north Africa
r Mostly in middle age men increased risk of stone formation cancer
r Solitary stone are present in 75% of cases
r In catheterized patients the incidence of developing DIAGNOSTIC TESTS & INTERPRETATION
bladder calculi is 25% in 5 yr ASSOCIATED CONDITIONS Lab
r The incidence in children has declined significantly r Foreign bodies in the bladder r Urine analysis: Hematuria, leukocytes, and
however in the developing countries they are r Intermittent catheterization crystalluria may be present
common in boys younger than 11 yr r Low phosphate diet r Urine culture and sensitivity in case of suspected
r Vaginal prolapse and urethral surgery are common r Low protein diet infection
causes in women r Urinary stasis (prostatic hypertrophy, stricture, r Urine cytology in the presence of calculi is
Prevalence congenital abnormalities [ureterocele], diverticulum, nonspecific
r Bladder calculi constitute 1015% of the stone cystocele) r Serum creatinine
r Urinary tract infection r Stone analysis should be considered when removed
burden in adult and 1530% in children
r Data on the world wide incidence are not available Imaging
GENERAL PREVENTION
r Adequate hydration r Calcified stones can be visible on plain x-ray (KUB)
RISK FACTORS
r Urinary stasis r Treatment of bladder outlet obstruction Stones may be densely radiopaque.
Bladder outlet obstruction r Prevention of urinary tract infection Occasionally laminations may be visible on plain
Benign prostatic hyperplasia r Prevention of urololithiasis as appropriate x-ray
r Uric acid and ammonium acid urate stones are
Urethral stricture Allopurinol for uric acid stones
Bladder neck contracture Reduce oxalate intake radiolucent but will be seen on ultrasounds or CT
Neurogenic bladder Increase urinary citrate scan.
r Foreign body such as urethral catheter and ureteric r Bladder calculi may not be visible on MRI
Low sodium low diet
r CT without contrast is highly sensitive and specific
stent that act as nidus for stone formation
r Urinary tract infection to detect calculi, however it is rarely used to
r Urinary diversion and bladder substitution diagnose bladder stones
Secondary to foreign body, infection, and systemic
acidosis
Rarely patients may place foreign bodies in
bladder that become calcified
Genetics
N/A
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Diagnostic Procedures/Surgery
r Cystoscopy to visualize the stone and guide
SURGERY/OTHER PROCEDURES
r Endoscopic cystolitholapaxy using stone
REFERENCES
subsequent removal of the stone fragmenting forceps 1. Huffman JL, Ginsberg DA. Calculi in the bladder
r Electrohydraulic, ultrasonic, laser, and pneumatic
Allows evaluation of bladder outlet obstruction or
other abnormality such as bladder diverticulum lithotrites are used for larger or harder stones
and urinary diversions. In: Coe FL, Favus MJ, Pak
CY, Parks JH, Preminger GM, eds. Kidney Stones:
B
r Large stones can be removed through small Medical and Surgical Management. Philadelphia,
Pathologic Findings
r Acute and chronic inflammation abdominal incision (open cystolitholapaxy) PA: Lippincott-Raven; 1996:10251034.
r Squamous metaplasia and squamous cell carcinoma r Cystolitholapaxy can be safely combined with 2. Douenias R, Rich M, Badlani G, et al. Predisposing
procedures such as TURP or TUIP for bladder outlet factors in bladder calculi. Review of 100 cases.
can result from chronic vesical calculus irritation
obstruction Urology. 1991;37(3):240243.
DIFFERENTIAL DIAGNOSIS 3. Paez E, Reay E, Murthy LN, et al. Percutaneous
r Bladder diverticulum ADDITIONAL TREATMENT treatment of calculi in reconstructed bladder.
r Bladder malignancy with or without calcification Radiation Therapy J Endourol. 2007;21(3):334336.
Urothelial carcinoma N/A
Other bladder malignancies Additional Therapies
r Chronic pelvic pain syndrome r ESWL has a limited role in treating bladder calculi ADDITIONAL READING
r Fungal bezoar or blood clot r Bladder outlet procedure may be necessary if urinary
r Interstitial cystitis Preminger GM, Tiselius HG, Assimos DG, et al;
stasis is causing vesical calculus to improve bladder EAU/AUA Nephrolithiasis Guideline Panel. 2007
r Lower urinary tract symptoms due to bladder outlet emptying
r Consideration to repair of bladder diverticulum or guideline for the management of ureteral calculi.
obstruction J Urol. 2007;178(6):24182434.
r Overactive bladder other anatomic abnormality if contributory
r Urinary tract infection See Also (Topic, Algorithm, Media)
Complementary & Alternative r Bladder Calculi (Vesical Calculi) Image
r Ureteral urolithiasis
Therapies r Bladder Diverticulum
Distal ureteral stone can cause significant vesical N/A r Bladder Filling Defect
irritation
r Bladder Wall Calcification, Differential Diagnosis
ONGOING CARE r Fungal Infections, Genitourinary
TREATMENT r Urolithiasis, Adult, General Considerations
PROGNOSIS
GENERAL MEASURES r Excellent with complete stone removal and
r Surgical removal is the mainstay treatment associated bladder outlet obstruction are treated
r Determining and correcting the cause (ie, bladder r Metabolic stone evaluation may be considered if CODES
outlet obstruction) should be a priority appropriate (ie, multiple upper tract calculi,
recurrent bladder calculi, etc.) ICD9
MEDICATION r 594.0 Calculus in diverticulum of bladder
First Line COMPLICATIONS r 594.1 Other calculus in bladder
r Medical therapy is used to treat associated urinary r Recurrent urinary tract infection
r 596.0 Bladder neck obstruction
tract infection r Squamous metaplasia
r Bladder outlet obstruction is treated with alpha r Chronic irritation may result in secondary ICD10
blockers such as tamsulosin 0.4 mg QD and 5 alpha malignancy (ie, squamous cell carcinoma) r N21.0 Calculus in bladder
reductase inhibitors such as dutasteride 0.5 mg QD r N32.0 Bladder-neck obstruction
FOLLOW-UP
Second Line Patient Monitoring
r Alkalinization of urine to a PH of 6.5 in case of uric r Urine analysis CLINICAL/SURGICAL
acid stones r Flowmetry and postvoid residual
Use potassium citrate 60 mEq/d PO r Renal ultrasound scan to screen for upper tract
PEARLS
urolithiasis If an otherwise healthy person is found to have a
bladder calculus, a complete evaluation is warranted
Patient Resources
to evaluate for causes such as urinary stasis.
PubMed Health: Bladder Stones http://www.ncbi.
nlm.nih.gov/pubmedhealth/PMH0002254/
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PATHOPHYSIOLOGY Imaging
BASICS r 70% of tumors present as nonmuscle-invasive r CT abdomen/pelvis
lesions Can detect lymphadenopathy and other
DESCRIPTION 70% of these are Ta, 20% T1, 10% CIS intra-abdominal disease
r Bladder cancer is the most common site of r Risk of recurrence Presence of hydronephrosis is suggestive of
malignancy in the urinary system CIS: 5090% muscle-invasive disease
r Includes multiple histologic types: Ta low grade: 5070% CT urography has replaced IVP as standard for
Urothelial cell carcinoma (formerly transitional cell Ta high grade: 60% evaluating upper tracts
carcinoma) is most common T1 high grade: 7080% r MRI may be useful for local staging
Other: Adenocarcinoma, squamous cell Risk of recurrence in upper tracts 24% r Chest x-ray (CXR): Metastasis with muscle invasion
carcinoma, and small-cell carcinoma r Risk of progression r Bone scan is recommended only in patients with
TNM staging: Initially based on clinical findings CIS >50% bone pain, elevated calcium, or elevated alkaline
(bladder biopsy) (See Section VII: Reference Ta low grade: 510% phosphatase
tables: TNM Classification: Urinary Bladder Ta high grade: 1540%
cancer.) Diagnostic Procedures/Surgery
T1 high grade: 3050% r Cystoscopy is the most accurate initial diagnostic
T staging: Primary tumor Most important prognostic factor is grade
Ta/Tis/T1: Superficial/nonmuscle invasive Concurrent upper-tract UCC in patients with procedure
bladder cancer (NMIBC) Can be done in office with local anesthesia
bladder cancer is 24% r Bladder biopsy
T2a/T2b: Muscle invasive bladder cancer (MIBC)
T3a/T3b/4a: Locally advanced ASSOCIATED CONDITIONS Establishes pathologic diagnosis
Regional lymph node (N) staging: Regional lymph Other smoking related illnesses (COPD) May be definitive treatment if tumor can be
nodes (the true pelvis); all others are considered GENERAL PREVENTION completely removed
distant metastasis r Avoid occupational exposure and smoking r Prostatic urethra biopsies are not routinely
Distant metastasis (M) staging r Urinalysis for hematuria screening performed unless there is:
Stage grouping: r High-fat diet has been associated with increased Multifocal disease of the bladder
Stage 0: Tis, N0, M0 CIS of the bladder
risk of bladder cancer
Stage 1: Ta-T1, N0, M0 r Vitamins A and B compounds have not shown Visible abnormality in the prostatic urethra
Stage II: T2, N0, M0 r Retrograde pyelography
Stage III: T3a-T4a, N0, M0 conclusive benefit for primary prevention
r Long-term hydration may be beneficial May be used in setting of renal impairment or
Stage IV: T4b, N0, M0 or any T, N1,2,3, M0 or contrast allergy
any T, any N, M1 Further evaluate equivocal findings on CT
EPIDEMIOLOGY DIAGNOSIS Pathologic Findings
r Carcinoma in situ (CIS) is a urothelial cancer that is
Incidence HISTORY
r American Cancer Society 2014 new case estimates: flat, high grade, and noninvasive but has metastatic
r Gross painless hematuria is the most common
74,690 (male: 56,390 female: 18,300) potential. Patients with bladder CIS have a 20% risk
presenting symptom of upper-tract disease
Estimated 155,800 deaths in 2014 r Irritative voiding symptoms (present in 20%)
r 3:1 malefemale ratio r Histologic types
r 4th most common cancer in males, 7th most Often associated with CIS Transitional cell carcinoma (urothelial carcinoma),
r Smoking history (quantify in pack years and if/when
common cancer in females 90%
r Median age of diagnosis is 70 yr patient quit) Squamous cell carcinoma, 37%
r Occupational exposures (see Risk Factors) Adenocarcinoma, <2%
Prevalence Small cell, sarcomas (leiomyosarcoma,
3rd most prevalent cancer in men (high recurrence) PHYSICAL EXAM
r Rarely abnormal in NMIBC rhabdomyosarcoma) uncommon
RISK FACTORS r General DIFFERENTIAL DIAGNOSIS
r Tobacco smoking confers a 24 times risk over r Hematuria
Weight loss, abdominal/pelvic masses,
those that have never smoked lymphadenopathy, flank tenderness Trauma: Iatrogenic, other
Risk reduction after quitting takes up to 20 yr r DRE with bimanual exam in men and women may Neoplasms: Malignancies: (30% of adults with
r Occupational exposures: painless, gross hematuria and 10% with
reveal palpable mass in bladder
Painters, leather, petroleum, chemical and metal painless microscopic hematuria have a
workers, dry cleaners, truck drivers, hairdressers DIAGNOSTIC TESTS & INTERPRETATION malignancy), benign tumors, endometriosis
Aromatic amines such as aniline dyes, benzidine, Lab Inflammatory causes: UTI (most common cause of
r Urinalysis with microscopy: RBCs
naphthylamine, 4-aminobiphenyl, and coal soot hematuria in adults), other infections
r Cyclophosphamide treatment r Urine cytology (Schistosomiasis, TB, syphilis) radiation cystitis
Caused by toxic metabolite, acrolein High specificity (96%), more sensitive for Renal/glomerular diseases: Nephritis,
r Pelvic radiation high-grade tumors (50%) Goodpasture syndrome, IGA nephropathy, lupus
r Risk for squamous cell carcinoma r Other urinary markers nephritis, glomerular diseases
Indwelling catheters, bladder calculi FISH (evaluate aneuploidy for chromosomes (membranoproliferative, poststreptococcal, or
Schistosomiasis (Schistosoma hematobium) 3,7,17 and 9p21) rapidly progressive glomerulonephritis)
Sensitivity 77%, specificity 98% Urolithiasis: 85% have hematuria
Genetics NMP-22 (marker of urothelial cell death) Congenital/Familial causes: Cystic disease, benign
r No clear hereditary causes identified
Sensitivity 56%, specificity 85% familial hematuria, etc.
r Tumor suppressor p53 is the most commonly altered r Renal function tests (BUN, Creatinine) Hematologic causes: Bleeding dyscrasias (eg,
gene in bladder cancer May indicate renal impairment secondary to hemophilia), Sickle cell anemia/trait (renal
ureteral obstruction papillary necrosis)
r Liver function tests Vascular causes: Hemangioma, AVM (rare),
May be abnormal due to metastasis Nutcracker syndrome, renal artery/vein
thrombosis, arterial emboli to kidney
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REFERENCES
TREATMENT ONGOING CARE
1. Kim SP, Frank I, Cheville JC, et al. The impact of
GENERAL MEASURES
r Treatment is related to stage
PROGNOSIS
r Related to pathologic stage (3)
squamous and glandular differentiation on survival
after radical cystectomy for urothelial carcinoma.
B
r In general, SCC of the bladder presents with locally Evidence suggests that patients with SCC of the J Urol. 2012;188:405409.
advanced disease, and radical cystectomy with bladder tend to present with higher-stage 2. Rausch S, Lotan Y, Youssef RF. Squamous cell
urinary diversion is an integral part of the treatment (pT3/T4) disease at the time of radical cystectomy carcinogenesis and squamous cell carcinoma of the
r Overall survival has ranged from 4.8 to 50% urinary bladder: A contemporary review with focus
paradigm
r Although uncommon, noninvasive lesions can be r 5-yr cancer-specific survival in contemporary series on nonbilharzial squamous cell carcinoma. Urol
treated with local resection and diligent surveillance has ranged from 57 to 64% Oncol. 2014;32(1):32.e116.
3. Xylinas E, Rink M, Robinson BD, et al. Impact of
MEDICATION COMPLICATIONS
r Related to radical cystectomy and urinary diversion histological variants on oncological outcomes of
First Line patients with urothelial carcinoma of the bladder
r Systemic chemotherapies have been used with Perioperative mortality approaches 2%
treated with radical cystectomy. Eur J Cancer.
limited experience in treating SCC of the bladder 4050% of patients will experience a
2013;49(8):18891897.
r Small series have reported positive responses to postoperative complication
Gastrointestinal complication is most common,
cisplatin-based therapies, similar to pure urothelial
eg, ileus, small bowel obstruction
carcinoma ADDITIONAL READING
r At present, role for neoadjuvant/adjuvant FOLLOW-UP
chemotherapy is poorly defined Patient Monitoring N/A
r Related to tumor stage at the time of radical See Also (Topic, Algorithm, Media)
Second Line
cystectomy r Bladder Cancer, General
N/A
In general, patients are followed with history, r Bladder Cancer, Squamous Cell Carcinoma Image
SURGERY/OTHER PROCEDURES (2) physical exam, laboratory studies (CBC and r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
r After diagnosis is confirmed, radical cystectomy is
comprehensive metabolic profile, including liver and Pathologic T2/T3/T4) (MIBC)
1st-line treatment function tests) and cross-sectional imaging of
r Bladder-preserving therapies can be considered if chest, abdomen, and pelvis every 36 mo after
tumor is nonmuscle invasive and completely surgery for the first 2 yr then semiannually for 2 yr
resected, and patient is willing to commit to then annually
CODES
intensive surveillance protocol Renal function needs to be followed annually as
r Limited experience with chemoradiotherapy as well ICD9
primary treatment modality 188.9 Malignant neoplasm of bladder, part
Patient Resources unspecified
ADDITIONAL TREATMENT Bladder Cancer Advocacy Network (www.bcan.org)
Radiation Therapy ICD10
C67.9 Malignant neoplasm of bladder, unspecified
Can be used in adjuvant setting for patients with
positive surgical margins at time of surgery
Additional Therapies CLINICAL/SURGICAL
N/A PEARLS
Complementary & Alternative r With the control of schistosomiasis in endemic
Therapies
regions, the rate of SCC is dropping relative to the
N/A
diagnosis of urothelial carcinoma.
r Radical cystectomy is the gold standard for
muscle-invasive SCC of the bladder.
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MEDICATION Complementary & Alternative 4. Bellmunt J, von der Maase H, Mead GM, et al.
First Line Therapies Randomized phase III study comparing
r Cisplatin-based chemotherapy combinations are the Supportive care includes adequate nutrition and paclitaxel/cisplatin/gemcitabine and
most active and superior to carboplatin regimens.
Survival outcome is similar in patients treated with
hydration, particularly for patients undergoing
multimodality therapy
gemcitabine/cisplatin in patients with locally
advanced or metastatic urothelial cancer without
B
standard multiday MVAC (methotrexate, vinblastine, prior systemic therapy: EORTC Intergroup Study
adriamycin, cisplatin) compared to cisplatin with 30987. J Clin Oncol. 2012;30:11071113.
gemcitabine (GC), with less toxicity in the GC group ONGOING CARE 5. Dodd P, McCaffrey JA, Herr H, et al. Outcome of
(1,2) PROGNOSIS postchemotherapy surgery after treatment with
r High-dose intensity chemotherapy with MVAC plus methotrexate, vinblastine, doxorubicin, and
For patients with metastatic disease, ECOG status 1,
GMCSF (HD-M-VAC) compared to classic M-VAC led hemoglobin 10, and visceral involvement are cisplatin in patients with unresectable or metastatic
to a better overall response rate (64 vs. 50%) and prognostic for overall survival (survival 14.2 mo for transitional cell carcinoma. J Clin Oncol 1999;
improved survival (21.8% in the HD-M-VAC vs. those with none of these features vs. 1.7 mo with all 3 17:25462552.
13.5%) at 7 yr. The toxicity profile of HD-M-VAC features) 6. Zaghloul M, Boutrus R, El-Hossieny H, et al. A
was superior with better dose intensity, and thus prospective, randomized, placebo controlled trial of
established HD-M-VAC as an alternative to standard COMPLICATIONS zolendronic acid in bony metastatic bladder cancer.
r Cisplatin: Nephrotoxicity, ototoxicity, peripheral
M-VAC (3) Int J Clin Oncol. 2010;15;382389.
MV chemotherapy is usually given every 14 days neuropathy, fatigue
r Adriamycin (in MVAC regimen): Cardiac toxicity
with the AC given along each 28 days
HD-M-VAC is also referred to as dose dense r MVAC toxicity is a major concern: ADDITIONAL READING
MVAC (DDMVAC) regimen gives the same drugs Myelosuppression, neutropenic fever, sepsis,
at the same doses closer together, all drugs every mucositis, nausea, and vomiting are common (up to NCCN guidelines for Bladder Cancer, version 1.2013,
14 days with hematopoietic growth factor support 54% may require readmission for toxicity) http://www.nccn.org/professionals/physician gls/
and is recommended by the NCCN guidelines r Neutropenia (including life-threating febrile pdf/bladder.pdf, Accessed January 28, 2014.
r Adding paclitaxel to cisplatin and gemcitabine, neutropenia) associated with multimodality See Also (Topic, Algorithm, Media)
compared to GC led to a modest but not significant chemotherapy for bladder cancer. Granulocyte r Bladder Cancer, General
improvement in survival and is not endorsed for growth factor is standard in the high-dose intensity r Bladder Cancer, Nonurothelial
most patients (4) MVAC regimen, and is used to support patients on r Bladder Cancer, Squamous Cell Carcinoma
r Some typical regimens reported in the literature gemcitabine and platinum combinations r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
include r Gemcitabine: Rash and cytopenias
and Pathologic T2/T3/T4) (MIBC)
MVAC: Methotrexate (30 mg/m2 on days 1, 15, r Taxanes (ie, paclitaxel): Fluid retention, neuropathy, r Reference Tables: TNM Classsification: Urinary
22), vinblastine (3 mg/m2 on days 2, 15, 22), myelosuppression Bladder Cancer
doxorubicin (30 mg/m2 on day 2), and cisplatin r Ureteral obstruction (tumor or lymphadenopathy) is
(70 mg/m2 ), repeated every 28 days for 6 cycles common and can be alleviated with ureteral
GC: Gemcitabine (1,000 mg/m2 days 1, 8, 15) stenting or percutaneous nephrostomy drainage CODES
plus cisplatin (70 mg/m2 day 2), repeated every
28 days for a maximum of 6 cycles FOLLOW-UP
Patient Monitoring ICD9
Second Line r 188.9 Malignant neoplasm of bladder, part
r There is no standard therapy for patients who have In patients with metastatic disease on chemotherapy,
imaging should be performed every 23 mo to assess unspecified
disease recurrence or progression following 1st-line r 196.9 Secondary and unspecified malignant
response. In patients with durable responses of
cisplatin/gemcitabine or MVAC chemotherapy, and neoplasm of lymph nodes, site unspecified
chemotherapy, imaging should be done every 3 mo for
clinical trial participation is encouraged. r 198.89 Secondary malignant neoplasm of other
r Small nonrandomized studies support the use of the 1st 2 yr of response.
Patient Resources specified sites
taxanes, gemcitabine, 5-fluorouracil, methotrexate, r Bladder Cancer Advocacy Network
pemetrexed, and others in the 2nd line with modest ICD10
benefit of single-agent therapy. http://www.bcan.org/ r C67.9 Malignant neoplasm of bladder, unspecified
r The substitution of carboplatin for gemcitabine in r C77.9 Secondary and unsp malignant neoplasm of
the palliative setting in the GC regimen is REFERENCES lymph node, unsp
reasonable for those felt unfit for cisplatin. r C79.89 Secondary malignant neoplasm of other
SURGERY/OTHER PROCEDURES 1. von der Maase H, Hansen SW, Roberts JT, et al. specified sites
r Retrospective series support consideration of Gemcitabine and cisplatin versus methotrexate,
salvage surgery for patients who initially present vinblastine, doxorubicin, and cisplatin in advanced
with unresectable or metastatic disease with robust or metastatic bladder cancer: Results of a large, CLINICAL/SURGICAL
chemotherapy response (5). Survival rates were randomized, multinational, multicenter, phase III PEARLS
consistently better in those with pathologic complete study. J Clin Oncol. 2000;18(17):30683077.
r Single-agent chemotherapy provides low response
response to induction chemotherapy and in those 2. Sternberg CN, de Mulder P, Schornagel JH, et al.
Seven year update of an EORTC phase III trial of rates of usually short duration.
with node only metastasis. (See Section I Bladder r For 1st-line chemotherapy, performance status and
cancer, urothelial, muscle invasive (clinical and high-dose intensity M-VAC chemotherapy and
pathologic T2/T3/T4)(MIBC) neoadjuvant therapy.) G-CSF versus classic M-VAC in advanced urothelial the presence or absence of visceral metastases are
tract tumours. Eur J Cancer. 2006;42(1):5054. independent prognostic factors for overall survival.
ADDITIONAL TREATMENT r Brisk diuresis helps limit cisplatin renal toxicity.
3. Galsky MD, Chen GJ, Oh WK, et al. Comparative
Radiation Therapy effectiveness of cisplatin-based and carboplatin-
Palliative radiotherapy is an option for patients with based chemotherapy for treatment of advanced
painful bony metastasis urothelial carcinoma. Ann Oncol. 2012;23:
Additional Therapies 406410.
r Studies suggest benefit of zolendronic acid or
denosumab in patients with metastatic bladder
cancer to bone (6)
r Granulocyte colony-stimulating factor can help limit
myelosuppression with cisplatin and others
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BLADDER CANCER, UROTHELIAL, MUSCLE INVASIVE (CLINICAL AND PATHOLOGIC T2/T3/T4) (MIBC)
SURGERY/OTHER PROCEDURES
r RC with pelvic lymphadenectomy and urinary
ADDITIONAL READING
ONGOING CARE r American Urological Association Clinical Guidelines,
diversion considered gold standard therapy for
PROGNOSIS Bladder Cancer, 2007. Available at: www.
MIBC (2)
Complete extirpation and pelvic
r Prognostic factors: auanet.org (accessed May 1, 2014). B
lymphadenectomy provide best chances for local Tumor cell type (SCC and adenocarcinoma less r Herr HW, Dotan Z, Donat SM, et al. Defining optimal
control and long-term survival favorable) therapy for muscle invasive bladder cancer. J Urol.
Ureteral frozen sections to ensure negative Tumor grade and stage 2007;177(2):437443.
margins before urinary tract reconstruction is Disease-free survival correlates with stage r Huang GJ, Stein JP. Open radical cystectomy with
standard practice Node burden (>8 positive) and node density lymphadenectomy remains the treatment of choice
Patients with T3 disease on clinical staging may (>20%) has worse prognosis for invasive bladder cancer. Curr Opin Urol.
be offered neoadjuvant chemotherapy r Survival rates after RC: 2007;17(5):369375.
RC gives no survival benefit in metastatic disease, Disease-free survival (5-yr) without positive nodes: r Smith ZL, Christodouleas JP, Keefe SM, et al.
but may be palliative in patients with intractable 72% (6284%) for pT2; 40% (1957%) for pT3; Bladder preservation in the treatment of
hematuria or pelvic pain 24% (036%) for pT4 muscle-invasive bladder cancer (MIBC): A review of
Lymphadenectomy may be prognostic and Disease-free survival with positive nodes: 30% the literature and a practical approach to therapy.
therapeutic: (1548%) BJU Int. 2013;112(1):1325.
Positive nodes in 25%
Patients with limited nodal burden have higher COMPLICATIONS See Also (Topic, Algorithm, Media)
r General: r Bladder Cancer, General
survival rates
Extended lymphadenectomy (to include Commonly due to local invasion and advancement r Bladder Cancer, Nonmuscle-Invasive Bladder Cancer
of disease (Ta, T1)
presacral, paraaortic, and paracaval nodes) may Urinary obstruction, hydronephrosis
improve survival r Bladder Cancer, Urothelial, Metastatic (Clinical and
Hematuria, clot retention
May identify patients most suited for adjuvant Pathologic N+, M+)
Malnutrition, infection, etc. r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
therapies r Associated with RC:
r Urinary diversion (3): and PathologicS T2/T3/T4) (MIBC) Image
90-day hospital readmission: 32% r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
Options include continent catheterizable stoma,
90-day mortality: 6%
continent orthotopic neobladder, or ileal conduit; and Pathologic T2/T3/T4) (MIBC) Neoadjuvant
Bowel obstruction (410%), ureteral anastomotic
each with advantages and disadvantages Therapy
Ileal conduit used most commonly, least stricture (510%), PE (2%) r Bladder Mass
complications FOLLOW-UP r Bladder Tumor Algorithm
Neobladders typically reserved for younger, Patient Monitoring r Bladder Tumors, Benign and Malignant, General
motivated patients who are able to perform r Follow-up remains controversial and dependent on
Considerations
self-catheterization if needed disease severity. Example: r Bladder Tumors, Benign and Malignant, General
r Partial cystectomy: T1/T2 disease: Semiannual physical exam, serum Considerations Algorithm
Strict patient selection criteria: Stage T2 only, chemistries, and CXR with CT scan every 2 yr (T1) r Reference Tables: TNM Classification: Urinary
solitary lesion allowing for 2-cm margins, lack of or yearly (T2)
Bladder Cancer
CIS, not involving trigone or ureteral orifices T3/T4 disease: Exam, labs, and CXR every 3 mo
Recurrence common within 2 yr with semiannual CT scan
Still allows for lymphadenectomy If disease free at 5 yr, surveillance can be lessened CODES
r Radical TURBT: per patient/practitioner comfort level
As a sole therapy, outcomes poor for MIBC Patients with intact urethra should be monitored
for urethral recurrence ICD9
Usually palliative in patients who will not tolerate
Consider urethral washing or cystoscopy 188.9 Malignant neoplasm of bladder, part
RC or systemic therapy (such as elderly with
unspecified
significant comorbidities) Patient Resources
r Urethrectomy: ICD10
Bladder Cancer Advocacy Network (BCAN):
Simultaneous (during RC) or delayed urethrectomy www.bcan.org C67.9 Malignant neoplasm of bladder, unspecified
if CIS or tumor involves prostatic urethra, ducts, or
stroma
Orthotopic reconstruction should not be made REFERENCES CLINICAL/SURGICAL
until negative frozen-section distal urethral margin
1. Siegel R, Ma J, Zou Z, et al. Cancer statistics, 2014.
PEARLS
is examined r MIBC represents an aggressive disease with lethal
CA Cancer J Clin. 2014;64(1):929. doi: 10.3322/
ADDITIONAL TREATMENT caac.21208. potential.
Radiation Therapy 2. Lerner SP, Sternberg CN. Management of r Surgical resection in the form of RC is the gold
r RT as a monotherapy is considered inferior to RC metastatic and invasive bladder cancer. In: Wein standard therapy.
r RT in combination with chemotherapy has a role in AJ, et al., eds., Campbell-Walsh Urology. 10th ed. r Role for multimodal treatment of MIBC with
selected patients undergoing organ preservation Philadelphia, PA: Elsevier, 2012. chemoradiotherapy and aggressive TUR is not as
(see below) 3. Dahl DM, McDougal WS. Use of intestinal well established as RC, however, has shown
Additional Therapies segments in urinary diversion. In: Wein AJ, et al., promising results.
r Combination RT and chemotherapy after TURBT is eds., Campbell-Walsh Urology. 10th ed.
the most efficacious bladder preservation technique Philadelphia, PA: Elsevier, 2012.
Developed for patients who are either not
candidates for or refuse RC. Ideal candidates for
bladder preservation:
Complete visual resection on TURBT
Solitary tumor
No hydronephrosis
5-yr overall survival 3050%; better in T2 disease
than T3T4
Complementary & Alternative
Therapies
N/A
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BLADDER CANCER, UROTHELIAL, MUSCLE INVASIVE (CLINICAL AND PATHOLOGIC T2/T3/T4) (MIBC) NEOADJUVANT THERAPY
r Fitness for cisplatin is not well defined, but 4. Sternberg CN, de Mulder P, Schornagel JH, et al.
includes: Renal function, hearing, performance ONGOING CARE Seven year update of an EORTC phase III trial of
status, baseline neuropathy, and cardiac function high-dose intensity M-VAC chemotherapy and
PROGNOSIS
Second Line
r For patients treated with perioperative
r Patients with pathologic complete response (pT0) at
G-CSF versus classic M-VAC in advanced urothelial
tract tumours. Eur J Cancer. 2006;42(1):5054.
B
radical cystectomy have superior relapse free and 5. Griffiths G, Hall R, Sylvester R, et al. International
cisplatin-based chemo with disease recurrence,
overall survival outcomes compared to those with phase III trial assessing neoadjuvant cisplatin,
particularly for those with recurrence >12 mo
residual invasive disease methotrexate, and vinblastine chemotherapy for
following chemo, cisplatin rechallenge can be
10.6% complete pathologic downstaging muscle-invasive bladder cancer: Long-term results
considered. r Treatment with neoadjuvant cisplatin-based chemo
With disease progression while on or closely of the BA06 30894 trial. J Clin Oncol. 2011;
following cisplatin-based chemo, there is no increases the rate of pT0 over patients treated with 29(16):21712177.
standard second line therapy. TURBT alone (38 vs. 15%) in the Phase III study of 6. Raghavan D. Neoadjuvant treatment and bladder
Many small studies have shown modest benefit of preoperative MVAC compared to cystectomy alone preservation options for muscle-invasive urothelial
single agent or combination regimens. COMPLICATIONS (transitional cell) bladder cancer www.UpToDate.
r Cisplatin: Nephrotoxicity, ototoxicity, peripheral com, Accessed January 26, 2014.
SURGERY/OTHER PROCEDURES 7. Bi L, Huang H, Fan X, et al. Extended vs
r Radical cystectomy is the treatment of choice for neuropathy, and fatigue
r Adriamycin (in MVAC): Cardiac toxicity non-extended pelvic lymph node dissection and
patients with invasive (T2T4) disease, patients with
r Neutropenia, including life threating febrile their influence on recurrence-free survival in
multifocal tumors, large tumors, hydronephrosis, patients undergoing radical cystectomy for bladder
node-positive disease (N1). neutropenia is associated with multimodality chemo
for bladder cancer. Granulocyte growth factor is cancer: A systematic review and meta-analysis of
Consider consolidative cystectomy in patients with comparative studies. BJU Int. 2014;113(5b):
extensive nodes or locally advanced disease with standard in the high-dose intensity MVAC regimen,
and can be used to support patients on GC/platinum E39E48.
durable responses to chemo.
r Bilateral pelvic lymphadenectomy: Include common combinations 8. Gakis G, Efstathiou J, Lerner SP, et al. ICUD-EAU
r GC: Rash and cytopenias International Consultation on Bladder Cancer
iliac, internal iliac, external iliac, hypogastric, 2012: Radical cystectomy and bladder preservation
presacral, and obturator nodes. r Ureteral obstruction (tumor or nodes): Alleviate with
for muscle invasive urothelial carcinoma of the
Increased number of lymph nodes removed and ureteral stenting or percutaneous nephrostomy to
bladder. Eur Urol. 2012;63:4557.
lymph node density (# positive LN / # total LN improve renal function
removed) in patients with positive lymph nodes r 30-day perioperative mortality following cystectomy
can improve recurrence rates (7). approximately 1%; average 30-day readmission rate
A randomized trial of standard vs. extended pelvic 2132%
ADDITIONAL READING
lymph node dissection is ongoing. r Perioperative morbidity following cystectomy: Ileus, NCCN Guidelines for Bladder Cancer, version 1.2013,
ADDITIONAL TREATMENT blood loss, infection, thromboembolism, wound http://www.nccn.org/professionals/physician gls/
dehiscence, ostomy complications. pdf/bladder.pdf, Accessed January 28, 2014.
Radiation Therapy
r Multimodality treatment with chemo-radiotherapy FOLLOW-UP See Also (Topic, Algorithm, Media)
can be considered for patients who are medically Patient Monitoring r Bladder Cancer, General
inoperable or selected patients who wish for bladder r Following radical cystectomy, imaging of the chest, r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
preservation (8). abdomen and pelvis including upper tract evaluation and Pathologic T2/T3/T4) (MIBC) Neoadjuvant
Tumors <5 cm, no carcinoma in situ, no and CBC and electrolyte assessment should occur Therapy Image
hydronephrosis, complete TURBT resection, every 36 mo. Based on recurrence risk for the first r Bladder Cancer, Urothelial, Metastatic (Clinical and
T2T3, functional bladder at baseline. 2 yr, then as clinically indicated. Pathologic N+, M+)
r Neoadjuvant chemo followed by cystectomy and r Urine cytology and electrolytes should be monitored r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
chemo-radiotherapy have not been compared head every 36 mo. Based on recurrence risk for the first and Pathologic T2/T3/T4) (MIBC)
to head in a prospective trial 2 yr., then as clinically indicated. r Bladder Tumor Algorithm
r Continent diversion: Monitor B12 deficiency r Reference Tables: TNM Classification: Urinary
Additional Therapies
r Multidisciplinary evaluation should be considered to Bladder cancer
Patient Resources
asses best plan of care Bladder Cancer Advocacy Network
r For patients who have received standard http://www.bcan.org/
cisplatin-based neoadjuvant chemo, there is CODES
currently no recommendation for adjuvant chemo
following cystectomy REFERENCES ICD9
r For patients with invasive or node-positive disease 188.9 Malignant neoplasm of bladder, part
1. Siegel R, Ma J, Zou Z, et al. Cancer statistics, 2014.
following cystectomy who did not receive CA Cancer J Clin. 2014 64(1):929. doi: 10.3322/ unspecified
neoadjuvant chemo, adjuvant platinum-based caac.21208.
therapy should be considered
ICD10
r Positive surgical margins can increase risk of local 2. Advanced Bladder Cancer Meta-analysis C67.9 Malignant neoplasm of bladder, unspecified
Collaboration. Neoadjuvant chemotherapy in
failure; consider adjuvant radiotherapy invasive bladder cancer: A systematic review and
Complementary & Alternative meta-analysis. Lancet. 2003;361(9373):1927 CLINICAL/SURGICAL
Therapies 3. Grossman HB, Natale RB, Tangen CM, et al. PEARLS
N/A Neoadjuvant chemotherapy plus cystectomy
r Outcomes after radical cystectomy indicate
compared with cystectomy alone for locally
advanced bladder cancer. N Engl J Med. 2003;349: increased survival in patients who had more, rather
859866. than fewer, lymph nodes resected.
r Patients with pathologic complete response
following neoadjuvant chemotherapy appear to
have the best long-term survival.
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PATHOPHYSIOLOGY Imaging
BASICS r CIS usually multifocal and can occur in the upper r No imaging specific for diagnosing CIS
tracts, prostatic ducts, and urethra as well as the r Renal/bladder ultrasound (US): Detects
DESCRIPTION bladder hydronephrosis that may be caused by ureteral
r Carcinoma in situ (CIS) of the bladder is a flat, r Natural historyhighly aggressive obstruction from bladder tumor; bladder US can
multifocal, velvety lesion of the urothelium Progression to MIBC in 5483% of untreated visualize larger bladder tumors
CIS is a flat, high-grade tumor that are confined to cases (3,4) r Computed tomography (CT) urogram: Triple phase
the mucosa Increase risk of recurrence if found with NMIBC CT abdomen/pelvis is the gold standard for
Can be occult and diagnosed by random biopsies papillary lesions evaluation of painless gross hematuria; can detect
of normal appearing mucosa r Bacillus Calmette-Guerin (BCG) reduces risk of more advanced bladder tumors, hydronephrosis, and
Although can occur alone, most often seen with progression by 35% compared with other upper tract filling defects that may represent upper
concomitant high-grade papillary lesions intravesical therapies (1,3) tract urothelial carcinoma
r Classified as nonmuscle-invasive bladder cancer r BCG confers disease-free rate approximately 51% at
(NMIBC) similar to stage Ta and T1, however CIS is Diagnostic Procedures/Surgery
3.75 yr (1) r Cystoscopy with bladder biopsy
considered high grade and aggressive with a r If concomitant muscle-invasive lesion, prognosis and
propensity to invade the bladder wall and Appearance can be flat, grossly erythematous,
treatment depends on invasive lesion granular or cobblestone mucosa or visually normal
metastasize
ASSOCIATED CONDITIONS May be performed in office at initial visit
EPIDEMIOLOGY r NMIBC (Ta,T1) TURBT under general or spinal anesthesia may be
Incidence r Invasive bladder cancer (T2,T3,T4) required if papillary bladder tumor present
True incidence not known given the flat superficial Retrograde pyelography also should be performed
nature of this lesion, which can be destroyed by GENERAL PREVENTION to assess the upper tracts if not already evaluated
r Smoking cessation with a CT urogram
cautery effect during transurethral resection of bladder
tumor (TURBT) r Increased fluid intake Positive cytology with no visible tumor and
r Avoid occupational exposures negative random bladder biopsies suggests
Prevalence
r Occurs as isolated CIS in 35% cases disease outside of bladder
r Estimated 510% of patients with noninvasive Biopsy of prostatic urethra indicated
DIAGNOSIS Selective cytology from upper tracts; evaluate
urothelial carcinoma have CIS (1) for urothelial carcinoma/CIS of renal pelvis or
r 4565% patients with invasive urothelial carcinoma HISTORY
r Age and sex ureters. CIS of upper tracts suspected in absence
have CIS (2) of solid tumor and with positive cytology, rarely
r Presence of gross hematuria
RISK FACTORS able to obtain adequate biopsy to confirm CIS
r No risk factors specific for CIS beyond that of r Irritative voiding symptomsdysuria commonly histologically
urothelial carcinoma occurs with CIS r Fluorescent Blue light cystoscopy
r Tobacco smoking-cigarettes r History of bladder cancer More sensitive than conventional white light
r Occupational exposure r Family history of bladder cancer cystoscopy for detecting CIS
r Smoking history In a prospective study additional detection rate
Organic chemicals: Aromatic amines, benzenes,
aniline dyes r Occupational risk factors of 20% for all tumors and 23% for CIS (6)
High-risk occupations: Petroleum, chemical, False-positives can result in the presence of
PHYSICAL EXAM inflammation, recent TUR, or BCG instillation
rubber, textile workers, hairdressers r Usually unremarkable
r Medications r Bimanual exam should be performed at time of Pathologic Findings
Phenacetin-containing analgesics r Arises from surface uroepithelium
cystoscopy/TURBTIf CIS is found in presence of r Severe cytologic atypia and nuclear aplasia (2)
Cyclophosphamide advanced stage/invasive bladder cancer may
r Pelvic radiation Large, irregular hyperchromatic nuclei
appreciate palpable mass
Genetics Mitotic activity common
r p53 mutation most important deletion/mutation DIAGNOSTIC TESTS & INTERPRETATION r Thought to be a precursor of invasive disease
Lab r Some pathologists use the term severe dysplasia
found with CIS (2) r Urinalysis, including microscopic evaluation
r Chromosome 9q deletions common to describe CIS
r Urine cytologyhighly specific and sensitive
r Loss of CDKN2/p16 (tumor suppressor gene) DIFFERENTIAL DIAGNOSIS
(>90%) for detecting CIS and high-grade urothelial r Nonurothelial cancers (squamous cell carcinoma,
carcinoma (5)
r UroVysion, HA-HAase, and BLCA-4 have a high adenocarcinoma)
r Inflammatory lesion from prior radiation, interstitial
sensitivity to detect CIS however should not replace
classic urine cytology (1) (Grade B) cystitis, infection
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MEDICATION
to muscle-invasive disease if untreated ADDITIONAL READING
See Pathophysiology
First Line r EAU Guidelines on Non-muscle-invasive (TaT1 and
BCG reduces risk of recurrence and progression to
r BCGlive suspension of the attenuated CIS) Bladder Cancer http://www.uroweb.org/gls/
muscle-invasive disease
Mycobacterium bovis vaccine strain r CIS of prostatic urethra unfavorable (1) pdf/07 Bladder%20Cancer LR%20II.pdf, Accessed
Standard of care for CIS Prostatic tissue stromal invasion worst January 28, 2014.
Therapy initiated no earlier than 24 wk after r Guideline for the Management of Nonmuscle
prognosiscystoprostatectomy advised
TURBT/biopsy to give uroepithelium time to heal r Disease-specific survival rates excellent if cystectomy Invasive Bladder Cancer: (Stages Ta, T1, and Tis):
and prevent systemic complications of BCG performed early (instead of BCG instillation), Update (2007) (Reviewed and validity confirmed
Administered as induction therapy6 however 4050% could be overtreated (4) 2010). AUA Clinical Practice Guidelines.
consecutive weekly bladder instillations; then (Grade A) http//www.auanet.org/content/clinical-practice
maintenance treatment recommended for at least guidelines/clinicalguidelines.cfm?sub=bc, Accessed
1 yr (1) (Grade A) COMPLICATIONS November 2013.
BCG has the highest complete response rate and r BCG toxicity
durable disease-free rate among all intravesical Low, but serious risk of systemic BCG infection See Also (Topic, Algorithm, Media)
r BCG Sepsis/BCGosis
treatments (1) (Grade A) (BCGosis)avoid treatment in presence of recent
r Bladder Cancer, General
Initial response rates approximately 7090%, TURBT, hematuria, foley trauma, or urinary tract
infection r Bladder Cancer, Intravesical Agents (table)
however up to 1/2 of patients will recur
Response to BCG instillation should be assessed Has side effect of dysuria and can be intolerable in r Bladder Cancer, Nonmuscle-invasive Bladder Cancer
at 3 mo some patients (Ta, T1)
If no response can give another 6-wk course of Usually experienced within the first 6 mo of r Bladder Cancer, Urothelial, Metastatic (Clinical and
BCG vs. proceed to radical cystectomy treatment Pathologic N+, M+)
Approximately 50% will respond to second r Bladder Cancer, Urothelial, Muscle Invasive (Clinical
FOLLOW-UP
course of BCG (1) (Grade B) and Pathologic T2/T3/T4) (MIBC)
Patient Monitoring r Bladder Cancer, Urothelial, Superficial Carcinoma In
Second Line At 3 mo patients should have cystoscopy and urine
r Intravesical chemotherapy Situ (CIS) (NMIBC) Image
cytology. If negative, this should be repeated every
Mitomycin Can alternative for patients who r Bladder Tumor Algorithm
3 mo 2 yr, every 6 mo thereafter until year 5 and
cannot tolerate BCG then yearly (3) r Bladder Tumors, Benign, and Malignant, General
Valrubicinan option for poor surgical r Bladder Tumors, Benign and Malignant, General
candidates with BCG-refractory disease
Patient Resources
American Cancer SocietyBladder Cancer Considerations
Gemcitabine r Reference Tables: TNM Classification: Urinary
http://www.cancer.org/acs/groups/cid/documents/
SURGERY/OTHER PROCEDURES webcontent/003085-pdf.pdf Bladder Cancer
r TURBTResection of all visible papillary bladder
tumors is essential prior to BCG therapy
r For CIS refractory to intravesical therapyradical REFERENCES CODES
cystectomy 1. van der Meijden AP, Sylvester R, Oosterlinck W,
Disease-specific survival rates excellent if et al. EUA guidelines on the diagnosis and ICD9
cystectomy performed early (instead of BCG treatment of urothelial carcinoma in situ. Eur Urol. 233.7 Carcinoma in situ of bladder
instillation), however 4050% could be 2005;48:363371. ICD10
overtreated (4) (Grade A) 2. Nese N, Gupta R, Bui MH, et al. Carcinoma in situ D09.0 Carcinoma in situ of bladder
ADDITIONAL TREATMENT of the urinary bladder: Review of clinicopathologic
Radiation Therapy characteristics with an emphasis on aspects related
to molecular diagnostic techniques and prognosis.
CLINICAL/SURGICAL
No role in treatment of CIS
J Natl Compr Canc Netw. 2009;7:4857. PEARLS
Additional Therapies 3. Babjuk M, Oosterlinck W, Sylvester R, et al. EAU
N/A r Urine cytology is the best marker (>90%
guidelines on non-muscle invasive urothelial
sensitivity/specificity) for diagnosis of CIS.
Complementary & Alternative carcinoma of the bladder, the 2011 update. Eur r Positive cytology in absence of visible bladder
Therapies Urol. 2011;59:9971008.
N/A lesionsdifferential includes CIS bladder, prostatic
4. Sylvester R, van der Meijden AP, Witjes JA, et al.
urethra, or upper tract urothelial carcinoma.
High-grade Ta urothelial carcinoma and carcinoma r BCG is the treatment of choice for CIS of the
in situ of the bladder. Urology. 2005;66(Suppl 1):
90107. bladder; highest response rate and most durable
disease-free rates of all intravesical therapies.
5. Lotan Y, Roehrborn CG. Sensitivity and specificity r To prevent systemic complications of BCG do not
of commonly available bladder tumor markers
versus cytology: Results of a comprehensive administer after TURBT until urothelium healed
literature review and meta-analysis. Urology. (approximately 2 wk).
r For BCG refractory CIS: A second induction course
2003;61:109118.
BCG can be administered vs. proceeding
immediately to radical cystectomy.
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Diagnostic Procedures/Surgery (1,4) r Transurethral procedure: 4. Gomez, Ceballos L, Coburn M, et al. Consensus
r Intraoperative diagnosis: Extraperitoneal perforation statement on bladder injuries. BJU Int. 2004;
Normal saline with indigo carmine into Foley and Exploratory laparotomy with repair for large 94(1):2732.
observe for extravasation (blue staining)
Avoid use of methylene blue due to extensive
perforation. Carefully inspect bowel for
potential injury
5. Corriere JN, Sandler CM. Diagnosis and
management of bladder injuries. Urol Clin North
B
tissue staining risk Small leak can be initially managed with Am. 2006;33:6771.
r Intraoperative cystoscopy can be useful in selected catheter drainage and close monitoring 6. Stav K, Dwyer PL, Rosamilia A, et al. Risk factors
situations and may be the most reliable method of Extraperitoneal perforation for trocar injury to the bladder during mid urethral
immediately assessing bladder wall integrity Usually managed with catheter drainage sling procedures. J Urol. 2009;182:174179.
r Cystoscopy Large perforations complicated by symptomatic
Recommended after suburethral sling operations collections require drainage, with or without
via the retropubic route formal closure of the perforation
r Bladder perforation during midurethral sling or
ADDITIONAL READING
May be considered after sling insertion via the
obturator route (controversial as bladder injuries transvaginal mesh placement EAU Guidelines on Iatrogenic Trauma - European
are rare with this technique). Sling reinsertion and urethral catheterization Association of Urology www.uroweb.org, Accessed
r Cystoscopy after transvaginal mesh procedures is (12 days) should be performed (4,6). February 2, 2014.
preferable. ADDITIONAL TREATMENT See Also (Topic, Algorithm, Media)
r Some authors have recommended routine r Bladder Trauma
In the setting of any bladder perforation during TURBT
cystoscopy due to the higher risk of bladder injuries intravesical postoperative chemotherapy should not be r TUR Syndrome
during hysterectomy or after any major gynecologic administered r Ureter, Intraoperative Injury
procedure. r Ureter, Trauma
Pathologic Findings ONGOING CARE
Rupture through mucosa, submucosa, and muscularis
of detrusor usually causes urine leak PROGNOSIS CODES
r Extraperitoneal: Usually heals with Foley catheter
DIFFERENTIAL DIAGNOSIS
r Prostatourethral injury drainage and without further intervention ICD9
r Intraperitoneal: Good prognosis if identified r 867.0 Injury to bladder and urethra, without
r Small or large bowel injury
r Ureteral injury intraoperatively and repaired. Prognosis worse if mention of open wound into cavity
delayed diagnosis r 867.1 Injury to bladder and urethra, with open
r Vascular injury
COMPLICATIONS wound into cavity
r Peritonitis or abscess r 998.2 Accidental puncture or laceration during a
TREATMENT r Ileus procedure, not elsewhere classified
r Fistula
GENERAL MEASURES r Reoperation
ICD10
r Prompt recognition improves opportunity for r N99.71 Acc pnctr & lac of a GU sys org during a GU
improved outcome. FOLLOW-UP sys procedure
r Bladder injury can be found intraoperatively or r N99.72 Accidental pnctr & lac of a GU sys org
Patient Monitoring
postoperatively, and will be intraperitoneal or r Foley catheter or suprapubic tube to monitor urine during oth procedure
extraperitoneal. r S37.23A Laceration of bladder, initial encounter
output
r For most bladder injures Foley catheter for r Usually no need for outpatient antibiotics
1014 days with follow-up cystogram is
recommended
CLINICAL/SURGICAL
REFERENCES PEARLS
MEDICATION
First Line r Intraoperatively, visual inspection is a reliable
1. Vakili B, Chesson RR, Kyle BL, et al. The incidence
r Consider antibiotics: Gentamicin or fluoroquinolone of urinary tract injury during hysterectomy: A method of assessing bladder injury.
for 24 hr prospective analysis based on universal cystoscopy. r Extraperitoneal: Usually heals with Foley catheter
r Anticholinergic for postoperative bladder spasm: Am J Obstet Gynecol. 2005;192:15991604. drainage and without further intervention.
Oral or suppository 2. Ostrzenski, Ostrzenska KM. Bladder injury during r Intraperitoneal: Good prognosis if identified
Second Line laparoscopic surgery. Obstet Gynecol Surv. intraoperatively and repaired. Prognosis worse if
N/A 1998;53(3):175180. delayed diagnosis.
3. Cass AS. Diagnostic studies in bladder rupture. r All cystograms must include a postcontrast
SURGERY/OTHER PROCEDURES (5) Urolog Clinc North Am. 1989;16:267273. evacuation study to evaluate for residual contrast
r Laparoscopic or robotic injury:
outside of the bladder.
1-layer laparoscopic or robotic repair
r Intraoperative intraperitoneal injury:
Open bladder/2-layer repair
r Intraoperative extraperitoneal injury:
Foley or 2-layer repair
r Postoperative intraperitoneal injury:
Exploratory laparotomy with repair
r Postoperative extraperitoneal injury:
Initial catheter drainage with antibiotics
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BLADDER TRAUMA
Brad Figler, MD
Hunter Wessells, MD, FACS
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BLADDER TRAUMA
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r Balanitis circinata ADDITIONAL TREATMENT 2. Buechner SA. Common skin disorders of the penis.
Dry and scaling lesions of the glans in circumcised Radiation Therapy BJU Int. 2002;90(5):498506.
or uncircumcised males Radiation therapy can be used for patients resistant to 3. Arlette JP. Treatment of Bowen disease and
Associated with Reiters syndrome
Can be moist and erythematous in uncircumcised
topical treatment or who are not surgical candidates. erythroplasia of queyrat. Br J Dermatol. 2003; B
149(Suppl 66):4349.
males Additional Therapies
r Candidal balanitis Additional therapies include cryotherapy, curettage,
and photodynamic therapy, although their
Usually found in uncircumcised diabetics
effectiveness is limited. ADDITIONAL READING
Reddened and edematous lesions
Complementary & Alternative r NCCN Guidelines Version 1.2013, Penile Cancer;
Usually treated with antifungal therapy
r Zoon balanitis Therapies from NCCN.org (Accessed April 10, 2014).
N/A r Pettaway CA, Lance RS, Davis JW, Tumors of the
Usually in elderly, uncircumcised males
Cayenne pepper-appearing red, raised lesion penis. In: Wein, et al. Campbell-Walsh Urology.
Usually distinguished from CIS on biopsy by 10th ed. Philadelphia, PA: Saunders, 2012.
ONGOING CARE
band-like infiltrate of plasma cells See Also (Topic, Algorithm, Media)
PROGNOSIS r Bowen Disease and Erythroplasia of Queyrat
r 533% of cases have been reported to transform to
Image
TREATMENT SCC r Penis, Cutaneous Lesion
510% risk in BD, 1033% in EQ r Penis, Squamous Cell Carcinoma
GENERAL MEASURES (3) Carries significant risk of death
Treatment based on multiple biopsy samples of r Cure can be achieved up to 80% of the time
adequate depth to rule out invasion r All therapies have recurrence rates of 2030%
MEDICATION CODES
COMPLICATIONS
First Line
r Topical therapy Progression to invasive squamous cell carcinoma ICD9
5-fluorouracil cream BID for 45 wk or FOLLOW-UP 233.5 Carcinoma in situ of penis
5% imiquimod cream daily for 16 wk Patient Monitoring ICD10
Proven effective for large lesions not amenable to r BD and EQ surveillance parallels localized, invasive D07.4 Carcinoma in situ of penis
surgery or for recurrent lesions SCC of the penis with clinical exam:
Utilized with rubber condom to increase contact Year 12, every 3 mo
time Year 35, every 6 mo CLINICAL/SURGICAL
Second Line Year 510, every 12 mo PEARLS
N/A r Consider re-biopsy of recurrent lesions to rule out
r EQ is SCC in situ arising on the glans or inner side of
transformation to invasive SCC
SURGERY/OTHER PROCEDURES the foreskin.
r Circumcision can decrease likelihood of recurrence Patient Resources r BD is SCC in situ of the penile shaft or scrotum.
r With lesions on the foreskin, circumcision, or Medline Plus: Cancer Penis http://www.nlm.nih. r 8090% of cases seen in uncircumcised men.
excision with 5-mm margin is adequate for local gov/medlineplus/ency/article/001276.htm r Progression to invasive SCC in 530%.
control
Lesions on the glans are difficult to excise with this REFERENCES
strategy when trying to preserve penile anatomy
Ensure adequate depth of resection to rule out 1. Wieland U, Jurk S, Weissenborn S, et al.
invasion Erythoplasia of queyrat: Coninfection with
r Mohs micrographic surgery has been utilized to cutaneous carcinogenic HPV type 8 and genital
accomplish adequate excision without disfigurement papillomaviruses in a carcinoma in situ. J Invest
r Nd:YAG, KTP, or carbon dioxide laser ablation has Dermatol. 2000;115(3):396401.
been shown to be effective
Nd:YAG preferred due to depth of penetration
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ADDITIONAL READING
TREATMENT ONGOING CARE r Michielsen DP, Lafaire C. Management of genital
GENERAL MEASURES
r Treat any life-threatening conditions (ABCD)
PROGNOSIS
r Based on degree and extent of burn
burns: A review. Int J Urol. 2010;17(9):755758.
r Peck MD, Boileau MA, Grube BJ, et al. The B
Do not attempt to cool wound as this may cause r Most burns have matured by 612 mo; additional management of burns to the perineum and genitals.
more extensive injury reconstruction may be required at that time J Burn Care Rehabilitation. 1990;11(1):5456.
r Shock may occur; IVF critical r Vanni AJ. Trauma to the external genitalia. In:
COMPLICATIONS
>20% total body surface area (TBSA), use r Erectile dysfunction Wessells, ed. Urological Emergencies. Totowa NJ:
modified Brooke formula: r Scarring/disfigurement Humana Press, 2013.
2 mL/kg/TBSA r Urethral strictures
r Most chemical burns should be copiously irrigated. If See Also (Topic, Algorithm, Media)
r Burns, External Genitalia and Perineum Image
agent is known use guidelines: FOLLOW-UP r Penis, Trauma
Hydrofluoric acid: Irrigate with calcium gluconate Patient Monitoring r Scrotum and Testicle, Trauma
Hydrochloric acid or sulfuric acid: Use bicarbonate r Follow-up as indicated
irrigation
Patient Resources
Phenol: No irrigation r American Burn Association
CODES
MEDICATION www.ameriburn.org
First Line r Phoenix Society for Burn Survivors
ICD9
r Silver sulfadiazine 1%: Apply to affected area www.phoenix-society.org r 942.05 Burn of unspecified degree of genitalia
Does not penetrate eschar r 942.15 Erythema [first degree] of genitalia
r Mafenide acetate (Sulfamylon) 11.1% r 942.25 Blisters, epidermal loss [second degree] of
Penetrates eschar REFERENCES
genitalia
r Pain control
1. Hettiaratchy, Dziewulski. ABCs of burns. Br Med J.
Narcotics 2004;328:14271429. ICD10
Anti-inflammatories r T21.06XA Burn of unsp degree of male genital
r Fluid resuscitation 2. Black PC, Friedrich JB, Engrav LH, et al. Meshed
region, init encntr
unexpanded split-thickness skin grafting for r T21.07XA Burn of unsp degree of female genital
r Electrolytes as needed
reconstruction of penile skin loss. J Urol.
r Tetanus prophylaxis 2004;172(3):976979. region, init encntr
r Antibiotic prophylaxis not necessary r T21.16XA Burn of first degree of male genital
3. Angel C, Shu T, French D, et al. Genital and perineal
Treat specific infections as they arise. burns in children: 10 years of experience at a major region, init encntr
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CALYCEAL DIVERTICULA
Yaniv Shilo, MD
Timothy D. Averch, MD, FACS
Pathologic Findings
BASICS DIAGNOSIS r Lined by nonsecretory transitional epithelium.
r Retrograde reflux of urine from the calyx via the
DESCRIPTION HISTORY diverticular neck can cause stasis with stones in
r Calyceal diverticula are nonsecretory, transitional r Mostly incidental finding on imaging
r Flank pain calyceal diverticula in up to 50% of cases
cell epithelium-lined cystic cavities within the renal
parenchyma. r Microhematuria or macrohematuria DIFFERENTIAL DIAGNOSIS
r The cavity is usually filled retrograde from urine in r Recurrent UTI r Calcified tumor
r Complicated renal cyst
the collecting system.
r Mostly unilateral. PHYSICAL EXAM r Kidney abscess
r Usually not suggestive r Nephrolithiasis
r Most prevalent in upper calyces (70%) r Possible flank pain
r No gender nor laterality predilection
r Bilateral in 3% DIAGNOSTIC TESTS & INTERPRETATION
r Sometimes called pelvicaliceal diverticula Lab
TREATMENT
r Urinalysis
GENERAL MEASURES
EPIDEMIOLOGY Microhematuria and pyuria r In case of uncomplicated, asymptomatic calyceal
Incidence r Urine culture diverticulum treatment can be conservative with no
<1% Bacterial persistence further imaging follow-up.
Prevalence r Indications for therapy include pain, recurrent
Imaging
Found in up to 0.45% of routine intravenous r Abdominal x-ray (KUB): infection, increased calculus growth, hematuria or
pyelogram studies. May demonstrate characteristic radiopaque milk large size that compresses or progressively damages
of calcium, which appears as a half moon or contiguous renal parenchyma
RISK FACTORS
N/A meniscus-shaped calcification MEDICATION
Milk of calcium should change its location when
Genetics First Line
changing positioning from erect to lateral
N/A Antibiotic treatment can be used for recurrent UTIs;
decubitus.
otherwise no specific role
PATHOPHYSIOLOGY Case reports of confusion as being diagnosed as
r Congenital in origin due to failure of regression of rib metastasis Second Line
ureteric bud. r Ultrasound (US): N/A
r Urine enters diverticulum passively via narrow Provide diagnosis in up to 80% of the cases. SURGERY/OTHER PROCEDURES
communication with collecting system. Shows cystic lesion with curvilinear, plaque-like r Shock wave lithotripsy (SWL):
r Urine trapped in diverticulum predisposes to calcification along its posterior wall.
Exam while changing positioning is needed to May be suitable for calyceal diverticulum with
infection and stone formation. small calculi and wide infundibulum (1)[B].
differ from complex cyst.
ASSOCIATED CONDITIONS r Intravenous pyelography (IVP): Can resolve flank pain.
r Flank pain Limitations are due to inadequate passage of
Delayed imaging demonstrates the diverticulum,
r Calyceal calculi (950%) stone fragments through the infundibulum and
as it fills retrogradely from its connection to the
r Recurrent urinary tract infection (UTI) renal pelvis or calyx. lack of anomaly repair.
r Hematuria r CT urography (CTU): r Ureteroscopy (URS):
Delayed imaging is critical to demonstrate Most suitable as initial treatment for calculi
GENERAL PREVENTION <1.5 cm located in the middle or upper pole
N/A contrast medium within an apparent cystic mass
diverticulum and specifically in the anterior aspect.
Diagnostic Procedures/Surgery Involves mechanical dilatation of the diverticular
r Retrograde pyelogram:
neck and removal of calculi if present.
Allows greater distension of the collecting system Ablation of diverticular cavity is not a common
than can be attained with IVP. practice.
Delineating anatomy and assist in planning the
appropriate treatment approach.
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CALYCEAL DIVERTICULA
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CHORDEE
Jennifer A. Hagerty, DO
EPIDEMIOLOGY
Incidence DIAGNOSIS TREATMENT
The incidence of chordee is unknown
HISTORY GENERAL MEASURES
Prevalence r Visualized curvature of the penis with an erection Chordee repair is the standard approach
r 44% of fetuses through the 2nd trimester r Presence of hypospadias
suggesting chordee is a normal part of
MEDICATION
development (1)[A] PHYSICAL EXAM First Line
r Observe the individuals erection if possible
r Chordee occurs without hypospadias in 410% of None usually indicated specifically for chordee
r Possible coexisting findings:
cases of congenital chordee (2)[C] Second Line
Hypospadias or epispadias N/A
r Hypospadias occurs in 1 of 250 live births (3)[A] Incomplete foreskin ventrally
Chordee is identified in 1/3 of these patients Penoscrotal webbing SURGERY/OTHER PROCEDURES
Penile torsion r Specific surgery dependant on the associated
(3)[A]
Hypoplasia of the ventral shaft skin conditions and the severity of the curvature
RISK FACTORS Cryptorchidism r Performed typically after 6 mo of age
r Congenital
r General points:
r Prior penile surgery DIAGNOSTIC TESTS & INTERPRETATION
r Trauma Following penile skin release, induce artificial
Lab
r Routine lab testing not typically indicated erection. This should be repeated to confirm
Genetics r Chromosomal testing and/or biochemical testing in correction.
r Found in syndromes associated with hypospadias Chordee without hypospadias often can be
r Chromosomal abnormalities found in 22% of the individual with a suspected syndrome or corrected by penile degloving with excision of the
disorder of sexual differentiation fibrous tissue superficial to Buck fascia.
individuals with severe hypospadias associated with
undescended testicles Imaging More moderate chordee requires simple plication
r 14% of hypospadias in siblings r Renal and bladder ultrasound routinely and/or excision of ellipses from the site of
r 8% incidence in offspring recommended only in individuals with: maximum curvature.
Severe hypospadias In the most severe cases, often associated with
PATHOPHYSIOLOGY Hypospadias associated with other organ system hypospadias the urethra may be foreshortened
r Chordee could be considered an arrest of normal anomalies and need to be transected.
embryologic development
r Different proposed etiologies for chordee without Diagnostic Procedures/Surgery
r Intraoperative artificial erection test at the time of
hypospadias (2,4): repair
Class I: Results when corpus spongiosum, dartos, Infusion of injectable saline into the corpora with
and Buck fasciae are deficient over the involved a tourniquet at the base of the penis
portion of the urethra; urethra is just below the
skin, and the dense fibrous tissue beneath the
urethra is responsible for the chordee.
Class II: Spongiosum is normal while the dartos
and Buck fasciae are dysgenetic.
Class III: Only the dartos fascia is deficient.
Class IV: Corporeal disproportion.
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CHORDEE
Chordee secondary to corporeal disproportion Patient Resources See Also (Topic, Algorithm, Media)
involves incising the tunica albuginea on the r http://men.webmd.com/guide/chordee-repair- r Chordee Image
ventral surface of the penis, transversely over the treatment r Disorders of Sexual Development (DSD)
point of maximal curvature; than covering the r http://www.mayoclinic.com/health/hypospadias/ r Epispadias
defect with either a free dermal, tunica vaginalis or DS00884 r Hypospadias
single ply small intestinal submucosal (SIS) graft.
It is critical to identify and preserve the
neurovascular bundles during dissection and REFERENCES CODES
plication.
Skin flaps may be required for penile skin 1. Kaplan GW, Lamm DL. Embyrogenesis of Chordee. C
coverage after correction of the chordee. J Urol. 1975;114:769772. ICD9
2. Kramer S, Aydin G, Kelalis P. Chordee without r 607.89 Other specified disorders of penis
ADDITIONAL TREATMENT hypospadias in children. J Urol. 1982;128: r 752.61 Hypospadias
Radiation Therapy 559561. r 752.63 Congenital chordee
N/A 3. Paulozzi LJ, Erickson JD, Jackson RJ. Hypospadias
Additional Therapies trends in two US surveillance systems. Pediatrics. ICD10
r N48.89 Other specified disorders of penis
N/A 1997;100:831834.
r Q54.4 Congenital chordee
Complementary & Alternative 4. Devine CJ Jr., Horton CE. Chordee without
r Q54.9 Hypospadias, unspecified
hypospadias. J Urol. 1973;110:264271.
Therapies
N/A 5. Vandersteen DR, Husmann DA. Late onset recurrent
penile chordee after successful correction at
hypospadias repair. J Urol. 1998;160:11311133.
CLINICAL/SURGICAL
ONGOING CARE PEARLS
PROGNOSIS r Chordee most commonly occurs with hypospadias.
r Excellent prognosis postoperatively with a low ADDITIONAL READING r Repair recommended after 6 mo of age.
complication rate Bologna RA, Noah TA, Nasrallah PF, et al. Chordee: r Consider ongoing monitoring after puberty.
r There may be progression of chordee after puberty Varied opinions and treatments as documented in a
(5)[C] survey of the American Academy of Pediatrics, Section
of Urology. Urology. 1999;53:608612.
COMPLICATIONS
Recurrence of chordee
FOLLOW-UP
Patient Monitoring
r Postoperative checkup within several weeks after
surgery
r Consider follow-up after puberty
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PATHOPHYSIOLOGY r Hyperparathyroidism
BASICS r Heterogenous condition with various causes Results from altered calcium and phosphorus
Diabetic kidney disease metabolism
DESCRIPTION Nondiabetic kidney disease r Anemia
r Defined as presence of kidney damage or impaired Glomerular disease r Proteinuria/albuminuria
GFR (<60 mL/min/1.73 m2 ) for >3 mo (1) Vascular diseases Albuminuria indicates increased glomerular
if less than 3 months considered acute kidney Tubulointerstitial disease permeability to macromolecules
injury (AKI) Cystic disease (polycystic kidney disease) Albumin to creatinine ratio >30 mg/g indicates
Irrespective of cause kidney damage defined as: Transplant nephropathy increased risk of CKD progression, ESRD,
Pathologic abnormalities Acute rejection cardiovascular and all cause mortality.
Urinary, blood, or imaging abnormalities Chronic rejection r GFR
Kidney transplantation Calcineurin toxicity
r Classification based on Kidney Disease Outcomes Estimated GFR (mL/min/1.73 m2 ) = 1.86
Glomerulonephropathy (SCR)1.154 (age)0.203 (0.742 if female)
Quality Initiative (NKF KDOQI): (1.1212 if African American)
ASSOCIATED CONDITIONS
Stage 1: Kidney damage with normal renal
See risk factors Imaging
function (GFR >90 mL/min/1.73 m2 ) r Broad range of findings depending on etiology and
Stage 2: Mild renal dysfunction GENERAL PREVENTION
(GFR 6089 mL/min/1.73 m2 ) r Screening and treatment of associated risk factors imaging modality (US, CT scan, MRI, angiography,
Stage 3: Moderate renal dysfunction Screening selected populations: isotope scans)
(GFR 3059 mL/min/1.73 m2 ) Age >50 Hydronephrosis: Potentially reversible
Stage 4: Severe renal dysfunction History of DM (diabetes melitus), HTN Polycystic kidneys
(GFR 1530 mL/min/1.73 m2 ) (hypertension), or CV (cardiovascular) disease Atrophic kidneys
Stage 5: Kidney failure (GFR <15 or dialysis Family history Increased echogenicity (on ultrasound)
mL/min/1.73 m2 ) Exposure to nephrotoxins Renal artery stenosis
Cortical scarring
EPIDEMIOLOGY
Diagnostic Procedures/Surgery
Incidence DIAGNOSIS r Renal biopsy is indicated in selected cases and
r Stage 1 or 2 CKD progress to more advanced stages
HISTORY choices can vary greatly with nephrologists
at 0.5% per year
r Stage 3 or 4 progress to end-stage renal disease at r Silent, asymptomatic until late stages Isolated glomerular hematuria with proteinuria
Evaluate for symptoms of associated Nephrotic syndrome
1.5% per year (3) Acute nephritic syndrome
conditions/risk factors
Prevalence r Symptoms of uremia in ESRD Acute/rapidly progressive kidney disease
r 10% in noninstitutionalized adults r Urologic evaluation if gross or microscopic
Anorexia
Corresponds to >20 million people (4) Decreased urine output hematuria
398,000 treated by dialysis in 2000, expected to Increased thirst Cystoscopy
increase to >2 million people by 2030 (2) Mental status changes Upper tract imaging
Prevalence in US population r Angiography (CT or MR angiogram) if suspected
Muscle cramps
Stage 1: 1.8% atherosclerotic renovascular disease (asymmetric
Nausea
Stage 2: 3.2% renal size)
Vomiting
Stage 3: 7.7%
Stage 4/5: 0.35% (3) PHYSICAL EXAM Pathologic Findings
r Physical exam findings uncommon until late stages r Renal biopsy findings
RISK FACTORS of disease Reveals tubulointerstitial, glomerular, or vascular
r Diabetes disease
r Hypertension Findings associated with increased risk
BP >130/85 May also be seen on nephrectomy/partial
r Cardiovascular disease Obesity/increased waist circumference nephrectomy specimens
r Family history Manifestations of advanced kidney disease DIFFERENTIAL DIAGNOSIS
r Age >60 Volume overload/edema r Kidney damage with duration >3 mo is diagnostic
r Urinary tract obstruction Pruritus regardless of cause
r Urinary calculi Visual disturbances r Differentiate from acute kidney disease based on
r Nephrotoxic drugs Weight Loss duration and underlying etiology
r Obesity Confusion Acute kidney injury
r Neoplasia DIAGNOSTIC TESTS & INTERPRETATION Alport syndrome
r Loss of kidney mass Lab Autosomal dominant polycystic kidney disease.
r Race r Chemistry Chronic glomerulonephritis
Elevated creatinine Diabetic nephropathy
African American, American Indian, Hispanic, Goodpasture syndrome
Asian, or Pacific Islander Elevated blood urea nitrogen
Hyperkalemia Multiple myeloma
Genetics Acidosis Nephrolithiasis
r Complex phenotype impacted by various genetic Nephrosclerosis
Hyperphosphatemia
factors in addition to environmental factors and r Urinalysis with microscopy Rapidly progressive glomerulonephritis
comorbid disease Renal artery stenosis
CYP4A11 gene involved in renal vasoconstriction Hematuria
Systemic lupus erythematosus
and natriuresis and is associated with increased Casts: RBC (glomerulonephritis), WBC (interstitial
Urinary obstruction
risk in African Americans (6) nephritis)
Wegener granulomatosis
APOL1 associated with focal segmental Fat bodies (nephrotic syndrome)
glomerulosclerosis and hypertension associated
ESRD (7)
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DIFFERENTIAL DIAGNOSIS
r As mentioned for TID, polyuria, polydipsia can also
ADDITIONAL TREATMENT REFERENCES
Radiation Therapy
be associated with diabetes, which is an easy N/A 1. National Kidney Foundation. K/DOQI clinical
diagnosis to exclude based on urinalysis and blood practice guidelines for chronic kidney disease:
work. Additional Therapies Evaluation, classification, and stratification. Am J
r Differential diagnosis of CKD is limited to underlying Dialysis (peritoneal or hemodialysis) or kidney Kidney Dis. 2002;39:S1S266.
transplant should be considered when GFR falls into
disease 2. Wesseling-Perry K. Bone disease in pediatric
the 20s or growth and metabolic control can no
Congenital renal anomalies: Obstructive uropathy, chronic kidney disease. Pediatr Nephrol.
longer be maintained with medical management.
renal hypoplasia or dysplasia, reflux nephropathy, 2013;28:569576.
polycystic kidney disease Complementary & Alternative C
Glomerular disease: Focal segmental Therapies
glomerulosclerosis (FSGS). r Nutrition is important: At least 2 g/kg/d protein ADDITIONAL READING
Others: Hemolytic uremic syndrome, genetic would be needed in order to maintain adequate
r Avner ED, Harmon WE, Niaudet P, et al., eds.,
diseases (cystinosis, oxalosis, Alport syndrome), protein stores and growth.
interstitial nephritis r Nutrition in CGD is associated with salt and water Pediatric Nephrology. 6th ed. Springer; 2009.
r Geary DF, Schaefer F, eds. Comprehensive Pediatric
Rare in childhood: Diabetic nephropathy and restriction. Therefore, the nutrition construction may
hypertension be opposite in terms of the sodium and water load. Nephrology. 1st ed. Mosby; 2008.
See Also (Topic, Algorithm, Media)
r Acute Kidney Injury, Pediatric (Renal Failure, Acute)
TREATMENT ONGOING CARE r Megaureter, Congenital
GENERAL MEASURES PROGNOSIS r Posterior Urethral Valves
r In chronic interstitial renal disease with r Difficult to predict early in life. r Prune Belly (Eagle-Barrett or Triad) Syndrome
polyuria/polydipsia, more fluid and sodium are r Lack of renal growth with a creatinine greater than 1
needed to maintain euvolemia. at a year of age, associated hematuria, proteinuria,
r In both groups, attention to potassium and and hypertension in patients with TID portends the CODES
phosphorus load is important. need for future dialysis and transplantation.
r All NSAIDS should be avoided. Levels of potential r CGD prognosis is directly related to underlying
ICD9
nephrotoxins, if measurable such as vancomycin or cause. r 582.89 Chronic glomerulonephritis with other
gentamicin, should be followed at least twice r Certain diseases such as lupus, Wegener,
specified pathological lesion in kidney
weekly; with an initial level no later than 2448 hr Goodpasture, membranous nephropathy, and IgA r 585.9 Chronic kidney disease, unspecified
after starting. nephropathy can be amenable to therapy. r 753.8 Other specified anomalies of bladder and
r Damage from CT contrast should be minimized with r Other renal diseases such as focal sclerosis maybe
urethra
pre- and postprocedure IV hydration. less amenable to therapy.
COMPLICATIONS ICD10
MEDICATION r N03.9 Chronic nephritic syndrome with unsp
r Growth impairment in children is a known
First Line morphologic changes
r Treatment of metabolic acidosis with either liquid complication, independent of the etiology of CKD (2) r N18.9 Chronic kidney disease, unspecified
form of Bicitra or the pill form of bicarbonate to r Hypertension is also a risk factor.
r Q64.79 Other congenital malformations of bladder
normalize the CO2 . This will preserve growth as well r Protein restriction, used in adult CKD to slow disease
as bone integrity. and urethra
progression, cannot be used with kids as this would
r Treatment of phosphorus restriction, phosphorus further hinder their growth and development.
binding (nonaluminum binders such as CaCO3 , FOLLOW-UP CLINICAL/SURGICAL
Calcium acetate, or sevelamer products), and r Newborns and infants should be seen as frequently PEARLS
institution of vitamin D to preserve and prevent as every 12 wk in order to ensure maintenance of
secondary hyperparathyroidism and prevent bone r Patients with interstitial disease or obstructive
euvolemia.
disease. r Primary care physicians need to be instructed that uropathy require IV hydration while NPO prior to
r Treatment with replacement doses of iron sucrose procedures.
patients with interstitial disease will get dehydrated r In contrast to adults, diabetic nephropathy and
13 mg/kg per dose and use of erythropoietic more quickly than the average patient; therefore,
agents (epoetin, darbepoetin) are used for anemia. attention to their care at the time of vomiting and hypertension are rare causes of CKD in children.
r Small changes in creatinine reflect large changes in
Second Line diarrhea is important for these patients will become
r Those that were mentioned above including volume depleted in a hurry. GFR for children with CKD.
antihypertensive agents if indicated. r Baseline creatinine with attention to any changes
r Often ACE inhibitors and angiotensin receptor Patient Monitoring
after potential renal insult (CT contrast,
Glomerular-based renal diseases are associated with
blockers (ARBS) are used for glomerular-based renal hypovolemia/hypertension, medications, etc.) is an
salt and water restriction as well as blood pressure
disease because of proteinuria but are important measure for monitoring and prevention of
control.
contraindicated in possible pregnancy (birth defects) progression of CKD.
and low GFR (renal failure, hyperkalemia) Patient Resources
r American Society of Pediatric Nephrology.
SURGERY/OTHER PROCEDURES www.aspneph.com/parentpatient.asp
In TID, patients may require cystoscopy with ablation r National Kidney Disease Educational Program.
of PUVs. UPJ or UVJ obstructions may need to be www.nkdep.nih.gov
relieved. Reflux may need to be corrected, if high r NKF Cares: Patient Information Center.
grade and not resolving spontaneously. www.kidney.org/patients
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CHYLOUS ASCITES
Brett S. Carver, MD
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CHYLOUS ASCITES
ADDITIONAL READING
TREATMENT ONGOING CARE r Bosl GJ, Feldman DR, Bajorin DF, et al. Cancer of the
GENERAL MEASURES PROGNOSIS testis. In: DeVita VT, Hellman S, Rosenberg SA, eds.
r All patients with abdominal distention following an The prognosis is excellent for the vast majority of cases Cancer: Principles and Practice of Oncology. 9th ed.
RPLND should be evaluated for: as most will respond to conservative management. Philadelphia, Pa: Lippincott Williams & Wilkins;
Ascites (nonchylous) COMPLICATIONS 2011:12801301.
Ileus r The complications of chylous ascites related to r Evans JG, Spiess PE, Kamat AM, et al. Chylous
Small-bowel obstruction
Recurrent disease in the abdomen or
increased abdominal pressure:
Renal failure
ascites after post-chemotherapy retroperitoneal
lymph node dissection: Review of the M. D.
C
retroperitoneum. Venous thrombosis Anderson experience. J Urol. 2006;176(4 Pt 1):
r The majority of chylous effusions will heal 14631467.
Pulmonary embolism
spontaneously. Abdominal paracentesis is diagnostic r Link RE, Amin N, Kavoussi LR. Chylous ascites
Atelectasis
and often therapeutic in relieving symptoms Pneumonia following retroperitoneal lymphadenectomy for
associated with increased abdominal pressures. r The gastrointestinal complications of chylous ascites testes cancer. Nat Clin Pract Urol. 2006;
include ileus and small-bowel obstruction. 3(4):226232.
MEDICATION
First Line Malnourishment and failure to thrive may also See Also (Topic, Algorithm, Media)
r Low lipid, high medium chain triglyceride oral diet. occur due to protein-losing enteropathy with r Chylous Ascites Image
MCT oil supplement chronic diarrhea (steatorrhea), malabsorption, and r Lymphatic Ascites
1 tablespoon (15 mL) 34 times/d malnutrition r Testis Cancer, Adult General Considerations
Mix with juices or otherwise incorporated into FOLLOW-UP
low-fat diet Patient Monitoring
Do not use in patients with advanced cirrhosis: r Follow-up protocols should be followed according to CODES
Risk of narcosis and coma guidelines established by the National
r Somatostatin analogs have been demonstrated to
Comprehensive Cancer Network for testicular cancer ICD9
be effective in reducing lymphorrhagia. patients. r 125.9 Unspecified filariasis
Octreotide 100 mcg administered subcutaneously r After initial treatment of chylous ascites, patients r 457.8 Other noninfectious disorders of lymphatic
3 times per day should be seen in follow-up to monitor for recurrent channels
Second Line ascites.
r Total parental nutrition is to be utilized in patients ICD10
Patient Resources r B74.9 Filariasis, unspecified
who fail oral diet modifications. N/A r I89.8 Oth noninfective disorders of lymphatic vessels
Bowel rest may enhance recovery if conservative
approaches are not successful and nodes
SURGERY/OTHER PROCEDURES
REFERENCES
r Abdominal paracentesis, repeated as necessary. CLINICAL/SURGICAL
1. Carver BS, Sheinfeld J. Germ cell tumors of the
Primarily for pain control and dyspnea
r Surgical exploration with direct ligation of lymphatic
testis. Ann Surg Oncol. 2005;12(11):871880. PEARLS
2. Castillo OA, Litvak JP, Kerkebe M, et al. Case r Chylous ascites occurs in 13% of patients
vessels for persistent chylous ascites (2). report: laparoscopic management of massive
r Peritoneal venous shunts for refractory chylous chylous ascites after salvage laparoscopic undergoing a RPLND.
r Risk factors include vena cava resection, suprahilar
ascites. retroperitoneal lymph-node dissection. J Endourol.
r Direct lymphatic vessel ligation or embolization of 2006;20(6):394396. dissections, and concomitant hepatic surgery.
r Initial management includes paracentesis for
large leaking vessels using interventional radiologic 3. Chen J, Lin RK, Hassanein T. Use of orlistat (xenical)
techniques. to treat chylous ascites. J Clin Gastroenterol. symptom of pain or pulmonary compromise and low
2005;39(9):831833. lipid, high medium-chain triglyceride oral diet.
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
N/A
Complementary & Alternative
Therapies
Orlistat (Xenical) has been used successfully in a
nontesticular cancer case of chylous ascites (3)
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CHYLURIA
Matthew A. Uhlman, MD, MBA
James A. Brown, MD, FACS
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CHYLURIA
Pathologic Findings ADDITIONAL TREATMENT 4. Kaur H, Matin SF, Javadi S, et al. Chyluria after
Lipid contents of chyluria are mainly chylomicrons, Radiation Therapy radiofrequency ablation of renal tumors. J Vasc
90% of which are in the form of triglycerides N/A Interv Radiol. 2011;22:924927.
DIFFERENTIAL DIAGNOSIS 5. Zhang CJ, Chen RF, Zhu HT, et al.
Additional Therapies
r Filariasis from W. bancrofti, B. malayi, or B. timori r Sclerotherapy with various agents instilled into
Retroperitoneoscopic renal pedicle lymphatic
r Pyelolymphatic fistula disconnection for chyluria in presence of complex
collecting system (1,3)[C][B] renal vasculature. Urology. 2012;80:12731276.
r Phosphaturia, most common cause of cloudy urine
Povidone iodine (5%) and dextrose (50%) in 6. Gomella LG, Shenot P, Abdel-Meguid TA.
r Pyuria renal pelvic instillation sclerotherapy; 87%
r Hyperuricosuria success reported
Extraperitoneal laparoscopic nephrolysis for the
treatment of chyluria. Br J Urol. 1998;81(2):
C
r Nephropathyurinary sediment can cause cloudy Silver nitrate (13%) instillation into the affected 320321.
appearing urine collecting system causes sclerosis of lymphatic
r Enterovesical fistula fistulas; 48% success reported
Case reports of successful sclerotherapy with: ADDITIONAL READING
N-butyl-2-cyanoacrylate (component of medical
TREATMENT cyanoacrylate glues) Kaul A, Bhadhuria D, Bhat S, et al. Chyluria: A
Radiographic contrast media mimicker of nephrotic syndrome. Ann Saudi Med.
GENERAL MEASURES 2012;32(6):593595.
r Nontropical Complementary & Alternative
Up to 50% of cases resolve spontaneously under Therapies See Also (Topic, Algorithm, Media)
dietary restriction (1)[C] N/A r Chyluria Image
Bed rest and/or use of abdominal binder to r Filariasis, Urologic Considerations
increase abdominal pressure may allow r Urine, Abnormal Colored
ONGOING CARE
spontaneous closure.
Medium-chain triglyceride (MCT) diet (avoidance PROGNOSIS
r Rarely fatal, with high success rates reported for
of long-chain triglycerides) CODES
MCTs are transported via portal system, not by surgical intervention
chylomicrons through lymphatics r Recurrence rates after surgery reported as high as
ICD9
Ureteral stent placement to reduce renal pelvis 25% r 125.0 Bancroftian filariasis
pressure r 125.9 Unspecified filariasis
COMPLICATIONS
MEDICATION r Hypoalbuminemia and anasarca from massive r 791.1 Chyluria
Nontropical protein loss (1)[C]
Dietary modifications to reduce chylomicrons in r Immunosuppression from fat soluble vitamin loss in ICD10
r B74.0 Filariasis due to Wuchereria bancrofti
dietrecommendations are often for fat-free or very chronic cases (1)[C]
r Underlying filariasis may cause epididymitis, r B74.9 Filariasis, unspecified
low-fat diet, though this should not be observed for
more than several weeks given the bodys need for r R82.0 Chyluria
hydrocele, and elephantiasis of the penis/scrotum
some fats and lower extremities
Parasitic Chyluria FOLLOW-UP CLINICAL/SURGICAL
r DEC and albendazole, or ivermectin and albendazole
r DEC fortified salt can be used to treat and prevent Patient Monitoring PEARLS
r Treatment failures are readily apparent as urine
lymphatic filariasis r Milky or cloudy urine (often after meals) is the most
returns to milky color (1,5)[C][A]
SURGERY/OTHER PROCEDURES common presentation, though phosphaturia is the
r Re-evaluate if chyluria recurs following treatment;
r Procedures of choice involve disconnection of renal most common cause of cloudy urine.
consider the contralateral kidney as the source r W. bancrofti, B. malayi, and B. timori are the
pedicle lymphatics (1,5,6)[C][A]
Patient Resources primary causes of filariasis, the most common cause
r Nephrolysis:
N/A of chyluria (parasitic chyluria).
Stripping and ligation of all lymphatic vessels to r Following renal surgery, incidence of chyluria (up to
the kidney and upper ureter; open and 41% on CT) is likely much higher than is clinically
laparoscopic techniques described REFERENCES significant (<<1%).
Laparoscopic transabdominal and r Up to 50% of cases of chyluria resolve
retroperitoneoscopic approaches described 1. Kim RJ, Joudi FN. Chyluria after partial
nephrectomy: Case report and review of the spontaneously with a medium chain fatty acid or
Success rates 8098%; recurrence rates 325% very low-fat diet.
r Endoscopic coagulation of fistula literature. ScientificWorldJournal. 2009;9:14.
r There is no best imaging technique, though
r Lymphangiovenous anastomosis with ligation of 2. Tandon V, Singh H, Dwivedi US, et al. Filarial
chyluria: Long-term experience of a university lymphangiography can demonstrate entrance of
renal lymphatics contrast from lymphatics into the collecting system.
r Renal autotransplantation hospital in India. Int J Urol. 2004;11:193199.
r Nephrectomy was described prior to minimally 3. Panchal VJ, Chen R, Ghahremani GG. Non-tropical
chyluria: CT diagnosis. Abdom Imaging. 2012;
invasive techniques 37(3):494500.
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Pathologic Findings
BASICS DIAGNOSIS r Branching, villous, papillary connective tissue stroma
covered by epithelium.
DESCRIPTION HISTORY r Superficial hyperkeratosis and thickening of the
r Anogenital epidermal lesions caused by the r Age and sex of patient
r History of recent sexual exposure epidermis (acanthosis).
transmission of human papilloma virus (HPV) r Clear vacuolization of the prickle cells (koilocytosis),
r The most common viral sexually transmitted r Number of partners and frequency of sexual
characteristic of HPV infection, is seen.
infection in the US, they are also called genital intercourse r There is no evidence of invasion of the underlying
warts, or venereal warts r Visible warts usually seen within 23 mo after
stroma
r Most common sites: Penis, vulva, vagina, cervix, exposure
perineum, and perianal area. r Practice of anal intercourse DIFFERENTIAL DIAGNOSIS
r Less commonly, urethra, bladder, oropharynx, larynx, r Immunocompromised state r Bowen disease and erythroplasia of Queyrat
r Bowenoid papulosis
and trachea
PHYSICAL EXAM r Buschke-Lowenstein tumor
EPIDEMIOLOGY r Lesions are pinkish to red-grayish white r Condyloma latum (syphilis)
Incidence (1) cauliflower-like lesions found on moist surfaces, r Extramammary Paget disease
r Most common STD often coalescing. r Fibroepitheliomas
r 1% of sexually active adults in the US r Lesions appear pearly white and granular
r Herpes simplex virus
r Larger lesions may be verrucous or flat in
Prevalence r Malignant melanoma
r Highest prevalence: 1828 yr olds and exceeds 50% configuration r Molluscum contagiosum
r HPV DNA can be detected in 1015% of the US r With magnifications, a central venule can be seen
r Nevi
population within each projection.
r Male: Examine penis, meatus, scrotum, perineum, r Pearly penile papules
r HPV 6 and 11 account >90% of visible genital r Seborrheic keratosis
warts. suprapubic, and perianal region
r Female: Vagina, introitus, perineum, cervix, and r Squamous cell carcinoma/basal cell carcinoma
RISK FACTORS perianal region
r Increased risk with number of sex partners, r Examine for evidence of coexisting STD (ulcers,
frequency of sexual activity, early coitus, and discharge, adenopathy).
TREATMENT
presence of condyloma on partners
r Age <25 DIAGNOSTIC TESTS & INTERPRETATION GENERAL MEASURES (5)
r Diagnosis usually based on observation of
r Immunocompromised status Lab
r Cigarette smoking and oral contraceptives may be r HPV cannot be readily grown in culture. characteristic lesions.
r Cytologic testing with Pap smear: Exfoliated genital r Main goal of treatment is to remove visual presence
associated with an increased risk. of warts.
r Onset of sexual activity at an early age cells are stained and examined for koilocytosis and
r Current therapies have an equally low effectiveness
neoplasia
PATHOPHYSIOLOGY r Serologic assays not useful in screening for HPV in preventing wart recurrence and may not reduce
r HPV is a double-stranded, circular DNA genome disease transmission.
injection, but may provide prognostic information
consisting of 8,000 base pairs. Subtypes 6 and 11 r Vaccine: HPV quadrivalent recombinant (types 6, 11,
for patients with abnormal Pap smears
are associated with the majority of genital warts. r Histologic analysis from biopsy specimens 16, and 18): Gardasil (Merck) is currently available
Types 16, 18 most often associated with potential r Rapid commercial screening tests available and are for administration to females of ages 926 for
for malignancy. prevention of condyloma acuminata and associated
r >80 different subtypes can potentially associate fairly accurate: ViraPap, ThinPrep Pap, Hybrid
diseases. HPV bivalent Cervarix (types 16 and 18)
capture II
with condylomata. r If necessary, molecular characterization for diagnosis (GSK)
r HPV subtypes associated with malignancy include: r Gardasil can also be used in males aged 926 to
and serotyping (eg, Southern and/or slot blot
16, 18, 31, 33, 35, 39, 45, 51, 52, 56, 58, 59, 68, prevent genital warts. Administration to males prior
hybridization, PCR)
and 82 (2) r Consider screening for other associated STDs: HIV, to start of sexual activity is optimal.
r Transmission is by direct sexual contact. r Topical therapy may take up to 3 mo to observe a
GC, chlamydia, syphilis
r Less common mode is autoinoculation from response.
nongenital lesions. Diagnostic Procedures/Surgery
r Magnification with colposcope or 10 handheld MEDICATION
r Basal layer of epidermis is invaded by the virus.
r Latent phase can last months to years. magnifying lens of the suspected region after First Line
r Podophyllin (Podoben 25%, Podocon, Podofin):
application of 35% acetic acid-soaked gauze pad
ASSOCIATED CONDITIONS for 5 min allows visualization of nonvisible lesions, Applied to lesion (concentration 1025%) by
r Penile cancer but has low specificity. (3,4) health care worker once weekly for up to 6 wk
r Anal cancer However, the Centers for Disease Control (CDC) r Podofilox (Condylox):
r Cervical cancer no longer recommends acetic acid soaks to Self-application of a 0.5% solution to warts twice
r Buschke-Lowenstein tumor improve diagnosis. The soaks are associated with daily for 3 days, followed by 4 days without
many false positives. treatment; can be repeated 46 times.
GENERAL PREVENTION r Subclinical lesions may appear shiny white. r 5-FU (Efudex, Fluoroplex):
r Sexual abstinence r Urethroscopy for any patients with suspected Topical treatment with 5% cream 13 times per
r Condoms week for several weeks, as needed. Maybe also
urethral warts, with care to occlude proximal urethra
r Pre-exposure vaccination (Gardasil, Cervarix, to prevent flushing of virus toward bladder used as an intraurethral instillation but not
Hepatitis B) r Proctoscopy for patients at risk for anal condyloma without irritative complications.
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Diffuse erythema: IV access, monitor vitals, pulse Furosemide 2040 mg IV over 2 min 3. Liccardi G, Lobefalo G, Di Florio E, et al. Strategies
oximeter, O2 mask Fexofenadine: 180 mg PO for the prevention of asthmatic, anaphylactic and
If hypotensive NS or LR IV bolus 1 L Glucagon: 1 mg IM anaphylactoid reactions during the administration
If no fluid response consider IM/IV epinephrine Hydrocortisone 200 mg IV over 2 min of anesthetics and/or contrast media. J Investig
Bronchospasm: IV access, monitor vitals, pulse Labetalol: 20 IV over 2 min; double dose every Allergol Clin Immunol. 2008;18(1):111.
oximeter, O2 mask 10 min PRN 4. Schabelman E, Witting M. The relationship of
Mild: -agonist inhaler (eg, albuterol); consider Lorazepam 24 mg IV slow push; 4 mg max radiocontrast, iodine, and seafood allergies: A
rapid response team or ER admit Methylprednisolone: 40 mg IV over 2 min medical myth exposed. J Emerg Med. 2010;39(5):
Moderate: Consider IM/IV epinephrine; consider Morphine: 13 mg IV, repeat every 510 min PRN
rapid response team or ER admit Nitroglycerine: 0.4 mg tablet SL repeat every
701707.
5. ACR Manual on Contrast Media Version 9 2013
C
Severe: IM/IV epinephrine and rapid response 510 min PRN (www.acr.org). Accessed October 2013.
team/911 Second Line
Laryngeal edema: IV access, monitor vitals, pulse N/A
oximeter, O2 mask, IM/IV epinephrine; consider ADDITIONAL READING
rapid response team/911 based on response SURGERY/OTHER PROCEDURES
Hypotension (systolic BP <90 mm Hg): IV access, N/A r Brockow K, Christiansen C, Kanny G, et al.
monitor vitals, pulse oximeter, O2 mask, elevate ADDITIONAL TREATMENT Management of hypersensitivity reactions to
legs 60 ; consider NS or LR IV bolus 1 L iodinated contrast media. Allergy.
With bradycardia (pulse <60 BPM [vasovagal] Radiation Therapy 2005;60:150158.
as above; give atropine; consider rapid response N/A r Schopp JG, Iyer RS, Wang CL, et al. Allergic
team/911 Additional Therapies reactions to iodinated contrast media:
With tachycardia (pulse >100 BPM For life-threatening reactions: ABCs of resuscitation, Premedication considerations for patients at risk.
[anaphylactoid reaction]) IM/IV epinephrine; IV fluids, vasopressors for BP support if IV fluids not Emerg Radiol. 2013;20(4):299306.
consider rapid response team based on response adequate
Hypertensive crisis (DBP >120 mm Hg; SBP >200 See Also (Topic, Algorithm, Media)
Complementary & Alternative r Contrast-Induced Nephropathy (CIN)
mm Hg; IV access, monitor vitals, pulse oximeter, Therapies r Nephrogenic Systemic Fibrosis/Fibrosing
O2 mask; IV labetalol or nitroglycerine SL and N/A
furosemide; rapid response team/911 Dermatopathy (NSF/NFD)
r Reference Tables: Contrast Agents, Genitourinary
Unresponsive and pulseless: Check for
responsiveness; rapid response team/911; initiate ONGOING CARE
CPR; apply AED device; epinephrine IV (10 mL
1:10,000) PROGNOSIS CODES
r Depends on severity of ADR
Pulmonary edema: IV access, monitor vitals, pulse
r Risk of death of 1 in 170,000 for both ionic HOCM
oximeter, O2 mask; elevate head of bed; IV ICD9
furosemide, IV morphine; rapid response and nonionic LOCM r 708.0 Allergic urticaria
team/911 COMPLICATIONS r 995.0 Other anaphylactic reaction
Seizures: Protect patient; turn on side to avoid r Renal failure occurs in up to 5% r 995.27 Other drug allergy
aspiration; suction airway as needed; IV access, r Generally supportive measures only with renal
monitor vitals, pulse oximeter, O2 mask; if function returning to normal in a few weeks ICD10
unremitting rapid response team/911 lorazepam r Contrast-induced nephropathy (CIN) r L50.0 Allergic urticaria
IV r T50.8X5A Adverse effect of diagnostic agents, initial
Hypoglycemia: IV access, O2 mask; oral 2 sugar FOLLOW-UP encounter
packets or 4 oz fruit juice or D50W I amp IV with Patient Monitoring r T88.6XXA Anaphylactic reaction due to adverse
D5W or D5NS 100 mL/h adjunctively; if no IV r Appropriate supportive measures until recovery
effect of correct drug or medicament properly
glucagon 1 mg IM depending on severity of ADR administered, initial encounter
Anxiety/panic attack: Diagnosis of exclusion; r For patients with renal insufficiency on metformin
monitor for evolving reactions if present; IV access, follow-up renal function monitoring recommended.
monitor vitals, pulse oximeter; reassure patient CLINICAL/SURGICAL
Reaction rebound prevention: IV steroids help Patient Resources
N/A PEARLS
short-term recurrence but not acute treatment
benefit; consider IV hydrocortisone/ A shellfish or iodine allergy does not correlate with
methylprednisolone with severe allergic reaction REFERENCES contrast media allergy.
prior to transport to ED
1. Cochran ST, Bomyea K. Trends in adverse events
MEDICATION
from iodinated contrast media. Acad Radiol.
First Line 2002;9(suppl 1):S65S68.
r Based on guidelines noted above (5):
2. Hagan JB. Anaphylactoid and adverse reactions to
Albuterol: 2 puffs (90 mcg/puff)
radiocontrast agents. Immunol Allergy Clin North
Atropine 0.61 mg slow IV with NS flush up to
Am. 2004;24:507519.
3 mg
Benadryl
2550 mg PO
2550 mg IV slowly over 2 min
Epinephrine
0.3 mg (0.3 mL 1:1,000 solution) IM
EpiPen or (equivalent) IM 0.3 mL 1:1,000
solution
1 mL 1:10,000 solution slow IV injection over
5 min repeated every 510 min as needed for
severe reaction
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Imaging
BASICS DIAGNOSIS r Brain MRI if pituitary lesion suspected
r CT abdomen/pelvis adrenal protocol for
DESCRIPTION HISTORY ACTH-independent hypercortisolism to evaluate for
r Cushing disease is hypercortisolism due to an r Progressive weight gain
r Fatigue adrenal adenoma/carcinoma
ACTH-secreting pituitary adenoma
r Cushing syndrome is the cluster of symptoms r Proximal limb weakness Diagnostic Procedures/Surgery
r Skin abnormalities: Easy bruisability, and striae Inferior petrosal vein sampling to diagnose and
attributable to hypercortisolism
r Pituitary adenomas account for 70% of patients r Abnormal menses/decreased libido localize pituitary adenoma
with endogenously elevated cortisol (15% primary r Impotence Pathologic Findings
r Pituitary adenoma
adrenal tumor, 15% ectopic ACTH production) r New-onset hypertension/diabetes
r Iatrogenic supplementation of glucocorticoids is the r Adrenal adenoma/carcinoma
r Frequent infections
r Micronodular/macronodular adrenal hyperplasia
most common cause of hypercortisolism r Osteopenia/osteoporosis
EPIDEMIOLOGY r Visual disturbances due to pituitary impinging optic DIFFERENTIAL DIAGNOSIS
nerves r Alcoholism (pseudo-Cushing)
Incidence
r Anorexia nervosa
N/A PHYSICAL EXAM
r Obesity/weight gain (80%) r Bulimia
Prevalence r Depression
r Cushing disease: 1.22.4 per million r Thin skin with striae (70%)
r Cushing syndrome 45 more common in women r Moon facies (75%) r Hypertension
r Buffalo hump (50%) r Obesity
than men
r In diabetics, prevalence 25% r Hypertension (75%) r Polycystic ovarian syndrome
r Truncal obesity (50%)
RISK FACTORS
r Iatrogenic exposure to glucocorticoids r Amenorrhea (60%)
r Loss of visual fields
TREATMENT
Includes steroid creams or nasal sprays
GENERAL MEASURES
Genetics DIAGNOSTIC TESTS & INTERPRETATION r Multidisciplinary approach: Endocrinologist,
r Associated with MEN-1, Carney complex
Lab neurosurgeon, adrenal surgeon
r GNAS1 gene mutation (McCune-Albright syndrome) r CBC, serum glucose, electrolytes, lipids r Surgical therapy is the mainstay of treatment
PATHOPHYSIOLOGY Hyperglycemia, hypokalemia, neutrophilia,
r Elevated serum levels of cortisol, either from lymphopenia, hyperlipidemia consistent with MEDICATION
exogenous intake or endogenous production Cushing First Line
r Initial screen: Late-night salivary cortisol and 24-hr r Medical therapy only indicated when surgery not
r Hypothalamus-pituitary-adrenal physiology
urinary free cortisol. (Note: Establishes possible
ACTH (anterior pituitary), stimulates cortisol
hypercortisolemia, not etiology) Ketoconazole: Considered medical treatment of
production in zona fasciculate of adrenal cortex
Elevated late-night salivary cortisol: In Cushing choice; not FDA approved for this indication.
ACTH release governed by CRH (hypothalamus) Inhibits cytochrome p450
Cortisolfeedback regulation of CRH/ACTH syndrome, diurnal variation of cortisol levels is lost
high levels of cortisol suggest Cushing 200400 mg 2 or 3 times a day
production Side effects: Reversible hepatotoxicity,
r Cushing syndrome causes: syndrome
24-hr urinary free cortisol 3 samples: Sensitivity headache, sedation, nausea, and vomiting.
Exogenous intake (most common) Reduced androgen production may lead to
9097%, specificity 8596%
Cushing disease (70% of endogenous Cushing) r Second-line tests: Late-night serum cortisol, gynecomastia, decreased libido, and impotence
Adrenal adenoma/carcinoma (10% of in males
endogenous Cushing) low-dose DST
r Determining etiology of hypercortisolemia Mitotane: Suppresses cortisol production by
Ectopic ACTH producer (10%): Neuroendocrine inhibiting 11-hydroxylase
tumor (small-cell lung cancer, thymoma, ovarian Plasma ACTH concentration
Elevated in Cushing disease/ectopic tumor Primarily used for adrenocortical carcinoma
tumors) 0.5 g start, gradually increase to 23 g/d
Other: ACTH-independent macronodular adrenal Decreased in adrenal adenoma/carcinoma,
nodular adrenal hyperplasia, steroid use Metyrapone: Inhibits 11-hydroxylase
hyperplasia, ectopic CRH production 500750 mg 3 or 4 times a day
High dose DST
ASSOCIATED CONDITIONS May distinguish pituitary from ectopic
r Pituitary tumor
ACTH-secreting tumor (failure to suppress
r Steroid administration cortisol suggests ectopic tumor)
r Adrenal adenoma/carcinoma Inferior petrosal vein sampling: Higher sensitivity
and specificity than high-dose DST
GENERAL PREVENTION
Diligent management of glucocorticoid administration
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r Pelvic radiation:
BASICS DIAGNOSIS Usually initiated by bladder distension, minor
trauma, infection, instrumentation
DESCRIPTION HISTORY Acute episodes wane within 1218 mo
r Inflammation leading to damage of the bladders r Gross hematuria (with or without pain)
Can occur as late as 1520 yr after exposure
urethelium and blood vessels, causing hematuria r Frequency, urgency, dysuria r Viral infection:
and irritative voiding symptoms. r Urinary retention from clots
Adenovirus 11 and 35, influenza A, CMV,
r Hemorrhagic cystitis (HC) is commonly caused by r Occasional mucosal sloughing Polyomavirus hominis 1, the BKV, and JC viruses
severe infection, cyclophosphamide, and radiation r Suprapubic pain Typically seen in immunocompromised patients
therapy induced. r Fevers with chills after BMT
r Previous history of cyclophosphamide therapy, pelvic May present dramatically, but usually resolves
EPIDEMIOLOGY
radiation, bone marrow transplant spontaneously in <2 wk
Incidence r Other infections rarely cause severe HC:
r Cyclophosphamide-induced HC: 57%
PHYSICAL EXAM Bacterial: Escherichia coli, Staphylococcus
r Radiation-induced HC: 1015% in patients with r Suprapubic pain/mass: Distended bladder, infected
saprophyticus, Proteus, Klebsiella, Mycobacterium
history of pelvic radiation. and/or clot-filled bladder tuberculosis
r 770% of hematopoietic stem cell transplants. r Signs and symptoms of hypovolemia, hemorrhagic
Fungal: Candida, Aspergillus, Cryptococcus,
RISK FACTORS shock, or anemia if severe Torulopsis
r No age, sex, or race predilection. r Ocular infections: Common with adenovirus Parasitic: Schistosoma haematobium,
r Infections. infection Echinococcus granulosus
r Exposure to certain industrial chemicals, such as r Large hypertrophied tongue: Amyloidosis r Systemic hematologic disease: Rare; often refractory
aniline or toluidine derivatives. DIAGNOSTIC TESTS & INTERPRETATION to fulguration and irrigation
r Previous treatment with oxazaphosphorine r Systemic amyloidosis associated with rheumatoid
Lab
alkylating agents (for lymphoproliferative disorders, r Urine for analysis, cytology, and cultures (including arthritis or Crohn disease
r Chemical toxins:
solid tumors, collagen diseases) such as fungal and viral cultures, if indicated)
cyclophosphamide, isophosphamide. r Coagulation factors (especially platelets, which can Anilines, toluidines, and chlordimeform are
r History of prior pelvic radiation (prostate and be depleted) common industrial exposures (dyes, pesticides).
cervical cancers). r Serial hematocrits Overdoses of methenamine mandelate; accidental
r Reactivation of BK virus (BKV) infection in bone r Serum creatinine urethral instillation of gentian violet douche or
nonoxynol-9 contraceptive
marrow transplant patients. r Blood tests for collagen disease markers, if indicated
Thiotepa and acetic acid intravesically
Genetics Imaging r Medications:
N/A r CT urogram: Penicillin, piperacillin, methicillin, carbenicillin,
PATHOPHYSIOLOGY Often done as part of hematuria workup danazol, bleomycin, allopurinol, busulfan
r Cyclophosphamide: Acrolein enters the urethelium. Rules out other urologic abnormalities r Prolonged high-altitude travel (Boon disease)
Activates platelet-activating factor, nitric oxide, Usually not able to diagnose HC, but may show r Carcinomas of the urinary tract
tumor necrosis factor-, and IL-1, eventually clots in the lumen, a thickened irregular bladder r Acute UTIs
forming peroxynitrite that causes damage. wall, and/or small capacity. r Benign prostatic hypertrophy
r Radiation-induced cystitis results from a progressive Diagnostic Procedures/Surgery r Trauma to the urinary system
obliterative endarteritis leading to mucosal r Cystoscopy biopsy, clot evacuation r Arteriovenous malformation, vascular fistulae
ischemia, ulceration, and neovascularity. r Consider electro- or laser fulguration if focal
r Penicillin toxicity is immune-mediated, whereas bleeding visualized.
danazol toxicity is likely from damaging vascular
Pathologic Findings
TREATMENT
changes. r Urothelial damage: Edema, necrosis, ulceration,
GENERAL MEASURES
ASSOCIATED CONDITIONS hemorrhage, leukocyte infiltration, and r Catheterization/bladder irrigation with normal saline
See Differential Diagnosis. neovascularization to clear bleeding and evacuate clots
r May reveal amyloid deposits; eosinophilic r Remove the offending toxin.
GENERAL PREVENTION
r Patients treated with cyclophosphamide once had a inflammatory response of schistosomiasis; IgG, IgM, r Treat the infectious agent.
very high incidence of HC (70%), with high and C3 depositions; penicillin toxicity; whitish r Hydration and diuresis
mortality rates (as high as 75%) if it became severe pseudomembranes or plaques of fungal infections; r Blood products transfusion, when necessary
inclusion bodies of viral infections
r IV hydration, frequent bladder emptying, and
DIFFERENTIAL DIAGNOSIS MEDICATION
sometimes indwelling catheters with bladder r Oxazaphosphorine agents (cyclophosphamide and First Line
irrigations are used to reduce the time toxins are in r Alum irrigation often considered 1st line:
contact with the bladder wall (1)[A] isophosphamide):
Most common cause of severe HC Astringent, forms precipitates over bleeding
r Mercaptoethane sulfonate Na (MESNA) and surface
Acrolein, a liver metabolite of the agents, is the
N-acetylcysteine (Mucomyst) bind to acrolein, toxin believed to be directly implicated. 14% solution at 3001,000 mL/h
creating nontoxic compounds. Higher dosages, IV route of administration (vs. No need for anesthesia
r WF-10 (2)[A], sodium pentosan polysulfate oral), and increased contact time between the Adverse effects: Spasms, precipitation and
(Elmiron), and amifostine (Ethyol) have been bladder wall and the acrolein (because of clogged catheters, rare encephalopathy from
investigated in prevention of radiation-induced dehydration and/or infrequent emptying) all aluminum toxicity
cystitis. worsen HC.
r Infectious: Minimize bacterial exposure, avoid
indwelling Foley catheter if possible; intermittent
catheterization if prolonged catheter needed
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CYSTOCELE
Alana M. Murphy, MD
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CYSTOCELE
SURGERY/OTHER PROCEDURES
r Preoperative preparation:
ADDITIONAL READING
ONGOING CARE r Chow D, Rodriguez LV. Epidemiology and
Optional hormone replacement with topical
estrogen PROGNOSIS prevalence of pelvic organ prolapse. Curr Opin Urol.
r Perioperative factors: r Recurrence rates as high as 3070% 2013;23:293298.
r Close to 30% of women will require reoperation for r Walters MD. Surgical correction of anterior vaginal
Single dose of preoperative antibiotics
DVT prophylaxis with sequential compression symptomatic POP (1) wall prolapse. In: Walters MD, Karram MM, eds.
devices COMPLICATIONS Urogynecology and Reconstructive Pelvic Surgery,
Optional vaginal packing
Optional temporary urethral catheterization
r Bladder injury
r Ureteral injury/obstruction
3rd ed. Philadelphia, PA: Mosby Elsevier; 2007.
C
Consider in the setting of a multicompartment
See Also (Topic, Algorithm, Media)
r Bleeding r Baden-Walker Staging
repair with or without an anti-incontinence r Dyspareunia r Cystocele, Grading
procedure r Cystocoele Enterocele Algorithm
r Transvaginal vs. transabdominal repair: r de novo stress UI
r Recurrent cystocele r Cystocele Image
Transvaginal repair:
Central defect repair: Plication of pubocervical r Pelvic Organ Prolapse (Cystocele and Enterocoele)
FOLLOW-UP r Pelvic Organ Prolapse Quantification System (POP-Q)
fascia in the midline with horizontal mattress
Patient Monitoring r Rectocele, Urologic Considerations
sutures
Lateral defect repair: Reattachment of the Evaluation for recurrent POP should largely be based r Vaginal Mesh Erosion
on symptoms or clinical signs (elevated PVR, urinary r Vaginal Pessaries, Urologic Considerations
endopelvic fascia to the ATFP
retention, recurrent UTIs)
Transvaginal mesh grafts provide a superior r Vaginal Prolapse
Patient Resources
anatomic outcome but are associated with r American Urogynecologic Society.
higher complication rates (5)[A]
http://www.voicesforpfd.org/p/cm/ld/fid=6
Transabdominal repair: r International Urogynecological Association. CODES
Only repair lateral defects www.iuga.org/resource/resmgr/Brochures/eng
r Closure of the vagina (colpocleisis): ICD9
pop.pdf r 618.01 Cystocele, midline
Excellent option for geriatric women who no
longer desire the ability to maintain sexual activity r 618.02 Cystocele, lateral
r Perform a simultaneous repair of all POP defects and REFERENCES
an anti-incontinence procedure for demonstrable ICD10
1. Olsen AL, Smith VJ, Bergstrom JO, et al. r N81.10 Cystocele, unspecified
stress UI r N81.11 Cystocele, midline
r Consider a prophylactic concomitant Epidemiology of surgically managed pelvic organ
prolapse and urinary incontinence. Obstet Gynecol. r N81.12 Cystocele, lateral
anti-incontinence procedure in patients with stage
1997;89:501506.
3 cystocele and/or history of stress UI
2. Swift SE. The distribution of pelvic organ support in
ADDITIONAL TREATMENT a population of female subjects seen for routine CLINICAL/SURGICAL
Radiation Therapy gynecologic healthcare. Am J Obstet Gynecol. PEARLS
N/A 2000;183:277285.
r Management of a cystocele should largely be based
Additional Therapies 3. Nygaard I, Barber MD, Burgio KL, et al. Prevalence
r Observation: of symptomatic pelvic floor disorders in US women. on patient preference and symptoms.
JAMA. 2008;300:13111316. r Always suspect concomitant apical prolapse in the
Appropriate if a patient is not symptomatic
4. Braekken IH, Majida M, Engh ME, et al. Can pelvic setting of stage 3 cystocele or a recurrent
r Pelvic floor exercises (Kegel exercises) (4)[B] cystocele.
floor muscle training reverse pelvic organ prolapse
r Vaginal pessary: and reduce prolapse symptoms? An r Mesh grafts for cystocele repair provide a superior
Good option for poor surgical candidates assessor-blinded, randomized, controlled trial. Am J anatomic outcome but they are associated with
May be used as a temporary solution Obstet Gynecol. 2010;203:e1e7. higher complication rates.
Risk of vaginal discharge, vaginal ulceration, 5. Altman D, Vayrynen T, Engh ME, et al. Anterior
vesicovaginal and rectovaginal fistula formation colporrhaphy versus transvaginal mesh for
Complementary & Alternative pelvic-organ prolapse. N Engl J Med. 2011;364:
Therapies 18261836.
N/A
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DETRUSOR OVERACTIVITY
Lysanne Campeau, MD, CM, PhD, FRCSC
Victor W. Nitti, MD, FACS
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DETRUSOR OVERACTIVITY
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r Mullerian remnants
Imaging
r Abdominal/Pelvic ultrasound (utero presence)
ADDITIONAL READING
Small asymptomatics are managed conservatively
r Cystogram/genitogram (visualization of vagina, Symptomatic remnants are treated surgically r Auchus RJ, Chang AY. 46,XX DSD: the masculinised
sinus) (endoscopic incision or unroofing, laparoscopic/ female. Best Pract Res Clin Endocrinol Metab.
r MRI robotic excision) 2010;24:219242.
r Barbaro M, Wedell A, Nordenstrom A. Disorders of
Diagnostic Procedures/Surgery ADDITIONAL TREATMENT
r Laparoscopy to define internal anatomy sex development. Semin Fetal Neonatal Med.
Radiation Therapy 2011;16:119127.
r Cysto/vaginoscopy to confirm anatomy and level of N/A r Barthold JS. Disorders of sex differentiation: a
confluence of urogenital sinus Additional Therapies pediatric urologists perspective of new terminology
r Gonadal biopsy to analyze presence of ovarian
N/A and recommendations. J Urol. 2011;185:393400.
and/or testicular tissue r http://www.ncbi.nlm.nih.gov/omim/ (Online
r Skin biopsy to obtain cellular lines Complementary & Alternative
Therapies Mendelian Inheritance in Man )
Pathologic Findings Some patient groups strongly advocate to delay any
Identification of ovarian tissue, testicular tissue, surgical procedures until patients are competent to
See Also (Topic, Algorithm, Media)
r Androgen Insensitivity Syndrome (AIS; OR Androgen D
ovotestes, or streak gonads according to related provide informed consensus Resistance Syndrome), Complete (CAIS) and Partial
specific disorders (PAIS)
DIFFERENTIAL DIAGNOSIS r Congenital Adrenal Hyperplasia
r Hypopituitarism ONGOING CARE r Disorders of Sexual Development (DSD) Image
r Hypospadias PROGNOSIS r Disorders of Sexual Development (DSD) Algorithm
r Hydrocele and hernia Many patients can remain fertile (CAH, some r Mullerian Duct Remnants and Persistent Mullerian
r Menstruation disorders ovotesticular DSD, XY DSD 5-RD) Duct Syndrome (PMDS)
r Microphallus r Pseudohermaphroditism, Male and Female
COMPLICATIONS
r Gonadoblastoma r Acute adrenal insufficiency in CAH not adequately
treated
TREATMENT
r Damages to clitoral innervation (clitoroplasty) CODES
r Stenosis of the vaginal introitus (vaginoplasty)
GENERAL MEASURES r Meatal stenosis, fistula (hypospadias repair) ICD9
r Gender assignment avoiding hasty decision r Rectal injury (urogenital sinus mobilization) r 255.2 Adrenogenital disorders
r Expert evaluation by an experienced r 259.50 Androgen insensitivity, unspecified
FOLLOW-UP r 752.7 Indeterminate sex and
multidisciplinary team
Patient Monitoring pseudohermaphroditism
MEDICATION r Sexual function (adequate vaginal introitus,
First Line adequate penis reconstruction) ICD10
r Newborn with salt-wasting CAH r Risk of gonadoblastoma in gonadal dysgenesis is r E25.0 Congenital adrenogenital disorders assoc w
Fluid and electrolytes replacement 12% (occurrence of neoplasia is primarily associated enzyme deficiency
Glucocorticoid and mineralocorticoid replacement with the Y chromosome containing karyotypes) r E34.50 Androgen insensitivity syndrome, unspecified
Hydrocortisone 10 mg/m2 /d r Lifelong psychosocial support mandatory for all r Q56.4 Indeterminate sex, unspecified
Fludrocortisone 0.10.2 mg/d patients with DSD
Oral sodium chloride, 12 g/d added to formula
or breast milk Patient Resources CLINICAL/SURGICAL
r http://www.hopkinschildrens.org (all DSDs)
Second Line r http://www.accordalliance.org (all DSDs) PEARLS
N/A r http://www.isna.org (all DSDs) r DSD should be managed by a specialized
SURGERY/OTHER PROCEDURES r http://www.caresfoundation.org (CAH) multidisciplinary team.
r Masculinizing genitoplasty (between the ages of 6 r http://www.ahn.org.uk (CAH) r Gender assignment should be made after thorough
and 18 mo) r http://heainfo.org (hypospadias and epispadias) investigation by the team.
Hormonal treatment with testosterone r http://www.aisdsd.org (androgen insensitivity DSD) r DSD is a heterogeneous group of conditions with
preparation to stimulate phallus different underlying molecular causes. Many disease
Surgical excision of mullerian structures genes remain to be identified.
Phalloplasty (hypospadias repair, chordee REFERENCES r Infants with a DSD and who present with truly
correction, scrotal transposition)
ambiguous genitalia are a rare occurrence.
Orchidopexy 1. Hughes IA, Houk C, Ahmed SF, et al. Consensus
r Feminizing genitoplasty (during the 1st 6 mo of life) statement on management of intersex disorders.
(3) Arch Dis Child. 2006;91:554563.
Clitoroplasty preserving innervation to reduce the 2. Ahmed SF, Rodie M. Investigation and initial
size of the gland and shaft management of ambiguous genitalia. Best Pract
Vaginoplasty and labioplasty to separate vagina Res Clin Endocrinol Metab. 2010;24:197218.
and urethra from the common urogenital sinus 3. Romao RL, Salle JL, Wherrett DK. Update on the
r Gonads management of disorders of sex development.
46XX DSD: Normal ovaries, no treatment Pediatr Clin North Am. 2012;59:853869.
necessary
46XY DSD:
Female gender assigned: Orchiectomy (timing is
subject of debate)
Male gender assigned: Orchidopexy
Ovotesticular DSD:
Gonadal biopsy
Excision of dysgenetic gonads (streak)
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ASSOCIATED CONDITIONS
BASICS r Chronic pelvic pain syndrome (NIH category III
TREATMENT
prostatitis)
DESCRIPTION r Erectile dysfunction (1) GENERAL MEASURES
r Dysorgasmia specifically refers to pain that occurs r Prostate cancer Reassurance that the condition is most often
immediately preceding, at or immediately following self-limiting
orgasm. GENERAL PREVENTION
r The pain is usually located in the penis or testicles None known MEDICATION
but may be present in the lower abdomen, groin, First Line
r -Blockers (daily initially; if successful attempt
perineum, or elsewhere.
r The severity of pain ranges from mild and of
DIAGNOSIS on-demand) (2).
r Up to 70% of men using -blockers will have
nuisance value to crippling and may last seconds to HISTORY
hours after orgasm. r Medical history significant improvement in pain.
r The condition is best identified and studied in the r Focusing on assessment of orgasmic pain location, r Side effects include syncope, orthostasis, retrograde
postradical prostatectomy setting. severity and duration. ejaculation, asthenia, and nasal congestion
r Ejaculatory pain may be seen in other conditions r Prior history of radical prostatectomy, radiation Alfuzosin 10 mg/d
therapy, or CPPS. Doxazosin start 1 mg/d to max 8 mg
such as chronic prostatitis/chronic pelvic pain
Silodosin 8 mg/d
syndrome (CP/CPPS), or NIH category III prostatitis, PHYSICAL EXAM Tamsulosin start 0.4 mg to max 0.8 mg
and is discussed in Section II (Ejaculation, painful). r General physical exam
Terazosin start 1 mg/d to max 20 mg
EPIDEMIOLOGY r Genital exam (although often there are no specific
Second Line
Incidence findings) r Centrally acting pain relievers
r The most frequent correlate of dysorgasmia is r Optimum dose and duration not established
DIAGNOSTIC TESTS & INTERPRETATION
radical prostatectomy and this condition occurs in r Gabapentin
Lab
about 1015% of patients.
r In this population, the pain is usually self-limiting None are useful 900 to 1,800 mg/d and given in divided doses
Imaging (3 times a day) using 300 or 400 mg capsules
with most sufferers experiencing complete r Pregabalin
resolution by 2 yr postoperatively None are useful
Begin dosing at 150 mg/d, increase to 300 mg/d
Prevalence Diagnostic Procedures/Surgery within 1 wk. Maximum dose of 600 mg/d
N/A None
SURGERY/OTHER PROCEDURES
RISK FACTORS Pathologic Findings Case reports exist of excision of retained seminal
r Radical prostatectomy None vesicle following radical prostatectomy with relief of
r Prostate radiation DIFFERENTIAL DIAGNOSIS symptoms (3)
r Chronic pelvic pain syndrome (CPPS) r Penile pain:
ADDITIONAL TREATMENT
Genetics Penile compressive neuropathy
Penile trauma Radiation Therapy
None known N/A
Peyronie disease
PATHOPHYSIOLOGY Sexually transmitted infection (STI) Additional Therapies
r While unproven one of the postulated mechanisms Ureteral stone N/A
is that the pain is related to pelvic floor or bladder r Testicular pain:
Complementary & Alternative
neck spasm. Epididymitis Therapies
This is the rationale for the use of -blockers. Orchitis
r Dysorgasmia decreases in frequency and degree N/A
Testicular tumor
over time after RP. Trauma
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DYSPAREUNIA, FEMALE
Bradley C. Gill, MD, MS
Sandip P. Vasavada, MD, FACS
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DYSPAREUNIA, FEMALE
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DYSURIA
Mohamed S. Ismail, MBChB, MRCS, PhD
Francis Xavier Keeley, Jr., MD, FRCS
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DYSURIA
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DIFFERENTIAL DIAGNOSIS
r Acute idiopathic scrotal edema
ADDITIONAL TREATMENT ADDITIONAL READING
Radiation Therapy
r Angioedema of the genital skin While this can be a cause of genital lymphedema, it Rabinowitz R, Hulbert WC Jr. Acute scrotal swelling.
r Cellulitis may have a role in primary palliative treatment of Urol Clin N Am. 1995:22(1):101105.
r Chemical or allergic dermatitis prostate, penile, and retroperitoneal malignancies See Also (Topic, Algorithm, Media)
r Elephantiasis causing scrotal edema. r Edema, Lower Extremity, Urologic Considerations
r Epididymo-orchitis Additional Therapies r Fournier Gangrene
r Fournier gangrene Supportive undergarments/briefs for patient comfort r Testicular Torsion
r Hydrocele r Paraphimosis
Complementary & Alternative
r Idiopathic scrotal edema (usually children) r Edema, External Genitalia (Lymphedema,
Therapies
r Inguinal hernia N/A Peno-Scrotal Edema) Image
r Paraphimosis
r Retroperitoneal mass
r Squamous carcinoma of the penis
ONGOING CARE CODES
r Testicular torsion PROGNOSIS
r Varicocele Depends on etiology ICD9
r 605 Redundant prepuce and phimosis
COMPLICATIONS r 607.83 Edema of penis
r Skin breakdown/ulceration
TREATMENT r Urinary retention/difficulty voiding r 608.86 Edema of male genital organs E
GENERAL MEASURES r Genital and scrotal compression is NOT
r Scrotal elevation
ICD10
recommended r N47.1 Phimosis
r Genital or scrotal compression NOT recommended r N48.89 Other specified disorders of penis
r Meticulous care of skin breakdown FOLLOW-UP
r N50.8 Other specified disorders of male genital
r Correction of hypervolemia Patient Monitoring
r Physical exam for resolution organs
r Dialysis if due to severe hypervolemia r Monitor underlying condition, appropriate labs,
r Evaluate for urinary retentionFoley catheter if
nutritional status CLINICAL/SURGICAL
indicated
r Immediate postoperative edema usually resolves Patient Resources PEARLS
Sterns RH. Patient information: Edema (swelling)
spontaneously r Determine the patients fluid status to rule out
(Beyond the Basics). In: UpToDate, Basow DS, ed.
MEDICATION UpToDate. Wolters Kluwer, Philadelphia hypervolemia as the cause of genital edema.
First Line (www.uptodate.com, accessed August 8, 2014). r Acute scrotal pain, swelling, lack of cremasteric
r Limited utility reflex, and a high-riding ipsilateral testis could
r Diuretics may be of some utility indicate testicular torsion.
r Chemotherapy for lymphoma
REFERENCES r Evaluate for paraphimosis in uncircumcised males.
1. Lachiewicz AM, Dawson DV. Do young boys with r Crepitance, induration, necrosis, and foul odor
Second Line
N/A fragile X syndrome have macroorchidism? suggest Fournier gangrene and require emergent
Pediatrics. 1994;93:992995. surgical debridement.
SURGERY/OTHER PROCEDURES 2. Sterns RH. Pathophysiology and etiology of edema r Complications of edema may include urinary
r Indicated to address the etiologic process: Testicular
in adults. In: UpToDate, Basow DS, ed. UpToDate. retention and skin breakdownthese should be
torsion, inguinal hernia, penile fracture, or Fournier Wolters Kluwer, Philadelphia (www.uptodate.com, evaluated for and treated accordingly.
gangrene accessed August 8, 2014).
r Manual reduction of foreskin or dorsal slit if
3. Weinberger LN, Zirwas MJ, English JC 3rd. A
necessary to address paraphimosis diagnostic algorithm for male genital oedema. J Eur
r Rarely, radical excision with gracilis flap may be Acad Dermatol Venereol. 2007;21(2):156162.
required for severe refractory cases
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PATHOPHYSIOLOGY
BASICS r Normal ejaculation: DIAGNOSIS
Central control in multiple brain regions
DESCRIPTION Can promote or inhibit ejaculation HISTORY
r Anorgasmia or delayed orgasm/ejaculation r Duration of symptoms
Sympathetic (T12L3):
Difficulty/inability to reach orgasm Hypogastric nerve (thoracolumbar) r No defined criteria for diagnosis of delayed
r Low volume ejaculate Seminal emission by contraction of epididymis, ejaculation
Suspect if <1.5 cc ejaculate volume vas deferens/ampulla, seminal vesicle (SV), and Mostly normal men ejaculate after 410 min of
r Aspermia prostate smooth muscle penetration
Orgasm with zero ejaculate volume Bladder neck closure preventing retrograde Presence of significant distress to patient or
r Retrograde ejaculation ejaculation partner important to diagnosis
Parasympathetic (S2S4): r Presence or absence of orgasm
Sperm seen in post-ejaculatory urine
r Ejaculatory duct obstruction (EDO) Pelvic nerve r Perceived ejaculate volume
Gland secretions of prostate SV r Sources of stress/psychological disturbance
Congenital, acquired, iatrogenic
r Failure of emissions Somatic (S2S4): r Past medical history
Pudendal nerve r Retroperitoneal and genitourinary operations
Can also cause low/zero volume Efferents from sacral cord r Family history of cystic fibrosis
EPIDEMIOLOGY Contraction of bulbocavernosal and
See vas deferens, congenital absence
Incidence ischiocavernosal muscles r Medications:
r Increased in aging (age 5080 yr) men with Relaxation of external urethral sphincter
Projectile expulsion of ejaculate Antidepressants/antipsychotics
BPH/LUTS (1)[B] Sensory Bladder outlet medications
46% decreased ejaculation Pudendal nerve Antihypertensives (clonidine)
5% anejaculation Tactile stimulation of penis can activate Methyldopa
r Men on tamsulosin 0.8 mg ejaculatory reflex PHYSICAL EXAM
r Anorgasmia/Delayed orgasm r Absence or diminished development of epididymides
90% report decreased ejaculatory volume
(2)[B] Hypogonadism and vasa deferentia
r Selective serotonin reuptake inhibitors (SSRIs) Medication side effect Congenital bilateral or unilateral absence of the
Psychological/Psychiatric (depression) vas deferens (CBAVD/CUAVD)
1637% delayed or difficult orgasm r Retrograde ejaculation r Enlarged SV
r Anorgasmia is rare:
Damage to ejaculatory nerves/reflexes EDO
0.140.4% in general population r Hypospadias or epispadias
Bladder neck surgery or dysfunction
Prevalence Medications affecting bladder neck Hypogonadism
N/A r Low volume ejaculate
DIAGNOSTIC TESTS & INTERPRETATION
RISK FACTORS Poor development/absence of accessory sex
r Age organs Lab
r Semen analysis
r Benign prostatic hyperplasia Retrograde ejaculation or functional problem
Medications affecting accessory glands Volume: Suspect if ejaculate volume <1.5 cc
r Lower urinary tract symptoms
Decreased prostate and SV secretions seen in Concentration: Low volume azoospermia
r Prostatitis/Ejaculatory duct stones suspicious for EDO
r Depression and related medications hypogonadism
r Ejaculation requires intact, properly developed, and Absence of seminal fructose suggests EDO
r Hypogonadism r Post-ejaculatory urinalysis (PEU): >1015
coordinated accessory sex organs, nerves, and
r Hypertension medications sperm/HPF demonstrates retrograde ejaculation
muscles
r Prostate/Urethral /Bladder neck surgery Congenital, acquired, iatrogenic, infectious, Imaging
r Retroperitoneal lymph node dissection (RPLND) inflammatory causes can all prevent normal r Transrectal ultrasound (TRUS)
r Cystic fibrosis ejaculation Usually done for low volume azoospermia
r Neurologic conditions/Diabetes Functional causes may lead to the complaint of For patients with negative PEU
Multiple sclerosis, spinal cord injury (SCI), spina decreased force of ejaculate Normal SV A-P diameter <1.5 cm
bifida, diabetes Ejaculate volume commonly decreases r MRI
r Rectal surgery by 0.03 mL each year with advanced age Can help identify structural abnormalities
r Radiation therapy ASSOCIATED CONDITIONS
r Psychological/Psychiatric conditions
Genetics r See Risk Factors
N/A
GENERAL PREVENTION
r Avoidance of bladder neck procedures
Transurethral prostate, bladder neck surgery
r Avoidance/decreased use of medications
SSRI, blockers, 5-reductase inhibitors
r Nerve sparing at time of RPLND
r Strict diabetic control
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ENURESIS, ADULT
Katie S. Murray, DO
Tomas L. Griebling, MD, MPH, FACS
DIFFERENTIAL DIAGNOSIS
BASICS DIAGNOSIS r Obstructive sleep apnea
r Anxiety or psychological disorders
DESCRIPTION HISTORY r Anatomic abnormalities
r Enuresis is repeated inability to control urine r Have never achieved nocturnal continence of urine
r Idiopathic detrusor instability
Primary: Starts in childhood and never resolves r Nonspecific urinary symptoms
r Neurologic disorders
and continues into adulthood r Ask about known or potential medical history
Secondary: New onset in adulthood r Complete surgical and trauma/incident history
r Nocturnal enuresis (NE) is involuntary urination r Obtain record of fluid intake habits TREATMENT
while asleep after the age at which bladder control r Review medications and times of administration
usually occurs r Voiding diaries to evaluate frequency, volume, and GENERAL MEASURES
r Conservative measures have varying success rates
EPIDEMIOLOGY patterns
r International prostate symptom score (IPSS) in men r Education is key when attempting to improve
2.3% of adult population affected (1)[A] enuresis without medical therapy
RISK FACTORS PHYSICAL EXAM r Timed voiding
r Family history of NE r Full urologic exam (pelvic exam in women and DRE r Complete bladder emptying
If both parents have NE, children have 80% in men) r If associated with BPH in men management with
r Full neurologic exam
chance -blockers for 5-reductase inhibitors
DIAGNOSTIC TESTS & INTERPRETATION r Avoidance of caffeine and alcohol
Genetics
r Possibly hereditary Lab r Adjust timing of fluid intake
r Related to site on chromosome 13 r Urinalysis and urine culture: Rule out urinary tract Restrict fluid intake in evening to reduce urine
infection, hematuria, proteinuria, glycosuria output at night
PATHOPHYSIOLOGY r Creatinine: Rule out renal insufficiency Take diuretic medications early in a day
r Unknown in most situations
r Urine cytology (if other symptoms such as irritative
r Recognized hypotheses MEDICATION
voiding symptoms make carcinoma a concern) First Line
Obstructive sleep apnea causing diminished
vasopressin secretion Imaging r If due to prostatic hypertrophy: See Section I
Disturbance in sensation, cortical arousal, or r Post-void residual bladder scan Bladder Outlet Obstruction (BOO).
urinary sphincter function r Renal/ureteral imaging to evaluate for abnormalities r Antimuscarinics or 3-agonists (3)[B]
Decreased bladder capacity initiating involuntary such as ectopic ureters Inhibit the effect of acetylcholine at postjunc-
voiding reflex CT urogram tional muscarinic receptors on detrusor muscle
Nocturnal polyuria because vasopressin secretion Renal ultrasound cells
or reduction in renal sensitivity to the antidiuretic Diagnostic Procedures/Surgery 3-Adrenergic agonist promotes detrusor
(2)[B] r Bladder diaries/frequencyvolume charts muscle relaxation
r Cystoscopy with retrograde pyelograms to evaluate Varying results (540%), depends on whether
Detrusor instability during filling phase
Urine production increased in recumbent position bladder and ureters detrusor instability is root cause of enuresis
in patients with peripheral edema or congestive r Urodynamic testing (3)[B] Side effects: Dry mouth, constipation, blurred
heart failure vision, confusion
Identify anatomical urethral abnormalities r Antimuscarinics
r Normal physiology decreases nighttime, relative to
Identify anatomical bladder abnormalities
daytime, urinary output. Excess production of urine Evaluate bladder function for possible neurogenic Tolterodine (24 mg/d)
at night, in the setting of a normal 24-hr urine bladder findings Trospium XR (60 mg/d)
output, is termed nocturnal polyuria May find abnormalities in up to 90% of patients Darifenacin (7.515 mg/d)
Nocturnal polyuria is nighttime excretion of (4)[B] Solifenacin(510 mg/d)
>35% of a 24-hr urine volume r Consider sleep medicine consultation and/or Oxybutynin (IR 7.520 mg/d, XL 530 mg/d,
patch twice weekly)
ASSOCIATED CONDITIONS polysomnography if clinical concern for sleep apnea Fesoterodine (48 mg/d)
r Benign prostatic hypertrophy
Pathologic Findings r 3-adrenergic agonist
r Daytime urinary incontinence
N/A Mirabegron (2550 mg/d)
r Psychological disorders including depression
r Sleep apnea
GENERAL PREVENTION
r Timed voiding
r Complete bladder emptying
r Avoidance of caffeine and alcohol
r Adjust timing of fluid intake
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ENURESIS, ADULT
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ENURESIS, PEDIATRIC
Ellen Shapiro, MD, FACS
Daniel A. Wollin, MD
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ENURESIS, PEDIATRIC
ALERT
COMPLICATIONS
r Recurrent UTI
CODES
Imipramine overdose can result in seizure, r Persistence of incontinence and LUTSrequires
hypotension, coma, and fatal arrhythmias; may ICD9
further investigation with VUDs and MRI r 307.6 Enuresis
prolong QT interval. r Persistence of enuresis into adulthood (23%) r 788.30 Urinary incontinence, unspecified
r Oxybutynin (anticholinergic) for NMNE: r Social consequences/withdrawal r 788.36 Nocturnal enuresis
2.55 mg BIDQID (0.2 mg/kg/dose) when PVR
negligible FOLLOW-UP
ICD10
Available in long-acting form (510 mg/d) Patient Monitoring r F98.0 Enuresis not due to a substance or known
r Children with history of UTI or organic causes of
Success primarily when the medication is used physiol condition
with a well-organized treatment program enuresis should be followed for the specific condition r N39.44 Nocturnal enuresis
r Monitor closely while on medication to treat the r R32 Unspecified urinary incontinence
including voiding 1st thing in the morning,
timed voiding during the day, and regular bowel enuresis (PVR and urinalysis)
habits. Patient Resources
Patients should be seen in 46 wk for evaluation International Childrens Continence Society. CLINICAL/SURGICAL
including urinalysis and PVR. If elevated PVR, http://i-c-c-s.org/parents/ PEARLS
lower the dose and institute double voiding.
r Tolterodine (anticholinergic) for polysymptomatic The primary therapy for all children with NE should be
or daytime incontinence: initial behavioral management before relying on
medications.
12 mg BID. Also available in long-acting form
(24 mg/d)
More success when the medication is used with
a well-organized treatment program
r Low-dose prophylactic antibiotics for NMNE:
Helpful for children with recurrent UTI or
bacteriuria with LUTS and voiding dysfunction
Nitrofurantoin recommended 12 mg/kg QHS
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ADDITIONAL READING
TREATMENT ONGOING CARE
Rubenstein RA, Dogra VS, Seftel AD, et al. Benign
GENERAL MEASURES PROGNOSIS intrascrotal lesions. J Urol. 2004;171(5):17651772.
r As most epididymal lesions are benign, observation r Adenomatoid tumors
Benign, excellent prognosis See Also (Topic, Algorithm, Media)
for asymptomatic cystic lesions r Adenomatoid Tumors, Testicular and Paratesticular
r Epididymitis r Rhabdomyosarcoma
r Epididymal Cystadenoma/Papillary Cystadenoma
Consider sexually transmitted infection as source In children with low stage disease survival r Epididymis, Mass (Epididymal Tumor and Cysts)
in young men and treat accordingly (See Sexually may be as high as 90%. Worst stage (IV), Images
Transmitted Infections [STIs] (Sexually Transmitted survival is 5.2% (4)[A] r Epididymis, Metastasis to
Diseases [STDs]), General) r Epididymitis
Older men more likely to be infected by enteric COMPLICATIONS
r Untreated epididymitis can cause severe systemic r Hydrocele
organisms Escherichia coli, other coliforms, and
Pseudomonas illness. r Paratesticular tumors
More advanced infections can present with r Scrotum and Testicle, Mass
MEDICATION testicular swelling and pain (epididymo-orchitis). r Sexually Transmitted Infections (STIs) (Sexually
First Line r If radiation or chemotherapy needed:
Transmitted Diseases [STDs]), General
r Epididymitis (3)[A] infertility, higher risk for secondary neoplasms r Sperm Granuloma
<35 year old: Consider gonorrhea and including lymphoma, leukemia, soft tissue r Spermatocele
sarcomas
chlamydia
Ceftriaxone IM 500 mg 1 AND
r Von HippelLindau Disease E
FOLLOW-UP
Azithromycin 1 g PO 1 Patient Monitoring
>35 year old: Enteric organisms r Oncologic follow-up if malignant disease
Levofloxacin 500 mg PO daily 10 days r Teach patient testicular self-exam
CODES
r TB: Treat according to current CDC guidelines
Patient Resources ICD9
(http://www.cdc.gov/tb/) r 222.3 Benign neoplasm of epididymis
National Cancer Institute. http://www.cancer.gov/
Second Line cancertopics/pdq/treatment/childrhabdomyosarcoma/ r 608.89 Other specified disorders of male genital
r Epididymitis (3)[A] Patient organs
Doxycycline 100 mg PO BID 10 days in lieu of ICD10
azithromycin REFERENCES r D29.30 Benign neoplasm of unspecified epididymis
SURGERY/OTHER PROCEDURES r D29.31 Benign neoplasm of right epididymis
r Excision of suspicious lesion via inguinal approach 1. Montgomery JS, Blood DA. The diagnosis and r N50.8 Other specified disorders of male genital
r Frozen section management of scrotal masses. Med Clin North
Am. 2011;95(1):235244. organs
r If positive for malignancy, radical orchiectomy
r Further surgical therapy guided by pathology but 2. Grimsby GM, Ritchey ML. Pediatric urologic
oncology. Pediatr Clin North Am. 2012;59(4): CLINICAL/SURGICAL
may include retroperitoneal lymph node dissection if 947959.
rhabdomyosarcoma 3. Workowski KA1, Berman S; Centers for Disease
PEARLS
ADDITIONAL TREATMENT Control and Prevention. Sexually transmitted r Most epididymal lesions are benign and should be
Radiation Therapy diseases treatment guidelines, 2010. MMWR. followed serially.
Use of radiation for local control of 2010;59(No. RR-12):1110. r Treatment for epididymitis is guided by risk of STIs
rhabdomyosarcoma in young patient is controversial 4. Oberlin O, Rey A, Sanchez de Toledo J, et al. as a source.
Randomized comparison of intensified six-drug r Ultrasound is important to delineate a testicular vs.
Additional Therapies versus standard three-drug chemotherapy for
r Chemotherapy paratesticular origin of the mass.
high-risk nonmetastatic rhabdomyosarcoma and r Ultrasound cannot reliably differentiate malignant
Vincristine, cyclophosphamide, dactinomycin other chemotherapy-sensitive childhood soft tissue
may have a role in rhabdomyosarcoma solid tumors from benign tumors.
sarcomas: Long-term results from the international r Rhabdomyosarcoma predominantly occurs in
depending on extent of disease and oncologist society of pediatric oncology MMT95 study. J Clin
recommendations (4)[A] Oncol. 2012;30(20):24572465. children.
Complementary & Alternative
Therapies
N/A
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EPIDIDYMITIS
Jonathan H. Huang, MD
Wayland Hsiao, MD
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EPIDIDYMITIS
r Epididymitis due to TB
Diagnostic Procedures/Surgery
r Testicular exploration
REFERENCES
Systemic antibiotics based on most current CDC
Not used as 1st-line diagnostic procedure guidelines or local guidelines if available local 1. Centers for Disease Control and Prevention.
Used when clinical suspicion for testicular torsion guidelines Epididymitis. MMWR. 2010;59(No. RR-12):6769.
is high r Epididymitis due to intravesical BCG 2. Hori S, Sengupta A, Shukla CJ, et al. Long-term
r In infants and children with epididymitis, up to 75% Fluoroquinolone (eg, levofloxacin 500 mg once outcome of epididymectomy for the management
have genitourinary abnormalities daily) of chronic epididymal pain. J Urol. 2009;182:
Renal ultrasound and voiding cystourethrography 14071412.
Second Line
are recommended when there are clinical signs of N/A 3. Tracy CR, Steers WD, Costabile R. Diagnosis and
epididymitis and a positive urine culture management of epididymitis. Urol Clin N Am.
SURGERY/OTHER PROCEDURES 2008;35:101108.
Pathologic Findings r Drainage if abscess present
r Inflammation 4. Wu HC, Sun SS, Kao A, et al. Comparison of
r Epididymectomy (2)
r Infection radionuclide imaging and ultrasonography in the
r Possible fibrosis Not used as 1st-line treatment differentiation of acute testicular torsion and
Reserved for severe acute or chronic inflammatory testicular disease. Clin Nucl Med.
DIFFERENTIAL DIAGNOSIS epididymitis/epididymalgia 2002;27:490493.
r Abscess Patient needs to understand that there is only at 5. Yin S, Trainor JL. Diagnosis and management of
r Chronic pelvic pain syndrome best a 50% chance of pain relief testicular torsion, torsion of the appendix testis,
r Epididymitis (acute vs. chronic) Outcomes appear improved in the setting of and epididymitis. Clin Pediatr Emerg Med.
r Interstitial cystitis postvasectomy chronic epididymitis 2009;10:3844. E
r Orchitis Fertility issues need to be addressed
r Testicular denervation
r Partial spermatic cord torsion
r Prostatitis Not widely used ADDITIONAL READING
Reserved for patients who failed conservative
r Referred pain (inguinal hernia renal colic, aneurysm, N/A
management
hip pain, lower back pain) Pain relief noted in 71% of cases
r Spermatocele See Also (Topic, Algorithm, Media)
ADDITIONAL TREATMENT r Acute Scrotum
r Testicular cancer
r Acute Scrotum Algorithm
r Testicular torsion Radiation Therapy
N/A r Epididymitis Image
r Varicocele
r Behcet Disease
Additional Therapies
r Scrotal elevation r Orchitis, General Considerations
TREATMENT r Limitation of activity r Scrotum and Testicle, Mass
r Ice packs r Scrotum and Testicle, Mass Algorithm
GENERAL MEASURES
r Acute epididymitis Complementary & Alternative
Treat infections Therapies
Decrease inflammation (NSAIDs) N/A
CODES
Pain control (NSAIDs, prescription pain
medications) ICD9
Scrotal support ONGOING CARE r 016.40 Tuberculosis of epididymis, unspecified
Ice/heat based on response r 098.0 Gonococcal infection (acute) of lower
PROGNOSIS
Avoid sexual activity for at least 1 wk following the r Pain often improves within 4872 hr after treatment genitourinary tract
initiation of therapy and until symptoms resolved r 604.90 Orchitis and epididymitis, unspecified
r Chronic epididymitis of acute epididymitis
Induration may remain for up to 4 wk ICD10
Course of antibiotics is appropriate initially; if no r Chronic cases can be difficult to treat r A18.15 Tuberculosis of other male genital organs
relief observation and reassurance are
COMPLICATIONS r A54.23 Gonococcal infection of other male genital
recommended for mild symptoms
Scrotal support r Chronic or recurrent epididymitis organs
Avoid aggravating activities r Epididymal and/or testicular abscess r N45.1 Epididymitis
Local heat therapy/Sitz baths r Infertility
r Testicular atrophy
MEDICATION (1)
r Fournier gangrene CLINICAL/SURGICAL
First Line PEARLS
r For infections tailor therapy to age and history
FOLLOW-UP
Ciprofloxacin and other quinolones are no longer r In men <35-yr-old STI/STD with C. trachomatis and
Patient Monitoring
recommended for gonococcal/nongonococcal r Patients should follow up within 37 days after N. gonorrhoeae are the most common organisms
infections due to resistance initiation of treatment, especially if symptoms have responsible for bacterial epididymitis.
r Empiric therapy to treat N. gonorrhea and r In older men suspect coliform bacteria.
not improved
C. trachomatis should be initiated pending lab r Infants and children may need to be assessed for r Testicular torsion needs to be ruled out in cases of
results genitourinary abnormalities acute scrotal pain (clinical exam and Doppler US as
Ceftriaxone 250 mg intramuscularly in a single r Elderly males may need to be assessed for urinary appropriate).
dose along with either tract obstructions
Azithromycin 1 g PO 1 dose OR r Screening and treatment of partners for STDs
Doxycycline 100 mg orally BID for 10 days
r Epididymitis due to enteric organisms Patient Resources
Levofloxacin 500 mg orally once daily for 10 days Urology Care Foundation. http://www.
or ofloxacin 300 mg orally twice daily for 10 days urologyhealth.org/urology/index.cfm?article=114
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EPISPADIAS
Sarah M. Lambert, MD
Pasquale Casale, MD, FACS
Paul H. Noh, MD, FACS, FAAP
Imaging
BASICS DIAGNOSIS r Plain x-ray to assess orientation of pelvic bones;
osteotomies should be done if pubic diastasis
DESCRIPTION HISTORY is >4 cm.
r Congenital anomaly characterized by a dorsal r Usually recognized at birth r Renal/bladder US to assess presence/absence of 2
opening of the urethra, resulting in dorsal chordee r Less severe forms, especially in females, may go
kidneys and presence/absence of hydronephrosis,
and widely displaced corporeal bodies. unrecognized until the child experiences persistent due to increased risk of renal agenesis, ectopic renal
r Often associated with the so-called urinary incontinence after toilet-training or UTIs location, and VUR.
exstrophyepispadias complex a wide spectrum r Urinary incontinence due to open bladder outlet and r Voiding cystourethrogram to assess bladder
of abnormalities that can include classic bladder absence of urinary sphincter. The more proximal the capacity, bladder outlet, presence/absence of VUR.
exstrophy, epispadias, and cloacal exstrophy. urethral meatus, the greater the degree of
Each of these anomalies is considered to arise incontinence Diagnostic Procedures/Surgery
from the same basic embryologic defect. r There may be a family history of Cystourethroscopy to assess length of urethra,
exstrophyepispadias, although rare presence/competency of sphincter, bladder
EPIDEMIOLOGY capacity/quality, location/quality of ureteral orifices.
Incidence PHYSICAL EXAM Pathologic Findings
r Males:
r 1 in 117,000 newborn males (1)[A] r N/A
Displaced meatus, ranging from glans to penile
r 1 in 484,000 newborn females (1)[A]
shaft to peno-pubic region to subsymphyseal DIFFERENTIAL DIAGNOSIS
r Male > Female (3:15:1) (1)[A] location r Varying degree of epispadias
Open urethral plate visible on dorsum of phallus r Classic bladder exstrophy
Prevalence
N/A Divergent peno-pubic attachments due to public
diastasis, resulting in splaying of corpora
RISK FACTORS cavernosa and a short, pendular penis with dorsal TREATMENT
None identified chordee, similar to that seen in exstrophy
Ventral hood of foreskin GENERAL MEASURES
Genetics r Usually managed along with bladder exstrophy,
None sporadic Assess position of testes
r Females: which is commonly present.
PATHOPHYSIOLOGY r Complete continence may not be achieved for
r On the same spectrum of exstrophy 3 degrees of female epispadias, according to
Davis (1) months to years after initial surgery.
r Failure of medial migration of mesenchyme between r In males, continence may not occur until puberty
I: Urethral orifice appears patulous
the ectodermal and endodermal layers of the cloacal II: Urethra split dorsally along most of urethra with maturation of prostate.
membrane due to premature rupture of the cloacal III: Urethra open dorsally along its entire length
membrane MEDICATION
into the bladder neck, rendering patient First Line
r The mesenchyme that forms the genital tubercles at
incontinent. Most common female type Anticholinergic therapy may help with bladder
the 5th wk of gestation fails to migrate completely Bifid clitoris
toward the midline, resulting in a defect in the development and modeling to promote increased
Mons pubis depressed and covered in glabrous capacity with good compliance once surgery has
dorsal urethral wall skin increased outlet resistance.
ASSOCIATED CONDITIONS Labia minora poorly developed and terminated
r Exstrophy anteriorly at clitoris Second Line
r Urinary incontinence Vagina and internal genitalia usually normal N/A
r VUR: Incidence of 3075% (1) r Other:
SURGERY/OTHER PROCEDURES
r Inguinal hernias: Incidence of 33% (1) Should assess for any degree of bladder prolapse r Goals:
r 2.8% concomitant renal anomalies, duplicated or exstrophy Protection of upper tracts, including correction of
Low-set umbilicus with exstrophy VUR and maintenance of a low-pressure system
collecting system most common Public diastasis due to outward rotation of
r Concomitant colorectal anomalies with 1.8% Achieve urinary continence
innominate bones, usually not as wide as in Reconstruction of external genitalia for optimal
(exstrophy/epispadias), imperforate anus most exstrophyepispadias complex functional and cosmetic results
common Evaluate for inguinal hernias r 1st stage:
GENERAL PREVENTION DIAGNOSTIC TESTS & INTERPRETATION Bladder closure between 3 to 6 mo of age. Patient
N/A Lab is left with an epispadias
CBC, renal profile Can also be done as a single stage with bladder
closure and urethral reconstruction known as the
Complete Primary Repair of Exstrophy. Higher
incidence of glanular loss than staged repair
Osteotomies are needed if the pubic diastasis is
4 cm or greater on plain x-ray. It was once
thought that if the surgery was done in the 1st
48 hr of life that osteotomies are not needed.
Current management favors osteotomies to
maximize continence
Patients need to be immobilized after surgery to
allow pelvic bone healing if osteotomies are
needed
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EPISPADIAS
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Second Line
r Intracavernosal injection therapy
Complementary & Alternative ADDITIONAL READING
Therapies
r Data does not support use of trazodone, yohimbine, r Mendenhall WM, Henderson RH, Indelicato DJ, et al.
Highly efficacious with up to an 89% response
rate post-RP (3)[C] and herbal therapies. These medications are not Erectile dysfunction after radiotherapy for prostate
Risks include priapism, penile pain, ecchymosis recommended for use in ED by the American cancer. Am J Clin Oncol. 2009;32:443447.
Used in a variety of formulations Urological Association r Mulhall JP, Bivalacqua TJ, Becher EF. SOP for the
Single agent: Prostaglandin E1 r Testosterone therapy preservation of erectile function outcomes after
Bimix: Papaverine and phentolamine May be useful in aiding erectile function recovery radical prostatectomy. J Sex Med. 2013;10:
Trimix: Papaverine, phentolamine, and only in patients with documented hypogonadism 195203.
prostaglandin E1 Controversial; must discuss risks/benefits of
r Intraurethral prostaglandin E suppository (MUSE) See Also (Topic, Algorithm, Media)
1 androgen supplementation before initiating r Erectile Dysfunction/Impotence, General
Variable efficacy therapy, particularly in patients with a history of Considerations
Penile pain frequently reported, especially in the prostate cancer r Penile Doppler Ultrasound, Indications and
immediate postoperative period
Parameters
SURGERY/OTHER PROCEDURES r Penile Rehabilitation
r Vacuum constriction devices ONGOING CARE r Reference Tables: International IIEF (Sex Function
Low patient satisfaction given cumbersome PROGNOSIS Survey)
application r Improvement in erectile function can be noted after
Cooler, cyanotic appearance of vacuum-assisted pelvic surgery, with maximal improvement noted
erection appears unnatural to some between 18 and 24 mo postoperatively. CODES
E
r Penile prosthesis implantation r Low likelihood of improvement in erectile quality
Definitive therapy for patients failing or refusing after 2 yr postoperatively. ICD9
1st- and 2nd-line treatments r Nadir of erectile function 3 to 5 yr after RT.
607.84 Impotence of organic origin
Generally, postponed until 2-yr post-RP as r Penile rehabilitation likely improves the prognosis of
regeneration of cavernous nerves during this time postsurgical/post-RT ED. Definitive data are pending. ICD10
may preclude need for surgical therapy r N52.31 Erectile dysfunction following radical
High patient satisfaction in appropriately selected COMPLICATIONS prostatectomy
r Significant effect on patient quality of life r N52.32 Erectile dysfunction following radical
population
Implant infection and malfunction risk must be Noted to be strongest predictor of patient cystectomy
discussed with patient preoperatively satisfaction after prostate cancer therapy r N52.39 Other post-surgical erectile dysfunction
r Depression
ADDITIONAL TREATMENT
Radiation Therapy FOLLOW-UP
CLINICAL/SURGICAL
N/A Patient Monitoring
r Variable dependent upon patient response to PEARLS
Additional Therapies treatment.
r Limited data on combining modalities has been r ED after pelvic surgery and RT is highly prevalent
r Close follow-up is recommended in patients on
reported and frequently underestimated in physician
Level 3 evidence: PDE5i + either transurethral or rehabilitation protocols to evaluate for erectile marketing materials.
intracavernosal injection therapy generate better recovery. r ED after pelvic surgery is immediate in onset with
efficacy rates than either monotherapy alone Patient Resources 18- to 24-mo time to maximal recovery.
Level 4 evidence: Enhanced efficacy with the Mulhall JP. Saving Your Sex Life: A Guide for Men with r ED after RT has an insidious onset, with nadir of
combination of vacuum-erection therapy + either Prostate Cancer. 1st ed. Chicago, IL: Hilton Publishing erectile function at 3- to 5-yr post-RT.
PDE5i or transurethral PGE1 or intracavernosal Company; 2008. r Data on penile rehabilitation is conflicting but
injection therapy increasingly shows an improvement in
REFERENCES posttreatment erectile recovery.
r The majority of postpelvic surgery/RT ED patients are
1. Alemozaffar M, Regan MM, Cooperberg MR, et al. effectively treated with PDE5i intracavernosal
Prediction of erectile function following treatment injection therapy.
for prostate cancer. JAMA. 2011;306(11):
12051214.
2. Candy B, Jones L, Williams R, et al.
Phosphodiesterase type 5 inhibitors in the
management of erectile dysfunction secondary to
treatments for prostate cancer: Findings from a
Cochrane systematic review. BJU Int. 2008;102(4):
426431.
3. Coombs, PG, Heck M, Guhring P, et al. A review of
outcomes of an intracavernosal injection therapy
programme. BJU Int. 2012;110(11):17871791.
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Genetics
BASICS r Several gene polymorphisms linked with ED DIAGNOSIS
r Angiotensin-converting enzyme (ACE)
DESCRIPTION polymorphisms may be risk factors for vasculogenic HISTORY
r Medical historycomorbid conditions, medications,
Consistent or recurrent inability to attain and/or ED and endothelial nitric oxide synthase (eNOS)
maintain an erection sufficient for satisfactory sexual polymorphisms alone or in combination with other alcohol, tobacco, recreational drug use, history of
activity genetic polymorphisms implicated in ED cycling
r Surgical history
EPIDEMIOLOGY PATHOPHYSIOLOGY r Psychosexual history
Incidence r Mechanism of erection
r Crude incidence: 26 cases/1,000 man years Status of current relationship
Relaxation of cavernosal smooth muscle Level of libido/interest in sex
Incidence increases with each decade above 40 (contracted in flaccid state inhibiting inflow of Quality of erection
12 cases/1,000 man years: 4049 yr blood) Duration of ED
30 cases/1,000 man years: 5059 yr Mediated by NO release from pelvic nerves and Onsent of ED (sudden vs. gradual)
46 cases/1,000 man years: 6069 yr endothelial cells Presence of nocturnal/early morning erections
Increased cyclic GMP (cGMP) and cyclic AMP
Prevalence Presence of penile curvature, plaque, pain
r Increases universally with age and medical (cAMP) trigger signaling pathways leading to International Index of Erectile Function
comorbidities (cardiovascular disease, hypertension, decreased intracellular calcium causing smooth Questionnaire (IIEF-5)
smoking, inactivity, obesity) muscle relaxation, increased penile blood flow, 5 questions scored individually from 05
Prevalence by age and tumescence (maximum of 25 points, higher score indicated
Below age 40: 19% Smooth muscle relaxation further promoted due better function)
4059 yr: 2030% to inhibition of Rho-kinase Classifies ED into severe (57), moderate
6069 yr: 2040% Veno-occlusive mechanism prevents outflow of (811), mild to moderate (1216), mild
>70 yr: 5075% blood from penis and maintains erection (1721), and no ED (2225)
cGMP degraded by phosphodiesterase type 5
RISK FACTORS (PDE5) PHYSICAL EXAM
r Probability of ED increases with presence of each r Organic ED r Neurologic: Stroke, CNS disease, visual field defects,
risk factor Vasculogenic neuropathy, perineal sensation
Diabetes mellitus Arteriogenicatherosclerotic lesions decrease r Endocrinologic: Atrophic testes, gynecomastia, loss
Prevalence of ED 3 times higher in diabetic men arterial inflow to penis of secondary sexual characteristics
ED occurs at earlier age and increases with Venogenicfailure of corporal vasoocclusion r Cardiovascular: Blood pressure, femoral/pedal
disease duration NeurogenicAlzheimer disease, Parkinson pulses, lower extremity ischemia
Associated with 14 times increased risk of disease, injury to central nervous system, spinal r Penile: Curvature, Peyronie disease plaques
cardiovascular morbidity and mortality cord, or peripheral nerves r Rectal exam
Cardiovascular disease (hyperlipidemia, Anatomic
hypertension, peripheral vascular disease) Endocrinologic (hyperprolactinemia, hyper or DIAGNOSTIC TESTS & INTERPRETATION
Lower urinary tract symptoms/Benign prostatic hypothyroidism, adrenal disorders/Cushing Lab
hyperplasia (BPH) r Complete blood count
syndrome)
Chronic renal failure, chronic liver disease r Psychogenic ED r Serum chemistries
Endocrinopathies (hypogonadism, Cushing Only accounts for 10% of ED r Fasting glucose level, hemoglobin A1C
disease) More common in men <35 yr old r Lipid profile
Prior abdominal/pelvic/penile surgery, radiation or May result from lack of interest in partner, r Serum total testosterone
trauma performance-related anxiety, negative mood, r Thyroid function tests (optional)
Priapism, Peyronie disease life stressors r PSA (suspect prostate pathology)
Neurologic disease (Parkinson disease, dementia,
ASSOCIATED CONDITIONS r Urinalysis (glucose as indicator of diabetes)
prior stroke)
r Atherosclerosis
Depression/Psychological disorders Imaging
Long-distance cycling r Diabetes mellitus r Duplex penile ultrasoundmost reliable and least
Smoking r Hypertension, stroke
invasive modality for assessing ED
Medications r Depression r Provides imaging evaluation and quantification of
Antihypertensives (thiazide diuretics, -blockers, r Parkinson disease, multiple sclerosis penile blood flow
2 -agonists) r Priapism
ACE inhibitors, angiotensin receptor blockers, r Peyronie disease
and calcium channel blockers cause less ED/may r Prostate cancer
improve erectile function
Psychotropics (monoamine oxidase inhibitors, GENERAL PREVENTION
selective serotonin reuptake inhibitors, lithium) r Avoidance of tobacco use
Antiandrogens r Optimal medical management of commonly
Miscellaneous (digoxin, cimetidine, associated conditions
spirolactone, marijuana) r Increase exercise/weight loss
Tobacco smoking r Split bicycle seat for long-distance cycling
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EXSTROPHY, CLOACAL
Jason C. Fisher, MD
Harry P. Koo, MD, FAAP, FACS
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EXSTROPHY, CLOACAL
MEDICATION ADDITIONAL TREATMENT 3. Stolar CH, Randolph JG, Flanigan LP. Cloacal
First Line Radiation Therapy exstrophy: Individualized management through a
r IVF support: Adjust for fluid losses across N/A staged surgical approach. J Pediatr Surg.
exstrophied mucosa 1990;25:505507.
r At risk for hyperchloremic metabolic acidosis Additional Therapies
r Large omphaloceles not amenable to primary 4. Zderic SA, Canning DA, Carr MC, et al. The CHOP
r Generally not candidates for epidural anesthesia due experience with cloacal exstrophy and gender
closure can be treated with Silvadene-mediated
to spinal dysraphism reassignment. Adv Exp Med Biol. 2002;511:
epithelialization of sac followed by delayed closure,
135147.
Second Line or by excision of sac and placement of a silo with
N/A gradual staged reduction of viscera
r Consider gastrostomy tube placement and/or
SURGERY/OTHER PROCEDURES tunneled central line during initial repair if patient
ADDITIONAL READING
r Open spinal anomaly: Prompt neurosurgical repair
appears at risk for short-gut syndrome r Mathews R, Jeffs RD, Reiner WG, et al. Cloacal
required before addressing exstrophy.
r Exstrophy repair at 4872 hrs of life if stable Complementary & Alternative exstrophy: Improving the quality of life: The Johns
Therapies Hopkins Experience. J Urol. 1998;160:24522456.
Combined pediatric urology and general pediatric r McHoney M, Ransley PG, Duffy P, et al. Cloacal
surgery N/A
Usually staged approach vs. single stage exstrophy: Morbidity associated with abnormalities
r Multistage approach (3)[B]: Creates classic bladder of the gastrointestinal tract and spine. J Pediatr
ONGOING CARE Surg. 2004;39:12091213.
exstrophy anatomy at conclusion of initial surgery r Reiner WG. Psychosexual development in genetic
described above; preferred for most cases. General PROGNOSIS
r Survival >90% over last 20 yr males assigned females: The cloacal exstrophy
E
principles of Stage 1:
r Nutrition and growth is the most important experience. Child Adolesc Psychiatric Clin N Amer.
Separate hemibladders from cecal plate 2004;13:657674.
Revert any prolapsed terminal ileum determinant of early survival and morbidity r Soffer SZ, Rosen NG, Hong AR, et al. Cloacal
Mobilize, rescue, and preserve any hindgut 3050% will have failure to thrive before age 5
Important to avoid using any bowel for GU exstrophy: A unified management plan. J Pediatr
Tubularize the cecum to bring terminal ileum,
reconstruction procedures until child is thriving Surg. 2000;35:932937.
cecum, and hindgut into closed continuity
Create end colostomy from distal hindgut r After 3 yr, quality-of-life issues outweigh nutritional See Also (Topic, Algorithm, Media)
Assess and preserve mullerian anatomy concerns r Epispadias
Excise and close omphalocele if possible Urinary continence: Rarely achieved; dependent r ExstrophyEpispadias Complex
Anastomose hemibladders in midline on a compliant reservoir and continent r Exstrophy, Bladder (Classic Exstrophy)
r Single-stage approach (4)[B]: In highly select catherizable conduit r Exstrophy, Cloacal Images
patients, can proceed with bladder and abdominal Fecal continence: Usually managed by enema
wall closure and phallic reconstruction which may regimen, rarely are perineal pull-through
avoid osteotomies, minimize bladder scarring. maneuvers associated with any continence
Gender assignment and reconstruction, especially CODES
Otherwise, Stage 2 is performed in late infancy,
mirroring a classic bladder exstrophy repair: for genetic males raised as females
Mobilize bladder plate and posterior urethra Ambulation impairments ICD9
deep into pelvis: Yields incontinent bladder. Gynecologic issues after onset of menarche 753.5 Exstrophy of urinary bladder
Orchiopexy with repair of inguinal hernias Psychosexual problems ICD10
Reconstruct gender-based external genitalia COMPLICATIONS Q64.12 Cloacal extrophy of urinary bladder
Pubic reapproximation +/ pelvic bone r Infection and breakdown of repair
osteotomies with fixation and traction for r Abdominal compartment syndrome
46 wk r Short-gut syndrome CLINICAL/SURGICAL
r Stage 3 involves procedures aimed at continence r Vesicoureteral reflux and hydronephrosis PEARLS
and genital cosmesis and is addressed in older r Hirschsprung-type enterocolitis in the dysmotile r Meticulous assessment of associated anomalies can
children, often involving bladder augmentation and hindgut, even after colostomy formation prevent early clinical and surgical mishaps,
catherizable conduits
r Surgical pitfalls to avoid: FOLLOW-UP particularly with regard to spinal defects.
r A multidisciplinary team is critical to the short- and
Injury to ureteral orifices: Place stents Requires a multidisciplinary team (see Diagnosis) to
coordinate regular follow-up through all stages of long-term outcomes of these children.
Overaggressive closure of a large omphalocele r A staged surgical repair remains the preferred
defect leading to compartment syndrome surgical repair, with careful attention to nutrition and
Excising/discarding diminutive male phalic growth in infancy, and both surgical and psychological approach for most children with cloacal exstrophy.
support for the multiple quality of life issues which r Do not underestimate the impact of early gender
remnants and assigning female gender:
Controversial and still occurs with unclear begin in childhood and persist into adulthood assignment: Avoid irreversible surgical resection of
long-term consequences Patient Resources structures that may be useful for genital
Excising/discarding the hindgut even if short Urology Care Foundation. http://www. reconstruction.
Primary perineal pull-through of the hindgut (can r Save as much bowel as possible, especially the short
urologyhealth.org/urology/index.cfm?article=91
be performed in highly select patients) hindgut, to maximize nutritional capability.
REFERENCES
1. Vermeij-Keers C, Hartwig NG, van der Werff JF.
et al. Embryonic development of the ventral body
wall and its congenital malformations. Semin
Pediatr Surg. 1996;5:8289.
2. Austin PF, Homsy YL, Gearhart JP, et al. The
prenatal diagnosis of cloacal exstrophy. J Urol.
1998;160:11791181.
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r Benign lesions
BASICS DIAGNOSIS Air: Iatrogenic, infectious, fistula
Blood clot
DESCRIPTION HISTORY Fibroepithelial polyp
r Radiographic diagnosis of a radiolucent entity r Flank pain or renal colic
Fungus ball
occupying the confines of the upper urinary tract r Hematuria
Hemangioma
including the intrarenal collecting system or ureter, Gross Inflammatory lesions: Granuloma, malakoplakia,
as seen against contrast within the intraluminal Microscopic TB
space r Pre-existing malignancy Inverted papilloma
r The finding itself is nonspecific but may represent r History or urinary diversion Calculus (usually radiolucent)
malignant or benign processes r Prior urinary tract manipulation (stent, stone Benign tumors (rare): Leiomyoma, neurofibroma,
r Ureteroscopic evaluation is the gold standard to treatment, etc.) cholesteatoma
establish definitive diagnosis Extrinsic compression of the ureter
PHYSICAL EXAM Mucous (urinary diversion patients)
EPIDEMIOLOGY r Costovertebral angle tenderness
Protein matrix
Incidence r Patient may by asymptomatic
Ureteritis or pyelitis cystica
r Difficult to define given the nonspecific nature of the
DIAGNOSTIC TESTS & INTERPRETATION Vascular impression
radiographic finding Fibroepithelial polyp
Upper tract urothelial carcinoma (UTUC) Lab
r Urinalysis Papilla
Estimated 5,9007,300 new cases in USA in Prominent papilla (ectopic or end on; normal
r Urine cytology may suggest malignancy
2014 (1)[C] anatomic variant)
r Urine culture including fungal cultures
Prevalence Sloughed papilla (may cause obstruction or
r Nephrolithiasis r Serum creatinine/BUN
hematuria)
Reported prevalence of kidney stones in USA Imaging Foreign body
between 1976 and 1994 was 13% in men and r Filling defects are found in imaging modalities Stent fragment (retained)
7% in women (2)[C] which utilize contrast that fills the intrarenal Staple/clip (more likely with urinary diversion)
collecting system
RISK FACTORS Intravenous injection of contrast
r For UTUC TREATMENT
CT urogram
History of smoking MR urogram
History of urothelial carcinoma of the bladder IVP
GENERAL MEASURES
Gene carrier or family history of Lynch syndrome r If any doubt exists about the etiology of the filling
Invasive arteriograms (cardiac catheterization,
(hereditary nonpolyposis colorectal cancer) defect then diagnostic ureteroscopy is indicated
r For nephrolithiasis aortogram, etc.) r Prominent papilla may appear as filling defects in
Intraluminal administration of contrast
Previous stone history Retrograde pyelogram the very peripheral aspect of renal calyces in an
Chronic dehydration Antegrade nephrostogram end on position
Dietary factors Cystogram (if reflux present) MEDICATION
Elevated sodium intake
Purine gluttony Diagnostic Procedures/Surgery First Line
r For stones composed purely of uric acid manifesting
r For sloughed papilla Ureteroscopic evaluation is required to obtain
definitive diagnosis and provides for direct visual as filling defects, alkalization of the urine can be
NSAID overuse
inspection with relatively low morbidity (3)[C] attempted and if pH of 6.5 or greater is achieved
Sickle cell disease or trait
then uric acids may dissolve over time
History of diabetes Pathologic Findings Potassium citrate
r For fungus ball Depends on underlying etiology Sodium bicarbonate
Immunosuppression DIFFERENTIAL DIAGNOSIS
r Malignant lesions Second Line
Genetics N/A
N/A UTUC
Rare primary cancers of the upper urothelial
PATHOPHYSIOLOGY
surface
Depends on underlying etiology Squamous cell carcinoma (often associated with
ASSOCIATED CONDITIONS chronic untreated infected staghorn calculi)
r Hematuria Adenocarcinoma
Gross Inverted papilloma (about 15% have malignant
Microscopic components)
r Flank pain Sarcoma
Leiomyosarcoma
GENERAL PREVENTION Angiosarcoma
r Depends on underlying etiology
Small cell carcinoma
UTUC Metastatic carcinoma
Smoking cessation/avoidance Melanoma
Nephrolithiasis Renal cell carcinoma
Adequate hydration
Diet and lifestyle modification to prevent future
stone formation
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SURGERY/OTHER PROCEDURES
r UTUC
ADDITIONAL READING
ONGOING CARE r Hubosky SG, Boman BM, Charles S, et al.
For low-grade UTUC which can be reached
ureteroscopically and completely ablated, 5-yr PROGNOSIS Ureteroscopic management of upper tract urothelial
r Depends on underlying etiology carcinoma (UTUC) in patients with Lynch syndrome
survival is equal to radical nephroureterectomy
(4)[C] Prognosis usually excellent for benign conditions (hereditary nonpolyposis colorectal cancer
After complete ablation, local recurrence can be UTUC (prognosis depends on pathologic staging) syndrome). BJU Int. 2013;112(6):813819. doi:
seen in up to 75% of patients if followed for at (5)[C]. TNM pathologic staging and prognosis is 10.1111/bju.12008
least 5 yr. as follows: r Matin SF, Margulis V, Kamat A, et al. Incidence of
Progression of low-grade to high-grade disease pTo, pTa, and pTis have 93% and 89% downstaging and complete remission after
occurs in about 15% of cases cancer-specific survival at 5 and 10 yr neoadjuvant chemotherapy for high-risk upper tract
These points should be stressed to patients pT1 has 91% and 85% cancer-specific survival urothelial cell carcinoma. Cancer. 2010;116:
when counseling on the management of UTUC at 5 and 10 yr 31273134.
For high-grade UTUC or very large-volume pT2 has 75% and 70% cancer-specific survival r Stower MJ, MacIver AG, Gingell JC, et al. Inverted
low-grade UTUC, radical extirpative surgery is at 5 and 10 yr papilloma of the ureter with malignant change. BJU.
considered the gold standard for cancer control pT3 has 54% and 45% cancer-specific survival 1990;65:13.
Nephroureterectomy (open or laparoscopic) at 5 and 10 yr r Xu AD, Ng CS, Kamat A, et al. Significance of upper
Segmental ureterectomy pT4 has 12% and 6% cancer-specific survival at
urinary tract urothelial thickening and filling defect
r Nephrolithiasis 5 and 10 yr seen on MDCT urography in patients with a history
Ureteroscopy with laser lithotripsy COMPLICATIONS of urothelial neoplasms. Am J Roentgenol.
ESWL with or without retrograde pyelogram to N/A 2010;195:959965.
assist with localization or with ultrasound
guidance FOLLOW-UP See Also (Topic, Algorithm, Media)
r Fibroepithelial Polyp, Genitourinary
PCNL Patient Monitoring
r Sloughed papilla r UTUC r Filling Defect, Upper Urinary Tract (Renal Pelvis and
Ureteroscopy confirms diagnosis For those undergoing ureteroscopic conservative Ureter) Image
r Fungal Infections, Genitourinary
F
Papilla can be removed primarily with treatment, regular surveillance including
cystoscopy and ureteroscopy is required given r Reference Tables: TNM: Renal Pelvis and Ureter
ureteroscopic grasper or basket
Coagulation with cautery or laser will achieve high chance of local recurrence Cancer
hemostasis Cross-sectional imaging (CT or MRI) is r Ureter and Renal Pelvic Tumors, General
Avoid overuse of NSAIDs recommended to check for locally advancing Considerations
r Fibroepithelial polyp disease r Ureter and Renal Pelvis, Squamous Cell Carcinoma
Can be removed with ureteroscopy using laser or For those undergoing radical nephroureterectomy r Ureter and Renal Pelvis, Urothelial Carcinoma
grasper (NU), surveillance cystoscopy and cross-sectional r Urolithiasis, Ureteral
r Fungus ball imaging are also needed on a regular basis
r Nephrolithiasis
Can be removed with ureteroscopy or
Renal ultrasound, serum electrolyte testing
percutaneous approach
For patients at high risk for stone recurrence,
CODES
Antifungals
24-hr urine electrolyte evaluation
ADDITIONAL TREATMENT ICD9
Patient Resources r 189.1 Malignant neoplasm of renal pelvis
Radiation Therapy N/A r 189.2 Malignant neoplasm of ureter
N/A
r 793.5 Nonspecific (abnormal) findings on
Additional Therapies
r UTUC REFERENCES radiological and other examination of genitourinary
organs
Neoadjuvant chemotherapy is currently under 1. Siegel R, Ma J, Zou Z, Jemal A. Cancer statistics,
investigation for suspected high-stage disease 2014. CA Cancer J Clin. 2014;64(1):929. ICD10
with preliminary data suggesting down staging on r C65.9 Malignant neoplasm of unspecified renal
2. Stamatelou KK, Francis ME, Jones CA, et al. Time
pathologic specimens pelvis
trends in reported prevalence of kidney stones in
Complementary & Alternative the United States: 19761994. Kidney Int. 2003; r C66.9 Malignant neoplasm of unspecified ureter
Therapies 63:1817. r R93.4 Abnormal findings on diagnostic imaging of
N/A 3. Conlin MJ. Diagnostic ureteroscopy. In: Smith AD, urinary organs
et al. Smiths Textbook of Endourology. 3rd ed.
Chichester: Wiley and Blackwell, 2012.
4. Grasso M, Fishman AI, Cohen J, et al. CLINICAL/SURGICAL
Ureteroscopic and extirpative treatment of upper PEARLS
urinary tract urothelial carcinoma: A 15-year r Up to 40% of patients with an upper tract urothelial
comprehensive review of 160 consecutive patients.
BJU Int. 2012;110:16181626. carcinoma will develop urothelial carcinoma of the
bladder.
5. Margulis V, Shariat SF, Matin SF, et al. Outcomes r Ureteroscopy can be both diagnostic and
of radical nephroureterectomy: A series from the
upper tract urothelial carcinoma collaboration. therapeutic.
Cancer. 2009;115:12241233.
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r Renal/ureters/bladder US COMPLICATIONS
Can diagnose hydronephrosis with a sensitivity of TREATMENT Longstanding ureteral obstruction can cause
8594% and a specificity of 100%. permanent loss of renal function.
Disadvantages: Sensitivity for detecting stones GENERAL MEASURES (3)
r Treatment varies based on etiology. FOLLOW-UP
only 2457%, limited in obese patients, and
operator-dependent. Rest and physical therapy may be recommended Patient Monitoring
r Nuclear scan: Helps to evaluate differential renal for flank pain cause by muscle spasms. Periodic renal imaging, urinalysis, or 24-hr urine may
function, degree of obstruction, and presence of NSAIDs are excellent 1st-line agents to control be indicated for patients with stone disease. Follow-up
renal scarring. pain secondary to inflammation, but caution must may be more or less intensive based on etiology.
r MRI: Not usually indicated for initial workup unless be used in the presence of acute ureteral Patient Resources
CT is contraindicated. It may be helpful for obstruction or in patients with advanced renal MedlinePlus http://www.nlm.nih.gov/medlineplus/
evaluation of renal masses or in the evaluation of disease as they can decrease intrarenal blood flow. ency/article/003113.htm
suspected spinal cord pathology. MEDICATION
Diagnostic Procedures/Surgery First Line
r Acute pain control (NSAIDs, opioids)
REFERENCES
These are dependent upon etiology
r Antiemetics, antipyretics, antibiotics as appropriate 1. Carter MR, Green BR. Renal calculi: Emergency
Pathologic Findings
These are dependent upon etiology r IV fluids if sepsis/hypovolemia. May also help with department diagnosis and treatment. Emerg Med
passage of stones Pract. 2011;13(7):1.
DIFFERENTIAL DIAGNOSIS 2. Coursey CA, Casalino DD, Remer EM, et al. ACR
r There are many causes of flank pain. It is useful to Second Line Appropriateness Criteria acute onset flank
differentiate between urologic and nonurologic -antagonists and calcium channel blockers may help painsuspicion of stone disease. Ultrasound Q.
causes. Renal/ureteral etiologies are the most with expulsion of ureteral stones. 2012;28(3):227233.
common and those that usually require urologic SURGERY/OTHER PROCEDURES 3. Marx JA, Hockberger RS, Walls RM, et al. Rosens
intervention. Some of the most common causes are r Prior to any diagnostics or intervention, the patient Emergency Medicine: Concepts and Clinical
listed below (2). must be stabilized. Practice. 7th ed. St Louis, MO: Mosby; 2010. F
Urologic r Surgical management may be required in some
Calculi: Mostly ureteral; however, renal pelvic
cases depending on the etiology and the patients
and calyceal stones (obstructing infundibulum)
can cause flank pain
medical condition. ADDITIONAL READING
r Examples of surgical management: If the collecting
Acute cortical necrosis N/A
Acute papillary necrosis system is infected and obstructed or renal abscess is
Ptotic kidney present, percutaneous drainage and antibiotics are See Also (Topic, Algorithm, Media)
the mainstays of treatment. If dealing with a r Calcifications, Abdominal and Pelvic
Polycystic kidney disease
Acute/chronic pyelonephritis ruptured AML, embolization should be considered. r Hydronephrosis/Hydroureteronephrosis (Dilated
Renal infarction (renal artery thrombus or Renal tumors should be treated on an elective basis. Ureter/Renal Pelvis), Adult
Emergent nephrectomy for ruptured AML or r Renal Mass
dissection)
Renal cyst (especially hemorrhagic; benign cysts XGP/emphysematous pyelonephritis may be r Urolithiasis, Adult, General
necessary. r Urolithiasis, Pediatric, General Considerations
rarely cause flank pain)
Renal neoplasm ADDITIONAL TREATMENT
Renal trauma Radiation Therapy
Renal vein thrombosis NA CODES
Retroperitoneal bleed or mass
Ureteropelvic junction obstruction Additional Therapies
Calyceal diverticulum NA ICD9
r 592.9 Urinary calculus, unspecified
Medullary sponge kidney Complementary & Alternative r 788.0 Renal colic
Other ureteral obstruction (extrinsic Therapies r 789.09 Abdominal pain, other specified site
compression, blood clot, necrotic material, etc.) NA
Nonurologic ICD10
Appendicitis r N20.9 Urinary calculus, unspecified
Abdominal aortic aneurysm ONGOING CARE
r N23 Unspecified renal colic
Diabetes r Follow-up for flank pain will also be dictated by the r R10.9 Unspecified abdominal pain
Diverticulitis
etiology and acuity of the clinical presentation.
Herpes zoster
Repeat imaging or other lab studies may be required
Musculoskeletal (muscle spasm, costochondritis,
depending on response to initial therapy. CLINICAL/SURGICAL
strain) r If clinical picture fails to improve or worsens, a
Myocardial infarction PEARLS
Ovarian torsion change in therapy should be instituted (ie, different
antibiotic, PCN, surgical intervention). r Flank pain associated with fever and chills may
Pancreatitis
represent urinary tract infection (pyelonephritis).
Peripheral nerve compression or trauma PROGNOSIS r Abdominal aortic aneurysm is a potentially
Peripheral neuropathy In general, for nephrolithiasis, the prognosis is good
Pleuritis life-threatening cause of flank pain.
but this may vary for other etiologies.
Tubal pregnancy
Vertebral or spinal cord/nerve root irritation
(herniated disc, sciatica, vertebral body fracture,
or collapse)
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FOURNIER GANGRENE
Brad Figler, MD
Bryan Voelzke, MD, MS
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FOURNIER GANGRENE
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r Phycomycosis:
BASICS DIAGNOSIS Stain tissue
r Blastomycosis:
DESCRIPTION HISTORY Stain tissue and visualize secretions
r Primary fungal infection of the genitourinary (GU) r Immunocompromised state:
r Coccidioidomycosis/histoplasmosis:
tract is common with Candida, but uncommon with Fungi are ubiquitous in the environment and can
Culture and stain tissue
other fungi. overwhelm those with weakened immune systems
r Other fungal infections are found in the GU tract but Those receiving chemotherapy, with AIDS, or Imaging
are seen more commonly with immunocompromised afflicted with diabetes CT abdomen with contrast and delayed imaging vs. US
r Recent antibiotic use: may elucidate bezoars, perinephric pathology, renal
patients or in setting of systemic disease.
Risk of candiduria is 6 after use of destruction
EPIDEMIOLOGY broad-spectrum antibiotics Diagnostic Procedures/Surgery
Incidence r Indwelling GU tubes or prosthesis: r Cystoscopy/retrograde urogram
r Difficult to determine because most cases are not r Urine culture
Risk of candiduria 12 with catheterization
reportable r GU tract abnormalities: r Tissue biopsy
Estimated 12 new cases per 100,000 population
per year involving the GU tract Risk of candiduria 6 with abnormalities (1)[A] Pathologic Findings
r Occupation: Positive histology staining for fungi in tissue
Prevalence
Difficult to estimate as cases are not reportable Exposure to aerosolized soil; spelunkers; bird DIFFERENTIAL DIAGNOSIS
handler r Blood clots in collecting system
RISK FACTORS r Recent travel or recreation (see image): r Cystitis
r Urinary tract drainage catheter
Blastomycosis found in Ohio, Missouri, and r GU TB
r Prior antibiotics Mississippi river basins; Great Lakes; Canada
r Diabetes/glucosuria r Nephrolithiasis
Coccidioidomycosis found in semiarid regions of r Squamous cell carcinoma (SCC)
r Urinary tract pathology the Western US, Mexico, Central and South
r Malignancy r Urothelial carcinoma (transitional cell carcinoma)
America
r Increased age Histoplasmosis found in Midwestern and Southern
r Neonates US in areas of high-nitrogen soil such as chicken
coops and bat caves TREATMENT
r Female sex
r Prior surgical procedures Cryptococcus thrives with birds GENERAL MEASURES
r UTI symptoms:
r Immunosuppression r Infectious Diseases Society of America recommends
Only 414% with symptomatic candiduria
Genetics treatment of candiduria in (3)[A]:
PHYSICAL EXAM Infants with low birth weight
No heritable form of transmission r CVA tenderness
Patients who will have GU procedures
PATHOPHYSIOLOGY r Abdominal tenderness Neutropenic patients
r Funguria to fungemia: r Boggy or firm prostate Symptomatic patients
Can occur with obstruction, reflux, or r Firm testicular or epididymal masses r Treat UTI symptoms empirically for funguria only if
instrumentation r GU tubes present the patient is unable to vocalize or perceive
r Fungemia to funguria: r Manifestations of disseminated disease symptoms
Disseminated disease seeds GU tract
Multiple microabscesses develop in the renal DIAGNOSTIC TESTS & INTERPRETATION r Asymptomatic candiduria: Assess for risk factors
cortex, with subsequent penetration into the Lab (3)[A]
glomeruli and shedding into the urine from the r Candida:
MEDICATION
proximal tubules No studies have established the importance of First Line
ASSOCIATED CONDITIONS pyuria or quantitative urine culture in diagnosing r Aspergillosis:
r Immunocompromised state: Candida UTI (2)[A] Amphotericin B 11.5 mg/kg/d for 10 wk
Diabetes Presence of pyuria helpful in those without r Blastomycosis:
AIDS catheter, however, up to 25% with candiduria also Itraconazole 200 mg PO BID for 612 mo
r Anatomic GU abnormalities: have bacteriuria/pyuria r Candidiasis:
Strictures Urine cultures positive for candiduria in otherwise Clotrimazole, miconazole, tioconazole,
Prostatic hypertrophy asymptomatic patients should be repeated with a terconazole topical for 1 wk
Diverticula r Candidemia (treat for 2 wk after afebrile and Cx
clean catch sample to rule out contamination
Indwelling tubes (2)[B] negative):
Stones Fluconazole 400800 mg/d IV, then PO
>10,000 CFU/mL could mean infection; r Candiduria:
GENERAL PREVENTION 10,000100,000 CFU/mL could mean
r Remove unnecessary catheters/tubes Fluconazole 200 mg/d IV/PO for 12 wk
colonization r Coccidioidomycosis:
r Narrow antibiotic coverage Check urine microanalysis looking for casts
r Improve nutritional status containing yeast: Very specific, not sensitive Itraconazole 200 mg PO BID. for 1 yr
r Control hyperglycemia r Aspergillosis:
Culture in Sabouraud medium or stain tissue with
methenamine silver or Periodic acidSchiff stain
(PAS); can PCR
r Cryptococcosis:
Culture; stain tissue with India ink, PAS,
methenamine silver; perform latex agglutination
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r Cryptococcosis: ADDITIONAL TREATMENT 3. Fisher JF, Sobel JD, Kauffman CA, et al. Candida
Amphotericin B 0.51 mg/kg/d IV + flucytosine Radiation Therapy urinary tract infectionstreatment. Clin Infect Dis
100 mg/kg/d PO for 2 wk None 2011;52:S457466.
Then fluconazole 400 mg/d PO for 8 wk OR 4. Wise GJ, Shteynshlyuger A. How to diagnose and
Then Itraconazole 200 mg PO BID for 8 wk Additional Therapies
r Irrigation may be necessary in aggressive infections treat fungal infections in chronic prostatitis. Curr
For cryptococcal suppression: Urol Rep. 2006;7:320328.
Fluconazole 200 mg/d PO OR when systemic medication is not excreted into the
Amphotericin B 0.51 mg/kg IV every week urine
r Histoplasmosis: Amphotericin B GU tract irrigation
50 mg in 1,000-mL water at 40 mL/hr ADDITIONAL READING
Itraconazole 200 mg PO BID for 618 mo
(over 24 hr) for 57 days r Kauffman CA. Candiduria. Clin Infect Dis. 2005;
For histoplasmosis suppression:
Itraconazole 200 mg/d PO BID OR In children, renal irrigation with 1024 mg/d 41:S371S376.
r Removing catheter may eradicate funguria in 40% r Kauffman CA, Vazquez JA, Sobel JD, et al.
Amphotericin B 0.51 mg/kg IV every week
r Mucormycosis: of cases Prospective multicenter surveillance study of
Amphotericin B 11.5 mg/kg/d IV for 610 wk Complementary & Alternative funguria in hospitalized patients. The National
Therapies Institute for Allergy and Infectious Diseases Mycoses
Second Line Study Group. Clin Infect Dis. 2000;30:1418.
r Aspergillosis None
r The Medical Letter: Antifungal drugs. Treatment
Voriconazole 6 mg/kg q12h for 2 days, then
Guidelines from The Medical Letter 2005;30:714.
4 mg/kg q12h (IV or oral) for 10 wk OR ONGOING CARE r Pappas PG, Rex JH, Sobel JD, et al. Guidelines for
Itraconazole 200 mg IV BID for 4 days, then
200 mg/d IV for 12 days, then 200 mg PO BID for PROGNOSIS treatment of candidiasis. Clin Infect Dis.
r Candiduria does not predict development of 2004;38:161189.
10 wk OR
Itraconazole 200 mg PO TID for 9 days, then candidemia in most people See Also (Topic, Algorithm, Media)
200 mg PO BID for 10 wk Rates 1.310.5% r Candidiasis, Cutaneous, External Genitalia
r Blastomycosis No different in renal transplant population: 5%
r Aspergillosis mortality 4090% with treatment
r Candiduria Algorithm F
Amphotericin B 0.51 mg/kg/d for 612 wk OR r Cryptococcus, Genitourinary
Fluconazole 400800 mg/d PO for 612 mo r Phycomycosis (mucormycosis, zygomycosis) r Filling Defect, Upper Urinary Tract (Renal Pelvis and
r Candidiasis mortality 90% if untreated, 24% with nephrectomy Ureter)
Fluconazole 150 mg in 1 dose and amphotericin B r Fungal Infections, Genitourinary Algorithm
r Candidemia (treat until 2 wk after afebrile and Cx r Fungal Infections, Genitourinary Image
COMPLICATIONS
negative): r Bezoar formation r Histoplasmosis, Genitourinary
Caspofungin 70 mg/d IV in 1 dose, then 50 mg/d r Emphysematous pyelonephritis r Urinary Tract Infection (UTI), Adult Female
OR r Obstruction, fungemia, death r Urinary Tract Infection (UTI), Adult Male
Amphotericin B 0.51 mg/kg/d r Papillary necrosis
r Candiduria: r Urinary Tract Infection (UTI), Pediatric
r Perinephric abscess
Amphotericin B 0.30.5 mg/kg/d IV for 12 wk r Renal scarring
OR
Flucytosine 25 mg/kg/d PO for 12 wk FOLLOW-UP CODES
r Coccidioidomycosis:
Patient Monitoring
Fluconazole 400800 mg/d PO for 1 yr OR r Surveillance cultures can be obtained to document ICD9
Amphotericin B 0.50.7 mg/kg/d IV for 1 yr r 112.1 Candidiasis of vulva and vagina
clearance of infection.
r Histoplasmosis: r Prostate can be fungal reservoir for recurrent r 112.2 Candidiasis of other urogenital sites
Fluconazole 400800 mg/d PO for 618 mo OR r 116.0 Blastomycosis
infection.
Amphotericin B 0.51 mg/kg/d IV for 1012 wk
Patient Resources ICD10
SURGERY/OTHER PROCEDURES CDC Fungal Infections Fact Sheet http://www. r B37.3 Candidiasis of vulva and vagina
r Obstructions from fungal bezoars require drainage. cdc.gov/ncezid/dfwed/PDFs/fungal-factsheet-508c.pdf r B37.4 Candidiasis of other urogenital sites
r Access to upper tracts can facilitate drainage, r B40.89 Other forms of blastomycosis
antifungal irrigation, and extraction if needed.
r Perinephric abscess can be drained percutaneously, REFERENCES
but may require operative drainage if multiple 1. Achkar JM, Fries BC. Candida infections of the CLINICAL/SURGICAL
loculations are present. genitourinary tract. Clin Microbiol Rev. 2010;23: PEARLS
r Severe aspergillus kidney infections may require 253273.
nephrectomy. r When fungal infections are found in the GU tract in
2. Kauffman CA, Fisher JF, Sobel JD, et al. Candida
r Treatment of fungal prostatitis may require surgical urinary tract infectionsdiagnosis. Clin Infect Dis. patients without risk factors, a search for systemic
2011;52:452456. disease is warranted.
intervention for prostate resection or drainage of r Disseminated fungal disease can seed the GU tract
abscess in addition to medical therapy (4)[B].
through the development of renal microabscesses.
r Fungal infections are encountered in varying
geographic locales based on type.
r Treat candiduria in infants with low birth weight,
those undergoing GU procedures, neutropenic
patients, and symptomatic patients.
r Surgical drainage of fungal infections is indicated in
cases of urinary tract obstruction.
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GLOMERULONEPHRITIS, ACUTE
Christopher E. Keel, DO
Raju Thomas, MD, MHA, FACS
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GLOMERULONEPHRITIS, ACUTE
Pathologic Findings
r IgG and C3 deposits are found at the capillary wall
SURGERY/OTHER PROCEDURES REFERENCES
Renal biopsy if indicated (see evaluation)
and in the mesangium on renal biopsy. 1. Tu WH, Shortliffe LD. Evaluation of asymptomatic,
r Rapidly progressive GN is characterized ADDITIONAL TREATMENT atraumatic hematuria in children and adults. Nat
pathologically by crescents forming from the cells of
Radiation Therapy Rev Urol. 2010;7(4):189194.
Bowman capsule. N/A 2. Lee MN, Shaih U, Butani L. Effect of
r Typically >50% of glomeruli should have crescents Additional Therapies overweight/obesity on recovery after
to be called rapidly progressive GN. This may result N/A post-infectious glomerulonephritis. Clin Nephrol.
from any of the immunologically mediated types of Complementary & Alternative 2009;71(6):632636.
GN, but most frequently occurs with antiglomerular Therapies
basement membrane disease, antineutrophil N/A
cytoplasmic antibody GN, and HenochSchonlein ADDITIONAL READING
purpura nephritis. r http://kidney.niddk.nih.gov/kudiseases/pubs/
ONGOING CARE
DIFFERENTIAL DIAGNOSIS glomerular/index.htm
r Anaphylactoid purpura PROGNOSIS r Kliegman RM. Nelson Textbook of Pediatrics.
r IgA nephropathy r Most patients have a complete recovery, with 18th ed. New York, NY: Saunders, 2007.
r Alports disease resolution of clinical signs within a few weeks. r Lau KK, Wyatt RJ. Glomerulonephritis. Adolesc Med
r Membranoproliferative glomerulonephritis r The reported incidence of chronic renal insufficiency Clin. 2005;16(1):6785.
r Other postinfective glomerulonephritis is 020%. r Wong W. Starship Childrens Health Clinical
r Infective endocarditis r Microscopic hematuria may persist for months up to Guidelines on Glomerulonephritis Acute, 2009
r Rapidly progressive glomerulonephritis 2 yr, and mild proteinuria may persist for years http://www.adhb.govt.nz/starshipclinicalguidelines/
r Systemic lupus erythematosus following an episode of poststreptococcal GN. Documents/Glomerulonephritis.pdf
COMPLICATIONS See Also (Topic, Algorithm, Media)
r Rarely does poststreptococcal GN progress to r Acute Kidney Injury, Adult (Renal Failure, Acute)
TREATMENT crescentic or rapidly progressive GN resulting in r Acute Kidney Injury, Pediatric (Renal Failure, Acute)
ESRD. Most cases resolve with no sequelae. Chronic r Glomerulonephritis, Chronic
GENERAL MEASURES renal failure or marked decline in glomerular
r Supportive care and reassurance.
r Monitor weight and serum sodium daily during filtration rate is very rare
r It is rare to result in severe HTN, seizures, anuria,
acute phase. hyperkalemia, or death.
CODES G
r Bed rest does not influence rate of recovery. r Hypertensive retinopathy or encephalopathy
r Antibiotics do not change the course of illness once r Rapidly progressive glomerulonephritis ICD9
r 446.21 Goodpastures syndrome
established but should be given to reduce r Microhematuria may persist for years
infection-related morbidity. r 580.4 Acute glomerulonephritis with lesion of
r Nephrotic syndrome (10%)
r Restrict protein until azotemia clears. rapidly progressive glomerulonephritis
r Obesity may increase risk for residual renal injury r 580.9 Acute glomerulonephritis with unspecified
MEDICATION pathological lesion in kidney
FOLLOW-UP
First Line
r Treatment is supportive for this condition and Patient Monitoring ICD10
r Subsequent urinalysis to ensure hematuria has r M31.0 Hypersensitivity angiitis
directed at the effects of renal insufficiency and HTN.
r Sodium and water restriction is indicated in patients resolved r N00.9 Acute nephritic syndrome with unsp
r Periodic BP monitoring
who show signs of fluid overload (400 mL/m2 /d). morphologic changes
r Loop diuretics, calcium channel blockers, and Patient Resources r N01.9 Rapidly progr nephritic syndrome w unsp
vasodilators are mainstays in the treatment of r National Kidney Foundation website morphologic changes
resultant HTN. http://www.kidney.org/atoz/content/glomerul.cfm
Furosemide 24 mg/kg/dose IV r MedlinePlus http://www.nlm.nih.gov/
Titrate dose based on clinical response. medlineplus/ency/article/000484.htm CLINICAL/SURGICAL
Second Line PEARLS
r Patients should be treated with a 10-day course of r Dysmorphic RBC on microscopic urinalysis suggest
penicillin antibiotics to prevent the spread of the the diagnosis.
nephritogenic organisms. This will not alter the r Prior pharyngitis or skin infection suggests diagnosis
course of the disease.
r Erythromycin is substituted if penicillin allergic. of acute glomerulonephritis.
r With supportive care, recovery is usually rapid and
r Family members of patients with acute GN should
complete with an excellent prognosis.
be cultured for group A -hemolytic streptococci
and treated if positive.
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GLOMERULONEPHRITIS, CHRONIC
Eric Langewisch, MD
John M. Barry, MD, FACS
164
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GLOMERULONEPHRITIS, CHRONIC
DIFFERENTIAL DIAGNOSIS ADDITIONAL TREATMENT 3. Appel GB. Glomerular disorders and nephrotic
r Aristocholic acid (for weight control) syndromes. In: Goldman L, Ausiello D, eds. Cecil
Radiation Therapy
r Chronic interstitial nephritis N/A Medicine. 23rd ed. Philadelphia, PA: Saunders
r Diabetic nephrosclerosis Elsevier; 2007; Chapter 122.
r Diuretic abuse
Additional Therapies
r Oral calcium supplements (1 g/d) and vitamin D 4. Peterson JC, Adler S, Burkart JM, et al. Blood
r Hypertensive nephrosclerosis (400800 IU/d) for prophylaxis against osteoporosis.
pressure control, proteinuria, and the progression
r Nephrotoxin exposure r Sodium bicarbonate has been shown to slow of renal disease. The Modification of Diet in Renal
r Obstructive uropathy Disease Study. Ann Intern Med. 1995;123(10):
progressive kidney damage. 754762.
r Prerenal disease
Complementary & Alternative
r Renal artery stenosis
Therapies
N/A ADDITIONAL READING
TREATMENT Kopp JB. Glomerular disease in 2012: More
ONGOING CARE mechanistic insights, but translational progress is
GENERAL MEASURES slow. Nat Rev Nephrol. 2013;9(2):6768.
r See GENERAL PREVENTION PROGNOSIS
r Referral to nephrology r Progression of glomerular disease to ESRD is See Also (Topic, Algorithm, Media)
r Treat specific glomerular disease (eg, prednisone or variable and dependent upon cause and response to Glomerulonephritis, Acute
other immunosuppressive agents) treatment
r Control blood pressure (4) r Prognosis has negative correlation with higher
r Renal replacement therapy may be necessary long blood pressure and degree of proteinuria CODES
term COMPLICATIONS
r ESRD ICD9
MEDICATION r 582.1 Chronic glomerulonephritis with lesion of
Uremia
First Line Volume overload membranous glomerulonephritis
r Angiotensin-converting enzyme inhibitors (ACEIs) r 582.89 Chronic glomerulonephritis with other
Hyperkalemia
and angiotensin receptor blockers (ARBs) slow the Anemia specified pathological lesion in kidney
decline of the glomerular filtration rate (GFR) in Acidosis r 582.9 Chronic glomerulonephritis with unspecified
patients with diabetic and nondiabetic proteinuric r Increased risk of cardiovascular disease pathological lesion in kidney
nephropathies
ACEIs: Benazepril, captopril, enalapril, fosinopril,
r Increased risk of mortality
ICD10
G
lisinopril, moexipril, quinapril, ramipril, others FOLLOW-UP r N03.2 Chronic nephritic syndrome w diffuse
ARB: Candesartan, eprosartan, irbesartan, Patient Monitoring membranous glomrlneph
losartan, telmisartan, valsartan r Lab monitoring r N03.9 Chronic nephritic syndrome with unsp
Use may be limited by drug-induced Estimate glomerular filtration rate (eGFR): BUN, morphologic changes
hyperkalemia, increased plasma creatinine due to plasma creatinine r N11.9 Chronic tubulo-interstitial nephritis,
decreased glomerular pressure, or anemia Basic metabolic panel + phosphorous unspecified
Second Line Random urine protein/creatinine ratio
r Diuretics to treat volume overload 24-hr urine protein
r Additional antihypertensive agents to reach blood r Blood pressure CLINICAL/SURGICAL
pressure goals r Signs or symptoms of uremia PEARLS
-Blockers, calcium channel blockers, central Patient Resources r Many cases of acute GN can progress to chronic GN.
2-agonists (eg, clonidine), 1-antagonists, and www.kidney.org/patients r ACEIs and ARB can slow the decline of the GFR in
direct vasodilators
patients with proteinuric nephropathies.
SURGERY/OTHER PROCEDURES
r Access for dialysis REFERENCES
AV fistula or graft 1. National Kidney Foundation. K/DOQI clinical
Hemodialysis access or peritoneal dialysis catheter practice guidelines for chronic kidney disease:
r Renal transplantation
Evaluation, classification, and stratification. Am J
Preemptive transplantation before dialysis results Kidney Dis. 2002;39(2 suppl 1):S1S266.
in better survival than transplantation after the 2. Remuzzi G, Bertani T. Pathophysiology of
initiation of dialysis progressive nephropathies. N Engl J Med.
1998;339(20):14481456.
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GONORRHEA
Arpeet Shah, MD
Ahmer V. Farooq, DO
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GONORRHEA
SURGERY/OTHER PROCEDURES
r Chronic gonorrheal infection may lead to bulbar
REFERENCES
TREATMENT
urethral strictures requiring urologic intervention 1. Klausner J, Hook III E. Current Diagnosis and
GENERAL MEASURES r Gonorrheal abscesses may require incision and Treatment of Sexually Transmitted Diseases, 1st ed.
r Patients with suspected active infection should debridement procedures New York, NY: McGraw-Hill Medical, 2007.
abstain from sex until diagnostically excluded or 2. Leone PA. Epidemiology, pathogenesis, and clinical
adequately treated ADDITIONAL TREATMENT manifestations of neisseria gonorrhoeae infection.
r Patient counseling regarding safe sex practices and
r All sexual partners who have contacted the infected In: UpToDate, Basow DS, ed. UpToDate. Waltham,
abstinence for 7 days following treatment initiation MA; 2013.
patient within 60 days of diagnosis should also be r Patients should also be offered additional STD
evaluated 3. Mandel G, et al. Bennetts Principles and Practice
r Treatment with penicillins and tetracycline are not testing and pregnancy testing of Infectious Diseases. 7th ed. New York, NY:
r Pregnancy considerations
effective due to the high level of penicillinase- Churchill Livingstone; 2010.
producing bacteria and plasmid-mediated high-level 1st line still remains ceftriaxone 250 mg IM in 1 4. Swygard H, et al., Diagnosis of Gonococcal
tetracycline-resistant bacteria dose PLUS azithromycin 1 g PO in 1 dose Infections. In: UpToDate, Basow DS, ed. UpToDate,
r Over the last decade, increasing mean minimum Doxycycline should be avoided during pregnancy Waltham, MA; 2013.
If the patient has a penicillin allergy,
inhibitory concentrations of selective cephalosporins
desensitization procedures or 2nd-line treatments
have indicated decreasing susceptibility and have
such as azithromycin monotherapy are alternatives ADDITIONAL READING
impacted current treatment recommendations
r Fluoroquinolone resistance has impacted treatment Microbiologic test of cure with culture is
recommended www.cdc.gov/std/Gonorrhea/STDFact-gonorrea.htm
options and is most prevalent in the states of r Ophthalmia neonatorum
California and Hawaii See Also (Topic, Algorithm, Media)
r Macrolide resistance has also been reported Prevented by routine screening for endocervical r Epididymitis
infection during pregnancy and prophylactic use of r Gonorrhea Image
MEDICATION erythromycin ophthalmic solution r Pelvic pain, Female
First Line (4) r Sexually Transmitted Infections (STIs) STDs, General
r For uncomplicated cases of urethral and
endocervical gonorrheal infection, patients must
ONGOING CARE r Urethra, Discharge
r Urethritis
also be treated for concomitant chlamydia infection PROGNOSIS
unless diagnostically excluded >95% of uncomplicated genitourinary gonorrheal
r Ceftriaxone 250 mg IM in 1 dose PLUS azithromycin
1 g PO in 1 dose is the current gold standard
infections are cured by 1 course of treatment
CODES G
r Ceftizoxime 500 mg IM in 1 dose, cefotaxime 500 COMPLICATIONS
r In males, may lead to bulbar urethral stricture and
mg IM in 1 dose, or cefoxitin 2 g IM with probenecid ICD9
sterility r 098.0 Gonococcal infection (acute) of lower
1 g PO in 1 dose are alternatives for ceftriaxone r In females, can cause PID leading to chronic pelvic
r If an injectable cephalosporin is not an option, genitourinary tract
pain, ectopic pregnancy, and sterility r 098.11 Gonococcal cystitis (acute)
alternatives include cefixime 400 mg PO in 1 dose or r Genital abscesses may occur in either sex requiring r 098.12 Gonococcal prostatitis (acute)
cefpodoxime 400 mg PO in 1 dose. However,
patients who receive these options should return in surgical intervention
r FitzHughCurtis Syndrome perihepatitis ICD10
1 wk for microbiologic test of cure with culture r A54.00 Gonococcal infection of lower genitourinary
r Doxycycline 100 mg BID PO for 7 days is an characterized by acute right or bilateral upper
quadrant tenderness may occur in either sex tract, unsp
alternative for azithromycin r Ocular infection with gonorrhea in adults may lead r A54.01 Gonococcal cystitis and urethritis,
Second Line to corneal scarring and vision loss unspecified
r The management of those with a penicillin allergy r The most common complication of DGI is septic r A54.22 Gonococcal prostatitis
depends on clinical suspicion of true allergy and the arthritis and arthritisdermatitis syndrome; extreme
severity of the allergy. Most patients with cases may lead to destruction of articular surfaces
documented penicillin allergy are not found to have r Hematogenous spread may lead to endocarditis and CLINICAL/SURGICAL
an allergy after further testing and only 2% of those PEARLS
meningitis
with a penicillin positive skin test cross react with
cephalosporins. Thus, the physician must decide FOLLOW-UP r Maintain a high degree of suspicion for gonorrhea,
whether to give a cephalosporin vs. an alternative r All patients diagnosed with gonorrhea should be especially in patients who are in their 20s.
therapy tested to rule out repeat infection 34 mo after r Most common symptoms include mucopurulent
r Azithromycin 2 g PO in 1 dose monotherapy treats treatment discharge and dysuria.
gonorrhea and chlamydia; however, due to GI side r All patients who undergo PO cephalosporin therapy r Culture has been the gold standard for diagnosis,
effects and growing macrolide resistance, it is not a and all pregnant patients should undergo however, NAAT is now being widely used as a
preferred regimen unless the patient has a severe microbiologic test for cure using a Gram stain and 1st-line diagnostic modality.
penicillin allergy culture 7 days after treatment r 1st-line treatment includes ceftriaxone 250 mg IM in
r Spectinomycin 2 g IM in 1 dose is a safe and
1 dose PLUS azithromycin 1 g PO in 1 dose.
effective alternative therapy for those with severe r Antibiotic susceptibilities continue to change and
penicillin allergies, but is only available outside the vary by geographical location.
United States r Always council patients regarding safe sex practices.
r Quinolones were once a 2nd-line therapy, but due
to drug resistance in 10100% of strains depending
on location, they are no longer recommended for
the treatment of gonorrhea
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GYNECOMASTIA
Samuel Walker Nickles
James S. Rosoff, MD
r GM of aging:
BASICS DIAGNOSIS The hypothalamicpituitarytestis axis is variable
in age-related decline. Some men will have
DESCRIPTION HISTORY elevated gonadotropins while others will be
r Gynecomastia (GM) is benign enlargement of the r Age of patient and onset of symptoms (pubertal,
normal.
male breast due to proliferation of ductal elements. GM of aging) Adiposity increases with age which leads to
r Pseudogynecomastia/lipomastia is an increase in r Associated fevers or chills, breast trauma, nipple
increasing peripheral conversion.
breast adipose tissue. This can be distinguished by discharge Sex hormonebinding globulin (SHBG) levels rise
careful physical exam of subareolar tissue and r Medical conditions (cirrhosis, chronic kidney disease, with age and decreasing bioavailable
comparison to adjacent adipose tissue. HIV, hyperthyroidism) testosterone.
r Medications/drugs Medications may also play a part in GM in older
EPIDEMIOLOGY r History of cryptorchidism men.
Incidence (1) r Sexual history: Sexual maturation, changes in libido, r Estrogen secreting tumors:
Approximately 2,000 cases of male breast cancer are Leydig cell tumors are rare tumors of the testis;
diagnosed in the United States annually erectile dysfunction, infertility
8590% are benign, most are nonpalpable. Some
Prevalence PHYSICAL EXAM Leydig cell tumors can directly secrete estradiol.
r 3065% of men have palpable breast tissue and at r General appearance, weight, amount of adipose
This increases estrogen levels and inhibits LH
autopsy 4055% of men have histologic evidence tissue (contains aromatase capable of peripheral secretion, suppressing testicular production of
of GM conversion of androgens to estrogen) testosterone.
r Age related: Asymptomatic GM is 6090% in r Secondary sexual characteristics such as body hair
Sertoli cell tumor: Converts androgens to
neonates, 5060% in adolescents, and up to 70% distribution and phallus size estrogens leading to a direct increase in
r Thyroid exam circulating levels of estrogens.
in men aged 5069 yr
r Breast exam: Special attention should be paid to Feminizing adrenal cortical tumors are generally
RISK FACTORS distinguish true GM from pseudogynecomastia; malignant and poorly differentiated. These cancers
r Alcoholism
r Endocrinopathies unilateral vs. bilateral (if unilateral should be directly secrete estrogens as well as steroid
concerned for potential male breast cancer), firm precursors that may be aromatized to estrogens in
r Medications
and mobile vs. fixed, skin dimpling, any nipple peripheral tissues. Increased estrogen suppresses
r Obesity discharge, palpation of axillary lymph nodes LH-mediated production of testosterone as well.
r Renal failure r Genitourinary exam with special attention to the r hCG-secreting tumor such as choriocarcinoma
Genetics testicular exam stimulates Leydig cells to preferentially secrete
r Klinefelter syndrome (47, XXY) is strongly associated estradiol. Many HCG-secreting tumors also will take
DIAGNOSTIC TESTS & INTERPRETATION
with GM up steroid precursors such as DHEA and convert
r An increased risk of male breast cancer has been
Lab them to active estrogens.
r Basic studies: Creatinine, LFTs, thyroid function
r Increased peripheral aromatization to estrogens:
reported in families with a BRCA2 mutation tests, serum testosterone
r Further testing as needed: Familial aromatase excess syndrome. The enzyme
PATHOPHYSIOLOGY aromatase (P450 aroma or CYP19A1) catalyzes the
r Male breast tissue has both androgen and estrogen Serum estrogens (estradiol, estrone) conversion of steroid precursors to estrogens.
receptors. LH, FSH, prolactin r Estrogen receptor agonists:
r Androgens inhibit breast development and Tumor markers: AFP, -hCG
Therapeutic administration of estrogens such as
estrogens stimulate it. GM develops when there is Adrenal androgens, serum DHEA, urinary
DES (diethylstilbestrol) may be used to treat men
an imbalance of these two influences (ie, androgen 17-ketosteroids
with prostate cancer and can lead to GM.
deficiency or excess estrogen) or lack of tissue Imaging Estrogens may also be used to stimulate breast
response to them. r Testicular US if abnormal tumor markers development in male-to-female transsexuals.
r CT of the abdomen and pelvis/chest if abnormal Unintentional exposure may occur
ASSOCIATED CONDITIONS
r Prostate cancer levels of adrenal androgens transcutaneously by sexual intercourse with a
r Testicular tumors r Mammography if cancer suspected partner that uses topical estrogen. Occupational
r Cirrhosis exposure is also possible. Estrogens can be found
Diagnostic Procedures/Surgery in hair creams, embalming creams, and in the
r Renal failure Breast biopsy for suspected breast cancer
production of medicinal estrogen products.
GENERAL PREVENTION Pathologic Findings Marijuana smoke, digitoxin, testosterone, or other
r Proliferation of ductules embedded in a connective aromatizable androgens.
With hormonally induced GM, prophylactic breast
irradiation may reduce GM tissue stroma r Androgen deficiency or resistance:
r Over about 12 months, the breast tissue evolves into Primary or secondary hypogonadism: Testicular
a quiescent stage, in which the amount of stroma failure from any cause may result in GM.
and fibrosis increases and the ductules become less Testosterone deficiency leads to elevated LH,
prominent. glandular acini are rare (2) which increases estradiol production by remaining
DIFFERENTIAL DIAGNOSIS Leydig cells. Increased estrogens lead to elevated
r Physiologic GM: Normal in neonatal boys secondary levels of SHBG, further decreasing free
to maternal estrogen exposure. testosterone.
Occurs in 6090% of neonatal boys and resolves Klinefelter syndrome is the most common genetic
within several weeks after delivery. disorder associated with hypogonadism and
Pubertal GM results from the earlier rise of infertility in men. GM is present in 5070% of
estrogens in early puberty. As the normal ratio of cases. Klinefelter syndrome is the only cause of
estrogen to testosterone is restored later in GM with an established risk of breast cancer
puberty the GM resolves. (20-fold increase).
5070% of boys develop GM during puberty. Defects in genes critical for testosterone
20% of men still have GM at 20 yr of age. production may also lead to decreased
testosterone production.
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GYNECOMASTIA
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HEMATOSPERMIA
Robert L. Segal, MD, FRCS(C)
Arthur L. Burnett, II, MD, MBA, FACS
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HEMATOSPERMIA
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r If intractable GH despite all other measures consider REFERENCES See Also (Topic, Algorithm, Media)
formalin bladder instillation (6) r Cystitis, Hemorrhagic (Infectious, Noninfectious,
Performed under anesthesia 1. Grossfeld GD, Litwin MS, Wolf JS Jr. Evaluation of Radiation)
Must rule out vesicoureteric reflux asymptomatic microscopic hematuria in adults: The r Glomerulonephritis, Acute
1stcontraindicated if positive American Urological Association best practice r Glomerulonephritis, Chronic
Side effects: Renal failure, bladder policypart II: Patient evaluation, cytology, voided r Hematuria, Athletic (Runners Hematuria)
contracture/decreased capacity, incontinence, markers, imaging, cystoscopy, nephrology r Hematuria Adult Algorithm
ureteral stenosis evaluation, and follow-up. Urology. 2001;57: r Hematuria, Gross and Microscopic, Pediatric
r For life-threatening hemorrhagic cystitis or 604610.
r Hematuria, Traumatic Algorithm
recurrent/refractory hemorrhagic cystitis stabilize 2. Sutton JM. Evaluation of hematuria in adults.
r Hematuria-Dysuria Syndrome
patient then consider JAMA. 1990;263:24752480.
r Hematuria-Loin Pain Syndrome
Unilateral selective arterial embolization 3. Khadra MH, Pickard RS, Charlton M, et al. A
Urinary diversion with or without cystectomy prospective analysis of 1,930 patients with r Urine, Abnormal Color
hematuria to evaluate current diagnostic practice.
ADDITIONAL TREATMENT
J Urol. 2000;163:524527.
Radiation Therapy 4. Grossfeld GD, Litwin MS, Wolf JS. Evaluation of CODES
N/A asymptomatic microscopic hematuria: The
Additional Therapies American Urological Association best practice ICD9
N/A policy part I: Definition, detection, prevalence, r 599.0 Urinary tract infection, site not specified
and etiology. Urology. 2001;57:599603. r 599.71 Gross hematuria
Complementary & Alternative
Therapies 5. Davis R, Jones JS, Barocas DA, et al. Diagnosis, r 599.72 Microscopic hematuria
Hyperbaric oxygen therapy (HBO) has been shown to evaluation and follow-up of asymptomatic
be effective in hematuria caused by radiation-induced microhematuria (AMH) in adults: AUA guideline. ICD10
J Urol. 2012;188:24732481. r R31.0 Gross hematuria
cystitis if delivered within 6 mo of initiation of r R31.2 Other microscopic hematuria
hematuria 6. Abt D, Bywater M, Engeler DS, et al. Therapeutic
options for intractable hematuria in advanced r N39.0 Urinary tract infection, site not specified
bladder cancer. Int J Urol. 2013;20:651660.
ONGOING CARE
CLINICAL/SURGICAL
PROGNOSIS
Based on etiology of the hematuria ADDITIONAL READING PEARLS
r Corman JM, McClure D, Pritchett R, et al. Treatment r Gross or microscopic hematuria in any patient
COMPLICATIONS
Hypotension and anemia may result on degree and of radiation induced hemorrhagic cystitis with should be evaluated, especially when significant risk
chronicity of blood loss hyperbaric oxygen. J Urol. 2003;169:22002202. factors are present (age >35, smoking history,
r Sieber PR, Rommel FM, Huffnagle HW, et al. The exposure to chemicals/dyes, irritative voiding
FOLLOW-UP
Patient Monitoring
treatment of gross hematuria secondary to prostatic symptoms).
r Risk of urologic malignancy is 5 times higher in
H
bleeding with finasteride. J Urol. 1998;159:
r Monitor hemodynamic status if severe gross
12321233. patients who present with gross hematuria.
hematuria persists or if associated with trauma r Cytology is recommended for patients with risk
Serial hemoglobin and hematocrit factors; however, a negative result does not rule out
Patient Resources malignancy.
r Hematuria: Blood in the Urine National Kidney r CTU is the imaging test of choice for evaluating
and Urologic Diseases Information Clearinghouse hematuria from the upper tract.
(NKUDIC). http//kidney.niddk.nih.gov/kudiseases/ r Cystoscopy should be performed on any patient
pubs/hematuria >35 yr of age presenting with unexplained MH
r Urology Care Foundation. http://www.
or GH.
urologyhealth.org/urology/index.cfm?article=113
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r Due to the sloughing of necrotic tissue in prostatic DIAGNOSTIC TESTS & INTERPRETATION
BASICS fossa or bleeding at the bladder neck Lab
r Size of tissue resected, duration of resection, or r CBC to assess for anemia
DESCRIPTION r Creatinine level for obstruction
presence of prostate cancer does not correlate with
Significant gross hematuria with or without clot r Urinalysis, urine culture
the incidence of hematuria post-TURP (1)[B]
retention that occurs following transurethral resection r Coagulopathy screen (platelets, PT/PTT) particularly
r Studies suggest that there is transient change in
of the prostate (TURP) or transurethral resection of
bladder tumor (TURBT) platelet count, prothrombin time, and fibrinogen if there is suggestion of bleeding from other sites
and serum sodium concentrations postoperatively, Imaging
EPIDEMIOLOGY which can be explained on the basis of dilution of Bladder US or pelvic CT to evaluate for large organized
r Occurs in up to 11% of patients, typically within the blood clot within bladder
the 1st 3 mo after TURP (1)[B] r Prostate cancer is known to trigger disseminated
r TURP is associated with a 2.9% transfusion rate Diagnostic Procedures/Surgery
intravascular coagulation (DIC), and this should be Bladder drainage and irrigation with large-caliber
(2)[B] kept in mind when performing resection in the face hematuria catheter
r 2.23.3% of patients require recatheterization, of known advanced prostate cancer
clot evacuation, or return to OR for bleeding after r In the absence of prostate cancer, up to 6% of DIFFERENTIAL DIAGNOSIS
r Bleeding from lower GU tract source: Urethra,
TURP (3)[A] patients undergoing TURP may develop mild
subclinical intravascular coagulopathy prostate, bladder
RISK FACTORS r Urinary fibrinolysis is a normal physiologic process. r Bleeding from upper GU tract source: Ureter, renal
r Excessive Valsalva/straining/constipation
Plasminogen is converted to plasmin by pelvis, kidney
r Inadequate hemostasis/coagulation of bleeding
plasminogen activators
vessels r The presence of a clot in the bladder causes the
r Infection TREATMENT
r Medications: Warfarin, heparin, low molecular release of additional plasminogen activators.
Evacuation of clot in the bladder is essential to GENERAL MEASURES
weight heaprins, aspirin, thienopyridine stopping the bleeding r Limit physical activity, encourage bed rest
(clopidogrel), etc. r Limit Valsalva and avoid constipation through stool
r Trauma ASSOCIATED CONDITIONS
r Undermining of bladder neck r BPH softeners
r Bladder cancer r Adequate hydration; IV fluid resuscitation
Genetics r Prostate cancer r Bladder drainage and clot evaluation with
Patients with deficiencies in the clotting cascade (eg, large-caliber hematuria catheter
hemophilia) or other coagulopathies are more prone r Continuous bladder irrigation (CBI) via 3-way Foley
to hemorrhage. DIAGNOSIS catheter to clear clots and prevent new clots from
GENERAL PREVENTION HISTORY forming in the bladder
r Obtain sufficient hemostasis intraoperatively r Color of urine, presence of clots r Foley traction, additional inflation of Foley balloon
r Stop anticoagulants or other blood-thinning r Patient is not able to void (clot retention) r Cessation of anticoagulants or blood-thinning
medications prior to surgery r History of TURP TURBTtiming, complications, medications
r Delay starting anticoagulant medications r Check CBC and coagulation profile
catheter removal
r Use of anticoagulation or similar medications r PRBC transfusions if necessary, vitamin K and/or FFP
postoperatively if possible, although this practice
has been questioned (4)[B] r Excessive straining or trauma; last bowel movement if coagulopathic
r Gentle postoperative catheter traction r History of clotting disorder r CBI with intravesical alum or silver nitrate
r History of prostate cancer r These are reported but rarely necessary:
r 5-reductase inhibitors, taken pre operatively
Hyperbaric oxygen
reduce surgical blood loss intraoperatively (5)[A] PHYSICAL EXAM Aminocaproic acid (Amicar) antifibrinolytic
r 5-reductase inhibitors, do not decrease rates of r General: Pallor, dehydrated, acutely ill
Hormonal manipulation: LHRH agonists
postoperative clot retention (6)[A] r Vitals: Hypotensive or tachycardic Urinary diversion with bilateral PCNs
r Abdomen: Bladder distended or palpable Salvage radical prostatectomy
PATHOPHYSIOLOGY r Genitalia: Edematous; ecchymotic
r Anesthetic technique (regional or general) appears Selective arterial prostatic embolization (SAPE)
to have no impact on TURP-related bleeding (8)[A]
r Inadequate hemostasis/coagulation of bleeding
vessels
r Narcotics may cause constipation and increased
intra-abdominal pressure
r NSAIDs are not contraindicated after TURP, they
do not increase risk of postoperative adverse
events (7)[A]
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Patient Resources
N/A
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r Episodic therapy for recurrent genital herpes (2) r HIV transmission 3. Bryson YJ, Dillon M, Lovett M, et al. Treatment of
Requires initiation of therapy within 1 day of When the sores come into contact with the first episodes of genital herpes simplex virus
lesion onset or during the prodrome that precedes mouth, vagina, or rectum during sex, they infection with oral acyclovir: A randomized
some outbreaks. increase the risk of HIV transmission if either double-blind controlled trial in normal subjects. N
Provide patient with a supply of drug or a partner is HIV-infected. Engl J Med. 1983;308:916921.
prescription for the medication with instructions r Some HSV-infected persons might express anxiety 4. Mattison HR, Reichman RC, Benedetti J, et al:
to initiate treatment immediately when symptoms concerning genital herpes that does not reflect the Double blind placebo controlled trial comparing
begin. actual clinical severity of their disease; the long term suppressive with short term oral acyclovir
Acyclovir 400 mg TID for 5 days OR 800 mg PO psychological effect of HSV infection frequently is therapy for management of recurrent genital
BID for 5 days OR 800 mg PO TID for 2 days substantial. herpes. Am J Med. 1988;85:2025.
Famciclovir 125 mg PO BID for 5 days OR
1,000 mg PO BID for 1 day OR 500 mg once, FOLLOW-UP
followed by 250 mg BID for 2 days Patient Monitoring ADDITIONAL READING
Valacyclovir 500 mg PO BID for 3 days OR 1 g r Patients education concerning the natural history of
PO QD for 5 days the disease, potential for recurrent episodes, Hofstetter AM, Rosenthal SL, Stanberry LR. Current
asymptomatic viral shedding, and the risks of sexual thinking on genital herpes. Curr Opin Infect Dis.
SURGERY/OTHER PROCEDURES transmission. 2014;27(1):7583.
r Sitz baths r At 1st episode of genital herpes, advise the patient
r Foley catheter for retention of urine associated with See Also (Topic, Algorithm, Media)
that suppressive therapy is available and effective in r Aphthous Ulcer, External Genitalia
sacral nerve root involvement preventing symptomatic recurrent episodes r Genital Ulcers
ADDITIONAL TREATMENT r Encourage patients to inform their current sex
r Genital Ulcers Algorithm
Radiation Therapy partners that they have genital herpes and to inform r Herpes Simplex, Genital Image
N/A future partners before initiating a sexual
r Penis, Cutaneous Lesion
relationship.
Additional Therapies r The risk for HSV-2 sexual transmission can be r Sexually Transmitted Infections (STIs) (Sexually
r In complicated HSV infection (central nervous
decreased by the daily use of valacyclovir by the Transmitted Diseases [STDs]), General
system disease, disseminated HSV), the Centers for infected person. Episodic therapy does not reduce
Disease Control recommend intravenous acyclovir the risk for transmission and its use should be
(510 mg/kg) every 8 hr for 27 days or until clinical discouraged for this purpose among persons whose CODES
improvement partners might be at risk for HSV-2 acquisition (4).
r Suppressive or episodic therapy with oral antiviral r Symptomatic sex partners should be evaluated and ICD9
agents is effective in decreasing the clinical treated in the same manner as patients who have r 054.10 Genital herpes, unspecified
manifestations of HSV among HIV-positive persons. genital lesions. Asymptomatic sex partners of r 054.11 Herpetic vulvovaginitis
Complementary & Alternative patients who have genital herpes should be r 054.19 Other genital herpes
Therapies questioned concerning histories of genital lesions
None noted to be effective and offered type-specific serologic testing for HSV ICD10
r A60.00 Herpesviral infection of urogenital system,
H
infection.
unspecified
ONGOING CARE Patient Resources r A60.04 Herpesviral vulvovaginitis
http://www.cdc.gov/STD/Herpes/
PROGNOSIS r A60.9 Anogenital herpesviral infection, unspecified
r Symptoms may last 24 wk if untreated
r Symptoms less severe in nonprimary compared to REFERENCES
person without pre-existing HSV immunity
CLINICAL/SURGICAL
1. Tobian AA, Kacker S, Quinn TC. Male Circumcision:
r Treatment during primary infection lessens A globally relevant but under-utilized method for
PEARLS
morbidity (1)[A] the prevention of HIV and other sexually r It is estimated that 1 in 5 adults in US is infected
transmitted infections. Annu Rev Med. 2014;65: with HSV, but that many are asymptomatic and do
COMPLICATIONS 293306.
r Aseptic meningitis not know that they are infected with the virus.
r Encephalitis 2. MMWR. December 17, 2010, Vol 59, no. RR-12. r Most infected individuals have recurrent episodes of
r Transverse myelitis Available at http://www.cdc.gov/std/treatment/ painful genital ulcers.
2010/STD-Treatment-2010-RR5912.pdf. Accessed r The 1st episode usually occurs a few weeks
r Hepatitis
January 3, 2014. following initial infection with the virus and may last
r Pneumonitis
r Disseminated HSV 23 wk.
r HSV recurrences generally decrease in frequency
over time.
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PATHOPHYSIOLOGY
ALERT r HIV-1 binds to cells expressing CD4, leading to DIAGNOSIS
The American Urological Association (AUA) policy decline in CD4 cells and immune function.
statement now considers circumcision to be of a r Immunosuppression allows opportunistic/unusual HISTORY
health benefit, citing a 5060% risk reduction in infections, decreases host defense against r Voiding history (1)[A]
HIV transmission in some African nations. malignancy. Dysuria
Frequency
ASSOCIATED CONDITIONS
r UTI Incontinence
BASICS Urethral discharge
Greater if CD4 count <500/mm3
Pelvic or testicular pain
DESCRIPTION Associated with typical bacteria (Escherichia coli,
r HIV disease results from the acquired deficiency of Flank pain
Enterococcus) and atypical pathogens such as
fungi, mycobacteria, and viruses r Neurologic history
cellular immunity caused by the human
immunodeficiency virus (HIV). r Epididymitis/orchitis Numbness
Hallmarks Chlamydia, gonorrhea, salmonella, toxoplasmosis Dysesthesias
Reduction of the helper T-lymphocytes in the r Fournier gangrene r Social history
blood and the lymph nodes r Prostatitis Sexual history
Development of opportunistic infections Up to 14% in patients with AIDS IV drug use
(Pneumocystis carinii pneumonia, Greater risk in AIDS patients for developing Blood product transfusions
cytomegalovirus infections, tuberculosis, r Generalized lymphadenopathy, fever, weight loss,
prostatic abscess
candida infections, cryptococcosis, others) r Urolithiasis and chronic diarrhea are common symptoms.
Development of malignant neoplasms r Review of systems (ROS): Constitutional symptoms,
Risk with use of indinavir or from metabolic
(non-Hodgkin lymphoma and Kaposi sarcoma) abnormalities skin lesions, confusion, urticaria
r A spectrum of HIV infections range from r Hepatitis B virus (HBV)
PHYSICAL EXAM
asymptomatic seropositivity to AIDS r Malignancies r General: Skin lesions, adenopathy
r Urologic manifestations of HIV/AIDS
Non-Hodgkin lymphoma r Neurologic exam: Numbness, alterations in
Bacterial and nonbacterial infections Usually B cell sensation
Urolithiasis May involve kidneys in 612% of AIDS patients r GU exam: Urethral discharge, testicular/epididymal
Increased risk of malignancy Kaposi sarcoma
Renal impairment exam for masses, prostate exam for nodule or
Up to 20% of untreated patients tenderness
Voiding dysfunction Testicular tumors r Penile lesions of Kaposi sarcoma present as
EPIDEMIOLOGY Usually seminoma
red/brown/purple nodules, macules, or patches
Up to 50 times more common
Incidence
4050,000 new cases per year in US Renal cell carcinoma DIAGNOSTIC TESTS & INTERPRETATION
Up to 8-fold increased risk vs. noninfected Lab
Prevalence individuals r HIV testing
r 0.5% of US adults <50 are infected r HIV associated nephropathy (HIVAN) Screening HIV-1 antibody titer
2.6% of African American men and 1.5% of Proteinuria >3.5 g/d, edema, and HTN If positive, need confirmation by Western blot or
African American women were HIV positive from Associated with focal segmental immunofluorescence.
1999 to 2006 glomerulosclerosis (FSGS) on renal biopsy Need separate consent for HIV testing.
r 33.2 million people worldwide with HIV/AIDS r UA, urine C+S
Progression to dialysis in <10 mo.
r 2.1 million deaths due to HIV/AIDS in 2007 r Voiding dysfunction May show rectangular crystals from indinavir
RISK FACTORS Can be retention, detrusor overactivity, and Common bacterial pathogens in HIV-infected
r Unprotected intercourse, anal or oral sex sphincter dyssynergia patients are E. coli, Enterobacter (enterococci),
r IV drug abuse and needle sharing Pseudomonas aeruginosa, Proteus spp., Klebsiella,
GENERAL PREVENTION Acinetobacter, Staphylococcus aureus, group D
r Transfusion of blood products r Barrier protection during sex (male and female
r Concomitant STD/STI Streptococcus, Serratia, and Salmonella spp.
condoms) If UTI suspected and C&S negative, consider
r Uncircumcised phallus Lab studies indicate that the female condom is an atypical organisms: Fungi, parasites, viruses
r Transmission of mother to infant at birth or via effective mechanical barrier to viruses, including r CBC
breast milk HIV, and to semen. r BUN/creatinine
r Health care workers r Avoid high-risk sexual behavior
r Specific testing for STD if urethral discharge present
r Male circumcision
Risk for HIV after percutaneous exposure to HIV
infected blood is 0.3%; after mucous membrane Although male circumcision should not be Imaging
substituted for other HIV risk-reduction strategies, r With flank pain: Noncontrast CT
exposure 0.09%.
it has been shown to reduce the risk for HIV and Indinavir stones may not show on CT: Consider
Genetics some STDs in heterosexual men. contrast study or retrograde pyelogram if renal
r 3 groups of HIV viruses: M, N, and O
Despite these data, male circumcision has not impairment.
Most infections are by class M been demonstrated to reduce the risk for HIV or r Scrotal US for palpable lesions
9 subtypes of M exist r Prostate abscess: CT scan
other STDs among men who have sex with men.
1520% genetic variation between viruses r Use of precautions by health workers
r Treating STDs
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Diagnostic Procedures/Surgery
r Measure PVR urine
SURGERY/OTHER PROCEDURES
r Indinavir and other protease inhibitor (PI) stones
REFERENCES
r Urodynamics for voiding dysfunction or retention. Stop indinavir 1. Breyer BN, Van den Eeden SK, Horberg MA, et al.
Distinguishes bladder outlet obstruction from Hydration HIV status is an independent risk factor for
acontractile bladder if in retention Stent if necessary reporting lower urinary tract symptoms. J Urol.
Bladder hypocontractility was seen in 3545% Stones are soft and may pass after stenting. 2011;185(5):17101715.
r Surgical drainage of prostatic abscess 2. Lebovitch S, Mydlo JH. HIV-AIDS: Urologic
at time of urinary retention (2)[A]
r Stenting for ureteral obstruction from considerations. Urol Clin North Am. 2008;35(1):
Common urodynamic findings: 5968.
Hypo- and hyperreflexia retroperitoneal NHL
3. von Wyl V, Yerly S, Boni J, et al. Emergence of
Acontractile hypoactive bladder ADDITIONAL TREATMENT HIV-1 drug resistance in previously untreated
Detrusor-sphincter dyssynergia Radiation Therapy patients initiating combination antiretroviral
Pathologic Findings Indicated in some cases of focal Kaposi sarcoma treatment: A comparison of different regimen
r Testicular tumors: Usually seminoma Additional Therapies types. Arch Intern Med. 2007;167:17821790.
r Lymphoma: B-cell non-Hodgkin lymphoma (NHL) r For health care worker exposure
r Penile lesions: Kaposi sarcoma from lymphatic r Post-Exposure Prophylaxis (PEP)
endothelial cells vs. squamous cell carcinoma Occupational PEP (oPEP), healthcare worker ADDITIONAL READING
potentially exposed to material infected with HIV r Cohen MS, Hellmann N, Levy JA, et al. The spread,
DIFFERENTIAL DIAGNOSIS
r Other systemic disease that cause fatigue: Chronic Non-occupational PEP (nPEP), someone is
treatment and prevention of HIV-1: Evolution of a
potentially exposed to HIV outside the workplace
fatigue syndrome, others global pandemic. J Clin Invest. 2008;118:
r Salmonella epididymitis pathognomonic for HIV (eg, from sexual assault, unprotected sex,
12441254.
needle-sharing injection drug use). r Izzedine H, Lescure FX, Bonnet F, et al. HIV
begin within 72 hrs of exposure; 23 antiretorviral
medications for 28 days medication-based urolithiasis. Clin Kidney J.
TREATMENT r Pre-Exposure Prophylaxis (PrEP) 2014;7(2):121126.
r Millett GA, Flores SA, Marks G, et al. Circumcision
GENERAL MEASURES For people who are HIV-negative and at
r Refer to neurology, nephrology, infectious diseases substantial risk for HIV infection (relationship with status and risk of HIV and sexually transmitted
HIV infected partner, gay or bisexual man who has infections among men who have sex with men: A
when appropriate
r Patient education about risk factors, transmission had sex without a condom or been diagnosed meta-analysis. JAMA. 2008;300:16741684.
r www.aidsinfo.nih.gov (Accessed August 11, 2014)
with a sexually transmitted infection within the
MEDICATION r www.cdc.gov/hiv/resources/factsheets/index.htm
past six months, others
First Line Along with other prevention methods like (Accessed August 11, 2014)
r Highly active antiretroviral therapy (HAART)
condoms, PrEP can offer good protection against See Also (Topic, Algorithm, Media)
Combination therapy to combat the ability of HIV HIV if taken daily r HIV/AIDS, Urologic Considerations Image
to generate drug-resistant mutants (3)[A]. r Kaposi Sarcoma, Urologic Considerations
10 million people now on antiretroviral therapy
Complementary & Alternative
Therapies r Sexually Transmitted Infections (STIs) (Sexually
H
according to the WHO None Transmitted Diseases [STDs]), General
HIV therapy should be started in patients with: r Tuberculosis, Genitourinary, General Considerations
AIDS r Urolithiasis, Indinavir, and Other Protease Inhibitors
New WHO guidelines recommend starting ONGOING CARE
therapy when CD4 count <500/mm3 (previous PROGNOSIS
guidelines were <350/mm3 ) Much improved prognosis leading to longer life CODES
Pregnant women expectancies, primarily due to newer drug
Patients with HIV nephropathy combination therapies
Coinfection with HBV regardless of CD4 count. ICD9
r 042 Human immunodeficiency virus [HIV] disease
New WHO guidelines also call for some people COMPLICATIONS
r Antiretroviral therapy r 599.0 Urinary tract infection, site not specified
to begin treatment as soon as they test positive r 592.9 Urinary calculus, unspecified
for HIV, regardless of CD4 count Risk of nephrotoxicity, crystal precipitation leading
to stones, hypocalcemia
Second Line Erectile dysfunction and decreased libido (caused ICD10
r General urologic conditions such as UTI, voiding r B20 Human immunodeficiency virus [HIV] disease
by increased estradiol) may be associated with
symptoms, calcium stones treat as per general HAART therapy r N20.9 Urinary calculus, unspecified
practice. r Drugs used for the treatment of HIV-infected r N39.0 Urinary tract infection, site not specified
Salmonella epididymitis patients have become the most frequent cause of
24 wk of doxycycline 100 mg PO BID plus
drug-containing urinary calculi.
Cipro 500 mg PO BID Among these agents, PIs are well known to induce CLINICAL/SURGICAL
If difficult to eradicate may need lifelong
suppression.
kidney stones, (indinavir, atazanavir, darunavir). PEARLS
r Kaposi sarcoma FOLLOW-UP r Urologic considerations in patients with HIV/AIDS
If focal, local radiation, cryosurgery, or retinoids. Patient Monitoring include bacterial infections, urolithiasis, malignancy,
If disseminated use chemotherapy (doxorubicin) or r CD4 counts renal impairment, and voiding dysfunction.
immunotherapy with interferons r Serum creatinine r Urolithiasis is associated with patients taking
Patient Resources indinavir and other protease inhibitors (PIs).
r CDC HIV/AIDS Fact Sheets. http://www.cdc.gov/ r HIV associated nephropathy (HIVAN) increases the
hiv/library/factsheets/index.html patients risk of dialysis.
r Salmonella epididymitis is pathognomonic for HIV.
r Highly active antiretroviral therapy (HAART)
increases estradiol and can lead to symptoms of
erectile dysfunction and decreased libido.
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r Diethylstilbestrol (DES): 0.301 mg/d: ADDITIONAL TREATMENT 3. Langenstroer P, Kramer B, Cutting B, et al.
7090% of men achieve excellent results (1)[B] Radiation Therapy Parenteral medroxyprogesterone for the
N/A management of luteinizing hormone releasing
Side effects include painful gynecomastia. hormone induced hot flashes in men with advanced
At low doses, thromboembolic events are not a Additional Therapies prostate cancer. J Urol. 2005;174:642645.
significant problem. Behavioral therapy: Slow, deep breathing may reduce
4. Naoe M, Ogawa Y, Shichijo T, et al. Pilot evaluation
Generic drug, inexpensive though difficult to frequency of hot flushes
of selective serotonin uptake inhibitor
obtain in US. Complementary & Alternative antidepressants in hot flash patients under
r Cyproterone acetate (Androcur) 100 mg/d
Therapies androgen-deprivation therapy for prostate cancer.
Not approved for use in US r Acupuncture: Prostate Cancer Prostatic Dis. 2006;9(3):275278.
Steroidal antiandrogen, antigonadotropin with 1012 wk (twice weekly 2 wk then once 5. Moraska AR, Atherton PJ, Szydlo DW, et al.
progestin-like activity weekly 810 wk) Gabapentin for the management of hot flashes in
May interfere with ADT regimen prostate cancer survivors: A longitudinal
4378% reduction in frequency of flushes,
1 study demonstrated resolution of symptoms in average 9-mo duration of effect (1)[B] continuation studyNCCTG Trial N00CB.
84% of patients, >50% improvement in 37% r Vitamin E: J Support Oncol. 2010;8:128132.
(2)[B]
30% reduction vs. 22% of women receiving
Side effects include fatigue, increased risk of placebo in 1 study (not studied in men)
thrombosis, anemia, potential hepatotoxicity, ADDITIONAL READING
gynecomastia May increase the risk of prostate cancer; unclear
effect on existing cancer (1)[B] r Baum NH, Torti DC. Managing hot flashes in men
Second Line (Nonhormonal Therapy) r Soy products: being treated for prostate cancer. Geriatrics.
r Venlafaxine (Effexor) 12.5 mg/d PO:
Contain phytoestrogens which might decrease 2007;62:1821.
Antidepressant of the serotonin-norepinephrine r Engstrom CA, Kasper CE. Physiology and
severity of hot flushes (1)[C]
reuptake inhibitor (SNRI) type endocrinology of hot flashes in prostate cancer. Am
Also have shown benefit with regard to cardiac
Median weekly hot flush scores decreased 54% and bone health J Men Health. 2007;1:817.
from baseline after 1 mo (2)[B] r Black cohosh: r Sharifi N, Gulley JL, Dahut WL, et al. Androgen
Side effects include lack of sexual desire, delayed Has been used in some postmenopausal women deprivation therapy for prostate cancer. JAMA.
orgasm, and increase in suicidal ideation. for treatment of hot flushes 2005;294:238244.
r Paroxetine (Paxil-CR) 12.537.5 mg/d: Mechanism is unknown See Also (Topic, Algorithm, Media)
Antidepressant of the selective serotonin reuptake In 1 trial, no difference found with men taking r Andropause (Late-Onset Hypogonadism)
inhibitor (SSRI) class placebo (1)[C] r Menopause, Urologic Considerations
In 1 study, daily hot flushes decreased from r Testosterone, Decreased (Hypogonadism)
6.22.5. (4)[B] ONGOING CARE
Side effects include sexual dysfunction,
somnolence
r Ergotamine/belladonna/phenobarbital (EBP): 1
PROGNOSIS
r Most men have symptom improvement with medical
CODES H
or complementary therapy. ICD9
tablet PO BID: r At 8 yr of treatment with ADT over 40% of men still
Ergot alkaloid used primarily to treat migraine r 780.2 Syncope and collapse
headache had flashes. r 780.8 Generalized hyperhidrosis
r In 1 study, 72% of patients noted that hot flashes r 782.62 Flushing
Use with caution with patients on monoamine
oxidase inhibitors (MAOIs), central nervous system interfered with sleep and 59% reported they
(CNS) depressants, anticholinergic agents interfered with the quality of life. ICD10
r R23.2 Flushing
Generally not recommended for the treatment of COMPLICATIONS
hot flushes in men r R55 Syncope and collapse
Some men have side effects from medications taken to
r R61 Generalized hyperhidrosis
r Gabapentin (Neurontin) 900 mg/d (300 mg TID, alleviate hot flushes (bloating, weight gain,
titrated) (5)[B] hypertension)
Anticonvulsant/treatment of neuropathic pain FOLLOW-UP CLINICAL/SURGICAL
Modest 46% reduction in hot flush symptom Patient Monitoring
score at target dose of 900 mg r Ask patients on androgen deprivation at each PEARLS
Side effects include nausea, vomiting, dizziness, follow-up clinical evaluation about the presence and r Symptoms are usually reversible with cessation of
and somnolence severity of hot flushes: ADT usually within 34 mo of stopping treatment.
r Clonidine (Catapres) 0.11 mg/d (PO or patch Inquire about side effects from therapy r Most effective treatment is hormonal therapy with
formulations): Intermittent cessation of treatment can be used if estrogen or progesterone derivatives.
Centrally acting -adrenergic agonist used for side effects become bothersome. r There are limited data to support alternative/
treatment of hypertension (HTN) May have positive effect on quality of life, but complementary therapies.
1/3 of men will report a partial response may decrease survival
although similar to placebo (1)[B]. Patient Resources
Side effects include hypotension, dry mouth, skin N/A
irritation from patches.
REFERENCES
SURGERY/OTHER PROCEDURES
N/A 1. Jones JM, Kohli M, Loprinzi CL, et al. Androgen
deprivation therapy-associated vasomotor
symptoms. Asian J Androl. 2012;14(2):193197.
2. Irani J, Salomon L, Oba R, et al. Efficacy of
venlafaxine, medroxyprogesterone acetate, and
cyproterone acetate for the treatment of vasomotor
hot flushes in men taking gonadotropin-releasing
hormone analogues for prostate cancer: A
double-blind, randomised trial. Lancet Oncol.
2010;11:147154.
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ADDITIONAL READING
TREATMENT ONGOING CARE
Parlakgumus A, Yalcinkaya C, Kilicdag E. Prenatal
GENERAL MEASURES (3) PROGNOSIS diagnosis of McKusick-Kaufman/Bardet-Biedl
r Incision of the hymen if due to imperforate hymen. If Excellent, especially with early diagnosis and syndrome. BMJ Case Rep. 2011;2011.
an imperforate hymen is present and no mass or treatment
See Also (Topic, Algorithm, Media)
hydronephrosis is present, surgical correction is COMPLICATIONS r Hydrocolpos and hydrometrocolpos Image
sometimes delayed until tissues become more r Renal compromise or acute kidney injury with severe r Hydronephrosis/Hydroureteronephrosis (Dilated
estrogenized. However, the correction of the hydronephrosis Ureter/Renal Pelvis), Pediatric
imperforate hymen must take place before there is r Abdominal ascites r Hydronephrosis/Hydroureteronephrosis (Dilated
development of hydrocolpos. r Urinary retention and voiding difficulty
r Address vaginal or cervical issues as the anatomic Ureter/Renal Pelvis), Prenatal
r Reports of increasing rates of infertility based upon
pathology presents
level of obstruction
MEDICATION r Respiratory compromise in neonates due to massive CODES
First Line abdominal distension
N/A r At menarche, retrograde flow may predispose ICD9
patient to endometriosis r 623.8 Other specified noninflammatory disorders of
Second Line
N/A vagina
FOLLOW-UP r 752.42 Imperforate hymen
SURGERY/OTHER PROCEDURES Patient Monitoring r 752.46 Transverse vaginal septum
r Simple incision of the imperforated hymen. A Usually none necessary. Follow for resolution of
cruciate incision with resection of excess tissue tags hydronephrosis if present. ICD10
as necessary r N89.8 Other specified noninflammatory disorders of
Patient Resources
r Cloacal anomalies require a coordinated surgical vagina
N/A
team and planned intervention. r Q52.3 Imperforate hymen
r Vaginal septum if present needs to be incised either r Q52.11 Transverse vaginal septum
endoscopically or through open surgery REFERENCES
ADDITIONAL TREATMENT 1. Ameh EA, Mshelbwala PM, Ameh N. Congenital
vaginal obstruction in neonates and infants:
CLINICAL/SURGICAL
Radiation Therapy
N/A Recognition and management. J Pediatr Adolesc PEARLS
Gynecol. 2011;24(2):7478.
Additional Therapies The diagnosis should be considered in the pubertal
2. Nazir Z, Rizvi RM, Qureshi RN, et al. Congenital female with amenorrhea.
N/A
vaginal obstructions: Varied presentation and
Complementary & Alternative outcome. Pediatr Surg Int. 2006;22:749753.
Therapies 3. Puhl AG, Steiner E, Kramer WW, et al. Fetal H
N/A urogenital sinus with consecutive
hydrometrocolpos because of labial fusion: Prenatal
diagnostic difficulties and postpartal therapeutic
management. Fetal Diagn Ther. 2008;23:287292.
193
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HYDRONEPHROSIS/HYDROURETERONEPHROSIS (DILATED
URETER/RENAL PELVIS), ADULT
Kelly A. Healy, MD
Demetrius H. Bagley, MD, FACS
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r CT urogram
REFERENCES
Good for incidental hydronephrosis TREATMENT
Noncontrast phase for ureterolithiasis 1. Arena S, Magno C, Montalto AS, et al. Long-term
Contrast phase-delayed nephrogram suggested GENERAL MEASURES follow-up of neonatally diagnosed primary
obstruction r Management is highly dependent on underlying megaureter: Rate and predictors of spontaneous
Defined parenchymal masses, evaluate for condition and the timing (acute vs. chronic) resolution. Scand J Urol Nephrol. 2012;46:
crossing vessels with UPJ obstruction r Urgent decompression is needed with: 201207.
Delayed (excretory) phase may look like site of Severe pain 2. Patatas K, Panditaratne N, Wah TM, et al.
obstruction and rule out filling defect depending Active urinary tract infection and acute kidney Emergency department imaging protocol for
on degree of renal impairment insufficiency suspected acute renal colic: Re-evaluating our
r Excretory urogram: Retrograde ureteral stent or percutaneous service. Br J Radiol. 2012;85:11181122.
Generally replaced by CT urogram nephrostomy can provide equally effective 3. Jandaghi AB, Falahatkar S, Alizadeh A, et al.
r Magnetic resonance imaging (MRI) drainage Assessment of ureterovesical jet dynamics in
Lack of ionizing radiation advantageous for r Hydronephrosis lower urinary tract etiology is obstructed ureter by urinary stone with color
children, pregnant patients, and those with renal typically bilateral and patients may be asymptomatic Doppler and duplex Doppler examinations.
insufficiency or contrast allergy r May warrant catheter drainage or endoscopic Urolithiasis. 2013;41:159163.
More time consuming, expensive, does not treatment 4. Piazzese EM, Mazzeo GI, Galipo S, et al. The renal
effectively image urolithiasis resistive index as a predictor of acute
r Pyelography: Antegrade or retrograde MEDICATION
hydronephrosis in patients with renal colic.
Can be used with ultrasound or noncontrast CT in First Line J Ultrasound. 2012;15:239246.
r Patients with infection and hydronephrosis require
patients with contrast allergies or renal
insufficiency; but invasive antibiotic therapy and drainage
r Functional studies to differentiate obstructive vs. Renal failure and electrolyte abnormalities should ADDITIONAL READING
nonobstructive uropathy be corrected in conjunction with drainage
Whitaker test: 1st described in 1980 Second Line Shapiro SR, Wahl EF, Silberstein MJ, et al.
Indwelling percutaneous nephrostomy tube N/A Hydronephrosis Index: A new method to track patients
Percutaneous puncture renal pelvis with hydronephrosis quantitatively. Urology.
Upper urinary tract is perfused at a rate of SURGERY/OTHER PROCEDURES 2008;72:536.
r Catheter drainage of obstructed system with
510 mL per minute with saline or contrast See Also (Topic, Algorithm, Media)
percutaneous nephrostomy or ureteral stent is r Hydronephrosis/Hydroureteronephrosis, (Dilated
media
Serial pressure recording is made in renal pelvis guided by the severity of the illness
Hydronephrosis and fever may be ominous signs Ureter/Renal Pelvis), Adult Image
and bladder; spot films aid in evaluation r Hydronephrosis/Hydroureteronephrosis, (Dilated
Pressure gradient: Obstruction >22 cm H2 O; requiring early drainage
Other surgical procedures can be guided by the Ureter/Renal Pelvis), Pediatric
equivocal 1522 cm H2 O; normal <15 cm H2 O r Ureter, Obstruction
findings on imaging studies
Nuclear renography: r Urolithiasis, Ureteral
Primary noninvasive study to distinguish ADDITIONAL TREATMENT H
obstructive vs. nonobstructive uropathy Radiation Therapy
DTPA: Freely filtered by glomerulus. Neither N/A
secreted nor resorbed by renal tubules CODES
MAG-3: Almost exclusively limited by proximal Additional Therapies
Hemodialysis may rarely be needed in the acutely ill ICD9
tubule secretion without resorption distally
Preparation of patient, maintain hydration, patient r 591 Hydronephrosis
place a Foley catheter if concern for lower Complementary & Alternative r 592.0 Calculus of kidney
urinary tract dysfunction or obstruction Therapies r 600.00 Hypertrophy (benign) of prostate without
Furosemide 2040 mg given 20 min after N/A urinary obstruction and other lower urinary tract
radiotracer administration to induce diuresis symptom (LUTS)
Half time (T1/2 clearance). Time it takes to
eliminate 50% of radiotracer: Obstruction
ONGOING CARE ICD10
r N13.2 Hydronephrosis with renal and ureteral
>20 min; equivocal >1020 min; normal PROGNOSIS
<10 min; false positives: More commonly with calculous obstruction
Cause specific r N13.30 Unspecified hydronephrosis
severe dilation or poor function
r Endoluminal ultrasound (ELUS): COMPLICATIONS r N40.0 Enlarged prostate without lower urinary tract
r Progressive renal deterioration
Evaluate periureteral anatomy, vessels of high symptoms
r With vesicoureteral reflux, scarring and hypertension
insertion and UPJ obstruction, define ureteral
stricture can occur
Study of choice for submucosal calculi
r Postobstructive diuresis seen only with bilateral CLINICAL/SURGICAL
r Voiding cystourethrogram (VCUG): obstruction or solitary functioning kidney PEARLS
Evaluate for reflux FOLLOW-UP r Hydronephrosis and fever especially sepsis may
Patients with recurrent urinary tract infections, Patient Monitoring require immediate drainage.
flank pain, nonobstructive hydronephrosis r The etiology for hydronephrosis will determine the r Hydronephrosis may be nonobstructive.
Diagnostic Procedures/Surgery appropriate surveillance regimen r Generally hydronephrosis in an adult can be
Cystoscopy, retrograde ureteropyelogram r Consider renal ultrasound and renal scan at 3 mo
considered a sign of a process that must be defined
Pathologic Findings after treatment and possibly treated.
r Postoperative imaging may demonstrate the
Nephropathy related to obstruction (see above)
dilatation persists despite relief of obstruction
DIFFERENTIAL DIAGNOSIS
r Obstructive vs. nonobstructive Patient Resources
hydroureteronephrosis N/A
r See also Risk Factors
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HYDRONEPHROSIS/HYDROURETERONEPHROSIS (DILATED
URETER/RENAL PELVIS), PEDIATRIC
Ahmad H. Bani-Hani, MD, FAAP, FACS
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TREATMENT
r Multiple UTIs
r Reflux
ICD9
r 591 Hydronephrosis
H
GENERAL MEASURES r Renal insufficiency r 596.54 Neurogenic bladder NOS
Depends on underlying pathology responsible for the r Renal scarring r 753.3 Other specified anomalies of kidney
hydronephrosis r Urinary incontinence
ICD10
MEDICATION FOLLOW-UP r N13.30 Unspecified hydronephrosis
First Line Patient Monitoring r N31.9 Neuromuscular dysfunction of bladder,
r Antibiotic prophylaxis may be needed in severe r Patients need meticulous follow-up once unspecified
cases of hydronephrosis and/or presence of reflux hydronephrosis is diagnosed before and after r Q63.1 Lobulated, fused and horseshoe kidney
Prophylactic daily antibiotics to keep urine sterile, treatment
preferentially given at bedtime to maximize r Referral to pediatric urology/nephrology for full
urinary retention assessment and treatment CLINICAL/SURGICAL
<2 mo age: Amoxicillin 20 mg/kg/d
2 mo of age: Trimethoprimsulfamethoxazole Patient Resources PEARLS
2 mg/kg/d (concentrates in urine); nitrofurantoin National Kidney Foundation. http://www.kidney.org/ r Hydronephrosis is not a diagnosis but a sign.
is an alternative, but liquid is expensive and atoz/content/hydronephrosis.cfm r Renal ultrasound, VCUG, and diuretic renal scan can
bad-tasting establish the underlying etiology of hydronephrosis.
Second Line
r Anticholinergics in cases of an overactive bladder
and/or raised intravesical pressures
r -blockers in some cases of bladder outlet
obstruction
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HYDRONEPHROSIS/HYDROURETERONEPHROSIS (DILATED
URETER/RENAL PELVIS), PRENATAL
Bruce J. Schlomer, MD
Laurence S. Baskin, MD, FACS, FAAP
198
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ADDITIONAL READING
TREATMENT ONGOING CARE
Davenport MT, Merguerian PA, Koyle M. Antenatally
GENERAL MEASURES PROGNOSIS diagnosed hydronephrosis: Current postnatal
r Prenatal management: Assessment of r Most neonates have an excellent prognosis. management. Pediatr Surg Int. 2013;29(3):207214.
hydronephrosis, oligohydramnios: Prognosis depends on etiology of the dilated system
and other associated anomalies. See Also (Topic, Algorithm, Media)
Unilateral cases: Serial prenatal US if severe; r Hydronephrosis/Hydroureteronephrosis (Dilated
deliver at term r Severe bilateral hydronephrosis is associated with
Ureter/Renal Pelvis), Pediatric
Bilateral cases obstruction and oligohydramnios early in gestation r Hydronephrosis/Hydroureteronephrosis (Dilated
No oligohydramnios: Observation, deliver at predicts an adverse outcome.
r Fetuses with bilateral hydronephrosis, a distended Ureter/Renal Pelvis), Prenatal Image
term r Megaureter, Congenital
Oligohydramnios: Termination, early delivery, bladder, and oligohydramnios are at highest risk of
prenatal treatment for pulmonary immaturity neonatal demise or pulmonary complications.
r Postnatal management: r Risk of UTI correlated with severity of PN
Pulmonary support if respiratory compromise Mild: 10% CODES
Antibiotic prophylaxis if moderate-severe Moderate-severe: 30%
unilateral or bilateral PN ICD9
Bilateral hydronephrosis with dilated bladder:
COMPLICATIONS r 753.6 Atresia and stenosis of urethra and bladder
r Pulmonary hypoplasia with severe oligohydramnios
Place catheter to drain bladder neck
r Renal impairment r 753.20 Unspecified obstructive defect of renal pelvis
All hydronephrosis: US, VCUG, MAG-3 renal scan
r UTIs
as indicated (see above for SFU recommendations) and ureter
r 753.29 Other obstructive defects of renal pelvis and
MEDICATION FOLLOW-UP
Patient Monitoring ureter
First Line r Based on initial evaluation, subsequent imaging
r No specific antenatal medications exist
ICD10
r Prophylactic antibiotics controversial. may be necessary (1) r Q62.0 Congenital hydronephrosis
r Most centers employ serial renal US every 36 mo r Q64.31 Congenital bladder neck obstruction
Recommended by SFU with moderate-severe PN,
bladder/urethral abnormalities, dilated ureter, for the 1st yr of life r Q62.39 Other obstructive defects of renal pelvis and
oligohydramnios(1) r If febrile UTI, consider VCUG and/or MAG-3 renal
ureter
Amoxicillin (20 mg/kg/d1 dose per day) scan
r Surfactant to assist lung function after birth with
Patient Resources
pulmonary hypoplasia r http://urology.ucsf.edu/patient-care/children/ CLINICAL/SURGICAL
Second Line Hydronephrosis PEARLS
r http://urology.ucsf.edu/patient-care/children/urinary-
N/A r Majority of prenatal/fetal hydronephrosis (especially
tract-obstruction/ureteropelvic-junction-obstruction
SURGERY/OTHER PROCEDURES mild) is transient with no clinical significance.
r Fetal intervention (cases with oligohydramnios only):
r http://urology.ucsf.edu/patient-care/children/
r VCUG is not recommended for unilateral mild H
additional/megaureter
Controversial(3) r http://urology.ucsf.edu/patient-care/children/urinary- hydronephrosis.
Tapping of fetal bladder r Prophylactic antibiotics have not been shown to be
Percutaneous shunting: Vesicoamniotic drain tract-obstruction/posterior-urethral-valves
effective. Not recommended in mild cases.
r Surgery is seldom necessary in the neonatal period r Emergent evaluation by urologist should occur with:
with the exception of severe bilateral obstruction REFERENCES Severe bilateral prenatal hydronephrosis
due to bladder outlet obstruction or severe UPJ or Severe unilateral prenatal hydronephrosis in
UVJ obstruction. 1. Nguyen HT, Herndon CD, Cooper C, et al. The solitary kidney
r Need for postnatal surgery based upon diagnosis Society for Fetal Urology consensus statement on Prenatal hydronephrosis with dilated bladder
and correlated with severity of PN the evaluation and management of antenatal consistent with posterior urethral valves
Mild: <10% hydronephrosis. J Pediatr Urol. 2010;6:212231. Severe prenatal hydronephrosis with pulmonary
Severe: 50% 2. Lee RS, Cendron M, Kinnamon DD, et al. Antenatal compromise from mass effect (rare)
ADDITIONAL TREATMENT hydronephrosis as a predictor of postnatal
outcome: A meta-analysis. Pediatrics. 2006;118:
Radiation Therapy 586593.
N/A
3. Morris RK, Malin GL, Khan KS, et al. Systematic
Additional Therapies review of the effectiveness of antenatal intervention
N/A for the treatment of congenital lower urinary tract
Complementary & Alternative obstruction. BJOG. 2010;117:382390.
Therapies
N/A
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DIFFERENTIAL DIAGNOSIS
r Other causes of HTN. In Cushing disease,
ADDITIONAL TREATMENT ADDITIONAL READING
Radiation Therapy r Calhoun DA. Aldosteronism and hypertension. Clin J
aldosterone and renin will both be low. In renal N/A
artery stenosis, there will be high renin and high Am Soc Nephrol. 2006;1:10391045.
aldosterone. Additional Therapies r Conn JW. Presidential address. I. Painting
r Other causes of HTN and hypokalemia, such as: Emerging therapies include developing drugs that background. II. Primary aldosteronism, a new
inhibit actions of aldosterone synthase enzyme, clinical syndrome. J Lab Clin Med. 1955;45:317.
Overingestion of licorice
encoded on the CYP11B2 gene r Lim PO, Young WF, MacDonald TM. A review of the
Use of chewing tobacco
Hyperdeoxycorticosterones Complementary & Alternative medical treatment of primary aldosteronism.
r Other subtypes of primary hyperaldosteronism: Therapies J Hypertens. 2001;19(3):353361.
Bilateral adrenal hyperplasia: Idiopathic N/A r Mattsson C, Young WF Jr. Primary aldosteronism:
GRA due to aldosterone-producing, Diagnostic and treatment strategies. Nat Clin Pract
renin-responsive adenoma. Familial ONGOING CARE Nephrol. 2006;2(4):198208.
hyperaldosteronism type I, autosomal dominant r Plouin PF, Amar L, Chatellier G. Trends in the
Familial occurrence of APA or bilateral idiopathic PROGNOSIS prevalence of primary aldosteronism,
hyperplasia or both r Patients with primary hyperaldosteronism have aldosterone-producing adenomas, and surgically
Adrenal cancer producing aldosterone: Extremely higher rates of prior stroke (12.9% vs. 3.4%) correctable aldosterone-dependent hypertension.
rare compared to those with essential HTN. Nephrol Dial Transplant. 2004;19:774777.
r Liddle syndrome: Autosomal dominant disorder. r Nonfatal MI (4% vs. 0.6%) r Wheeler MH, Harris DA. Diagnosis and management
Mimics hyperaldosteronism and involves problems r Atrial fibrillation (7.3% vs. 0.6%) of primary aldosteronism. World J Surg. 2003;
with excess resorption of Na and loss of K. 27:627631.
COMPLICATIONS
r Related to HTN (left ventricular hypertrophy, See Also (Topic, Algorithm, Media)
r Adrenal Mass
TREATMENT coronary artery disease, heart failure, stroke,
r Aldosteronism (Hyperaldosteronism, Conn
intracerebral hemorrhage, etc.)
GENERAL MEASURES r Related to low K (tetany, headache, arrhythmias, Syndrome) Algorithm
r Treatment selected based on etiology of r Hypertension, Urologic Considerations
etc.)
hyperaldosteronism
r Control HTN FOLLOW-UP
Patient Monitoring
MEDICATION BP and serum electrolytes should be evaluated
CODES
First Line (2) postoperatively and following medical therapy.
r Mineralocorticoid receptor antagonist used in those ICD9
Patient Resources r 255.10 Hyperaldosteronism, unspecified
with bilateral adrenal hyperplasia and unilateral
Hyperaldosteronism - primary and secondary r 255.12 Conns syndrome
hyperplasia or APA who are not surgical candidates:
MedlinePlus. http://www.nlm.nih.gov/ r 255.14 Other secondary aldosteronism
Spironolactone: Limited due to affinity for
androgen and progesterone receptors. Can cause
medlineplus/ency/article/000330.htm H
gynecomastia, sexual dysfunction, menstrual ICD10
r E26.01 Conns syndrome
irregularities REFERENCES r E26.1 Secondary hyperaldosteronism
Eplerenone: No active metabolites, shorter
half-life than spironolactone, 5075% as potent 1. Mantero F, Mattarello MJ, Albiger NM. Detecting r E26.09 Other primary hyperaldosteronism
as spironolactone but less adverse effects and treating primary aldosteronism: Primary
r Thiazide diuretics, ACE inhibitors, calcium channel aldosteronism. Exp Clin Endocrinol Diabetes.
antagonists, angiotensin blockers 2007;115:171174. CLINICAL/SURGICAL
Second Line
2. Young WF Jr. Minireview: Primary aldosteronism PEARLS
changing concepts in diagnosis and treatment.
Amiloride, an epithelial Na channel blocker and r Hypertension (HTN) with serum K <3 meq/L is
Endocrinology. 2003;144(6):22082213.
K-sparing diuretic, may also be used, especially if highly suspicious of an aldosterone-producing
spironolactone or eplerenone are intolerable. More adenoma (APA).
often used in conjunction with the above. r Treatment selection is based on etiology of
SURGERY/OTHER PROCEDURES hyperaldosteronism.
r Unilateral adrenalectomy is indicated in patients r Surgical excision provides excellent control of
with hyperaldosteronism due to an adenoma. hypertension in aldosterone-producing adenoma
r HTN is cured or improved significantly in up to 90% (APA).
of such cases. Usually takes 36 mo to see an effect. r Control of the adrenal vein is the most important
r Adequate control of BP (see Medications) for step during adrenalectomy.
several weeks and correction of metabolic
abnormalities should be done before surgery.
r Obtain PAC after surgery to confirm cure
r Monitor K closely postoperatively
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MEDICATION REFERENCES
First Line ONGOING CARE
r Cabergoline or bromocriptine (dopamine agonists): 1. Melmed S, Casanueva FF, Hoffman AR, et al.
Usually will lower prolactin levels, regardless of PROGNOSIS Diagnosis and treatment of hyperprolactinemia: An
r 9095% of prolactin-secreting pituitary
cause, and shrink prolactinomas Endocrine Society clinical practice guideline. J Clin
In general, both cabergoline and bromocriptine microadenomas will not grow further, even without Endocrinol Metab. 2011;96(2):273288.
are effective. Cabergoline is usually medical therapy. 2. Klibanski A. Clinical practice. Prolactinomas. N Engl
r Medical therapy is usually successful in normalizing
better-tolerated, more convenient, and more J Med. 2010;362(13):12191226.
effective than bromocriptine, whereas prolactin levels, normalizing menses, reducing or 3. Casanueva FF, Molitch ME, Schlecte JA, et al.
bromocriptine is less expensive and has been used stopping galactorrhea, inducing ovulation, and Guidelines of the Pituitary Society for the diagnosis
longer. shrinking pituitary tumors. and management of prolactinomas. Clin Endocrinol
Use dopamine agonists with caution in patients r >90% of microadenomas do not grow significantly
(Oxf). 2006;65:265273.
on psychotropic drugs that inhibit dopamine during pregnancy, even after medical therapy is
action. stopped.
Cabergoline dosing (0.5-mg tablets): Start with r Some microadenomas disappear with time ADDITIONAL READING
0.250.5 mg once or twice weekly and increase (especially after menopause) or do not recur after
r Klibanski A. Clinical practice. Prolactinomas. N Engl
the dose at monthly intervals until prolactin medical therapy.
normalizes (>3 mg/wk is rarely needed). r Pituitary macroadenomas usually do not disappear J Med. 2010;362(13):12191226.
Bromocriptine dosing (2.5-mg tablets): Start r Mancini T, Casanueva FF, Giustina A.
completely with medical therapy and require
with 0.625 or 1.25 mg with food before continuous medical therapy. Hyperprolactinemia and prolactinomas. Endocrinol
bedtime and gradually increase at weekly Metab Clin North Am. 2008;37:6799.
intervals until prolactin level is controlled COMPLICATIONS r Melmed S, Casanueva FF, Hoffman AR, et al.
r Dopamine agonists can worsen underlying
(usually 2.5 mg BIDTID). Diagnosis and treatment of hyperprolactinemia: An
Side effects include nausea and postural psychiatric problems in patients taking psychotropic Endocrine Society clinical practice guideline. J Clin
hypotension medications. Endocrinol Metab. 2011;96(2):273288.
r Pregnancy considerations r Pituitary macroadenomas can secrete other r Molitch ME. Drugs and prolactin. Pituitary. 2008;
More experience with bromocriptine. hormones or become resistant to medical therapy. 11:209218.
Neither bromocriptine nor cabergoline has been FOLLOW-UP r Schlecte JA. Long-term management of
associated with teratogenicity. Patient Monitoring prolactinomas. J Clin Endocrinol Metab.
Nevertheless, either drug is usually stopped at the r Drug-induced HPRL: 2007;92:28612865.
1st evidence of pregnancy, except in patients with Prolactin should normalize after switching r Zeitlin S, Rajfer J. Hyperprolactinemia and erectile
macroadenomas in whom previous mass effects medications and no further follow-up is needed. dysfunction. Rev Urol. 2000;2(1):3942.
may recur if tumor enlarges. r Microadenomas:
Significant enlargement of microadenomas is See Also (Topic, Algorithm, Media)
Some microadenomas resolve spontaneously. r Erectile Dysfunction
uncommon during pregnancy. Measure prolactin every 612 mo to ensure
Lactation: Dopamine agonists will inhibit lactation. r Gynecomastia H
continued drug efficacy.
Second Line No need for repeat pituitary MRI unless prolactin
N/A increases markedly on therapy.
Consider stopping dopamine agonist after at least CODES
SURGERY/OTHER PROCEDURES
r Often performed transsphenoidally a year of successful therapy; some
r For microadenomas, generally reserved for patients microadenomas do not recur ICD9
r Macroadenomas: r 253.1 Other and unspecified anterior pituitary
intolerant of drug therapy. Tumors may recur. hyperfunction
r Only indicated for pituitary macroadenomas when If prolactin normalizes, repeat pituitary MRI after
r 256.39 Other ovarian failure
36 mo to ensure tumor shrinkage and establish
medical therapy is ineffective, including persistent new baseline. r 257.2 Other testicular hypofunction
visual field abnormalities: No consensus on frequency of further MRIs in
Usually not curative patients whose prolactin is well-controlled ICD10
r E22.1 Hyperprolactinemia
ADDITIONAL TREATMENT medically.
r E28.39 Other primary ovarian failure
Radiation Therapy Repeat prolactin measurements every 36 mo.
Follow visual fields in patients who have visual r E29.1 Testicular hypofunction
Usually only indicated for pituitary macroadenomas
that have failed medical therapy, and where response field defects at baseline.
to surgery is inadequate or surgery is contraindicated. Some macroadenomas resolve spontaneously.
CLINICAL/SURGICAL
Additional Therapies Patient Resources
Patient guide to hyperprolactinemia diagnosis and PEARLS
Men with ED or persistent hypogonadism may require
additional therapies. treatment. J Clin Endocrinol Metab. 2011;96: r Women present early in the disease course, while
35A36A. men present late.
Complementary & Alternative r Dopamine agonists are the 1st-line treatment of
Therapies
Vitex agnus-castus extract can be tried in cases of mild prolactinomas.
r Surgical excision is reserved for refractory cases.
HPRL
r All women should be screened for pregnancy before
initiating treatment.
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HYPOSPADIAS
Steve J. Hodges, MD
Anthony Atala, MD
PATHOPHYSIOLOGY
BASICS r Normal penile development: DIAGNOSIS
Urogenital folds form on either side of the cloacal
DESCRIPTION membrane, and fuse anteriorly at the genital HISTORY
r Common congenital disorder of male external r Family history of hypospadias
tubercle
genitalia characterized by a ventrally displaced Lateral labioscrotal folds fuse posteriorly and Any associated congenital anomalies
urethral meatus separate the urogenital and anal membranes Exposure of mother to hormonally active agents
Associated conditions may include: Under influence of testosterone and DHT, phallus during pregnancy
Ventral chordee r IVF may increase risk of hypospadias
elongates and the genital folds fuse in the midline
Incomplete foreskin with dorsal hood and to enclose urethral proximally to distally PHYSICAL EXAM
ventral deficiency Canalization of the glans occurs distally, fusing r Determine location of urethral opening
May be an isolated defect or may be associated with urethra r Evaluate for chordee
with a significant underlying abnormality Process complete by 20th wk of gestation r Evaluate foreskin
Classification: r Glandular hypospadias likely represents failure of
r Evaluate presence of inguinal hernia, hydrocele, or
Anterior (distal) 50%: Glandular, coronal, distal canalization
subcoronal, megameatus intact prepuce r Proximal hypospadias due to failure of fusion of cryptorchidism
Middle (midshaft) 30% r Severely proximal hypospadias may be associated
genital folds
Posterior (proximal) 20%: Penoscrotal, scrotal, r Scrotal or perineal variants result in cleft scrotum with bifid scrotum and/or penoscrotal transposition
perineal Look for other congenital anomalies
ASSOCIATED CONDITIONS DIAGNOSTIC TESTS & INTERPRETATION
EPIDEMIOLOGY r Growth restriction (low birth weight and length,
Incidence small head circumference) has been associated with Lab
r 1 in 250300 live male births Karyotype and hormonal evaluation to rule out
hypospadias
r 1 in 80100 in family history of hypospadias r Associated anomalies are more common in cases of intersex is needed in cases of severe hypospadias and
cryptorchidism
Prevalence severe hypospadias
Prevalence in US for hypospadias ranges between Cryptorchidism (79%) Imaging
Inguinal hernia/hydrocele (916%) r No routine imaging necessary for routine
2.01 and 56.17 per 10,000
Syndromes: hypospadias evaluation
RISK FACTORS 49 described in which hypospadias is frequent In setting of intersex evaluation, genitogram or
r 5 incidence in IVF births compared to controls
or occasional (Aniridia-Wilms tumor association, pelvic US may be performed
r Environmental: VCUG in proximal hypospadias may demonstrate
BeckwithWiedemann, SmithLemliOpitz,
Because of increases in rates in certain areas an Trisomy syndromes [4p, 9p, 13, 18], VACTERL prominent prostatic utricle
association with chemicals with estrogenic or association, XXY, Zellweger, and many others) Diagnostic Procedures/Surgery
antiandrogenic effects has been suggested 78% of these have associated micropenis, N/A
Examples include environmental chemicals such cryptorchidism, and/or scrotal anomaly
as bisphenol A (BPA) and hormones used during In presence of hypospadias and cryptorchidism Pathologic Findings
pregnancy such as progesterone. must rule out intersex condition (15% with N/A
Genetics (see below) palpable undescended testicle, 50% with DIFFERENTIAL DIAGNOSIS
Genetics nonpalpable) r In cases of proximal hypospadias associated with an
r <5% of cases have genetic cause (2) r 212% have upper tract anomalies (horseshoe undescended testicle differential should include:
r Can be seen in isolated and syndromic genetic kidney, renal ectopia, duplicated ureters, others) r Congenital adrenal hyperplasia
abnormalities r Enlarged prostatic utricle can be associated with r Mixed gonadal dysgenesis
r Caused by mutations of genes controlling severe cases and may increase risk of UTI or r Partial androgen insensitivity
development of male gonads or penis (eg, prostatic utricle stone formation r True hermaphroditism
homeobox, FGF, and Sonic hedgehog genes) GENERAL PREVENTION
r 5-reductase mutations
Not possible except by avoidance of environmental ALERT
r Familial propensity Do not perform circumcision in the setting of
agents or medications with estrogenic effects by
10% have affected 1st3rd degree relative pregnant women (see Risk Factors) hypospadias. Hypospadias in the presence of
14% of male siblings affected cryptorchidism may signal an intersex disorder.
27% concordance in monozygotic twins
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HYPOSPADIAS
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r HIV/AIDS
BASICS Unprotected high-risk sexual contact DIAGNOSIS
Blood transfusion
DESCRIPTION Uncircumcised Men HISTORY
r Immunocompromised patients have attenuated r HC
Occupational exposure
immune responses caused by: r Tuberculosis Ranging from pink urine to clot retention
Immunosuppressive drugs (chemotherapy) HIV infections: 14% of TB patients have HIV Can also have bladder pain and or lower urinary
Radiation (bone marrow irradiation) tract symptoms (LUTS)
Hematopoietic stem cell transplant PATHOPHYSIOLOGY r HIV/AIDS (2)
r Cyclophosphamide/busulfan
Malnutrition Increased risk of transmission and acquisition of
Disease processes (HIV, lymphoma, congenital Metabolized in the liver to acrolein sexually transmitted infections
immune deficiencies, autoimmune disorders) toxic to urothelium; prolonged exposure in the Atypical and prolonged course; genital lesions
r Immunocompromised patients are at risk for bladder causes increased inflammatory do not respond to normal treatments
opportunistic infections mediators: Bladder mucosal edema, vascular Can present with symptomatic genitourinary
r Hematopoietic stem cell transplant dilation, and increased capillary fragility tract infections
Long-term increased bladder cancer risk Testicular pain: Epididymitis/orchitis are
Need preparative regimens to prevent rejection of r Polyoma virusrelated hematuria (see Polyoma virus
transplanted graft: Complete myeloablative, common findings
Nonmyeloablative or +/ chemotherapy [BK, JC]), urologic considerations Can be positive for LUTS with prostatitis
r Miliary tuberculosis r HIV/AIDS Cystitis: Increased risk of bladder infections
Virus targets CD4+ T cells Urolithiasis: Can present with typical complaints
Hematogenous dissemination of Mycobacterium
Virus targets CCR5 expressing CD4+ cells; of ureteral calculus (flank pain, nausea,
tuberculosis
HIV coinfection is common; 38% with military TB with decreased CD4 lymphocyte count. Mucosal vomiting, dysuria, increased frequency, urgency)
tissues preferentially targeted, leads to Can present with voiding dysfunction
patients also have HIV
immunosuppression Commonly present with urinary retention
EPIDEMIOLOGY r TB genitourinary involvement Detrusor hyperreflexia, LUTS for bladder outlet
Incidence Hematogenous spread renal capillaries syndrome
r HIV infections renal cortex immune response chronic r Miliary tuberculosis
2.7 million new HIV infections worldwide inflammation granuloma with central caseous Failure to thrive, fever of unknown origin and
r Tuberculosis necrosis inflammation into renal tubules and night sweats, anorexia, weight loss
11,182 reported cases in US in 2010 medulla renal papilla sloughing calyceal Subacute or chronic presentation more
22% of cases were extrapulmonary ulceration fibrosis from healing calyceal common; can have dysfunction of one or more
2.7% were miliary TB infundibular narrowing or UPJ scarring organ system
hydronephrosis 50% have pulmonary disease with dyspnea or
Prevalence
Tubercles can also form in distal ureter leading to cough
HIV/AIDS: 1.2 million Americans
stricture Genitourinary involvement
RISK FACTORS Hematuria; small percentage may be passing
r Hemorrhagic cystitis (HC) (1) ASSOCIATED CONDITIONS
r HIV/AIDS; indinavir calculus material in urine (caseous material)
Increased degree of immunosuppression r TB Flank pain, symptoms of cystitis, LUTS (storage
BK virus Hemorrhagic cystitis (HC) symptoms), scrotal pain, male infertility workup;
r Any cause requiring bone marrow transplant
Early onset Hemorrhagic cystitis (HC) hematospermia
Conditioning regimen used for Hematopoetic r Urethritis: Reiter syndrome, arthritis
stem cell transplant (HSCT) with GENERAL PREVENTION
cyclophosphamide, busulfan, or with r HIV/AIDS: Protection during sexual activity; universal
antithymocyte globulin (4) precautions for healthcare professionals
Donorrecipient gender mismatch r Miliary tuberculosis: Treatment of latent TB can
Late onset Hemorrhagic cystitis (HC)
Allogenic HSCT transplant prevent miliary TB
Graft versus host disease (GVHD)
Use of corticosteroids or cyclosporine for GVHD
Use of T-cell depleted grafts
Need for blood transfusions
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r Stress incontinence r Absorbent products (pouches, absorbant pants, 5. Moore, KC, Lucas, MG. Management of male
No generally accepted drug therapy small pads) were evaluated in a multi-center, urinary incontinence. Indian J Urol. 2010;26(2):
Tricyclics sometimes used multi-crossover study (7) 236244.
Imipramine 1025 mg PO BID-TID The conclusion was that no one product suits 6. Moore KN, Schieman S, Ackerman T, et al.
Second Line every patient although small pads came closest Assessing comfort, safety, and patient satisfaction
r Urge incontinence Washable absorbant pants for men with light with three commonly used penile compression
Tricyclic antidepressants incontinence have economic advantages devices. Urology. 2004;63:150154.
Imipramine 1025 mg PO BID-TID 7. Fader M, Macaulay M, Pettersson L, et al. A
DDAVP for nocturnal symptoms multi-centre evaluation of absorbent products for
0.10.5 mg PO or intranasal QHS (off label)
ONGOING CARE men with light urinary incontinence. Neurourol
Avoided in patients with cardiac disease and PROGNOSIS Urodyn. 2006;25:689695.
older patients Continence can be improved in almost all patients
Risk of significant hyponatremia
Intradetrusor botulinum toxin injections COMPLICATIONS ADDITIONAL READING
r Candidiasis
SURGERY/OTHER PROCEDURES r Dermatitis Burden H, Warren K, Abrams P, et al. Diagnosis of
r Urge incontinence r Skin breakdown male incontinence. Curr Opin Urol. 2013;23(6):
Sacral neuromodulation 509514.
Augmentation cystoplasty FOLLOW-UP
r Stress incontinence Patient Monitoring See Also (Topic, Algorithm, Media)
r Bladder Areflexia (Detrusor Areflexia)
Urethral bulking agent Monitor post-void residual in patients on r Cunningham Clamp
Male sling procedure anticholinergic medications
r Incontinence Clamps
Promising short-term results Patient Resources r Incontinence, Urinary, Adult Male Image
No long-term studies (3)[B] Urology Care Foundation. Surgical Management of r Incontinence, Urinary, Following Radical
Artificial urinary sphincter Urinary Incontinence http://www.urologyhealth.
org/urology/index.cfm?article=33, Accessed April Prostatectomy
Excellent long-term continence rates (4)[A] r Incontinence, Urinary, with Orgasm (Climcaturia)
2013.
r Lower Urinary Tract Symptoms (LUTS)
ADDITIONAL TREATMENT
Radiation Therapy REFERENCES
N/A
1. Stewart WF, Van Rooyen JB, Cundiff GW, et al. CODES
Additional Therapies
r Pelvic floor exercise (Kegels) Prevalence and burden of overactive bladder in the
US. World J Urol. 2003;20:327336. ICD9
Significantly improve SUI/UUI r 788.30 Urinary incontinence, unspecified
r Biofeedback 2. Anger JT, Saigal CS, Stothers L, et al. Urologic
Diseases of America Project. The prevalence of r 788.31 Urge incontinence
r Timed voiding in UUI
urinary incontinence among community dwelling r 788.32 Stress incontinence, male
r Overflow incontinence due to poor bladder
men: Results from the National Health and
contractility with urinary retention Nutrition Exam survey. J Urol. 2006;176(5): ICD10
Indwelling catheter r N39.3 Stress incontinence (female) (male)
21032108.
Intermittent catheterization r N39.41 Urge incontinence
3. Comiter CV. The male sling for stress urinary
Evaluate for outlet obstruction incontinence: A prospective study. J Urol.
r R32 Unspecified urinary incontinence I
Complementary & Alternative 2002;167:597601.
Therapies 4. Elliott DS, Barrett DM. Mayo Clinic long-term
r Penile clamps, condom catheters, and pads are analysis of the functional durability of the AMS 800
CLINICAL/SURGICAL
occasionally used artificial urinary sphincter: A review of 323 cases. PEARLS
r They should be reserved for minor degrees of J Urol. 1998;159:12061208. r Prevalence of incontinence in males is 50% that of
incontinence or in patients who have multiple other
women.
comorbidities in whom surgery may be thought r Continence can be improved in almost all patients.
inappropriate (5)
r Penile compression clamps r An Artificial urinary sphincter has excellent
Applied externally to the penis to exert long-term continence rates.
nonsurgical compression of the urethra, thereby
preventing leakage of urine
3 types of commercially available penile
incontinence clamps (C3, U-Tex Male Adjustable
Tension Band and Cunningham clamp) have been
studied in a small trial (6)
No device completely eliminated leakage when
applied at a comfortable pressure
Complications of penile clamps can include
edema, pain, urethral erosion, and obstruction
Penile clamps should not be used for more than
4 hrs at a time
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r Delayed voiding/defecation lead to bladder r Rule out urine in vaginal vault or labial adhesions in
BASICS overactivity, constipation girls
r Bladder overactivity/constipation compounded by r Ectopic perineal ureteral orifice can be cause of
DESCRIPTION dyssynergy of pelvic floor, with failure to relax pelvic constant wetness in girls
r Incontinence: Involuntary leaking of urine due to r Rectal exam to rule out rectal stool, evaluate normal
floor completely with emptying
any cause sensation and tone
r A wet child is the most common problem seen by ASSOCIATED CONDITIONS r Neurologic exam
pediatric urologists. Most wetting children will See risk factors
r Measure or observe urinary stream for force, caliber,
appear to no inciting cause, and some will improve GENERAL PREVENTION straining, duration (may obtain flow/PVR)
spontaneously r Aggressively prevent and treat constipation
r Enuresis: Involuntary leaking of urine while sleeping r Ensure an environment where children are not DIAGNOSTIC TESTS & INTERPRETATION
Nocturnal enuresis implies night wetting alone, delaying micturition or defecation Lab
diurnal implies day and night, although these r Urinalysis
terms are outdated according to International Rule out UTI, microhematuria, proteinuria,
Childrens Continence Society (ICCS) DIAGNOSIS glucosuria
Primary enuresis: Child was always wet at night r If any of above discovered, require thorough
Secondary enuresis: Child has had a dry interval ALERT evaluation and treatment
for at least 6 mo before wetting again r Urine culture if UA shows signs of infection
Incontinence in the presence of an abnormal back
EPIDEMIOLOGY exam may signal a neurologic abnormality. Imaging
Prevalence r KUB to rule out spinal anomalies and rule out occult
r Day or night wetting occurs in up to 25% of 46 yr HISTORY constipation
r Childs agemore common in young r Renal US to evaluate for normal GU anatomy
old children (daytime incontinence is present in
approximately 510%) r Childs sexbedwetting is more common in boys, r VCUG only needed in the setting of febrile UTI (or
r Resolution rates of approximately 15% a year daytime wetting more common in girls any UTI in boys), hydronephrosis; allows evaluation
r At 12 yr of age 4% of children are enuretic at least r When did the symptoms begin? What is the of urethra in males
once a week, at 15 yr old it is 2% pattern? Severity? r MR urography may be needed when concerned for
r Enuresis is 3 more common in boys than girls, r Primary enuresis is highly associated with ectopic ureter
however daytime incontinence is more common in constipation r Renogram rarely needed to evaluate for urinary
females in all age groups r Secondary nocturnal enuresis implies an acquired obstruction, renal function
cause or stressor
RISK FACTORS r Dribbling upon standing or activity in girls may imply Diagnostic Procedures/Surgery
r Spinal dysraphism r Urodynamics indicated in setting of known
r Urinary tract anomalies vaginal voiding neurologic disorder
r Determine associated daytime symptoms (urgency, r Cystoscopy only needed if evaluation demonstrated
r Developmental delay
r Family history of enuresis frequency, weak or intermittent stream, or anatomic abnormality such as posterior urethral
infrequent voiding) valves
r Attention deficit disorder r History of UTI? Functional constipation?
r Urinary tract infection r History of large or firm bowel movements, or Pathologic Findings
r Constipation r Incontinence classified as structural, neurogenic,
encopresis, may signify constipation even in setting
complicated, or uncomplicated
Genetics of normal frequency of bowel movements r Structuralanatomic cause of incontinence (eg,
r Children whose parents were not bed-wetters have r Does child show holding behavior? (curtsey or
ectopic ureter in girls)
a 15% incidence of bedwetting. When one or both squatting in girls, holding genitals in boys) r Neurogenicincontinence due to spinal dysraphism
parents were bed-wetters, the rates jump to 44% r History of neurologic disorder?
and 77%, respectively. r Family history of incontinence? UTIs? Enuresis? or other neurologic cause
r Genetic research shows that bedwetting is r Uncomplicatednocturnal enuresis in appropriate
associated with the genes on chromosomes 13q and PHYSICAL EXAM age group in the presence of no obvious causes on
r Level of physical and emotional development diagnostic and physical exam (least
12q (possibly 5 and 22 also). r Abdominal examrule out masses, constipation commonalmost all cases have cause if look
PATHOPHYSIOLOGY r Back examrule out signs of occult spinal closely)
r Daytime control attained before nighttime r Complicatedfunctional voiding disorders;
dysraphism (dimples, short sacrum, spinal defect,
r Constipation plays a major role in urinary continence significant incontinence without anatomic or
hairy patches)
r Normal bladder control involves 3 basic r Flattened buttocks, low gluteal cleft, or nonpalpable neurologic cause
components: Intact neurologic system, normal coccyx suggest sacral agenesis
anatomy, and a mature, motivated child r GU exam, rule out genital or perineal sensation
r Normal urinary control occurs in stages:
disorders, signs of abuse, hypospadias, or epispadias
Infantile voiding (06 mo) low-pressure filling,
reflex detrusor contractions, simultaneous
relaxation of external sphincter, complete
emptying, uninhibited voids
Transitional voiding (12 yr) conscious sensation
of bladder filling, continence achieved by
controlling external sphincter, increasing bladder
capacity (60 cc at birth + 30 cc/yr till 12 yr old)
Adult voiding: Supraspinal inhibition of voiding
reflex, voluntary inhibition/initiation of voiding
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Genetics
BASICS r Genetic factor might be indicated. DIAGNOSIS
r Latex allergy is less frequent in adults with spinal
DESCRIPTION cord injury and multiple surgical procedures than in HISTORY
r Detailed clinical history of allergic reactions that are
r Localized or systemic reaction to latex, a natural children with similar conditions.
r Interleukin-13 (IL-13) and IL-18 promoter temporarily associated with exposure to Hevea
substance from the sap of the rubber tree, Hevea latexcontaining products (eg, prior history to
brasiliensis (1)[A]. polymorphisms more likely to be found in healthcare
anaphylaxis and/or intraoperative shock, itching,
r Latex is a common ingredient in many medical and workers in comparison to nonatopic controls or
redness, or swelling following dental, rectal, or
patients with anorectal/urologic malformations.
dental products (eg, bladder catheters, blood pelvic exam; itching or swelling with condoms,
pressure cuffs, face mask, gloves, endotracheal PATHOPHYSIOLOGY diaphragms, or latex sexual aids)
tubes, IV infusion sets, etc.) r Presensitization with Hevea latex allergens is r Detailed history of associated risk factors: Healthcare
r Patients with spina bifida or congenital urogenital prerequisite to initiate an allergic response. workers, hair dressers, rubber handling, eczema/hay
abnormalities have the highest risk. r A number of proteins found in the cytoplasm of fever, multiple surgeries, food allergies, etc.
r Mild forms include pruritus and swelling. The most r 3080% of patients with latex allergy also have
H. brasiliensis are known potent allergens that can
severe form of allergic reaction is anaphylaxis: A elicit human IgE antibody, leading to sensitization food allergy
severe, life-threatening, generalized or systemic in exposed patients and a spectrum of allergic r Allergic symptoms can include the following
hypersensitivity reaction characterized by rapidly reactions upon subsequent exposure (4)[A]. symptoms:
developing life-threatening airway and/or breathing r Symptoms of delayed (type IV) hypersensitivity Dizziness
and/or circulation problems usually associated with Dyspnea
usually develop within 12 days of exposure.
skin and mucosal changes. Pruritus
Immediate (type I) hypersensitivity causes symptoms
Rhinitis
within minutes of exposure.
ALERT r Immediate hypersensitivity reactions to latex (type I) Tearing
All patients with neurogenic bladder should be Swelling at the site of contact
are caused by cross-linking of latex protein-specific Abdominal cramps
considered for latex precautions.
IgE antibody with mast cells and basophils. r In the most extreme cases, anaphylaxis can develop
r Cross-reactivity between various proteins is
EPIDEMIOLOGY
responsible for the clinical associations between
Incidence ALERT
r Latex sensitivity (assessed by serum latex IgE or skin latex allergy and allergic responses to a number of
Use caution when examining any child for
fruits and vegetables.
prick test) in the general population is <1% r Type IVc (T-cellmediated type), delayed dysfunctional voiding especially if the child has
Spina bifida population is 1872% neurologic symptoms or suspected spina bifida.
r Latex allergy (eg, anaphylactic symptoms) is rare in hypersensitivity reaction can occur and usually
manifest as contact dermatitis 2496 hr after
healthy population. exposure.
PHYSICAL EXAM
r Healthcare community: Up to 12.1% which fell to r Use nonlatex exam gloves
4% with the introduction of powder-free gloves GENERAL PREVENTION Mucocutaneous manifestations:
r Exposure to multiple surgeries: 1/32/3 of children r Facility: Erythema
who underwent surgeries in the 1990s developed Avoidance is the most effective and least Edema
latex sensitivity; this risk decreased dramatically expensive method. Papules, macules, urticaria
since the implementation of latex avoidance in Establishment of a latex-safe environment should Allergic rhinitis
hospitals and products be a priority for institutions by replacing all Hevea Allergic conjunctivitis
latexcontaining products with nonHevea-based Angioedema
Prevalence synthetic products or powder-free latex products. Cardiopulmonary manifestations:
N/A Synthetic alternatives to rubber include butyl Tachypnea
RISK FACTORS rubber, a petroleum-based product with no Stridor, wheezing
r Occupational exposure: allergenic protein, neoprene, and copolymers of Tachycardia
Healthcare butadiene and acrylonitrile. Hypotension
Food handlers/restaurant workers Non-Hevea source of natural rubber is the guayule Shock
Hairdressers plant (Yulex). Yulex-based products pose no risk to
individuals allergic to Hevea latex and is approved
DIAGNOSTIC TESTS & INTERPRETATION
Construction workers
by the Food and Drug Administration (FDA). Lab
Painters
r Individuals with latex allergy: Routine resuscitation lab studies (blood gas, etc.) if
First responders
Should wear a medical alert bracelet indicating during acute anaphylaxis
Security personal
Gardeners latex allergy Imaging
r Atopic disease Should be encouraged to have self-injectable N/A
r People with food allergies mainly to kiwi, epinephrine if they have a clinical history of Diagnostic Procedures/Surgery
strawberry, banana, avocado, chestnut systemic reaction to latex r These are performed on a routine basis and not
r Multiple surgeries at young age Should avoid latex-containing products during an acute event
Should report their allergies prior to any medical r Prick skin test:
r Children with anorectal or urologic malformations or surgical procedure
(5)[A]. Extracts of Hevea latex, at least 3
Spinal dysraphism Commercial extracts are available
Bladder exstrophy/cloacal anomalies Test involves performing a puncture with a lancet
Patients on clean intermittent catheterization device through a drop of latex extract at
sequential concentrations ranging from
0.0011 mg/mL of protein
Results are read after 15 min and compared with
the positive histamine and negative saline controls
Small risk of anaphylaxis
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PHYSICAL EXAM
BASICS DIAGNOSIS r Assessment of nongenital sexual characteristics
Breast development
DESCRIPTION HISTORY Axillary hair
r Diminished libido, low sexual drive, or hyposexuality r Details of low libido r Signs of endocrinologic disorder
are defined by a lack of desire for sexual activity. Acquired or lifelong problem Cushingoid appearance
r Hypoactive sexual desire disorder or subjective Always or intermittently present Hypothyroid skin and hair changes
sexual arousal disorder may be implicated. With only specific sexual partners Diabetic neuropathy
After a new diagnosis or procedure r Visual inspection of the external genitalia
EPIDEMIOLOGY Following use of a new medication
Distribution of pubic hair
Incidence Association with life events
r More common with advancing age and especially r Reproductive information Ulcerations, pustules, discharge, or bleeding
Prolapsed urethra, vagina, or cervix
following menopause Age of menarche or onset of menses r Speculum exam
r A congenital syndrome may be causative at a young Pregnancies and deliveries
Mucosal rugae, moisture, thinning, or excoriation
age. Contraception use and type
Ulcerations, pustules, discharge, or bleeding
Prevalence Infertility and treatment
r Other sexual information Cystocele, rectocele, or enterocele
Estimated prevalence of 2575% of women varies by Vaginal wall masses
study sample and assessment Sexually transmitted infection r Palpation of the external genitalia, vaginal sidewalls,
Pain or discomfort with sexual activity
RISK FACTORS (1) pelvic floor muscles, cervix, and ovaries
Problems with sexual function of the partner
r Low testosterone (physiologic or iatrogenic) r Current or prior abuse Urethral or vaginal sidewall masses
r Advanced age Surgically placed foreign bodies
Sexual Pelvic floor muscle tension, spasm, or tenderness
r Menopause (physiologic or iatrogenic) Verbal or physical
r Pelvic floor disorder (incontinence or prolapse) r Symptoms of androgen insufficiency Cervical motion, ovarian, or adnexal tenderness
r Physical or psychological trauma (sexual assault, Vaginal cul-de-sac mass or tenderness
Dysphoria, fatigue, low sense of well-being
physical abuse, or verbal abuse) Reduced sexual receptivity and pleasure DIAGNOSTIC TESTS & INTERPRETATION
r Pregnancy (multifactorial per hormonal, emotional, Decreased vaginal lubrication despite estrogen Lab
treatment r Estrogens: Estradiol and estrone
and physical changes)
r Signs of androgen insufficiency r Androgens: Dehydroepiandrosterone,
Genetics
Early onset menopause may be implicated Bone loss, decreased muscle mass, less strength androstenedione, testosterone, and
Memory changes and altered cognitive function dihydrotestosterone
PATHOPHYSIOLOGY (2,3) r Other endocrine disorders r Proteins: Sex hormonebinding globulin (SHBG)
r Testosterone drops 50% from age 30 to 60 years
Hypothyroidism with free testosterone and total testosterone
and is linked to low libido as are other androgens. Cushing syndrome r Adjunctive: Thyroid-stimulating hormone,
r Estrogen can increase sex hormonebinding Diabetes glycosylated hemoglobin
globulin concentrations and lower free testosterone. r Urogenital conditions
r Progesterone may lower mood and decrease sex Imaging
Urinary incontinence or fecal incontinence Brain magnetic resonance imaging to assess the
drive as seen with some contraceptives. Pelvic organ prolapse
r Follicle-stimulating and luteinizing hormone hypothalamus and pituitary gland
r Medications
reduction by contraceptives lowers androgen Oral contraceptives, estrogens, progestins, DIFFERENTIAL DIAGNOSIS
r Hormonal
creation. gonadotropin-releasing hormone agonists
r Serotonin level alterations from certain Antidepressants, amphetamines, anticonvulsants, Decreased free testosterone or increased sex
antidepressants can decrease sex drive. antiepileptics, psychotropics hormonebinding globulin
Antihypertensives, antilipidemics, antiarrhythmics Decreased androgen
ASSOCIATED CONDITIONS Hypogonadotropic hypogonadism
r Vaginal atrophy Steroids, narcotics
r Chronic medical conditions Adrenal insufficiency or adrenal suppression
r Congenital syndromes
Psychiatric conditions Adrenal suppression or glucocorticoid excess
r Posttraumatic stress disorder (prior physical or Hypothyroidism or hyperthyroidism
Substance abuse
psychological trauma)
GENERAL PREVENTION
Exercise, balanced diet, healthy lifestyle
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SURGERY/OTHER PROCEDURES
Only useful for pituitary adenomas causing REFERENCES CODES
hyperprolactinemia or in cases of Cushings disease
1. Shimon I, Benbassat C. Male prolactinomas
ADDITIONAL TREATMENT presenting with normal testosterone levels. ICD9
r 302.71 Hypoactive sexual desire disorder
Radiation Therapy Pituitary. 2014;17(3):246250. r 752.89 Other specified anomalies of genital organs
N/A 2. Malavige LS, Jayaratne SD, Kathriarachchi ST, et al. r 799.81 Decreased libido
Additional Therapies Erectile Dysfunction among men with diabetes is
N/A strongly associated with premature ejaculation and ICD10
reduced libido. J Sex Med. 2008;5(9):21252134. r F52.0 Hypoactive sexual desire disorder
Complementary & Alternative 3. Aydogan U, Aydogdu A, Akbulut H, et al. Increased r Q55.0 Absence and aplasia of testis
Therapies frequency of anxiety, depression, quality of life and r R68.82 Decreased libido
L-arginine and yohimbine are touted but not proven sexual life in young hypogonadotropic hypogonadal
males and impacts of testosterone replacement
ONGOING CARE therapy. Endocr J. 2012;59(12):10991105. CLINICAL/SURGICAL
PEARLS
PROGNOSIS
The prognosis is good when there is a treatable ADDITIONAL READING r Decreased libido can be from a number of causes
underlying cause for loss of libido. Otherwise it can be r Carey JC. Pharmacological effects of sexual (medications, hormonal or psychiatric disorders,
permanent. etc.).
function. Obstet Gynecol Clin North Am. 2006; r A thorough history (including sexual history and
COMPLICATIONS 33(4):599620.
Loss of libido can result in depression, infertility, and r Swerdloff R, Wang C, Goldman. Cecil Medicine. SHIM score) and physical exam (assessment of
erectile dysfunction. secondary sex characteristics and testicular volume)
23rd ed. Philadelphia, PA: WB Saunders; 2007. are critical and can often point to a diagnosis.
r Wilson B. The effect of drugs on male sexual r It is very important to distinguish decreased libido
FOLLOW-UP
Patient Monitoring function and fertility. Nurse Pract. from other disorders of sexual function (arousal,
Men treated with androgens should be followed 1991;16(9):1217, 2124. erectile dysfunction, premature ejaculation, orgasm,
closely with digital rectal exam and prostate-specific See Also (Topic, Algorithm, Media) and sexual pain disorders) but patients can often
antigen every 6 mo r Andropause (Late Onset Male Hypogonadism) have multiple issues simultaneously.
r Erectile Dysfunction r Surgery is usually designated for pituitary adenomas
Patient Resources
r http://www.merckmanuals.com/home/ r Female Hypoactive Sexual Desire Disorder (ie, prolactinomas), which can be found on brain
mens health issues/sexual dysfunction in men/ r Testosterone, Decreased (Hypogonadism) MRI.
decreased libido in men
r http://men.webmd.com/mens-libido-directory
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PATHOPHYSIOLOGY r In men:
BASICS r BOO necessitates generation of higher bladder Inspection of the urethral meatus should be
pressures to overcome outlet resistance. performed to rule out meatal stenosis
DESCRIPTION r Bladder remodeling secondary to longstanding Digital rectal exam (DRE) should be performed to
r The lower urinary tract infection (LUTS) complex evaluate for:
outlet obstruction results in overactive bladder
includes both obstructive and storage urinary syndrome, storage symptoms, and over time, Prostatic enlargement
symptoms. decreased contractility. Nodularity or firmness suggestive of prostate
Obstructive urinary symptoms include urinary r LUTS may result from numerous conditions of the cancer
hesitancy, intermittency, post-void dribbling, and central and peripheral nervous systems. Bogginess or tenderness suggestive of
straining to void. r Result in either detrusor overactivity (storage prostatitis
Storage urinary symptoms include urinary Anal sphincter tone, abnormalities of which
symptoms) or detrusor hypocontractility (urinary
frequency, nocturia, and urinary urgency. retention/inadequate emptying). suggest neurologic disease
r While benign prostatic hyperplasia (BPH) frequently r In women:
contributes to the development of LUTS, there are ASSOCIATED CONDITIONS Speculum exam should be performed to evaluate
numerous other etiologies that must be considered Erectile dysfunction for mass, prolapse, and urethral abnormalities
in patients presenting with new urinary symptoms. GENERAL PREVENTION
r LUTS may result from structural or functional DIAGNOSTIC TESTS & INTERPRETATION
NA Lab
abnormalities of the genitourinary tract. r Urinalysis should be performed to evaluate for
EPIDEMIOLOGY DIAGNOSIS urinary tract infection or hematuria.
Incidence r Serum PSA should be considered as a diagnostic test
r There is a well-described relationship between age HISTORY (as opposed to a screening test).
r Essential to quantify LUTS for both diagnosis and r Serum creatinine is not recommended in the
and the development of LUTS.
r A few data specifically address the incidence of treatment planning evaluation of routine LUTS associated with BPH.
LUTS, given the basically negligible low case-fatality Use the validated AUA Symptom Score (AUASS)
often referred to a the AUA Symptom Index Imaging
rate and the often slow onset of symptoms. r Imaging with CT or ultrasound (US) is not
[AUA-SI]) or International Prostate Symptom Score
Prevalence (I-PSS) (17 mild; 819 moderate; 2035 severe) recommended as routine procedure.
r Disease prevalence is highly variable due to r Upper tract imaging with either CT or US may be
Attention should be paid to nature (obstructive/
differences in disease definition. storage) and duration of LUTS considered in the context of:
r The Olmsted County Study revealed age-dependent r Consider voiding diary (frequency/volume charts) if Acute symptom onset
increases in the prevalence of moderate-to-severe the patient is unable to elaborate the nature of his History of upper urinary tract infection or stone
LUTS from 26% (4049 yr) to 46% (7079 yr) or her symptoms disease
r 21.1% of patients in the National Health and r Elicit history of prior urinary tract infection or History of renal insufficiency
Nutrition Examination Survey (NHANES) reported at prostatitis Recent onset of nocturnal enuresis
least one symptom of LUTS. r Elicit history of prior hematuria (gross or r Prostate imaging with transrectal or transabdominal
r Various community-based studies estimate the US may provide information for treatment planning
microscopic)
age-stratified prevalence of moderateto-severe r Elicit history of prior urologic/pelvic surgery and is considered optional
LUTS in men as follows: Diagnostic Procedures/Surgery
Prior lower urinary tract intervention predisposes
4050 yr old: 20% r Assessment of post-void residual urine with US
to stricture/bladder neck contracture
5060 yr old: 30% imaging or catheterization is optional.
Disruption of pelvic plexus with pelvic surgery may
6070 yr old: 40% May aid in the noninvasive assessment of bladder
result in detrusor hypocontractility
7080 yr old: 5060% r Elicit history of other medical conditions function
RISK FACTORS Neurologic diseaseoveractivity or bladder r Assessment of urinary flow rate is optional that may
r Bladder outlet obstruction (BOO; male) hypocontractility predict response to invasive therapy.
Benign prostatic hyperplasia Diabetesbladder hypocontractility r Pressure flow urodynamic studies are not indicated
Urethral stricture disease/bladder neck contracture History of sexually transmitted in the evaluation of the uncomplicated patient with
Prostate/bladder cancer infection(s)urethral stricture disease LUTS
Bladder calculi History of pelvic radiationurethral stricture May be useful in patients with mixed symptoms or
r BOO (female) disease or bladder hypocontractility neurologic disease to develop a therapeutic
Pelvic organ prolapse r Elicit family history of genitourinary disease strategy
Bladder calculi (BPH/LUTS, prostate cancer, prostatitis) r Cystourethroscopy is not recommended for the
Urethral stricture disease r Review medications as certain antihistamines, uncomplicated patient with LUTS.
r Bladder (detrusor) hypocontractility antimuscarinics, sympathomimetics, and May be helpful to assess prostate configuration as
Idiopathic bronchodilators may exacerbate LUTS. it relates to invasive therapies
Neurogenic r Elicit history of sexual dysfunction May be useful in patients with mixed symptoms or
r Obesity, diabetes, and caffeine intake all have been r Evaluate overall fitness to undergo invasive neurologic disease to develop a therapeutic
associated with increased risk of LUTS procedure(s) strategy
May be useful in patients with a history suggestive
Genetics PHYSICAL EXAM
r Increased risk of moderate-to-severe LUTS in men of urethral stricture/bladder neck contracture
r Abdominal exam to assess suprapubic region for
with a family history of BPH. bladder distension Pathologic Findings
r The precise contribution of genetic and r Focused neurologic exam should be performed with Histopathology of BPH reveals proliferation of both
environmental factors to the development of LUTS stromal and glandular prostatic elements.
particular attention to:
remains largely unknown. General mental status
Ambulatory status
Motor and sensory function of the lower
extremities and perineum
Anal sphincter tone
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DIFFERENTIAL DIAGNOSIS
r BOO:
SURGERY/OTHER PROCEDURES
r Urethroplasty or directly visualized incision of
REFERENCES
Urethral Stricture/bladder neck contracture urethral stricture (DVIU) should be considered for 1. AUA Guideline on the Management of Benign
Bladder stone stricture/bladder neck contracture Prostatic Hyperplasia (BPH). 2012. http://
Cancer (prostate, bladder, urethral) r Prolapse repair should be considered for women www.auanet.org/content/clinical-practice-
Prostatitis with urinary symptoms and prolapse guidelines. Accessed November 23, 2013.
Urinary tract infection r Numerous surgical options exist for men with 2. McConnell JD, Roehrborn CG, Bautista OM, et al.
Detrusor-sphincter dyssynergia BPH/BOO. Some of these include: The long-term effect of doxazosin, finasteride, and
Pelvic organ prolapse Transurethral resection of the prostate (TURP) combination therapy on the clinical progression of
r Detrusor hypocontractility Transurethral microwave therapy (TUMT) benign prostatic hyperplasia. N Engl J Med.
Diabetes mellitus Transurethral laser vaporization of the prostate 2003;349:23872398.
Parkinson disease Transurethral laser enucleation of the prostate 3. Barry MJ, Meleth S, Lee JY, et al. Effect of
Multiple sclerosis Simple open or laparoscopic prostatectomy increasing doses of saw palmetto extract on lower
Radiation cystitis (generally reserved for men with prostate volume urinary tract symptoms: a randomized trial. JAMA.
Spinal cord injury > 80100 cc) 2011;306:13441351.
Lumbosacral disc disease r There are a few high-quality
Bladder stone comparative-effectiveness data upon which clinical
decisions can be based ADDITIONAL READING
TREATMENT Patients and physicians must weigh potential r McNicholas TA, Kirby RS, Lepor H. Evaluation and
benefits and harms of treatments.
nonsurgical management of benign prostatic
GENERAL MEASURES (1,2) ADDITIONAL TREATMENT hyperplasia. In: Wein AJ, Kavoussi LR, Novick AC,
r Treatment should be offered to men with moderate
Radiation Therapy et al. Campbell-Walsh Urology. 10th ed.
to severe symptoms (AUASS or IPSS 8) who are Philadelphia, PA: Saunders Elsevier; 2010.
N/A
bothered enough to consider therapy. r Roehrborn CG. Benign prostatic hyperplasia:
r Men with demonstrable sequelae of BPH/BOO (renal Additional Therapies
Behavioral interventions including timed voiding, etiology, pathophysiology, epidemiology, and
failure secondary to obstruction, bladder calculi, natural history. In: Wein AJ, Kavoussi LR, Novick AC,
etc.) should be counseled on benefits of treatment. double voiding, and biofeedback may improve
et al. Campbell-Walsh Urology. 10th ed.
r Treatment is tailored to symptom type (obstructive, symptoms.
Philadelphia, PA: Saunders Elsevier; 2010.
storage, mixed). Complementary & Alternative
Therapies See Also (Topic, Algorithm, Media)
MEDICATION r Bladder Outlet Obstruction (BOO)
Saw palmetto is widely used to treat LUTS with little r LUTS Algorithm
First Line benefit in randomized trials (CAMUS trial) (3).
r -Adrenergic blockers: relax prostatic/bladder neck r Prostate, Benign Hyperplasia/Hypertrophy (BPH)
smooth muscle tone and improve symptoms (all r Reference Tables: AUA Symptom Index/International
appear to have equal effectiveness) ONGOING CARE Prostate Symptom Score (I-PSS)
Alfuzosin 10 mg/d
Doxazosin start 1 mg/d to max. 8 mg PROGNOSIS
r 20% of men with untreated LUTS experience
Silodosin 8 mg/d
Tamsulosin start 0.4 mg to max. 0.8 mg progression within 5 yr (MTOPS trial). Options for CODES
Terazosin start 1 mg/d to max. 20 mg men with BPH/BOO include:
Side effects include syncope, orthostasis, Combination therapy reduces risk of progression ICD9
by 66% (2). r 788.41 Urinary frequency
retrograde ejaculation, asthenia, and nasal
congestion r 510% of men with moderate-to-severe LUTS will r 788.64 Urinary hesitancy
r 5-Reductase inhibitors: reduce prostatic volume require surgical intervention (MTOPS). r 788.99 Other symptoms involving urinary system
Finasteride or dutasteride COMPLICATIONS ICD10
Side effects include decreased libido and sexual
dysfunction
r Complications of BPH/LUTS include: r R35.0 Frequency of micturition L
Recurrent UTIs r R39.9 Unsp symptoms and signs involving the
Reduce PSA by 50% and correction should be Renal insufficiency
used when evaluating risk for cancer genitourinary system
Bladder stone formation r R39.11 Hesitancy of micturition
r Combination therapy (-adrenergic blocker +
Urinary retention
5-reductase inhibitor) should be considered in men Secondary bladder dysfunction
with moderate to severe symptoms and prostatic
enlargement. FOLLOW-UP CLINICAL/SURGICAL
r Tadalafil 2.55 mg/d can treat combined LUTS and Patient Monitoring PEARLS
r Monitoring with serial AUASS or IPSS to quantify
erectile dysfunction (ED). r Quantification of symptoms is paramount in the
r Antimuscarinic agents can be used alone or in symptom intensity and bother
r Urinalysis, serum PSA, urinary flow rate, and management of LUTS.
combination for overactivity/storage symptoms r Treatment should be offered to men with moderate
post-void residual as clinically indicated
Second Line to severe symptoms (AUASS 8).
N/A Patient Resources r Treatment should be tailored to symptoms and
Urology Care Foundation. http://www.urologyhealth. prostate volume and may include behavioral
org/urology/index.cfm?article=59&display=1 intervention, medical management, or surgical
intervention.
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LYMPHADENOPATHY, INGUINAL
Michael E. Woods, MD
Raj S. Pruthi, MD, FACS
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LYMPHADENOPATHY, INGUINAL
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LYMPHOCELE, PELVIC
Rafael E. Yanes, MD
Fernando J. Bianco, Jr, MD
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LYMPHOCELE, PELVIC
r Open marsupialization (internal drainage) into the 3. Loeb S, Partin AW, Schaeffer EM, et al.
TREATMENT peritoneum is the historic gold standard: Complications of pelvic lymphadenectomy: Do the
A window of peritoneum is excised, allowing the risks outweigh the benefits? Rev Urol. 2010;12(1):
GENERAL MEASURES lymph to be reabsorbed by the peritoneum. 2024.
r Treat DVT if present. r Infected lymphoceles require percutaneous or open 4. Hamilton BD, Winfield HN. Laparoscopic
r Foley catheter if the patient has significant voiding surgical drainage. marsupialization of pelvic lymphoceles. Tech Urol.
dysfunction r Omentoplasty: 1997;2(4):220224.
r Asymptomatic small lymphoceles should be Placing a portion of omentum in the window
monitored (<100150 mL volume). Many will decreases recurrence maintaining patency.
resolve spontaneously. Success: 75100% ADDITIONAL READING
MEDICATION Taneja SS. Complications of lymphadenectomy. In:
First Line ONGOING CARE Taneja SS, ed. Complications of Urologic Surgery:
r Lymphocele management is primarily interventional Prevention and Management. 4th ed. Philadelphia,
with limited role for medications unless associated PROGNOSIS PA: Saunders/Elsevier; 2010.
r Most smaller asymptomatic lymphoceles resolve
with infection or sclerosis (see below) (2).
r Systemic antibiotics (with percutaneous drainage) if spontaneously. See Also (Topic, Algorithm, Media)
r >90% success with marsupialization r Edema, External Genitalia
lymphocele is infected. r Lymphocele, Pelvic Images
Second Line COMPLICATIONS r Urinoma (Perinephric Pseudocyst)
r DVT/PE
N/A
r Lymphostasis of the lower extremity
SURGERY/OTHER PROCEDURES r Infection
r Treatment of symptomatic or large lymphoceles is
r Ureteral obstruction CODES
immediate percutaneous drainage (3). r Bowel obstruction
Reported success rates with aspiration and ICD9
drainage tube are approaching 80%, with a mean FOLLOW-UP 457.8 Other noninfectious disorders of lymphatic
drainage duration ranging from a few days to Patient Monitoring channels
several months. Repeat imaging: Ultrasound or CT in 24 mo after
Increased risk of infection, especially in ICD10
treatment to detect recurrence. I89.8 Oth noninfective disorders of lymphatic vessels
immunocompromised (transplant) patients.
r Sclerosis therapy can be used to treat Patient Resource and nodes
extraperitoneal lymphoceles http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2498000
Sclerotherapy (povidoneiodine, 95% ethanol, CLINICAL/SURGICAL
tetracycline 0.52 g in 50 mL NS, bleomycin REFERENCES PEARLS
1 U/mL, fibrin glue):
Cavity is aspirated, then filled gently with a 1. Musch M, Klevecka V, Roggenbuck U, et al. r Use of clips on identifiable lymphatic channels can
sclerosing agent. Complications of pelvic lymphadenectomy in 1,380 minimize the occurrence of postoperative
Sclerosis is usually contraindicated. patients undergoing radical retropubic lymphoceles.
Multiseptated lymphoceles: Drainage, lack of prostatectomy between 1993 and 2006. J Urol. r A transperitoneal approach for lymphadenectomy is
access to all chambers 2008;179:923928. not protective against the formation of a lymphocele
When the ureter is in close contact with a wall 2. Glass LL, Cockett AT. Lymphoceles: Diagnosis and because loculation of lymphatic fluid can still occur.
of the lymphocele (periureteral fibrosis, ureteral management in urologic patients. Urology. r Symptomatic lymphoceles may require percutaneous
obstruction) 1998;51(5A):135140. or laparoscopic drainage.
Incomplete lymphoceles should not be treated
by sclerosis.
r Transperitoneal laparoscopic marsupialization (4)
If unsuccessful sclerosis or not amenable to L
percutaneous drainage
Three transperitoneal ports provide access for
excision of the peritoneal window and optional
omental wick placement to keep peritoneal
window open.
Success: 77100%
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Diagnostic Procedures/Surgery
BASICS DIAGNOSIS Percutaneous renal biopsy confirms the diagnosis
HISTORY Pathologic Findings
DESCRIPTION r Gross findings
r Medullary cystic kidney disease (MCKD) is a rare r Polyuria
Usually the 1st clinical manifestation Initially before disease progression, the kidneys
congenital, cystic disease of the kidneys which
Occurs due to reduced urinary concentrating are of normal size
results in progressive renal deterioration and to
ability of the kidney Cortical atrophy with progression
eventual end-stage renal disease (ESRD)
r Symptoms develop insidiously and diagnosis is not r Polydipsia Cysts develop at the corticomedullary junction and
r Family history of ESRD, or renal cysts range in size from 1 to 10 mm.
common until renal insufficiency is detected and With disease advancement the kidneys become
initiates evaluation (1)[C] PHYSICAL EXAM very small and demonstrate a granular exterior
EPIDEMIOLOGY Hypertension may be noted with disease progression surface
r Microscopic findings
Incidence DIAGNOSTIC TESTS & INTERPRETATION
Less than 1: 100,000 Interstitial nephritis
Lab Dilated, atrophic tubules
Prevalence r Urinalysis
Inflammatory cell infiltrates
N/A Proteinuria and hematuria are usually absent
r CBC DIFFERENTIAL DIAGNOSIS
RISK FACTORS r Juvenile nephronophthisis
Positive family history Anemia present in advanced cases due to lack of
erythropoietin Clinically and anatomically similar to MCKD
Genetics r Serum electrolytes Autosomal recessive inheritance
r Mode of inheritance is autosomal dominant (2)[C] ESRD usually manifests as early as age 13 yr
Elevated creatinine
Medullary cystic kidney disease-1 (MCKD1) Hyperkalemia or metabolic acidosis in later stages Extrarenal manifestations are common
Mutation in MCKD1 gene localized to Retinal disorders (retinitis pigmentosa)
due to renal insufficiency
chromosome 1q21 Hepatic fibrosis
MCKD2 Imaging BardetBiedl syndrome (obesity, retinitis
r Renal ultrasound
Mutation in MCKD2 gene localized to pigmentosa, mental retardation, polydactyly)
chromosome 16p12 Kidneys may be atrophic depending on stage of r Polycystic kidney disease
disease Autosomal recessive polycystic kidney disease
PATHOPHYSIOLOGY Cysts may be visible at the corticomedullary
r Unlike other renal cystic diseases such as autosomal (infantile form)
junction in later disease but are usually not Autosomal dominant polycystic kidney disease
dominant polycystic kidney disease (ADPKD), there detectable in early stages
is no clear correlation between genetic mutation (adult form)
Increased parenchymal echogenicity from r Multicystic dysplastic kidney
and identifiable protein product responsible for the tubulointerstitial fibrosis r Benign multilocular cyst (cystic nephroma)
MCKD phenotype (3)[C] r CT scan
r Medullary sponge kidney
ASSOCIATED CONDITIONS Can detect cysts at the corticomedullary junction
r Hyperuricemia and gouty arthritis are associated better than ultrasound
with MCKD2 Need for IV contrast is suboptimal in patients with
r In contrast to juvenile nephronophthisis, MCKD does ESRD
not have many extrarenal manifestations
GENERAL PREVENTION
N/A
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ADDITIONAL READING
TREATMENT ONGOING CARE
Hildebrandt F, Otto E. Molecular genetics of
GENERAL MEASURES PROGNOSIS nephronophthisis and medullary cystic kidney disease.
r Same as for any patient with renal insufficiency r MCKD1 patients manifest with ESRD at median age J Am Soc Nephrol. 2000;11:17531761.
Control hypertension if present of 62 yr
r MCKD2 patients manifest with ESRD at median age See Also (Topic, Algorithm, Media)
Monitor fluid balance/daily weights r Nephronophthisis (Juvenile, Infantile, and
Monitor serum electrolytes of 32 yr
Adolescent)
MEDICATION COMPLICATIONS r Renal Cysts (Intrarenal, Peripelvic, and Parapelvic)
First Line Similar to any patient with renal insufficiency or ESRD r Renal Mass
r None for primary treatment
FOLLOW-UP
r Antihypertensive regimens sometimes necessary
Patient Monitoring
Second Line Close nephrology follow-up is essential
CODES
N/A Patient Resources
r National Kidney Foundation ICD9
SURGERY/OTHER PROCEDURES r 585.6 End stage renal disease
r Dialysis when ESRD develops www.kidney.org/patients r 753.16 Medullary cystic kidney
r Renal transplant r 788.42 Polyuria
Allograft is not affected by MCKD after
transplantation
REFERENCES ICD10
r N18.6 End stage renal disease
ADDITIONAL TREATMENT 1. Scolari F, Ghiggeri GM. Nephronophthisis-
r Q61.5 Medullary cystic kidney
Radiation Therapy medullary cystic kidney disease: From bedside to
bench and back again. Saudi J Kidney Dis r R35.8 Other polyuria
N/A
Transplant. 2003;14:316327.
Additional Therapies 2. Hildebrandt F, Omram H. New Insights:
N/A Nephronophthisis-medullary cystic kidney disease. CLINICAL/SURGICAL
Complementary & Alternative Pediatr Nephrol. 2001;16:168176. PEARLS
Therapies 3. Kim CM, Glassberg KI. Molecular mechanisms of r MCKD has an insidious disease onset.
N/A renal development. Cur Urol Rep. 2003;4: r Symptoms usually not present until patient has renal
164170.
insufficiency documented on serum testing.
r Polyuria is commonly the 1st clinical manifestation.
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MEDICATION
r Potassium citrate is employed in patients with
FOLLOW-UP ADDITIONAL READING
Patient Monitoring r Fabris A, Anglani F, Lupo A, et al. Medullary sponge
hypocitraturia (5) r Imaging at every 612 mo in stone formers to
r Thiazide diuretics are used in patients with stones evaluate for change in existing stones or appearance kidney: state of the art. Nephrol Dial Transplant.
and nonresponsive hypercalciuria of new ones 2013;28:11111119.
r Specific antibiotics are indicated for the treatment of r In some patients, renal ultrasound (RUS) can be r McPhail EF, Gettman MT, Patterson DE, et al.
UTIs used to monitor stones and avoid radiation Nephrolithiasis in medullary sponge kidney:
Suppressive antibiotics may be necessary in r 24-hr urine collections are used to monitor stone risk Evaluation of clinical and metabolic features.
patients with persistent or multiply recurrent UTIs factors during treatment for urinary abnormalities Urology. 2012;79:277281.
Second Line r Serum studies are used to monitor changes related See Also (Topic, Algorithm, Media)
N/A to medication r Dent Disease
r Distal Renal Tubular Acidosis
SURGERY/OTHER PROCEDURES Patient Resources
r Shock wave lithotripsy has been utilized for r Hypercalciuria
National Kidney and Urologic Diseases
Information Clearinghouse (NKUDIC) http://kidney. r Medullary Cystic Kidney Disease (MCKD)
treatment of collecting duct stones that can be
niddk.nih.gov/kudiseases/pubs/medullaryspongekidney/ r Medullary Sponge Kidney (MSK) Image
distinguished from nephrocalcinosis as well as
symptomatic intraluminal calculi r Nephrocalcinosis
r Endoscopy with ureteroscopy or occasionally r Polycystic Kidney Disease
REFERENCES r Urolithiasis, Adult
percutaneous nephrostolithotomy can treat
collecting system stones and unroof mucosa to 1. Torregrossa R, Anglani F, Fabris A, et al.
remove obvious and accessible collecting duct Identification of GDNF gene sequence variations in
stones patients with medullary sponge kidney disease. CODES
r SWL and endoscopy have been advocated to reduce Clin J Am Soc Nephrol. 2010;5:12051210.
the frequency of symptomatic episodes but is 2. Fabris A, Bernich P, Abaterusso C, et al. Bone ICD9
unproven disease in medullary sponge kidney and effect of r 588.89 Other specified disorders resulting from
potassium citrate treatment. Clin J Am Soc Nephrol. impaired renal function
ADDITIONAL TREATMENT
2009;4:19741979. r 592.0 Calculus of kidney
Radiation Therapy 3. Maw AM, Megibow AJ, Grasso M, et al. Diagnosis r 753.17 Medullary sponge kidney
N/A
of medullary sponge kidney by computed
Additional Therapies tomographic urography. Am J Kidney Dis. 2007; ICD10
N/A 50:146150. r N20.0 Calculus of kidney
Complementary & Alternative 4. Miller NL, Humphreys MR, Coe FL, et al. r N25.89 Oth disorders resulting from impaired renal
Therapies Nephrocalcinosis: Re-defined in the era of tubular function
N/A endourology. Urol Res. 2010;38:421427. r Q61.5 Medullary cystic kidney
5. Fabris A, Lupo A, Bernich P, et al. Long term
treatment with potassium citrate and renal stones
ONGOING CARE in medullary sponge kidney. Clin J Am Soc Nephrol. CLINICAL/SURGICAL
PROGNOSIS 2010;5:16631668. PEARLS
r Urinary calculi are the most common risk but can be
r Be suspicious of MSK in patients with multiple
followed and also may be controlled with medical
papillary calculi.
treatment r Use contrast study for diagnosis.
r Recurrent UTIs can usually be treated
r Search for metabolic defects.
r Development of renal failure is very uncommon
r Treat metabolic factors in stone formers.
COMPLICATIONS r Consider treatment of renal stones in patients with
r Stone formation and subsequent obstruction
recurrent symptomatic stones.
r Recurrent/chronic flank pain r Significant benefit in endoscopic inspection and
r UTI treatment.
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MEGAURETER, CONGENITAL
Ahmad H. Bani-Hani, MD, FAAP, FACS
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MEGAURETER, CONGENITAL
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Diagnostic Procedures/Surgery
BASICS DIAGNOSIS r Biopsy
The role of biopsy in pediatric renal tumors is
DESCRIPTION HISTORY controversial as nephrectomy is the mainstay of
r Congenital mesoblastic nephroma (CMN) is a renal r History of prenatal ultrasound finding of unilateral
treatment and preoperative biopsy upstages to
tumor arising from nephrogenic mesenchyme renal mass stage III
r Usually a solid lesion, but cystic varieties have been r History of polyhydramnios
r Neonate with abdominal mass Pathologic Findings
reported r Three histologic subtypes
r Majority are benign with a favorable prognosis r Hematuria, jaundice, hypertension, anemia,
Classic
r First reported in 1967, referred to in older literature hypercalcemia 1/3 of cases
as Bolandes tumor or Bolande disease PHYSICAL EXAM Similar macro- and microscopically to
r Palpable abdominal mass leiomyoma
EPIDEMIOLOGY
r Hematuria Entrapped nephrons and blood vessels seen at
Incidence
r Most common real tumor in children <6 mo of age, r Jaundice the tumor periphery
Not associated with metastasis
usually diagnosed prior to 3 mo DIAGNOSTIC TESTS & INTERPRETATION
r Accounts for 310% of all pediatric renal Cellular
Lab 2/3 of cases
neoplasms (1) r Complete blood count More aggressive than classic with high mitotic
r More common in males (1) r Basic metabolic panel index and atypical growth pattern
r Usually unilateral Associated with local invasion/recurrence and
Serum creatinine
r Often detected prenatally by ultrasound metastasis
Serum calcium
Prevalence r Urinalysis Mixed (3)
N/A Imaging DIFFERENTIAL DIAGNOSIS
r Abdominal ultrasound r Solid renal mass
RISK FACTORS
Preferred modality Wilms tumor
Genetics
r ETV6-NTRK3 gene fusion Ring pattern Rhabdoid tumor
Hypoechoic mass with hyperechoic rim Metanephric adenofibroma
Results from translocation t (12;15) (p13;q25)
signifying vessels at the tumor periphery Renal cell carcinoma
Found only in the cellular variant
Seen only in the classic variant Angiomyolipoma
Also found in congenital fibrosarcoma (2,3)
Homogeneous or heterogeneous solid mass seen Clear cell sarcoma
PATHOPHYSIOLOGY in cellular variant Multilocular cystic nephroma
r Tumor classification r CT r Autosomal recessive polycystic kidney disease
Stage I: Tumor limited to kidney without Homogeneous mass r Cross-fused ectopia
involvement of capsule or hilar vessels May have peripheral enhancement or focal r Renal vein thrombosis
Stage II: Tumor extends beyond capsule with enhancement at sites of hemorrhage or necrosis r Solitary kidney with compensatory hypertrophy
invasion into perinephric fat or blood vessels, but r MRI r BeckwithWeidemann syndrome
margins of resection are negative Signal similar to normal parenchyma with r Adrenal mass
Stage III: Tumor not completely resectable, tumor exception of areas of hemorrhage (4) r Retroperitoneal mass (3)
spillage occurs at time of resection, or tumor was
biopsied preoperatively
Stage IV: Hematogenous metastases or lymphatic
spread outside of abdomen
Stage V: Bilateral tumors
ASSOCIATED CONDITIONS
r Polyhydramnios
r Hydrops fetalis
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MICROPENIS (MICROPHALLUS)
Bruce J. Schlomer, MD
Laurence S. Baskin, MD, FACS, FAAP
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MICROPENIS (MICROPHALLUS)
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SURGERY/OTHER PROCEDURES r As of 2012, the American Academy of Pediatrics r Schreuder MF, Westland R, van Wijk JA. Unilateral
r Mostly nonoperative: states that contact-sport participation is generally multicystic dysplastic kidney: A meta-analysis of
25% totally involute OK for children who have only one functional observational studies on the incidence, associated
60% regress kidney. In a very large published series, none of the urinary tract malformations and the contralateral
15% remain stable kidney injuries were catastrophic or needed surgery kidney. Nephrol Dial Transplant. 2009;24(6):
A very small number may increase in size r Long-term follow-up for hypertension and 18101818.
r Indications for nephrectomy: microalbuminuria by informed pediatrician or family r Thomas DF. Prenatally diagnosed urinary tract
Kidneys which remain large, increase in size, or physician; referral to nephrology for renoprotective abnormalities: Long-term outcome. Semin Fetal
show an increased amount of solid tissue medications when indicated Neonatal Med. 2008;13(3):189195.
(especially with function) Patient Resources See Also (Topic, Algorithm, Media)
Kidneys easily removed (especially r National Kidney and Urologic Diseases r Multicystic Dysplastic Kidney Image
laparoscopically) or cysts may be sequentially r Information Clearinghouse (NKUDIC) http://kidney. r Potter Syndrome/Potter Facies
decompressed r Renal Cysts
niddk.nih.gov/kudiseases/pubs/kidneydysplasia/
Hypertension: Uncommon (5%) in childhood and
r Renal Dysplasia, Hypodysplasia, and Hypoplasia
not likely caused by MCDK
May persist after nephrectomy depending on REFERENCES
the patient age at onset of hypertension and the
presence of CAKUT (see Prognosis) 1. Westland R, Schreuder MF, Bokenkamp A, et al. CODES
ADDITIONAL TREATMENT Renal injury in children with a solitary functioning
kidneythe KIMONO study. Nephrol Dial ICD9
Radiation Therapy Transplant. 2011;26(5):15331541. r 593.70 Vesicoureteral reflux unspecified or without
N/A
2. Sanna-Cherchi S, Ravani P, Corbani V, et al. Renal reflux nephropathy
Additional Therapies outcome in patients with congenital anomalies of r 753.15 Renal dysplasia
N/A the kidney and urinary tract. Kidney Int. 2009;76(5): r 753.19 Other specified cystic kidney disease
Complementary & Alternative 528533.
Therapies ICD10
r Q61.4 Renal dysplasia
N/A
ADDITIONAL READING r Q62.7 Congenital vesico-uretero-renal reflux
r Q62.39 Other obstructive defects of renal pelvis and
ONGOING CARE r Abou Jaoude P, Dubourg L, Bacchetta J, et al.
ureter
Congenital versus acquired solitary kidney: Is the
PROGNOSIS difference relevant? Nephrol Dial Transplant.
r Usually excellent in unilateral disease
2011;26(7):21882194. CLINICAL/SURGICAL
r KIMONO study (1) reports renal injury (hypertension, r Hains DS, Bates CM, Ingraham S, et al.
PEARLS
albuminuria and/or the use of renoprotective Management and etiology of the unilateral
medication) present in 32% at young age (mean multicystic dysplastic kidney: A review. Pediatr r MCDK occurs as a result of renal maldevelopment
age 9.5 yr). Nephrol. 2009;24(2):233241. due to possible mutation(s) in genes responsible for
Increased renal injury in individuals with a solitary r Hayes WN, Watson AR; Trent & Anglia MCDK Study ureteral bud formation.
functioning kidney in later life when CAKUT Group. Unilateral multicystic dysplastic kidney: Does r Large cysts of varying sizes present with no
present. initial size matter? Pediatr Nephrol. 2012;27(8): identifiable parenchyma; ureter usually atretic.
Study suggests long-term clinical follow-up for 13351340. r Most involute or become significantly smaller; rare
hypertension and microalbuminuria especially into r Mansoor O, Chandar J, Rodriguez MM, et al. enlargement.
puberty and adulthood. Long-term risk of chronic kidney disease in unilateral r Almost none require nephrectomy; consider for
These findings are supported by an early study (2). multicystic dysplastic kidney. Pediatr Nephrol. functioning solid component or increasing size.
COMPLICATIONS 2011;26(4):597603. r Not associated with increased risk of hypertension
r Malignant transformation extremely low with only r Psooy K. Multicystic dysplastic kidney in the during childhood or Wilms tumor in large series.
15 cases of tumors reported neonate: The role of the urologist. Can Urol Assoc J. r Long-term follow-up recommended for hypertension
r Rare hypertension; usually associated with 2010;4(2):9597. and microalbuminuria especially at puberty and in
contralateral renal injury adulthood.
FOLLOW-UP
Patient Monitoring
r Renal US to document involution and contralateral
M
renal growth
Every 6 mo in the 1st 3 yr and then every 12 yr to
assure appropriate renal growth until puberty
Protocol personal preference of physician since
there is no consensus; some follow patients every
312 mo. No frequency of follow-up has been
shown to be beneficial or cost-effective
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NEPHROCALCINOSIS, ADULT
Jennifer E. Heckman, MD, MPH
Stephen Y. Nakada, MD, FACS
PATHOPHYSIOLOGY
r Caused by increase in urinary excretion of calcium,
phosphate, and/or oxalate (1)
May occur with or without hypercalcemia
CaOx and CaPhos crystals result from urinary
supersaturation
CaOx and CaPhos crystals precipitate,
aggregate, and move to interstitium
May result in acute or chronic renal damage
and/or lead to calculus formation
Renal ischemia or injury may augment
nucleation of CaOx or CaPhos crystals
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NEPHROCALCINOSIS, ADULT
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NEPHROTIC SYNDROME
Michael Perrotti, MD
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NEPHROTIC SYNDROME
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NEUROBLASTOMA
Nilay M. Gandhi, MD
Arthur L. Burnett, II, MD, MBA, FACS
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NEUROBLASTOMA
Pathologic Findings r High risk (stage 2 with age >1 yr with unfavorable REFERENCES
r Gross: Solid/cystic vascular, poorly encapsulated histopathology, or stage 3, 4, 4s with N-MYC
purple mass amplification regardless of age): 1. Brodeur GM, Seeger RC, Barrett A. International
r Histology: Intensive chemotherapy with or without bone criteria for diagnosis, staging, and response to
Small round blue cells marrow ablation and repeated surgery treatment in patients with neuroblastoma. J Clin
Mitosis-Karyorrhexis index is prognostic Oncol. 1988;6(12):18741881.
ADDITIONAL TREATMENT
HomerWright pseudorosettes 2. Park JR, Eggert A, Caron H. Neuroblastoma:
r Histopathologic markers: Radiation Therapy Biology, prognosis, and treatment. Pediatr Clin
r Reserved for primary or secondary chemotherapy
N-MYC North Am. 2008;55(1):97120.
failures in low-risk patients 3. Montclair T, Brodeur GM, Ambros PF, et al. The
DNA ploidy r Utilized for local control in bulky stage 3 or
Shimada classification (stroma poor/rich) International Neuroblastoma Risk Group (INRG)
Stroma-poor (based on age, histologic advanced stage 4 staging system: An INRG Task Force report. J Clin
maturation, mitotic rate) Avoid if spinal cord compression due to adverse Oncol. 2009;27(2):298303.
Stroma-rich (nodular, intermixed, well effects on spine growth 4. Maris JM. Recent advances in neuroblastoma.
r Intraoperative radiation therapy not better than
differentiated) NEJM. 2010;362:22012211.
r Neuron-specific enolase (NSE) staining is specific for external beam irradiation 5. Wagner LM, Danks MK. New therapeutic targets
neuroblastoma Additional Therapies for the treatment of neuroblastoma. J Cell
r Periodic acid-Schiff (PAS) staining can distinguish Bone marrow transplantation Biochem. 2009;107(1):4657.
sarcomas Complementary & Alternative
DIFFERENTIAL DIAGNOSIS Therapies (5)
r Ganglioneuroma (benign form) r 13-cis-retinoic acid improves 5-yr overall survival ADDITIONAL READING
r Ganglioneuroblastoma (intermediary between (OS) in children with advanced stage disease after r Matthay KK, George RE, Yu AL. Promising
ganglioneuroma and neuroblastoma) transplantation or intensive chemotherapy therapeutic targets in neuroblastoma. Clin Cancer
r Intra-abdominal mass in childhood: r Iodine131 MIBG targeted delivery for metastatic
Res. 2012;18(10):27402753.
Wilms tumor disease r Wylie L, Philpott A. Neuroblastoma progress on
r Anti-GD2 antibodies (research pending)
Pheochromocytoma many fronts: The neuroblastoma research
Rhabdomyosarcoma symposium. Pediatr Blood Cancer. 2012;58(4):
Lymphoma 649651.
Teratoma
ONGOING CARE
Ewing sarcoma
See Also (Topic, Algorithm, Media)
PROGNOSIS r Abdominal mass, newborn/child, urologic
Rare primary neoplasms of liver and pancreas r Dependent on risk status
considerations
Low risk: Resection is curative, 97% 5-yr OS r Neuroblastoma Image
Intermediate risk: neoadjuvant chemo followed by r Pheochromocytoma
TREATMENT >50% resection, 7090% 5-yr OS r Wilms tumor (nephroblastoma)
GENERAL MEASURES High risk: Neoadjuvant chemo 4 cycles (restage
r Multimodal treatment approach involving surgery, after 2 cycles), >50% resection, radiation,
chemotherapy, radiotherapy, and/or bone marrow or peripheral stem cell transplant, monoclonal Ab,
stem cell transplantation 2040% 5-yr OS CODES
r Nearly all stage 4s patients spontaneously resolve r Better survival in nonadrenal primary tumors
r Shimada classification ICD9
(observation) r 194.0 Malignant neoplasm of adrenal gland
r INSS surgical stage and more recently the Stroma-poor: <10% survival
r 197.7 Malignant neoplasm of liver, secondary
International Neuroblastoma Risk Group (INRG) COMPLICATIONS r 198.5 Secondary malignant neoplasm of bone and
pretreatment system dictates treatment (3) r Dumbbell neuroblastoma with spinal cord
bone marrow
MEDICATION compression
Best treated with chemotherapy ICD10
First Line (4) r C74.90 Malignant neoplasm of unsp part of
r Low risk: None unless surgical failure Neurosurgical intervention only for emergent
decompression unspecified adrenal gland
Cyclophosphamide, Adriamycin, and r Associated with tumor presentation and with r C78.7 Secondary malig neoplasm of liver and
Cisplatin/VM-26 in low-dose cycles
r Intermediate risk: Induction with Cyclophosphamide treatment modalities intrahepatic bile duct
r C79.52 Secondary malignant neoplasm of bone
and Adriamycin with or without radiotherapy FOLLOW-UP
r High risk: Cyclophosphamide, Adriamycin, VM-26, Patient Monitoring marrow
Doxorubicin, Cisplatin, Etoposide in various r Low risk:
combinations Imaging + lab markers 12 mo after therapy, CLINICAL/SURGICAL
every 6 mo for 5 yr, then annually after 5 yr
Second Line
r Intermediate risk: Cisplatin/VM-26 r Intermediate risk: PEARLS N
r High risk: Alternative use of above-listed Imaging + lab markers 12 mo after therapy, r Most common malignancy in infants <1 yr.
combinations every 13 mo for 1st yr, then every 46 mo for r INSS stage determines optimum treatment modality.
25 yr, then annually after 5 yr r Urine HVA and VMA are diagnostic.
SURGERY/OTHER PROCEDURES r High risk: r N-MYC associated with poor prognosis.
r Low risk (stages 1, 2, or 4s with age <1 yr or >1 yr
Imaging + lab markers 12 mo after therapy, r Neuroblastoma requires a minimum of 5-yr
with favorable pathology): every 13 mo for 5 yr, every 6 mo after 5 yr
Surgery is curative follow-up.
Chemotherapy indicated if recurrence, N-MYC Patient Resources
r National Cancer Institute (http://www.cancer.gov/
amplification, or unfavorable histology
r Intermediate risk (stage 3 age <1 yr or >1 yr with cancertopics/types/neuroblastoma)
r National Cancer Comprehensive Network
favorable pathology, or stage 4 <1 yr):
Surgery + multiagent chemotherapy (http://www.nccn.com/living-with-cancer/265-
neuroblastoma.html)
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NOCTURIA
Garjae D. Lavien, MD
Michael J. Naslund, MD
r Nocturnal polyuria
BASICS Relative increased production of urine at night DIAGNOSIS
that is often offset by lowered daytime urine
DESCRIPTION production resulting in normal 24-hr urine volume. HISTORY
r Nocturia is a symptom describing an individual who r Number of times getting up at night to urinate from
Age-related loss of the normal diurnal secretion of
awakens at night one or more times to void. Each vasopressin, resulting in increased nocturnal urine time of going to bed until time of waking in the
void is preceded and followed by sleep. output. morning
r Can negatively impact quality of life. r Degree of bother assessment
Peripheral edema:
Can be associated with depression, daytime Fluid that accumulates in the lower extremities r Differentiate between awakening due to the urge to
fatigue, and increased orthopedic morbidity when upright during the day is mobilized when void vs. awakening due to other sleep disturbances
among the elderly. supine at night, due to an increase in GFR and r Fluid intake habits
r Underlying etiologies of nocturia excretion. r Timing, volume
Nocturnal polyuria: Conditions: CHF, liver disease, nephrotic r Caffeine and alcohol consumption
The rate of urine output is excessive only at night syndrome, hypoalbuminemia, venous r Previous pelvic surgery or radiation
and total 24-hr output is within normal limits. insufficiency, lymphedema, lower extremity r Daytime fatigue and depression
Reduced bladder capacity injury/swelling. r Review of medications known to contribute to
24-hr polyuria Sleep apnea:
Transient periods of hypoxia lead to increased nocturia: such as diuretics, excessive calcium
Sleep disorder
pulmonary vascular resistance and secretion of supplementation, antacids, or lithium.
EPIDEMIOLOGY r Swelling of lower extremities
atrial natriuretic peptide, a potent diuretic.
Incidence Medications: Poorly timed/dosed diuretics that PHYSICAL EXAM
r The incidence of nocturia and total number of r Global or focal neurologic deficits
exert maximal effect during sleeping hours.
voiding episodes increases with age Excessive fluid intake prior to bedtime, resulting in r Digital rectal: Assess anal tone, prostate exam in
Overall: 28% a physiologic large volume excretion. men
Age >60: 41% r Reduced bladder capacity r Pelvic exam in women: Anterior prolapse causing
r Body mass index >29: 36%
Nonneurogenic or Neurogenic OAB (over active retention, urethral diverticulum, atrophic vaginitis
r Black and Hispanic > White bladder) causing irritative urinary symptoms
Prevalence Inflammatory: UTI, radiation cystitis, bladder r Lung auscultation for rales, crackles
r Higher prevalence in women than men among calculi, interstitial cystitis r Dependent edema, pedal edema
young adults Neoplastic: Bladder cancer, prostate cancer, r Suprapubic distension consistent with urinary
r Higher prevalence in men than women among extrinsic compression from pelvic masses
Traumatic: Spinal cord injury, urethral stricture, retention
elderly population groups r Obesity and a wide neck circumference raises the
injury to pelvic nerves or bladder, foreign body
RISK FACTORS (1) within bladder possibility of sleep apnea
r Advanced age Obstructive; BPH, urethral stricture
r Diuretic usage
DIAGNOSTIC TESTS & INTERPRETATION
r Lower urinary tract dysfunction ASSOCIATED CONDITIONS Lab
r Bladder outlet obstruction r Urinalysis: Low specific gravity (polyuria), RBCs (rule
r Cardiac disease r OAB: Idiopathic and neurogenic out stones, bladder cancer, foreign body, etc.),
r Obesity, sleep apnea r Detrusor hyperactivity with impaired contractility proteinuria (nephrotic syndrome), glucosuria
Genetics r Radiation cystitis (diabetes mellitus), pyuria (UTI)
r Diabetes mellitus r Urine culture: UTI
None
r Psychogenic polydipsia r Urine osmolality: Dilute low values suggest
PATHOPHYSIOLOGY inappropriate excretion of ADH or excess intake of
r 24-hr polyuria: r Depression
r Obesity water
Excessive total urine production where the total r PSA if indicated
24-hr urinary volume >40 mL/kg) r See also Pathophysiology
r Serum electrolytes: Hypokalemia with diuretic use,
Diabetes mellitus:
Secondary to polydipsia and osmotic diuresis GENERAL PREVENTION CHF, or hyperaldosteronemia
r Avoid excessive evening fluid intake, alcohol, and
from hyperglycemia Imaging
Diabetes insipidus: caffeine r Bladder US with PVR volume for suspected urinary
r Closely monitor and control the underlying
Under-secretion (central) or impaired response retention, especially if considering antimuscarinics
(nephrogenic) to ADH conditions that cause nocturia r Renal US may demonstrate hydronephrosis in cases
Medications: of urinary retention or poorly compliant bladders
Lithium, diuretics, caffeine, nephrotoxic
medications
Hypercalcemia: Can cause osmotic diuresis
Hyperaldosteronism
Psychogenic polydipsia
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NOCTURIA
Diagnostic Procedures/Surgery SURGERY/OTHER PROCEDURES 4. Van Kerrebroeck PE, van Voskuilen AC, Heesakkers
r Voiding diaries JP, et al. Results of sacral neuromodulation therapy
Sacral neuromodulation for nocturia secondary to
All voiding episodes and volumes should be reduced voided volumes is associated with refractory for urinary voiding dysfunction: Outcomes of a
recorded for a 24-hr period; the time the patient daytime frequency and urgency (4)[B]. prospective, worldwide clinical study. J Urol.
actually goes to sleep and awakens for the day 2007;78:2029.
should also be noted. ADDITIONAL TREATMENT 5. Johnson TM 2nd, Burgio KL, Redden DT, et al.
Nocturnal urine volume (NUV) is the total volume Radiation Therapy Effects of behavioral and drug therapy on nocturia
of urine voided during the night (the 1st morning N/A in older incontinent women. J Am Geriatr Soc.
void is included in this sum since it represents Additional Therapies 2005;53:846.
urine excreted during sleep hours). r Behavioral training:
Nocturnal polyuria index (NPi): NUV divided by
the total volume voided over the 24-hr period: Pelvic floor muscle exercises, +/ biofeedback: ADDITIONAL READING
NPi >33% = nocturnal polyuria More effective than both drug therapy and
Nocturnal Bladder Capacity index (NBCi) placebo in treatment of nocturia associated with r Cornu JN, Abrams P, Chapple CR, et al. A
NBCi = (NUV/Maximal volume per void)1 daytime urgency and urge incontinence (5)[A] Contemporary assessment of nocturia: definition,
NBCi >0 suggests that the nocturnal bladder r CPAP for obstructive sleep apnea epidemiology, pathophysiology, and
capacity cannot store the amount of urine made managementa systematic review and
Complementary & Alternative meta-analysis. Eur Urol. 2012;62;877890.
at night.
r Urodynamics Therapies r Weiss JP, Blaivas JG, Bliwise DL, et al. The
None
Helpful when empiric treatment for overactive evaluation and treatment of nocturia: a consensus
bladder (OAB) or bladder outlet obstruction has statement. BJU Int. 2011;108:621.
failed to improve nocturia ONGOING CARE See Also (Topic, Algorithm, Media)
r Polysomnographic sleep studies: Differentiate r Bladder Outlet Obstruction
PROGNOSIS
between sleep disorder and true nocturia r Diabetes Mellitus
Although it is often difficult to completely eliminate
Pathologic Findings episodes of nocturia, characterizing nocturia according r Incontinence, Adult Female
N/A to cause-specific etiologies allows for cause-specific r Incontinence, Adult Male
treatment. r Neurogenic Bladder
DIFFERENTIAL DIAGNOSIS
r Sleep disorders: r Nocturia Algorithm
COMPLICATIONS
Most patients awaken due to the sleep r Traumatic falling accidents, including hip fractures, r Nocturnal Polyuria
disturbance, but recall this as an awakening to from rising from sleep to urinate r Overactive Bladder
void. r DDAVP can lead to hyponatremia r Urgency, Urinary (Frequency and Urgency)
May need polysomnography r Urinary retention secondary to antimuscarinics r Urodynamics
r Urologic r Voiding Diary (see Section VII: Reference Tables)
Bladder outlet obstruction, OAB, incomplete FOLLOW-UP
bladder emptying. Patient Monitoring
r Nonurologic: r Bladder sonography with PVR as needed,
Renal failure, idiopathic nocturnal polyuria, particularly when treating men with antimuscarinics CODES
diabetes mellitus, central diabetes insipidus, r Repeat 24-hr voiding diaries
nephrogenic diabetes insipidus, primary r Regular monitoring of serum electrolytes with ICD9
r 596.59 Other functional disorder of bladder
polydipsia, hypercalcemia, drugs, autonomic DDAVP, starting 3 days after initiation of treatment r 788.42 Polyuria
failure, obstructive sleep apnea. Patient Resources r 788.43 Nocturia
r Medline Plus Excessive Urination at Night
TREATMENT http://www.nlm.nih.gov/medlineplus/ency/ ICD10
article/003141.htm r N31.9 Neuromuscular dysfunction of bladder,
GENERAL MEASURES unspecified
r Nocturnal polyuria secondary to diuretics r R35.1 Nocturia
Change to afternoon dosing to induce an early REFERENCES r R35.8 Other polyuria
evening diuresis rather than a nocturnal diuresis 1. Fitzgerald MP, Litman HJ, Link CL, et al. The
r Treatment of underlying condition associated with
association of nocturia with cardiac disease,
nocturia diabetes, body mass index, age and diuretic use:
CLINICAL/SURGICAL
MEDICATION Results from the BACH survey. J Urol. 2007;177: PEARLS
First Line 13851389. r The etiology of nocturia is not prostate or bladder
r Antimuscarinics are appropriate for reduced voided 2. Johnson TM 2nd, Burrows PK, Kusek JW, et al. The
related in the majority of men. Poor sleep pattern
volumes. effect of doxazosin, finasteride and combination
and fluid consumption/mobilization need to be
r Men only: -blocker alone or combined with a therapy on nocturia in men with benign prostatic
considered. N
hyperplasia. J Urol. 2007;178:2045. r A voiding diary is extremely helpful to determine the
5--reductase inhibitor (only modest benefit) 3. Mattiasson A, Abrams P, Van Kerrebroeck P, et al.
(2)[A]. cause of nocturia.
Efficacy of desmopressin in the treatment of
Second Line nocturia: A double-blind placebo-controlled study
r DDAVP for nocturia associated with nocturnal in men. BJU Int. 2002;89:855.
polyuria (3)[B]:
Dosing: 0.01 mg PO; titrate up to 0.04 mg.
DDAVP has a high risk of hyponatremia.
Greatest risk seen in men >65 yr old.
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DIFFERENTIAL DIAGNOSIS
r Epididymitis
ADDITIONAL TREATMENT ADDITIONAL READING
Radiation Therapy r Zipprich J, Murray EL, Winter K, et al. Mumps
r Granulomatous orchitis, infectious and noninfectious There is no role for radiation therapy
r Reactive hydrocele outbreak on a university campus - California, 2011.
r Scrotal pyocele
Additional Therapies MMWR 2012;61:986989.
Interferon-2B has been investigated in bilateral r Yapanoglu T, Kocaturk H, Aksoy Y, et al. Long-term
r Testicular malakoplakia mumps orchitis, given that the mumps virus replicates
r Testicular torsion efficacy and safety of interferon-alpha-2B in patients
with a virion-associated transcriptase
r Torsion of testicular appendage with mumps orchitis. Int Urol Nephrol 2010;42:
Complementary & Alternative 867871.
r Testicular tumor
Therapies
Patient specific referral for psychologic evaluation and
See Also (Topic, Algorithm, Media)
r Acute Scrotum
TREATMENT support for chronic refractory orchitis r Mumps Orchitis
r Orchitis, General Considerations Image
GENERAL MEASURES r Orchitis, Granulomatous
r Supportive in nature ONGOING CARE
r Scrotum and Testicle, Mass
Bed rest PROGNOSIS
Hot or cold packs for analgesia r Most cases of mumps orchitis are self-limited, r Testis, Pain (Orchalgia)
Applied for 1015 mins q.i.d or until pain r Testis, Tumor and Mass, Adult, General
resolving within 3-10 days
subsides r With appropriate antibacterial coverage, most cases r Testis
Scrotal elevation and support with tight fitting of bacterial orchitis resolve without complication
underwear or athletic support Analgesics
Nonsteroidal anti-inflammatory drugs (NSAID) COMPLICATIONS
r Unilateral testicular atrophy in up to 60% with
CODES
Antiemetics
Counsel patient on safe sex practices if STD mumps orchitis ICD9
suspected r Sterility is rarely a sequel of unilateral orchitis r 604.90 Orchitis and epididymitis, unspecified
r Impaired fertility reported rates of 713% r 604.91 Orchitis and epididymitis in diseases
MEDICATION r No definitive evidence for increased risk of testicular
First Line classified elsewhere
r There are no targeted medications indicated the tumor with history of orchitis r 604.99 Other orchitis, epididymitis, and
treatment of viral orchitis. Supportive care is FOLLOW-UP epididymo-orchitis, without mention of abscess
essential. Patient Monitoring
r Bacterial orchitis requires coverage with appropriate r Most patients can be safely monitored in an ICD10
r N45.1 Epididymitis
antibiotic for suspected pathogen(1)[C] outpatient setting r N45.2 Orchitis
<35 years old, suspected STD as causative agent: r A patient with a STD as the cause of orchitis should
r N45.3 Epididymo-orchitis
Ceftriaxone 125-250 mg IM once and either be tested for other STDs including Human immune
doxycycline 100 mg PO b.i.d. for 7 days or deficiency virus (HIV)
azithromycin 1-2 g PO once
>35 years old, or epididymo-orchitis secondary to Patient Resources CLINICAL/SURGICAL
r http://www.mayoclinic.com/health/orchitis
UTI: PEARLS
r http://www.urologyhealth.org
Additional gram-negative coverage with a r Most cases of orchitis are viral in nature and
fluoroquinolone or
self-limited, other cases are bacterial and most
trimethoprim-sulfamethoxazole (TMP-SMX)
r Tailor antibiotic prescription to local resistance REFERENCES commonly associated with epididymitis.
r Physical exam findings include tender, swollen testes
patterns of most common UTI pathogens 1. Nicholson A, Murray-Thomas T, Hughes G, et al. with associated erythema of the scrotum with or
Second Line Management of epididymo-orchitis in primary care: without fever.
N/A results from a large UK primary care database. Br J r Testicular ultrasonography is important to rule out
Gen Pract 2010;579:407422. torsion and malignancy.
SURGERY/OTHER PROCEDURES 2. Nariculam J, Minhas S, Adeniyi A, et al. A review of
r Surgical intervention is generally not indicated in the r Medical therapy for orchitis is largely supportive;
the efficacy of surgical treatment for and antibiotic coverage should be targeted to cover STDs
treatment of acute or chronic orchitis pathological changes in patients with chronic
r Associated scrotal pyocele or symptomatic hydrocele in the young and sexually active and UTIs in the
scrotal pain. BJU Int 2007;99:10911093. elderly.
may require surgery 3. Larsen SM, Benson JS, Levine LA. Microdenervation r The role for surgical management of orchitis is
r Orchidectomy for chronic orchitis refractory to of the spermatic cord for chronic scrotal content limited.
supportive measures is an option, but patients pain: single institution review analyzing success
must be counseled surgery may not alleviate pain rate after prior attempts at surgical correction.
(2)[B] J Urol 2013 189:554558.
r Consider microsurgical denervation of cord for
chronic refractory orchitis/orchalgia following
favorable response to spermatic cord block (3)[A]
10 mL of 0.5% bupivacaine injected to cord for
block
O
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Diagnostic Procedures/Surgery
BASICS DIAGNOSIS r Symphysogram of joint
Pain on injection of contrast diagnostic (4)[C]
DESCRIPTION HISTORY Generally replaced by MRI
r Osteitis pubis is a painful sterile inflammatory r Inciting event such as a pelvic procedure or trauma r Aerobic/anaerobic culture of joint aspirate to rule
condition affecting the pubic symphysis r Insidious onset of suprapubic pain
out infection if clinically indicated
Most commonly seen in athletes r Pain radiating to thigh adductors, lower abdomen,
r First described with suprapubic surgery and remains perineum Pathologic Findings
r Pain worse when walking or when rising from a Sclerotic changes in bony architecture and
a potential complication of pelvic procedures
degeneration of hyaline cartilage with normal
seated position (1)[C]
EPIDEMIOLOGY periosteum (5)[C]
Incidence PHYSICAL EXAM DIFFERENTIAL DIAGNOSIS
r Overall incidence in nonathlete populations r Point tenderness over pubic symphysis
r Osteomyelitis (the most critical)
unknown r Waddling gait
r Neoplasia of pelvic rami
0.16% in procedures using bone anchors r Low-grade fever
r Bony metastases
r Increased pain with coughing or Valsalva
Prevalence r Pubic osteolysis
r Painful hip abduction
Overall prevalence in nonathlete populations r Sports hernia (athletic pubalgia, sportsmans hernia)
unknown DIAGNOSTIC TESTS & INTERPRETATION r Adductor strain
RISK FACTORS r Muscle tears
r Invasive pelvic procedures ALERT r Avulsion injuries
Several urologic procedures implicated Must rule out osteomyletis, especially in r Stress fractures
Radical prostatectomy postoperative patients. r Tears of acetabular labrum
Prostate cryotherapy
TRUS Bx of prostate Lab
r Not generally required to make diagnosis
TURP
r May see moderate leukocytosis and an increased
TREATMENT
Retropubic urethropexy: Specifically
MarshallMarchettiKrantz procedure erythrocyte sedimentation rate (2)[C] GENERAL MEASURES
Sling procedures Raised levels of acute phase proteins (fibrinogen, r Rest, heat, or ice
Pelvic radiation C-reactive protein), and increased erythrocyte r Physical therapy to strengthen pelvic girdle can be
r Trauma sedimentation rate are more suggestive of considered
r Rheumatic disorders osteomyelitis
MEDICATION
r Pregnancy/parturition Imaging
r Pelvic radiograph First Line
r Overuse syndrome in athletes r Nonsteroidal anti-inflammatory
Typically normal in acute phase Ibuprofen 200800 mg 24/d (max dose
Genetics Articular surface erosion, sclerosis, osteophyte
No known genetic predisposition 2.4 g/d)
formation Naproxen 250500 mg 2/d (max dose 1.5 g/d
PATHOPHYSIOLOGY r Scintigraphy
r Symphysis pubis is a nonsynovial amphiarthrodial for limited time)
Increased uptake around pubic symphysis r Cyclooxygenase-2 (COX-2) inhibitor
joint at the confluence of the two pubic bones, r Symphysogram of joint
Celecoxib 100200 mg 12/d
consisting of an intrapubic fibrocartilaginous disc Extravasation of contrast material Adverse CV events noted with COX-2 inhibitors,
between thin layers of hyaline cartilage Diagnostic and therapeutic use lowest effective dose for shortest duration
r Etiology unknown but may be related to periosteal r Magnetic resonance imaging (MRI) most sensitive possible
trauma and considered gold standard
Second Line
ASSOCIATED CONDITIONS Acute (<6 mo): Bone marrow edema, fluid in r Oral glucocorticoids such as prednisone if local
r Ankylosing spondylitis joint, periarticular edema
glucocorticoid injections fail
r Rheumatoid arthritis Chronic (>6 mo): Subchondral
Typical short course (ie, 60 mg for 5 days)
sclerosis/resorption, bony margin irregularities,
Can use a taper dose
GENERAL PREVENTION osteophytes (3)[C]
N/A
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PROGNOSIS
r Typically a drawn out and variable clinical course
Symptoms can last several months to several years
Operative procedures may be needed in 510% of
cases
COMPLICATIONS
r Wedge or wide resection of pubic symphysisrisk
of posterior instability of pelvic girdle leading to
damage to sacroiliac joints
r Arthrodesisrisk of nonunion or death of bone
graft site requiring additional surgery
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PATHOPHYSIOLOGY Imaging
BASICS r The renal papilla normally exists in the state of r CT has become the imaging modality of choice
hypoxia because of the blood flow in the vasa recta r Contrast images show:
DESCRIPTION which can be affected further with conditions that Ring shadows in the medullae
r Renal papillary necrosis is ischemic necrosis of the reduce blood flow Contrast-filled clefts in the renal parenchyma
papillae and occasionally the medullary pyramids. Perfusion compromise in diabetes mellitus Renal pelvic filling defects
r The clinical course may be acute and rapidly Diminution in blood flow because of sickling of r Excretory urography has historically been the gold
progressive or chronic blood cells (sickle cell disease) standard for diagnosis
Acute forms are symptomatic and may present Infection that causes inflammation of the Findings include shrinkage and irregularity of
with hydronephrosis, pyelonephritis, and interstitium can lead to compression of the papilla defined by contrast materials as a ring
hematuria medullary vasculature shadow often in a triangular shape
Typically chronic forms are asymptomatic and r Analgesic use causes COX inhibition and decreased A calix without a papilla
discovered incidentally on radiographic studies prostaglandin production. This leads to decreased Filling defect in the renal pelvis or ureter
r Acute presenting symptoms include hematuria, flank vascular perfusion, vasoconstriction and can cause Contrast containing rice-grainsized cavities in the
or abdominal pain, and fever and chills ischemic necrosis papilla
r Some medications can cause direct interstitial cell r Retrograde pyelogram:
EPIDEMIOLOGY
necrosis and decrease in prostaglandin production Useful in patients with azotemia, contrast
Incidence r The necrotic, soft tissue can cause unilateral or
r Most cases occur after the 6th decade of life and sensitivity, or other situations where intravenous
bilateral ureteral obstruction contrast is contraindicated
papillary necrosis is uncommon in patients <40 yr
Findings may reveal a club-shaped calyx or a
r Female > Male (1.1:1.0) (1)[B] ASSOCIATED CONDITIONS
r Analgesic abuse filling defect in the ureter
Prevalence r Diabetes mellitus Diagnostic Procedures/Surgery
N/A r Pyelonephritis Patient presenting with hematuria needs a full urologic
RISK FACTORS r Sickle cell disease workup even if papillary necrosis is confirmed.
r Include any condition causing ischemia that can r Urinary tract obstruction Pathologic Findings
predispose to the development of renal papillary r The cortex features depressed areas of cortical
necrosis. Many have >2 risk factors GENERAL PREVENTION atrophy (3)
r Treatment of underlying disorders including diabetes r Papilla shows various stages of necrosis,
r Diabetes mellitus
r Sickle cell trait or disease or sickle disease desquamation, and sloughing
r Avoidance of analgesic use
r Analgesic abuse: Focal necrosis: Involves only the tip of the papilla
Most commonly phenacetin and NSAIDs Diffuse necrosis: The entire papilla and portions of
r Antiretroviral treatment: DIAGNOSIS the medulla are involved
r Microscopically, changes of papilla may be a patchy
Indinavir
r Urinary tract obstruction of any cause HISTORY appearance or complete coagulative necrosis.
r Pyelonephritis r May present with hematuria or obstruction with Glomeruli are typically unchanged
r Systemic vasculitis flank pain (2)
r With infection, fever, chills, dysuria, frequency, DIFFERENTIAL DIAGNOSIS
r Lupus nephritis r Acute tubular necrosis
r Wegener granulomatosis urgency, flank pain, and renal colic can occur r Nephrolithiasis
r Rarely, bilateral ureteral obstruction with necrotic
r Renal artery stenosis r Carcinoma of the ureter or bladder
r Systemic vasculitis tissue can present as acute oliguric renal failure r NSAID abuse and/or overuse
r Global ischemia: PHYSICAL EXAM r Pyelonephritis
r Costovertebral angle tenderness r Renal trauma
Shock, hypoxia, dehydration r Fever r TB
Genetics r Ureteral stricture disease
N/A DIAGNOSTIC TESTS & INTERPRETATION
Lab
r Urinalysis and urine culture:
Proteinuria, pyuria, bacteriuria, and low
urine-specific gravity
Epithelial cells and casts may be present
r CBC may demonstrate leukocytosis
r Metabolic panel can demonstrate azotemia and
elevated creatinine
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ADDITIONAL READING
TREATMENT ONGOING CARE
Chung DC, Kim SH, Jung SI, et al. Renal papillary
GENERAL MEASURES PROGNOSIS necrosis: Review and comparison of findings at
r Hydration, oral or intravenous Depends on the basis for the ischemia, the multi-detector row CT and intravenous urography.
r Glycemic control, if diabetic compounding factors, and the amount of necrosis Radiographics. 2006;26:18271836.
r Definition and treatment of sickle disease COMPLICATIONS See Also (Topic, Algorithm, Media)
r Infection may develop in the desquamated necrotic r Diabetes Mellitus, Urologic Considerations
MEDICATION
papilla r Filling Defect, Upper Urinary Tract (Renal Pelvis and
First Line r Calculi can develop on the base of the sloughed
r Cessation of any associated/causative medications Ureter)
papilla r Hematuria, Gross and Microscopic, Adult
including analgesics r Obstruction can develop along the ureter from
r Treatment of underlying cause of ischemia r Nephropathy, Analgesic
r Broad-spectrum antibiotics, if associated with multiple sloughed papilla r Papillary Necrosis, Renal Image
FOLLOW-UP r Sickle Cell Disease, Urologic Considerations
pyelonephritis
Second Line Patient Monitoring
r Monitoring includes the kidney itself for further
N/A
necrosis and for changes in function CODES
SURGERY/OTHER PROCEDURES r Causes of ischemia should be closely monitored
r When a patient presents with acute urinary
Patient Resources ICD9
obstruction, drainage is indicated with percutaneous r 584.7 Acute kidney failure with lesion of renal
nephrostomy, ureteral stent placement, or http://www.scripps.org/articles/1151-renal-papillary-
necrosis medullary [papillary] necrosis
endoscopic/ureteroscopic removal of obstructing r 590.80 Pyelonephritis, unspecified
sloughed tissue r 591 Hydronephrosis
r In the nonacute case, renal pelvic or ureteral filling
REFERENCES
defect can be electively evaluated with ureteroscopy ICD10
r Nephrectomy is rarely warranted 1. Vijayaraghavan SG, Kandasamy SV, Mylsamy A, r N12 Tubulo-interstitial nephritis, not spcf as acute or
et al. Sonographic features of necrosed renal chronic
ADDITIONAL TREATMENT papillae causing hydronephrosis. J Ultrasound Med. r N13.30 Unspecified hydronephrosis
Radiation Therapy 2003;22(9):951956. r N17.2 Acute kidney failure with medullary necrosis
N/A 2. Gordon M, Cervellione RM, Postlethwaite R, et al.
Additional Therapies Acute renal papillary necrosis with complete
N/A bilateral ureteral obstruction in a child. Urology. CLINICAL/SURGICAL
2007;69:575e11575e12. PEARLS
Complementary & Alternative
Therapies 3. Amuluru K, Erickson BA, Okotie O, et al. Bilateral
N/A ureteral obstruction from papillary necrosis Gross hematuria in a patient with sickle cell disease
secondary to household cleaner ingestion. Can J suggests papillary necrosis.
Urol. 2009;16(3):47014703.
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PARATESTICULAR TUMORS
Mohamed T. Ismail, MD
Sallyanne M. Fisher, MSN, FNP-C, CUNP
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PARATESTICULAR TUMORS
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r Urine
BASICS DIAGNOSIS Urine pregnancy test
Urine analysis
DESCRIPTION HISTORY Urine culture
r Chronic pelvic pain (CPP) is defined as discomfort r History of present illness
Nucleic acid amplification test for gonorrhea and
below the umbilicus lasting 6 mo Onset/pallaition or provocation Chlamydia
r Etiology often unclear and symptom severity often quality/radiation/severity/timing (OPQRST) of pain Cytology, if hematuria to evaluate for bladder
out of proportion to objective findings Alleviating or aggravating factors cancer
r Bears impact on physical, mental, emotional, and Ask if symptomatic during sexual intercourse r Others
sexual well-being Menstrual history Cervical culture
r Past medical and surgical history
Vaginal wet mount
EPIDEMIOLOGY Check history of PID, STIs, ectopic pregnancy PAP smear
Incidence Obtain history of trauma Fecal occult blood test
N/A Abdominal and pelvic surgeries contribute to
adhesions Imaging
Prevalence r Ultrasound
r Difficult to ascertain due to varied definition Check trigger points from incisional scars
r Family and social history Transvaginal and/or pelvic ultrasound: Modality of
Affects 1 in 7 women choice in the initial evaluation of pelvic pain
39% prevalence rate in primary care setting 1st-degree family with CPP
Inquire about physical and/or sexual abuse Renal and bladder ultrasound: Assess
Accounts for 10% of all gynecologic referrals hydronephrosis, renal stone disease, and bladder
Number of sex partners, method of contraception
RISK FACTORS Substance dependence, exposure to analgesics distension
r Depression, anxiety r Plain films
r Personal history of abuse PHYSICAL EXAM Kidney, ureter, bladder x-ray (KUB): Assess urinary
r Vital signs
r Prior sexually transmitted infections (STIs) stone burden or dermoid cyst
r Prior pelvic inflammatory disease (PID) increases risk Fever, hypotension, and tachycardia suggest Spinal and bony x-ray: Indicated when osseous
infectious etiology and skeletal etiologies of pelvic pain are suspected
4-fold, prior STI/STD r Abdominal exam r Hysterosalpingography: Allow anatomic evaluation
r Substance dependence
r 1st-degree family with CPP Search for trigger points of the uterus and fallopian tubes
Assess peritoneal signs r Pelvic venogram: Assess pelvic vascular anatomy
Genetics Sensory evaluation of dermatomes and venous congestion
r Twin studies and familial clustering do suggest r Back and musculoskeletal exam r Axial imaging (CT, MRI)
genetic basis for increased nociception Evaluate posture and gait Indicated when ultrasound negative or
r No established inheritance pattern Rule out scoliosis or lordosis inconclusive
r Pelvic exam
PATHOPHYSIOLOGY With intravenous and/or oral contrast
r Exact mechanism unknown Inspect vulva for skin lesions, signs of trauma, and More sensitive evaluation of the gastrointestinal
r Complex and multifactorial, combining, biologic, irritation and genitourinary systems
Speculum exam to assess vaginal mucopurulent
psychological, and social factors discharge and erythema Diagnostic Procedures/Surgery
r Diagnostic laparoscopy
ASSOCIATED CONDITIONS One-hand pelvic exam to identify muscular trigger
r Endometriosis, ectopic pregnancy, ovarian cysts, points, cervical motion tenderness, urethral Endometriosis most common (33%)
tenderness, and to delineate bladder base and Adhesions (24%)
adhesions
r Urinary tract infections (UTIs), STIs, and PID vaginal fornix Negative 3566% of the time
r Irritable bowel syndrome (IBS) Bimanual exam to assess uterine shape, direction, Negative findings do not exclude somatic cause
tenderness and mobility; assess adnexal masses and positive findings do not necessarily represent
r Interstitial cystitis (IC)
and tenderness true etiology of CPP
r Rectal exam r Barium enema or colonoscopy
GENERAL PREVENTION
r Prompt recognition r Urodynamics
Check rectal tone, rectovaginal septum,
r Safe sex practices r Cystoscopy, bladder biopsy, hydrodistension
cul-de-sac, and uterosacral ligaments
DIAGNOSTIC TESTS & INTERPRETATION Pathologic Findings
Based on diagnosis
Lab
r Serum DIFFERENTIAL DIAGNOSIS
Complete blood count: Leukocytosis and left shift r Gynecologic: Accounts for 20% of CPP
suggest infection Cervical stenosis
Erythrocyte sedimentation rate: Nonspecific Chronic PID (occurs after 30% of acute PID)
markers of subacute or chronic inflammation Endometriosis/chronic endometriosis
Cancer antigen-125: Marker for endometriosis, Gynecologic cancers
PID, and certain cancers Pelvic congestion syndrome
-Human chorionic gonadotropin: Becomes Uterine fibroids
positive 7 days after conception, a negative test
excludes ectopic pregnancy
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r Malfunction
BASICS Mechanical failure rates are 15% at 5 yr and 30% DIAGNOSIS
at 10 yr
DESCRIPTION Common reasons include aneurysm, tubing HISTORY
r While generally very reliable, penile prostesis can breakage, reservoir leakage, and connector failure Assess for fever, chills, pain, lethargy, fatigue, change
become infected, undergo extrusion and suffer form Auto inflation is usually due to improperly in bowel or bladder function, dysuria, frequency,
mechanical failure. positioned reservoir urethral discharge.
r 2 types of penile prosthesis, malleable (semirigid,
ASSOCIATED CONDITIONS PHYSICAL EXAM
noninflatable, nonhydraulic) and inflatable. r Conditions associated with erectile dysfunction r Assess penis/scrotum for erythema, edema,
Inflatables consist of 2-piece (pump and cylinders) induration, pain in palpation of penis/scrotum,
Adrenal disorders
and 3-piece (pump, cylinders, and reservoir). presence of wound drainage, adherence of
r Implanted via suprapubic or penoscrotal approach. AIDS-associated neuropathy
Alzheimers prosthesis components to skin.
r Meticulous sterility is required. r Erosion/extrusion of device through glans, urethral
Cardiac arterial disease
r Infections of any or all parts of the device meatus, scrotal skin, or perineum.
CNS infections
components require removal of the entire device. CNS tumors r Assess functionality of device by
r Extrusion/erosion of the device may occur into or Diabetes mellitus (Type I and II) inflation/deflationif suboptimally rigid or deflated
through the urethra, penile glans, proximal crura, History of kidney or liver transplant pump, consider fluid leak.
bladder or bowel, or adjacent vascular structures. History of myocardial infarction r Assess penile contour/morphology upon inflation:
r Mechanical breakdown may manifest as inability to History of prostatectomy, cystectomy, or colectomy Buckling of cylinder or S-shaped deformity
inflate/deflate device, abnormal erectile morphology, Hyperprolactinemia suggests oversizing of cylinders.
or auto inflation. Hypertension Floppy glans (SST deformity) suggests undersized
Hyperthyroidism cylinders or inadequate corporal dilation.
EPIDEMIOLOGY Hypogonadism
Incidence Hypothyroidism
DIAGNOSTIC TESTS & INTERPRETATION
N/A Liver failure Lab
r Urinalysis
Prevalence Multiple sclerosis
r Overall infection rate: 18% r Urine culture and sensitivity
Peripheral vascular disease
r Prosthesis revision infection rate: 1013% Renal failure r CBC with differential
r Prosthesis revision through infected field infection r Metabolic profile
GENERAL PREVENTION r Erythrocyte sedimentation rate
rate: 18% r The preoperative assessment should include issues
r Mechanical failure rate 2-piece: 5% @ 5 yr such as the patients needs and expectations of the Imaging
r Mechanical failure rate 3-piece: 18% @ 15 yr device (1,2) r Usually not necessary
Issues such as complications and the irreversibility r Ultrasound scrotummay reveal abscess
RISK FACTORS of the procedure should be exhaustively discussed r MRI (with device inflated)useful in assessment of
r Infection: Diabetes, spinal cord injury, previous
and documented through informed consent corporal abnormalities.
penile prosthesis, immunocompromised state, h/o r Infection
UTI, obesity Diagnostic Procedures/Surgery
r Extrusion/erosion: Previous surgery, previous pelvic Ensure UTI or infectious skin rash is absent
Cystourethroscopy may reveal urethral erosion of
Tight control of serum glucose and HbA1C
radiation, penile fibrosis, aggressive dilation, lack of cylinders or erosion of device component into bladder.
Preoperative parenteral antibiotic of vancomycin
surgical experience, Peyronie disease, previous + aminoglycoside or imipenem Pathologic Findings
penile prosthesis, upsizing of cylinders Meticulous adherence to sterile technique N/A
r Mechanical failure: Inadequate dilation of reservoir
Limit OR traffic DIFFERENTIAL DIAGNOSIS
space 10-min scrub of operative area r Intraoperative complications (4)
Genetics 10-min scrub for OR staff During corporal body dilation: Urethral
N/A Use of alcohol-based solution for final prep perforation, cross over perforation of opposite
Avoid having prosthesis contact skin crura during dilation
PATHOPHYSIOLOGY Use antibiotic-coated/antibiotic dripped
r Infection Reservoir position: Bladder perforation or
prosthesis (3) improper positioning during the implant procedure
18% this percentage increases with number of Postoperative oral antibiotics 710 days
revision surgeries Component failure: Check device function before
postoperatively implantation; careful technique to avoid cylinder
Most common bacteria Staphylococcus r Extrusion/erosion
epidermidis injury during corporal body closure
Avoid aggressive corporal dilation r Postoperative complications:
Other bacteria: MRSA, Pseudomonas,
Avoid upsizing of cylinders Infection
Enterococcus, Prevotella, Morganella
Avoid early/premature inflation of device Erosion (oversized cylinder): Often associated with
Gram-negative bacteria may be associated with r Malfunction
rapid infection pain and buckling
Biofilm plays important role in bacterial adherence Place corporotomy closing sutures before device Undersized cylinder (concorde deformity or
and infection insertion to avoid iatrogenic puncture floppy glans) whereby there is excess mobility
r Extrusion/erosion Demonstrate proper function and placement of of the glans
the device prior to conclusion of surgery Cylinder aneurysm
Erosion through skin is inherently infected
Pre-existing infection may hasten erosion Fluid leak
Iatrogenic-facilitated erosion may result from Auto inflation/inability to deflate or inflate
overaggressive dilation
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MEDICATION Additional Therapies 3. Johnson TV, Hsiao W, Delman KA, et al. Extensive
r Adjuvant chemotherapy inguinal lymphadenectomy improves overall 5-year
First Line
r Antibiotics 3 courses of cisplatin and 5-fluorouracil for pN23 survival in penile cancer patients: Results from the
Use of antibiotics has become controversial for No adjuvant chemotherapy for pN1 surveillance, epidemiology, and end results
enlarged LNs Complementary & Alternative program. Cancer. 2010;116:29602966.
Historically, a 46-wk course of antibiotics (such Therapies 4. Master VA, Jafri SM, Moses KA, et al. Minimally
as a cephalosporin or augmentin) was None are effective invasive inguinal lymphadenectomy via endoscopic
recommended to rule out infection in Tis and Ta groin dissection: Comprehensive assessment of
tumors with palpable LNs immediate and long-term complications. J Urol.
Delay in LAD, a potentially curative treatment, has ONGOING CARE 2012;188:11761180.
brought the use of antibiotics into question 5. Kattan MW, Ficarra V, Artibani W, et al.
FNAC can help determine if LNs are due to
PROGNOSIS
r 5-yr cancer-specific survivals: Nomogram predictive of cancer specific survival in
metastasis or infection patients undergoing partial or total amputation for
90100% in pN0 disease
Second Line squamous cell carcinoma of the penis. J Urol.
7080% in pN1 disease
N/A 2006;175:21032108; discussion 2108.
<30% in pN2pN3 disease
SURGERY/OTHER PROCEDURES 15% in patients with positive pelvic LNs who had
r Radical LAD (2,3) inguinal and pelvic LAD
r Predictors of cancer-specific survival: Pathologic ADDITIONAL READING
Superior margin: Superior margin of the external
ring to the anterior superior iliac spine (ASIS) stage of LNs, vascular and/or lymphatic involvement, Spiess PE, Horenblas S, Pagliaro LC, et al. Current
Lateral margin: ASIS to 20 cm inferiorly primary tumor thickness concepts in penile cancer. J Natl Compr Canc Netw.
Medial margin: Pubic tubercle to 15 cm inferiorly COMPLICATIONS 2013;11:617624.
Inferior margin: 20 cm inferior from the ASIS to 15 r LAD complications
See Also (Topic, Algorithm, Media)
cm inferior from the pubic tubercle Wound infection r Groin/Inguinal Mass, Male and Female
r Modified LAD (after Catalona) r Lymphadenopathy, Inguinal
Skin necrosis
Excludes area lateral to the femoral artery and Wound dehiscence r Penis, Cancer, General Considerations
caudal to the fossa ovalis Thigh numbness r Penis, Cancer, lymphadenopathy Image
Preservation of the saphenous vein Lymphedema r Penis Cutaneous Lesion
No transposition of the sartorius muscles Lymphorrhea r Penis, Squamous Cell Carcinoma
Conversion to radical LAD if there are positive LNs Scrotal swelling
r Techniques to minimize complications of LAD r Reference Tables: TNM: Penis Cancer
Suprapubic swelling
Careful skin-flap management Pulmonary embolism
Meticulous LN dissection
Prophylactic antibiotics: Cephalosporins for 2 mo
FOLLOW-UP CODES
Vacuum drains Patient Monitoring
r Recurrences occur most often within 2 yr after
Elastic and/or pneumatic stocking ICD9
Early ambulation inguinal LAD r 187.4 Malignant neoplasm of penis, part unspecified
r Endoscopic LAD (4) r Nomograms available (5) r 196.5 Secondary and unspecified malignant
r Evaluation should include an exam and
Complete radical LAD can be completed through 3 neoplasm of lymph nodes of inguinal region and
endoscopic ports ultrasound-guided FNAC lower limb
Node yield is equivalent to open surgery r Maximum follow-up length of 5 yr r 785.6 Enlargement of lymph nodes
Decreased complications r Surveillance (patient did not have LAD)
r The viability of the skin flaps developed during an Every 3 mo for yr 1 and 2 ICD10
r C60.9 Malignant neoplasm of penis, unspecified
inguinal LN dissection are based on the anastomotic Every 6 mo for yr 3, 4, and 5
vessels within the superficial fatty layer of Campers r LAD and pN0 disease r C77.4 Sec and unsp malig neoplasm of inguinal and
fascia which course lateral to medial along the skin Every 6 mo for yr 1 and 2 lower limb nodes
lines. This is a key anatomic dissection plane as the Every 6 mo for yr 3, 4, and 5 r R59.0 Localized enlarged lymph nodes
lymphatic drainage of the penis lies beneath r LAD and pN+ disease
Campers fascia allowing this superficial fatty layer Every 3 mo for yr 1 and 2
to remain attached to the skin flaps during a groin Every 6 mo for yr 3, 4, and 5 CLINICAL/SURGICAL
dissection
Patient Resources
PEARLS
r When performing an inguinal LN dissection for a
National Cancer Institute: Penile Cancer Treatment r Penile cancer metastasizes to regional LNs before
clinically negative groin, a modified technique (http://www.cancer.gov/cancertopics/pdq/treatment/
should be used to decrease morbidity. The key disseminating systemically.
penile/HealthProfessional) r Treatment is dependent on the clinical presence of
components of this technique include the following:
Shorter incision (10 cm), preservation of the LNs, tumor stage, and tumor grade.
saphenous vein, minimizing dissection lateral to the REFERENCES r Inguinal LAD is potentially curative and can improve
femoral artery, and avoiding transposition of the long-term outcomes in penile cancer with nodal
Sartorious muscle 1. Pizzocaro G, Algaba F, Horenblas S, et al. EAU metastases.
penile cancer guidelines 2009. Eur Urol. 2010;57: r Careful tissue management, antibiotics, vacuum
ADDITIONAL TREATMENT 10021012. drains, and compression stockings can minimize the
Radiation Therapy 2. Horenblas S. Lymphadenectomy for squamous cell morbidities associated with LAD.
r Adjuvant radiotherapy may improve locoregional
carcinoma of the penis. Part 2: The role and
control in patients with extensive metastases and/or technique of lymph node dissection. BJU Int.
extranodal disease 2001;88:473483.
Side effects include edema and pain
r Radiotherapy in clinical N0 patients is not
recommended P
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Imaging
BASICS DIAGNOSIS r If locally advanced lesion or internal
lesions/tumors/malignancies suspected
DESCRIPTION HISTORY r Workup for associated abnormalities
r 3 categories: Benign, premalignant, malignant r Age
r May be male genitalia-specific (primary) or r Symptoms: Pain, pruritus, burning, discharge Diagnostic Procedures/Surgery
r Location: Scrotum, glans, shaft, preputial skin, r Cytologic smears: Potassium hydroxide or periodic
associated with other cutaneous lesions or systemic
urethral/bladder lining, other sites acid-Schiff staining for fungal infections
disease (secondary) r Tzanck smear: Herpes, varicella, Molluscum
r May occur at any age r Duration
r Rate of onset: Acute or chronic contagiosum
EPIDEMIOLOGY r Microscopic exam: Scabies, pubic lice, pinworm, and
r Exposures: New bath/laundry soap, lotions, oils,
Incidence other infections
travel (exotic plants, animals, insects, people), r Gram stain, bacterial, and fungal cultures of lesion:
Varies widely by etiology
shared towels/clothes, new medications, industrial,
Prevalence chemical Infections
r Depends on etiology r Sexual history: Sexual partner with lesions r Excisional or incisional biopsy
Pearly penile papule found in 1448% of r Trauma Pathologic Findings
postpubertal males r History of systemic diseases or cancers r Depth of lesion
Genital warts found in 0.11% of men r Allergies r Exam of epidermis, dermis, and subcutaneous tissue
RISK FACTORS r Family history and any changes noted
Systemic disease, irritant or allergen, sexual contact, r Previous treatment Infiltration with other cells or infectious agents
trauma, uncircumcised penis, family history, DIFFERENTIAL DIAGNOSIS
inflammation, infections, medications, local skin PHYSICAL EXAM
r Examine and describe lesion(s): r Common benign lesions (1)[A]
hygiene, obesity, age, smoking
Elevated, nonelevated Acrochordon: Skin tag
Genetics Color of lesion Angiokeratoma of Fordyce: Red papules on penis,
r Reiter syndrome: Associated with HLA-B27
Morphology of lesion scrotum; ectasia of dermal blood vessels
haplotype Configuration of lesion (linear vs. serpiginous) Epidermoid cysts: Most common cysts of genital
r HaileyHailey disease: Autosomal dominant
Degree of margination area. Filled with keratin. Postsurgical after
r Penile cancer: Associated with altered expression of Degree of firmness circumcision or hypospadias repair
P53, P21, c-ras, myc, Ki-67 genes Examine genitalia: Circumcised, uncircumcised, Fordyce spots: Sebaceous glands on genitalia
PATHOPHYSIOLOGY proper placement of foreskin Pearly penile papule: Small, white/flesh colored,
r Idiopathic, allergic, infectious, autoimmune, Describe primary lesion(s) (1)[A]: multiple, on glans or corona
inflammatory, systemic, sexual transmission, genetic Papule: 0.5 cm, solid, elevated Seborrheic keratoses: Stuck on appearance
r Lesions appear similar; biopsy often needed for Plaque: >0.5 cm, solid, elevated Vitiligo: Patchy depigmentation of skin
Nodule: >0.5 cm, solid, dome-shaped Zoon balanitis: Nonelevated, erythematous,
diagnosis
Vesicle: 0.5 cm, fluid-filled, well- glistening plaques on glans in uncircumcised
ASSOCIATED CONDITIONS circumscribed men. Biopsy to distinguish from SCC in situ
r LeslerTrelat syndrome: Increase in size and number Sclerosing lymphangitis: Cordlike lesion of
Bulla: >0.5 cm, fluid-filled, well-circumscribed
of seborrheic keratosis lesions sometimes signaling Pustule: Vesicle with purulent fluid, coronal sulcus; after vigorous sexual activity
internal malignancy r Allergic dermatitis, eczematous lesion with
well-circumscribed
r StevensJohnson syndrome and toxic epidermal Wheal: Hive, edematous plaque erythema, discharge, excoriations (1)[A]
necrolysis: Prodromal upper respiratory illness Describe secondary lesion(s) (1)[A]: Atopic dermatitis: Also known as lichen simplex
followed by life-threatening desquamating lesions Scale: Flakes on lesion surface chronicus, pruritic, red/scaly lesion on posterior
due to medications, infections, or cancers Crust/scab: Collected cellular debris scrotum. Atopic triad of eczema, allergic
r Psoriasis: Lesions under preputial skin, glans, or Atrophy: Thinning of skin causing depression rhinitis, asthma
prepuce Scar: Connective tissue collection Contact dermatitis: Irritant or allergic. Scaly with
r Reiter syndrome (reactive arthritis): Urethritis, Cyst: Lesion with wall and lumen crust. Direct cytotoxic effect of irritant or local
arthritis, conjunctivitis. Circinate balanitis Erosion: Defect with red/moist base type IV hypersensitivity reaction
r Behcet disease: Painful ulcers found in 5793% of Fissure: Thin linear defect Erythema multiforme: Red papules and target
patients, mostly scrotum (90%), but glans and shaft Ulcer: Deep defect lesions, blisters. Minor or major. Major:
also affected r Full dermatologic exam: StevensJohnson syndrome, toxic epidermal
r Inflammatory bowel disease: Arterial thrombosis of necrolysis
Single or multiple lesions r Papulosquamous disorders, scaly lesion on
penis, penile swelling, and noncaseating Organ-specific or generalized
granulomas on biopsy; also pyoderma gangrenosum r Lymph node exam erythematous base (2)[C]
r HaileyHailey disease: Vesiculobullous rash Psoriasis: Thick plaque with silver scales. Corona
r Diabetes: Phimosis DIAGNOSTIC TESTS & INTERPRETATION and glans lesions in circumcised, underneath
r HIV: Kaposi sarcoma, seborrheic dermatitis Lab preputial skin in uncircumcised
r Urinalysis, Gram stain, culture Reiter syndrome: Circinate balanitis, urethritis,
GENERAL PREVENTION r Complete blood count arthritis, ocular, oral, and skin lesions. History of
r Circumcision helpful in some cases r Serum chemistry profile infection with Chlamydia, Gonococcus,
r Proper hygiene r STD screening if suspected Ureaplasma, or GI bacteria
r Safe sex practices Lichen planus: Idiopathic autoimmune reaction
r Avoid contact with allergens or irritants against basal keratinocytes; small, flat, shiny,
violaceous papules on glans
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Lichen sclerosis: Pruritic pearly white papules and Psoriasis: Topical corticosteroids, clobetasol, or FOLLOW-UP
plaques on glans and inner prepuce that scar. Late systemic treatment Patient Monitoring
stage called balanitis xerotica obliterans. Biopsy to Lichen sclerosis: Biopsy to exclude SCC. Follow patients to monitor response to intervention
exclude SCC Long-term follow-up required. Circumcision and any change in lesion
Fixed drug eruption: Hypersensitivity reaction to curative. Patient Resources
medication 12 wk after starting. Lesions occur in Pemphigus vulgaris: Oral corticosteroids, r http://www.cdc.gov/std/general/default.htm
same location after challenge immunosuppressive therapy r http://www.aad.org/skin-conditions/dermatology-a-
r Vesicobullous disorders, autoimmune blisters and Behcet disease: Oral corticosteroids,
to-z
erosions (1)[A] immunosuppressive therapy
Genital warts: Topical 0.5% podofilox, 5%
Pemphigus vulgaris: Extensive painful blisters and
erosions. Difficult to treat and may be fatal imiquimod, green tea polyphenol extract, 25% REFERENCES
Bullous pemphigoid: LgG mediated, typically in podophyllin or trichloroacetic acid, cryotherapy,
patients older than 70 yr electrosurgery, laser ablation, surgical excision 1. Choi JM. Common benign dermatologic genital
Balanoposthitis: Circumcision curative lesions: Diagnosis and treatment. AUA Update
Dermatitis herpetiformis: Associated with celiac
Hidradenitis suppurativa: Skin care, topical Series. 2011;30:367371.
disease; IgA mediated
HaileyHailey disease: Familial, seen in 2030s, clindamycin or oral clindamycin and rifampin. 2. Gogstetter D and Mercurio MG. Common penile
blisters in axilla, inguinal, perianal areas Surgical excision for recurrent lesions lesions: Tips to the differential. Med Aspects of
r Noninfectious ulcers, lesions extending to dermis Fourniers gangrene: Broad-spectrum Human Sexuality. 2001;1(2):4551.
intravenous antibiotic coverage and emergent 3. Teichman JM, Sea J, Thompson IM, et al.
(1)[A]
surgical debridement Noninfectious penile lesions. Am Fam Physician.
Behcet disease: Painful oral and genital ulcers, Infestation with scabies or pubic lice: 5%
uveitis and other systemic involvement 2010;81(2):167174.
permethrin cream overnight and repeated a
Pyoderma gangrenosum: Chronic painful ulcer
week later
associated with Crohns, ulcerative colitis,
collagen vascular disease MEDICATION ADDITIONAL READING
Traumatic ulcers: Direct impact, sexual activity, First Line r Buechner SA. Common skin disorders of the penis.
body piercings, cleansing techniques r Varies with etiology (see above) (3)[B]
r Infections and infestations (1)[A] BJU Int. 2002;90:498506.
Low-potency topical corticosteroids for symptoms, r Kohn FM, Pflieger-Bruss S, Schill WB. Penile skin
STDs: Herpes simplex, syphilis, chancroid, genital eg, Hydrocortisone 1%, 2.5% diseases. Andrologia. 1999;31(suppl 1):311.
warts, granuloma inguinale, lymphogranuloma Specific antibiotics aimed at pathogen
venereum, molluscum contagiosum, Chlamydia, See Also (Topic, Algorithm, Media)
gonorrhea, trichomoniasis Second Line r Balanitis and Balanoposthitis
Genital warts: HPV 6 and 11.4 variants: r Varies with etiology (3)[B] r Bowen Disease and Erythroplasia of Queyrat
Condylomata acuminate, common warts, flat- High-potency or oral corticosteroids r Chancroid
topped papules/plaques, BuschkeLowenstein Immunosuppressive mediations r Condylomata Acuminata (Venereal Warts)
tumor (giant condyloma). Biopsy for flat-topped Intravenous antibiotics r Genital Ulcers
and giant condylomas to rule out SCC r Penis, Cancer, General Considerations
Balanoposthitis: Inflammation of glans and SURGERY/OTHER PROCEDURES
r Excision of lesion(s) and surrounding tissue r Penis, Cutaneous Lesion Image
foreskin in uncircumcised males. Can be due to r Laser ablation, electrocautery, cryotherapy r Penis, Squamous Cell Carcinoma
bacteria, yeast, irritants, trauma
r Circumcision r Phimosis and Paraphimosis
Cellulitis: Infection of deep dermis and
subcutaneous tissues due to Staphylococcus r More extensive surgeries if neoplastic lesion r Sexually Transmitted Infections (STIs) (Sexually
aureus and Streptococcus pyogenes ADDITIONAL TREATMENT Transmitted Diseases [STDs]), General
Folliculitis: Infection of hair-bearing follicles
Furunculosis: Boil Radiation Therapy
Limited role in nonneoplastic lesions
Hidradenitis suppurativa: Painful, firm, red CODES
nodules with draining sinuses; chronic Additional Therapies
inflammation of gland-bearing skin, N/A ICD9
superinfection possible Complementary & Alternative r 078.11 Condyloma acuminatum
Fourniers gangrene: Necrotizing fasciitis; Therapies r 608.89 Other specified disorders of male genital
progresses from cellulitis to blisters to N/A organs
foul-smelling necrotic lesions. Surgical emergency r 709.8 Other specified disorders of skin
Infestation: Pubic lice (Pediculosis pubis) or
scabies (Sarcoptes Scabiei mite); very pruritic ONGOING CARE ICD10
r Neoplastic lesions, see Section I Bowen disease and r A63.0 Anogenital (venereal) warts
PROGNOSIS r N48.89 Other specified disorders of penis
Erythroplasia of Queyrat, Penis, cancer, general r Most cutaneous lesions have a good prognosis but
considerations, Penis, squamous cell carcinoma r R23.8 Other skin changes
should be addressed promptly
r Widespread lesions difficult to control
TREATMENT COMPLICATIONS CLINICAL/SURGICAL
r See Differential Diagnosis section
GENERAL MEASURES r If left untreated, lesions may progress locally or
PEARLS
r Common benign lesions (1)[A]: r Do a complete skin exam when genital cutaneous
distally and cause symptoms
No treatment if asymptomatic lesion found.
If inflamed or infected, treat with antibiotics r Skin lesions appear similar and excisional or
Topical corticosteroids or emollient for incisional biopsies are often necessary for diagnosis
symptomatic relief and to rule out cancer.
Recurrent infections or cosmetic reasons: Excise,
remove with laser or cryotherapy
r StevensJohnson syndrome, toxic epidermal P
necrolysis, Fournier gangrene, pemphigus vulgaris
Zoon balanitis: Topical steroids for symptoms, can be life-threatening.
circumcision for cure
Contact dermatitis: Remove offending agent
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r Metastatic disease r Mohs FE, Snow SN, Larson PO. Mohs micrographic
TREATMENT TIP surgery for penile tumors. Urol Clin N Am. 1992;
Clinical trial 19:291304.
GENERAL MEASURES Supportive/palliative care r Ornellas AA, Seixas AL, Marota A, et al. Surgical
r Treatment typically based on grade and stage of treatment of invasive squamous cell. J Urol.
Complementary & Alternative
primary tumor (1) Therapies 1994;151(5):12441249.
r Palpable lymphadenopathy r Pagliaro LC, Williams DL, Daliani D, et al.
N/A
Fine-needle aspiration (FNA) Neoadjuvant paclitaxel, ifosfamide and ciplatin
6-wk course of oral antibiotics followed by repeat chemotherapy for metastatic penile cancer. A phase
physical exam ONGOING CARE II Study. J Clin Oncol. 2010;38513857.
r Pettaway CA, Pisters LL, Dinney CP, et al. Sentinel
MEDICATION PROGNOSIS
First Line r Depends on T-stage and nodal status lymph node dissection for penile carcinoma: The MD
r Tis/Ta lesions r Overall survival for men with node-negative disease Anderson Cancer Center experience. J Urol.
1995;154:19992003.
Topical: Imiquimod 5% cream applied for 5 d/wk is 8090%.
for 46 wk or 5-FU 5% cream every other day for r 2030% of men with inguinal lymph node See Also (Topic, Algorithm, Media)
46 wk metastasis will have pelvic lymph node metastasis r Balanitis Xerotica Obliterans (BXO)
Pelvic nodal metastasis have a 10% 5-yr survival r Bowen Disease and Erythroplasia of Queyrat
Second Line
N/A r When applicable, ILND associated with improved r Genital Ulcer Algorithm
disease-specific survival r Penis, Bowenoid Papulosis
SURGERY/OTHER PROCEDURES r Penis, Lesion, General
r Primary lesions COMPLICATIONS
r Infections r Penis, Leukoplakia
Tis/Ta lesions
Laser ablation: CO2 or neodymium r Erosion of lymphadenopathy into femoral vessels r Penis, Mass (Corporal Body Mass)
Circumcision (preputial lesions) r Partial penectomy and total penectomy r Penis, Squamous Cell Carcinoma Algorithm
Wide local excision, Mohs surgery, glansectomy, Urethral stenosis r Penis, Squamous Cell Carcinoma Images
glans resurfacing Loss of erective function r Reference Tables: TNM: Penis Cancer
T1 grade 12 r ILND
Mohs, wide local excision Infection (43%)
External beam radiation therapy Seroma (24%) CODES
Brachytherapy (with interstitial placement) Wound breakdown (16%)
Laser ablation Lymphedema ICD9
T1 grade 34 or T2 Vascular injury r 176.0 Kaposis sarcoma, skin
Partial penectomy (with intraoperative frozen r 187.4 Malignant neoplasm of penis, part unspecified
section) FOLLOW-UP
r 233.5 Carcinoma in situ of penis
Traditionally 2-cm margin is required Patient Monitoring
Total penectomy with perineal urethrostomy r Close inspection for local recurrence usually every
ICD10
r Regional nodes (2) 3 mo for 5 yr (frequency depends on grade and r C46.0 Kaposis sarcoma of skin
Sentinel node biopsy stage) r C60.9 Malignant neoplasm of penis, unspecified
High false-negative rate (25%) r Consider imaging for ambiguous findings on
r D07.4 Carcinoma in situ of penis
Nonpalpable nodes physical exam
High-risk T2 or G3 and intermediate-risk cancer Patient Resources
with lymphovascular invasioninguinal node National Cancer Institute. http://www.cancer.gov/ CLINICAL/SURGICAL
dissection (ILND)
Unilateral palpable nodes <4 cm
cancertopics/types/penile PEARLS
FNA or ILND if high risk r Grade and stage associated with prognosis FNA of
6 wk of oral antibiotics less recommended REFERENCES palpable nodes is preferred over 6-wk course of oral
Palpable nodes 4 cm antibiotics.
Standard or modified ILND 1. National Comprehensive Cancer Network. Penile r Modified ILND is associated with improved
Possible preoperative EBRTchemotherapy Cancer Version 1.2013. http://www.nccn.org/
professionals/physician gls/pdf/penile morbidity.
r Pelvic lymph nodes r Bulky inguinal lymph node metastases should be
Pelvic Lymph node dissection if >2 inguinal nodes 2. Johnson TV, Hsiao W, Delman KA, et al. Extensive
inguinal lymphadenectomy improves overall 5-year managed by multimodal therapy consisting of
positive on frozen section at the time of ILND neoadjuvant systemic chemotherapy followed by
survival in penile cancer patients: Results from the
ADDITIONAL TREATMENT surveillance, epidemiology, and end results surgical resection ( radiotherapy).
Radiation Therapy program. Cancer. 2010;29602966
r External radiation to primary lesion or inguinal
lymph nodes
r Typical doses are 5060 Gy over 46 wk ADDITIONAL READING
r Interstitial brachytherapy for clinically indicated
r Burgers JK, Badalament RA, Drago JR. Penile cancer:
lesions
Clinical presentation, diagnosis, and staging. Urol
Additional Therapies Clin N Am. 1992;19:247256.
r Neoadjuvant chemotherapy r McDougal WS, Kirchner FK Jr, Edwards RH, et al.
TIP: Ifosfamide, paclitaxel, cisplatin Treatment of carcinoma of the penis: The case for
r Adjuvant for high-risk disease primary lymphadenectomy. J Urol. 1986;136:3841.
Bilateral inguinal nodal disease
Pelvic lymph node involvement
Extranodal extension P
>4 cm nodes
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PENIS, TRAUMA
Hunter Wessells, MD, FACS
Brad Figler, MD
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PENIS, TRAUMA
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PEYRONIE DISEASE
Irvin H. Hirsch, MD
ASSOCIATED CONDITIONS
BASICS r ED: Occurs in 20% men with PD (1)[C]
TREATMENT
Comorbidities: Diabetes, hypertension,
DESCRIPTION dyslipidemia, smoking, coronary disease GENERAL MEASURES
r An idiopathic, localized connective tissue disorder r PD is found in 10% men with ED r A small percent of men will undergo spontaneous
with increased collagen deposition in the tunica Urethral stricture may coexist remission.
albuginea, resulting in a fibrous plaque that leads to r Surgery is not a common 1st-line option but
pain and penile angulation (1)[C] GENERAL PREVENTION
ultimately offers definitive resolution of curvature
r Plaque (2)[C]: Avoidance of penile trauma during intercourse
and deformity.
Most commonly dorsal plaque on side of penis to r The lack of randomized, placebo-controlled trials
which curvature directed DIAGNOSIS makes evaluation of efficacy and comparison
r Penile angulation may cause dyspareunia and even between any medical therapies for PD difficult.
preclude sexual intercourse HISTORY r Patients most likely to respond to medical therapy:
r First described by French surgeon Francois Gigot de r Distress and depression resulting from Peyronie
Young patients in acute phase. (1,2)[C]
Peyronie disease r All medical therapies provide varying decrease in
r Synonym(s): Acquired penile curvature, chronic r Duration and onset of symptoms, history of erectile
pain, curvature, or plaque size; complete resolution
inflammation of the tunica albuginea (CITA), penile trauma. Severe pain or snap or popping during of curvature is uncommon.
induration, Induratio penis plastica intercourse
r Pain: With or without erection; during intercourse MEDICATION
EPIDEMIOLOGY r Penis: Induration; degree and direction of penile First Line
Incidence r Oral therapy (2):
r Affects males 4070 yr old, with 0.43.2% angulation; hourglass deformity; lateral indentation;
shortening; sensory loss, partners perception No therapy has proven more or less effective than
incidence (1)[C] r Erections: Quantify rigidity; sufficient for intercourse another
r 37% men 4070 yr old have PD (3)[C] Vitamin E (tocopherol):
r History of Dupuytren contractures or hand surgery
r Mean age: 53 8001,000 U/d PO in divided doses
for deformity Antioxidant effects; may cause bleeding
Prevalence
PHYSICAL EXAM Potassium aminobenzoate (Potaba):
Estimated 388 in 100,000 men (1,2)[C] r Penile exam noting plaque size, tenderness and 3 g PO q6h
RISK FACTORS location May increase monoamine oxidase, decrease
r Inherent tendency to produce abnormal fibrous r Autophotography may be helpful in assessing serotonin, or increase utilization of oxygen by
tissue degree of angulation tissues; Expensive, GI side effects
r Erectile trauma or injury to the tunica albuginea of Examine the palmar fascia for associated Colchicine:
the penis may incite fibrotic reaction from repetitive Dupuytren contracture 0.6 mg PO q8h
microvascular injury and healing May decrease collagen synthesis and increase
r Intracorporal injection therapy and oral DIAGNOSTIC TESTS & INTERPRETATION collagenase activity
pharmacotherapy for ED not implicated as risk (2)[C] Lab Pentoxifylline:
N/A Growth factor blocker and anti-inflammatory
Genetics 400 mg PO BID
Association with Dupuytren contracture (in 939%) Imaging
No imaging necessary for diagnosis/medical therapy Other reported oral therapy: Tamoxifen,
and HLA-B7 antigens (1)[C] acetyl-L-carnitine,
Diagnostic Procedures/Surgery r Intralesional therapy (2):
PATHOPHYSIOLOGY r Preoperative assessment of stretched penile length
r Idiopathic (1,2)[C] Collagenase clostridium histolyticum (CCH (4)[C]:
r Origin of initial inflammatory process that leads to and sensory threshold (Biothesiometry) Breaks down collagen, promotes remodeling
r Preoperative Doppler US with intracavernous
fibrosis, calcification, elastic fiber alterations, and FDA approved for curvature deformity of the
vasoactive challenge: Assess plaque size, penis due to the presence of a plaque in PD.
plaque formation in tunica albuginea unknown, but
calcification, vascular hemodynamics of penis and Restricted distribution through Risk Evaluation
likely predisposing genetic alteration with inciting
erectile curvature and Mitigation Strategy (REMS) due to the risks
trauma r Preoperative intracavernous injection of vasoactive
r Acute phase: of serious adverse reactions, including penile
agent and genital sexual stimulation with fracture and other serious penile injury
Occurs in 1st 618 mo
measurement of erectile curvature A cycle consists of 2 CCH injection procedures
Proliferation of fibroblasts, myofibroblasts, and r Photographic confirmation by the patient of degree
collagen deposition and a penile modeling procedure
Pain with erections, slight penile curvature, and of curvature is often helpful Induce a penile erection (eg, intracavernosal
nodule formation Pathologic Findings injection of 1020 mcg of alprostadil)
Medical therapy most effective in acute phase Excess collagen deposition and inflammatory infiltrate With the erection, identify and mark the target
r Chronic phase: is found in the tunica albuginea area in the Peyronie plaque
The penis should be in a flaccid state before
Remodeling of connective tissue into a dense DIFFERENTIAL DIAGNOSIS
fibrotic plaque r Cancer: Primary or metastatic to corpora injecting CCH. Inject 0.58 mg CCH into the
Stable plaque size, penile curvature possibly target plaque of a flaccid penis once on each of
r Chordee: Usually associated with hypospadias
causing ED, erections less painful 2 days, 13 days apart
r Kelami syndrome: Fibrosis of the corpus spongiosum
r Natural history: Minority of patients (10%) will have
that limits expansion of the ventral corpora
spontaneous regression, yet most patients will not
cavernosa
develop disease significant enough to require r Penile fracture (hematoma)
surgery
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PEYRONIE DISEASE
Perform a manual penile modeling procedure r Inflatable penile prosthesis placement: 5. Levine LA and Constabile RA. Is intralesional
13 days after the 2nd injection of each Candidates: Significant erectile dysfunction, verapamil effective therapy for peyronies disease?
treatment cycle. For each plaque causing the severe curvature J Urol. 2012;188:704706.
curvature deformity, up to 4 treatment cycles Modeling: 6. Hellstrom WJ, Kendirci M, Matern R, et al.
may be administered. Each treatment cycle may When prosthesis placement alone fails to Single-blind, multicenter, placebo controlled,
be repeated at approximately 6-wk intervals. If straighten penis, manual modeling is parallel study to assess the safety and efficacy of
the curvature deformity is <15 degrees after the recommended, with good outcomes intralesional interferon -2b for minimally invasive
1st, 2nd, or 3rd treatment cycle, or if further Forcible manual manipulation of penis treatment for Peyronies disease. J Urol. 2006;
treatment is not indicated, then subsequent (modeling over inflated prosthesis) 176:394398.
treatment cycles should not be administered Complications: Infection (13%), erosion (<5%), 7. Nelson JN, Diblasio C, Kendirci M, et al. The
10,000 U in 0.25 cm3 per injection mechanical malfunction (510%), urethral injury chronology of depression in men with Peyronies
No other intralesional therapy has proven more or disease. J Sex Med. 2008;5:19851990.
ADDITIONAL TREATMENT
less effective than another
Verapamil (5)[C]: Radiation Therapy
12 injections (10 mg/10 mL) given once every Mixed results reported; not recommended
ADDITIONAL READING
24 wk Additional Therapies
Calcium blockage inhibits extracellular transport Extracorporeal shockwave therapy: No good Abern MR, Larsen S, Levine LA. Combination of penile
of collagen; increases collagenase activity in placebo-controlled studies to document efficacy; traction, intralesional verapamil, and oral therapies for
vitro; must commit to full course studies report decreased pain after ESWL therapy (2) Peyronies disease. J Sex Med. 2012;9:288295.
Applicable for young patients in acute phase
Complementary & Alternative See Also (Topic, Algorithm, Media)
Interferon 2a or 2b (6)[C]: r Chordee
5 106 U biweekly for 36 mo Therapies
r Penile traction therapy may have utility when r Erectile Dysfunction/Impotence (ED)
Inhibits fibroblast proliferation, diminishes
combined with other therapies r Penis, Curvature and/or Pain
collagen production, increases collagenase r Carnitine supplementation: Mixed results
Applicable for young patients in acute phase r Penis and Corporal Body Mass
Flu-like side effects r Peyronie Disease Image
Intralesional corticosteroids no longer ONGOING CARE
recommended due to local side effects
Second Line PROGNOSIS CODES
N/A See PathophysiologyNatural History
COMPLICATIONS ICD9
SURGERY/OTHER PROCEDURES r PD can impact quality of life and cause relationship r 607.84 Impotence of organic origin
r Indications: Curvature or erectile dysfunction that
r 607.85 Peyronies disease
precludes intercourse (2)[C] difficulties
r Depression can be associated r 608.89 Other specified disorders of male genital
r Patient must be in chronic phase with stable
organs
painless plaques FOLLOW-UP
r Preoperative US with intracavernous vasoactive ICD10
Patient Monitoring
challenge is useful to evaluate vasculature and r Patients should be re-examined frequently to assess r N48.6 Induration penis plastica
anatomy of penis, as described above disease status and response to therapy. r N52.9 Male erectile dysfunction, unspecified
r Plication procedures: r Recent studies suggest up to 48% of men with PD r N53.12 Painful ejaculation
Candidates: Longer penis, mild, distal curvature, have clinically relevant signs of depression and
good erectile function should be considered for mental health screening.
Relative to corporal plaque, plication of opposite (7) CLINICAL/SURGICAL
aspect of corpora cavernosa with a 1224-point PEARLS
plication. A relaxing incision of plaque is rarely Patient Resources
r AUA Urology Care Foundation. http://www. r Diagnosis of Peyronie disease is exclusively based on
required
Complications/side effects: Hematoma, stitch urologyhealth.org/urology/index.cfm?article=115 history and physical exam.
r The Peyronie Disease Society. r Patients with mild curvature and no evidence of
erosion/granuloma, penile shortening
r Plaque excision with grafting: www.peyroniessociety.org erectile dysfunction should be observed.
Candidates: Shorter penis, proximal plaque, severe r New data suggests that CCH can significantly
curvature, hourglass deformity, lateral indentation REFERENCES reduce the symptoms of Peyronie disease.
and good erectile function r The ideal candidate for CCH is having Peyronie
Plaque incised/excised and corporotomy defects 1. Hellstrom WJ, Bivalacqua TJ. Peyronies disease: disease for at least 12 mo, has stable disease, and a
grafted with small intestine submucosal graft Etiology, medical, and surgical therapy. J Androl. curvature of 30 degrees or greater.
(Surgisis, Cook Biotech) 2000;21(3):347354.
Complications: Loss of sensitivity, infection, 2. Wespes E, Eardley I, Giuliano F, et al. Guidelines on
hematoma, shortening, de novo venoocclusive penile curvature. Eur Urol. 2012;62:543552.
erectile dysfunction 3. Sommer F, Schwarzer U, Wassmer G, et al.
Epidemiology of Peyronies disease. Int J Impot Res.
2002;14:379383.
4. Gelbard M, Goldstein I, Hellstrom WJ, et al. Clinical
efficacy, safety and tolerability of collagenase
clostridium histolyticum for the treatment of
Peyronie disease in 2 large double-blind,
randomized, placebo controlled phase 3 studies.
J Urol. 2013;190(1):199207.
P
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PHEOCHROMOCYTOMA
Shaun G.S. Grewal, MD
Gerald L. Andriole, MD, FACS
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REFERENCES
ONGOING CARE CODES
1. Jaret TW, Vaughan ED Jr. Accuracy of computerized
PROGNOSIS tomography in the diagnosis of colovesical fistula ICD9
r Gas-producing UTI (2)[A] secondary to diverticular disease. J Urol. 1995; r 596.1 Intestinovesical fistula
Parenteral antibiotic therapy is successful in the 153:4446. r 599.0 Urinary tract infection, site not specified
majority of patients with gas limited to bladder 2. Huang JJ, Tseng CC. Emphysematous r 599.84 Other specified disorders of urethra
Patients with gas in the upper urinary tract are at pyelonephritis: Clinicoradiological classification,
increased risk of mortality and require management, prognosis and pathogenesis. Arch ICD10
percutaneous drainage with parenteral antibiotics Intern Med. 2000;160:797805. r N32.1 Vesicointestinal fistula
Gas in the perinephric space and pararenal 3. Bartus CM, Lipof T, Sarwar CM, et al. Colovesical r N39.0 Urinary tract infection, site not specified
tissues are at increased risk of mortality fistula: Not a contraindication to elective r R39.89 Other symptoms and signs involving the
r Diverticular abscess and enteric vesical fistula laparoscopic colectomy. Dis Colon Rectum. genitourinary system
Patients have an excellent prognosis after 2005;48:233236.
elective resection of the disease bowel segment
CLINICAL/SURGICAL
In many cases this can be performed ADDITIONAL READING
laparoscopically (3)[B] PEARLS
Golabek T, Szymanska A, Szopinski T, et al. r Pneumaturia is the distinct sensation by the patient
COMPLICATIONS
r Patients with inflammatory disorders such as Crohn Enterovesical fistulae: Aetiology, imaging, and of passage of air from the urinary tract.
may have complex and recurrent fistulae management. Gastroenterol Res Pract. 2013; r Pneumaturia should be considered secondary to an
r Patients with fistulae following radiation therapy 2013:617967. enteric vesical fistula until proven otherwise.
may have impaired healing and experience See Also (Topic, Algorithm, Media) r The CT scan finding of air in the bladder is abnormal
recurrence r Cystitis, Emphysematous and of high diagnostic value in the evaluation of the
r Fistula, Enterovesical patient with suspected pneumaturia and is likely to
FOLLOW-UP r Inflammatory Bowel Disease (Ulcerative Colitis and reveal associated pathology.
Patient Monitoring r The most common cause of pneumaturia is
r Management of associated illness Crohn disease), Urologic Considerations
r Prompt treatment of disease flare r Pneumaturia (Gas in Urine) Image diverticular disease.
r Urinary Tract Infection (UTI), Adult Female
Patient Resources r Urinary Tract Infection (UTI), Adult Male
NA
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r Older children: Palpable flank mass, abdominal 4. Levine E, Hartman DS, Mellstrup JW, et al. Current
TREATMENT mass, Potter phenotype, GI bleed, hematuria, pyuria, concepts and controversies in imaging of renal
polydipsia, polyuria, HTN, nonspecific GI complaints, cystic disease. Urol Clin North Am. 1997;24:
GENERAL MEASURES edema, growth retardation, fatigue, infection. Will 523543. (Level III evidence)
r No specific therapy for ARPKD. Treatments are eventually develop renal failure and HTN 5. Roy S, lon MJ, Trompeter RS, et al. Autosomal
supportive r All patients with ARPKD have liver involvement. recessive polycystic kidney disease: Long-term
r Pulmonary issues 1st priority initially; survival better Those with severe ARPKD have mild congenital outcome of neonatal survivors. Pediatr Nephrol.
with advances in perinatology hepatic fibrosis and those with severe congenital 1997;11:302306. (Level II-3 evidence)
r Goals: Delay progression to renal failure, liver hepatic fibrosis have milder ARPKD
failure, and portal HTN COMPLICATIONS
r Avoid nephrotoxic mediations r Renal: Renal failure (concentrating defect with ADDITIONAL READING
r Social support and respite care
polydipsia and polyuria), HTN, anemia, occasional Brinkert F, Lehnhardt A, Montoya C, et al. Combined
MEDICATION metabolic acidosis, hyponatremia, osteodystrophy, liver-kidney transplantation for children with
growth failure, UTI autosomal recessive polycystic kidney disease
First Line r Hepatobiliary: Hepatosplenomegaly, bleeding
r Thiazides to help urine-concentrating defect (ARPKD): Indication and outcome. Transpl Int.
r Treatment of renal osteodystrophy with vitamin D esophageal varices, portal thrombosis, 2013;26(6):640650.
hypersplenism, choledochal cysts, bacterial
and phosphate binders See Also (Topic, Algorithm, Media)
r Recombinant human erythropoietin cholangitis r Acquired Renal Cystic Disease
r Pulmonary: Respiratory failure, pulmonary
r Growth hormone treatment r MeckelGruber Syndrome (Meckel Syndrome)
hypoplasia, pneumothorax, atelectasis, poor
r Multicystic Dysplastic Kidney
Second Line diaphragmatic excursion
r GI: Feeding intolerance, failure to thrive r Nephronophthisis (Juvenile, Infantile, and
N/A
Adolescent)
SURGERY/OTHER PROCEDURES FOLLOW-UP r Polycystic Kidney Disease, Autosomal Dominant
r Preemptive bilateral nephrectomy and peritoneal
Patient Monitoring r Polycystic Kidney Disease, Autosomal Recessive
dialysis catheter (significant pulmonary distress) r Progressive renal failure in most patients requiring
r Unilateral nephrectomy (improve feedings, help with Image
ongoing renal assessments r Renal Cysts (Intrarenal, Peripelvic, and Parapelvic)
breathing) r Blood pressure r Renal Dysplasia, Hypodysplasia, and Hypoplasia
r Gastrostomy tube placement (improve feedings) r Liver functions and ultrasound at least annually
r Splenorenal shunt or portocaval shunt procedures r Renal Mass
r Overall assessment of growth and nutritional status
(portal HTN) r Parental counselling is critical as there is a 1 in 4
r Renal transplantation (ESRD)
r Liver transplantation (hepatic failure)
chance of another child having the disease CODES
r Progressive liver fibrosis with portal hypertension Patient Resources
PKD Foundation http://www.pkdcure.org/learn/arpkd ICD9
may require combined liver and kidney r 751.69 Other anomalies of gallbladder, bile ducts,
transplantation and liver
ADDITIONAL TREATMENT
REFERENCES r 753.14 Polycystic kidney, autosomal recessive
Radiation Therapy r 777.8 Other specified perinatal disorders of
1. Ward CJ, Hogan MC, Rossetti S, et al. The gene
N/A mutated in autosomal recessive polycystic kidney digestive system
Additional Therapies disease encodes a large, receptor-like protein. Nat
r Adequate hydration Genet. 2002;30:259269. ICD10
r P78.89 Other specified perinatal digestive system
r Correct acidbase and electrolyte abnormalities 2. Adeva M, El-Youssef M, Rossetti S, et al. Clinical
disorders
r Aggressive HTN control and molecular characterization defines a r Q44.5 Other congenital malformations of bile ducts
r Peritoneal dialysis broadened spectrum of autosomal recessive r Q61.19 Other polycystic kidney, infantile type
r Enteral feedings polycystic kidney disease (ARPKD). Medicine
r Advanced pulmonary support as required (Baltimore). 2006:85:121. (Level II evidence)
3. Bergmann C, Senderek J, Kupper F, et al. PKHD1 CLINICAL/SURGICAL
Complementary & Alternative mutations in autosomal recessive polycystic kidney
Therapies disease (ARPKD). Hum Mutat. 2004:5:453463. PEARLS
N/A r Renal and liver involvement is typical.
r Often fatal if present at birth.
ONGOING CARE r Treatments are supportive; no specific therapy.
PROGNOSIS
r Prenatal: Abnormal prenatal US (oligohydramnios,
enlarged reniform kidneys, absent urine in bladder,
seen after 30-wk gestation) (5)
r Neonates: If present at birth, the usual clinical
course is death. Patients have feeding intolerance,
respiratory distress
r Infants: Palpable flank masses, abdominal mass,
respiratory distress, HTN, polydipsia, polyuria,
edema, feeding intolerance, Potter phenotype,
nonspecific GI complaints, failure to thrive, growth
retardation, infection
P
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POLYHYDRAMNIOS/OLIGOHYDRAMNIOS
Bruce J. Schlomer, MD
Laurence S. Baskin, MD, FACS, FAAP
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POLYHYDRAMNIOS/OLIGOHYDRAMNIOS
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PATHOPHYSIOLOGY
BASICS r Route of transmission is unknown but seems to DIAGNOSIS
occur early in life most likely oral/respiratory
DESCRIPTION exposure (1,2) HISTORY
r JC and BK viruses are 2 of 10 different human r Hypothesized that subclinical infection leads to r Hemorrhagic cystitis
polyoma viruses viremia that seeds the kidneys From pink colored urine to clot retention
Small DNA viruses in the papovaridae family r Pathology is postulated to occur from reactivation of Pt can also have bladder pain
JC and BK viruses named after 1st patients the Pt may have LUTS
latent infection and not reinfection r BK virus nephropathy
viruses were isolated from in 1971 r Immuno-reconstitution inflammatory syndrome
These viruses typically manifest clinical sequelae Typically occurs 1013 mo after transplant
Dominant inflammatory response to abundant
only in immunocompromised hosts Often asymptomatic
polyoma virus antigen followed by brisk recovery
BK virus has a tropism for genitourinary May have hematuria
of the cellular immune response
epithelium Seen in BKV-associated hemorrhagic cystitis May have decreased urine output
Clinical manifestations: Hemorrhagic cystitis r Transplanted ureteral stenosis (3)
after allogeneic stem cell transplantation
(HC), ureteral stenosis, nephropathy, and rare r Cytopathic-inflammatory polyomavirus pathology Typically occurs 24 mo after transplant
GU-associated malignancies Often asymptomatic
High-level virus replication and a significant
May have decreased urine output
EPIDEMIOLOGY inflammatory response due to cytopathic lysis,
Incidence necrosis, with infiltration of granulocytes and PHYSICAL EXAM
lymphocytes. Dominant inflammatory response to r Hemorrhagic cystitis
N/A
abundant polyoma virus antigen followed by brisk May present with palpable bladder if in clot
Prevalence retention
r BK virus has an 8299% seroprevalence in adults of recovery of the cellular immune response
Seen in BKV-associated nephropathy in kidney r BK virus nephropathy
the United States, Italy, and Australia
allografts No significant findings on exam
50% @ 2 yr of age; 90% @ 10 yr of age r Oncogenic polyoma virus pathology r Ureteral stenosis of kidney transplant
r JC virus has a 3981% seroprevalence in same
Early viral gene expression activating host cells but May have no significant findings
regions
without sufficient late gene expression to cause Pelvic mass bulge from transplant hydronephrosis
Clinically manifest only in immunocompromised
rapid host cell lysis Due to denervation of transplanted kidney, patient
subjects Seen in rare BKV-associated urothelial and renal
r Ureteral stenosis due to BK virus infection among may not present with pain
tubular cancers
allograft recipients is approximately 3% There is conflicting evidence of BK virus DIAGNOSTIC TESTS & INTERPRETATION
r BK-induced nephropathy:110% of transplants Lab
involvement in these tumors
r Hemorrhagic cystitis r Hemorrhagic cystitis r Virus culture mostly used in research setting
Reported to cause hemorrhagic cystitis in Another theory suggests 3 phases Takes weeks to months to grow
5.77.7% of bone marrow transplant recipients Conditioning regimen for stem cell transplant r Urine cytology
damages the bladder mucosa providing Detects virus shedding
RISK FACTORS
r Immunocompromised host environment for virus replication Characteristic finding is an enlarged nucleus with
Viral replication unchecked in the absence of a single large basophilic intranuclear inclusion
Degree of immunosupression
functional immunity (decoy cells)
Transplant recipients Does not distinguish between various types of
Solid organ (especially kidney), stem cell Further damage to the bladder mucosa with
immune reconstitution and return of anti-BK polyoma virus
transplants r Urine quantitative PCR
HIV/AIDS immunity
Predilection toward hemorrhagic cystitis Correlates with BK virus associated nephropathy
ASSOCIATED CONDITIONS Can be positive in normal controls, elderly patients
Autoimmune disorders requiring r BK virus has a tropism for genitourinary epithelium
immunosuppression and HIV-infected patients without clinical
Kidney transplant recipients manifestations
Multiple sclerosis Tubulointerstitial nephritis Difficult to assess clinical significance
Systemic lupus erythematous Ureteral stenosis r Plasma quantitative PCR
Genetics Stem cell transplant recipients r Hemorrhagic cystitis
r Small nonenveloped icosahedral particles of Hemorrhagic cystitis
r JC virus has a tropism for neural tissue Urinalysis positive for blood/RBCs
4045-nm diameter, with a nonenveloped, circular r BK virus nephropathy
double-stranded DNA genome Causes progressive multifocal
r Polyoma viruses encode 6 proteins leukoencephalopathy Elevated creatinine
r 3 structural capsid proteins Not as common, but JC can also be related to Urinalysis
Pyuria, hematuria, and/or cellular casts of renal
r 3 noncapsid regulatory proteins genitourinary manifestations like BK virus and vice
versa tubular cells and inflammatory cells
Large and small T antigen (cell immortalization r Transplanted ureteral stenosis
and latency), and agnoprotein (assembly of viral GENERAL PREVENTION Can have elevated creatinine
particles) r Route of transmission is unknown so difficult to
Proteins interact with cellular target proteins prevent Imaging
r Hemorrhagic cystitis
and impair pathways involved with cell cycle r Competent immune system will prevent clinical
and DNA repair Ultrasound or CT can show bladder thickening
sequelae
and possibly clot if present
r Transplanted ureteral stenosis
Hydronephrosis seen on renal ultrasound, CT or
MRI
Obstruction seen on renogram
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ADDITIONAL READING
TREATMENT ONGOING CARE r Heikkila J, Holmberg C, Kyllonen L, et al. Long-term
GENERAL MEASURES PROGNOSIS risk of end stage renal disease in patients with
r Place urethral catheter immediately after birth to r Depends on the amount of congenital renal posterior urethral valves. J Urol. 2011;186(6):
drain the bladder (1) dysplasia, vesicoureteral reflux, bladder function 23922396.
r Measure daily weights, I/Os (fluid balance), routine r Incontinence and later ESRD correlated r Taskinen S, Heikkila J, Rintala R. Effects of posterior
vital signs r Cr >1 mg/dL at the end of the 1st yr of life urethral valves on long-term bladder and sexual
r Fluid and electrolytes as needed correlated with ESRD function. Nat Rev Urol. 2012;9(12):699706.
r Long-term bladder dysfunction may progress to
MEDICATION See Also (Topic, Algorithm, Media)
overactive/fibrotic bladder or possibly eventual r Anterior Urethral Valves
First Line myogenic failure r Bladder Outlet Obstruction
r Prophylactic antibiotics r Sexual function and fertility seems to be normal in
r Hydronephrosis/Hydroureteronephrosis, (Dilated
<2 mo age: Amoxicillin 20 mg/kg/d most patients
2 mo of age: Trimethoprim-sulfamethoxazole Ureter/Renal Pelvis), Pediatric Incontinence, Pediatric
2 mg/kg/d (concentrates in urine); nitrofurantoin is COMPLICATIONS r Hydronephrosis/Hydroureteronephrosis, (Dilated
an alternative r End-stage renal disease Ureter/Renal Pelvis), Prenatal
r Anticholinergics for bladder dysfunction r Voiding dysfunction r Posterior Urethral Valves Image
r Incontinence r Urethra, Obstruction
SURGERY/OTHER PROCEDURES r VURD Syndrome
r Transurethral ablation of urethral valves is possible FOLLOW-UP
in 80% of neonates Patient Monitoring
r Cutaneous vesicostomy in children too small for r Follow-up for observation of progress of renal
endoscopy (usually <2,000 g) function, as high risk of ESRD CODES
r Bilateral cutaneous pyelostomies of mostly historical Usual late or difficulty toilet training; treat voiding
significance, but may be used in extreme cases dysfunction, incontinence ICD9
Patients need serial electrolyte and Cr r 599.69 Urinary obstruction, not elsewhere classified
ADDITIONAL TREATMENT measurements, US evaluations, VCUG following r 753.8 Other specified anomalies of bladder and
Radiation Therapy ablation to monitor success of surgery, resolution urethra
N/A of reflux r 753.15 Renal dysplasia
Additional Therapies UDS for bladder function
r Prenatal surgical intervention remains Prophylactic antibiotics as needed ICD10
r N13.8 Other obstructive and reflux uropathy
investigational Patient Resources r Q61.4 Renal dysplasia
Associated with risk of fetal and maternal http://www.chop.edu/healthinfo/posterior-urethral-
morbidity; long-term renal benefit proven r Q64.79 Other congenital malformations of bladder
valves-puv.html
r In children with persistent worsening renal function and urethra
and hydroureteronephrosis following valve ablation
may require upper tract diversion if possible to REFERENCE
salvage renal function
CLINICAL/SURGICAL
r Persistent vesicoureteral reflux following valve 1. Hodges SJ, Patel B, McLorie G, et al. Posterior PEARLS
urethral valves. Scientific World Journal. 2009;
ablation may require vesicoureteral reflux 9:11191126. r No benefit to early delivery as children with
r Low compliance fibrotic bladder or myogenic failure
pulmonary hypoplasia also have severe renal
(valve bladder) may require enterocystoplasty and/or dysplasia.
clean intermittent catheterization (CIC) r Select centers offer prenatal interventions with
Complementary & Alternative dubious efficacy.
Therapies r Poor kidney function at presentation is associated
r Behavioral measures with worse renal prognosis.
Timed voiding, constipation therapy
r Biofeedback
Physical therapy to relax external sphincter
r Diet
Avoid bladder irritant, caffeine
r Perineal hygiene
Voiding positioning
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POSTOBSTRUCTIVE DIURESIS
John J. Pahira, MD
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POSTOBSTRUCTIVE DIURESIS
ADDITIONAL READING
TREATMENT ONGOING CARE
Nyman MA, Schwenk NM, Silverstein MD.
GENERAL MEASURES PROGNOSIS Management of urinary retention: Rapid versus
r After the obstruction is relieved, admit the patient to r The rate of recovery is largely determined by the gradual decompression and risk of complications.
the hospital to closely monitor hemodynamic status duration and severity of obstructive disease. Mayo Clin Proc. 1997;72(10):951956.
and electrolytes, I/Os and daily weights r Extent of recovery can be estimated by the
r Monitor urine output q2h and replace with oral See Also (Topic, Algorithm, Media)
improvement in renal function within 714 days r Hydronephrosis/Hydroureteronephrosis, (Dilated
fluids or if oral intake is not keeping up then with IV after the obstruction has been relieved:
Ureter/Renal Pelvis), Adult
fluids (0.51.0 mL of 1/2 NS/mL of urine output) in Some patients may require short-term treatment r Polyuria
addition to PO fluids with dialysis, until their renal function recovers. r Urinary Retention, General
If urine output decreases to <250 mL/hr replace COMPLICATIONS
fluids volume <50 mL of the urine output per r Uremic death
hour. Adjust accordingly as the diuresis resolves r Hypovolemic circulatory collapse
r If patient at risk of congestive heart failure or has r Bladder mucosal bleeding secondary to vein rupture
CODES
pulmonary edema, replace at a slower rate resulting from rapid bladder decompression
r Check serum sodium and potassium q612h and r Arrhythmia secondary to electrolyte abnormalities
ICD9
r 592.0 Calculus of kidney
replace as needed
r Follow BUN and creatinine values until normal: r 599.60 Urinary obstruction, unspecified
FOLLOW-UP
r Replace sodium, potassium, magnesium, and r 788.42 Polyuria
Patient Monitoring
bicarbonate as needed Serial (weekly to monthly) renal function testing
r Diuresis is usually self-limiting and typically lasts ICD10
(creatinine, BUN), renal US imaging if lab values do r N13.9 Obstructive and reflux uropathy, unspecified
<48 hr not return to normal range r N20.0 Calculus of kidney
r If diuresis lasts >48 hr, usually due to impaired Patient Resources r R35.8 Other polyuria
proximal tubular reabsorption of sodium causing a N/A
salt diuresis
If outputs remain elevated, obtain a follow-up
REFERENCES CLINICAL/SURGICAL
renal US to rule out hydronephrosis
PEARLS
1. Li C, Wang W, Kwon TH, et al. Downregulation of
ALERT r Maintain a high degree of suspicion for the potential
AQP1, -2, and -3 after ureteral obstruction is
If there is persistent hydronephrosis, consider associated with a long term urine concentrating for postobstructive diuresis when relieving chronic
persistent obstruction of ureter(s) above the level of defect. Am J Physiol Renal Physiol. 2001;281: obstruction of the urinary tract.
the bladder or a nonfunctioning stent/percutaneous F163F171. r Diuresis is usually self-limiting and typically lasts
tube (3)[A]. 2. Pais VM, Strandhoy JW, Assimos DG. <48 hr.
Pathophysiology of urinary tract obstruction. In:
MEDICATION Wein AJ, ed. Campbell-Walsh Urology. 9th ed.
First Line St. Louis, MO: WB Saunders; 2007.
None needed beyond replacement of fluid and 3. Gulmi FA, Felson D, Vaughan ED. Management of
electrolyte losses as noted above post-obstructive diuresis. AUA Update Series.
Second Line Lesson 23. 1998;27:177183.
N/A
SURGERY/OTHER PROCEDURES
N/A
ADDITIONAL TREATMENT
Radiation Therapy
N/A
Additional Therapies
Management of any renal insufficiency
Complementary & Alternative
Therapies
N/A
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PREGNANCY, UROLITHIASIS
Demetrius H. Bagley, MD, FACS
Kelly A. Healy, MD
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PREGNANCY, UROLITHIASIS
Pathologic Findings r Shock wave lithotripsy has generally not been 3. Bozkurt Y, Soylemez H, Atar M, et al. Effectiveness
N/A employed because of concerns of safety and the and safety of ureteroscopy in pregnant women: A
readily available alternatives comparative study. Urolithiasis. 2013;41:3742.
DIFFERENTIAL DIAGNOSIS
r Acute pyelonephritis ADDITIONAL TREATMENT 4. Deters LA, Belanger G, Shah O, et al. Ultrasound
r Appendicitis guided ureteroscopy in pregnancy. Clin Nephrol.
Radiation Therapy 2013;79(2):118123.
r Cholecystitis N/A
r Gastroenteritis 5. Laing KA, Lam TB, McClinton S, et al. Outcomes of
Additional Therapies ureteroscopy for stone disease in pregnancy:
r Hydronephrosis of pregnancy N/A Results from a symptomatic review of the literature.
r Neurologic/musculoskeletal pathology
Complementary & Alternative Urolo Int. 2012;89:380386.
r Obstetric etiology of pain
r Other intra-abdominal conditions
Therapies
r Dietary changes including:
r Renal vein thrombosis Limiting high oxalate foods and purines ADDITIONAL READING
Increase in fluid intake
Evan AP, Lingeman JE, Matlaga BR. Surgical
Limiting salt and sodium intake
TREATMENT May be best preserved until metabolic evaluation
management of upper urinary tract calculi. Urology.
2007;2:14561458.
GENERAL MEASURES postpartum
r Often misdiagnosed initially See Also (Topic, Algorithm, Media)
r Pregnancy, Bacteruria, Pyuria, and UTI
(appendicitis/diverticulitis/placental abruption)
r Conservative measures are taken initially to manage ONGOING CARE r Pregnancy, Hematuria
r Pregnancy, Radiologic Considerations
pain and infection so that the stone may pass (2) PROGNOSIS
r Hydration and analgesia, antiemetics and antibiotics r Pregnancy, Urinary Tract Obstruction
Pregnancy outcome is not appreciably worsened
are used because of symptomatic urolithiasis with appropriate r Pregnancy, Urologic Considerations
r Approximately 6080% of renal calculi pass management (5) r Pregnancy, Urologic Medications
spontaneously. Among pregnant patients with r Urolithiasis, Adult General
COMPLICATIONS
dilated ureter, the passage rate is not defined r Premature labor, fetal loss r Urolithiasis, Ureteral Calculi Algorithm
r Ureteral calculi associated with obstruction and r Urosepsis, renal insufficiency
upper tract infection demand immediate treatment
with drainage and antibiotics FOLLOW-UP CODES
Patient Monitoring
MEDICATION r During gestation:
First Line ICD9
r Narcotics including morphine, hydromorphone, Conservative management with hydration r 592.1 Calculus of ureter
Indications for intervention: r 592.9 Urinary calculus, unspecified
butorphanol, meperidine, and acetaminophen can Worsening renal function associated with
provide short-term pain relief without fetal harm r 646.80 Other specified complications of pregnancy,
persistent obstruction
r Avoid codeine during pregnancy because of its Intractable pain unspecified as to episode of care or not applicable
association with fetal defects Obstruction of a solitary kidney
r Nonsteroidal anti-inflammatory drugs are ICD10
Persistent infection associated with an r N20.1 Calculus of ureter
contraindicated because of the increased risk of obstruction r N20.9 Urinary calculus, unspecified
miscarriage in the 1st trimester and other risks Renal colic, precipitation premature labor that is r O99.89 Oth diseases and conditions compl
including fetal renal anomalies, fetal pulmonary refractory to treatment
hypertension and premature closure of the ductus preg/chldbrth
Preventive medications for stone disease have
arteriosus when used near term unacceptable side effects during pregnancy
r Medical management for the prevention of calcium Thiazides: Can cause fetal thrombocytopenia, CLINICAL/SURGICAL
stones should be delayed until after delivery hypoglycemia, and hyponatremia
Xanthanine oxidase inhibitors: No adverse PEARLS
Second Line
N/A effects on fetal animals but effects on human r Most urinary stones pass.
fetus known r Intractable pain or infection with obstruction may
SURGERY/OTHER PROCEDURES Penicillamine: Teratogenic in rats; fetal defects
r Intervention may be required in 2030% of cases (3) have been found in infants of mothers who took
necessitate drainage.
r Drainage may be necessary r Catheters, ureteral stent, or percutaneous
this during gestation
r Cystoscopy/stent placement can be done with or r Postpartum: nephrostomy (must be changed frequently at 4 to
6 wk because of the risks of encrustation).
without ultrasound guidance (4) Metabolic screening should be undertaken r Ureteroscopic treatment with endoscopic lithotripsy
Stents must be changed every 6 to 8 wk because postpartum and should be delayed until
of rapid encrustation in the pregnant womens completion of lactation period appears to be the most efficacious and possibly
urine safest treatment.
r Percutaneous nephrostomy placement can be done Patient Resources
N/A
under ultrasound:
To minimize radiation exposure
The stone or obstruction can be addressed REFERENCES
postpartum
The tube should be changed every 68 wk 1. Masselli G, Derme M, Laghi F, et al. Imaging of
Clearly tube drainage alone must consider the stone disease in pregnancy. Abdom Imaging.
duration of pregnancy 2013;38(6):14091414.
r Ureteroscopy with laser lithotripsy or impact 2. Hoscan MB, Ekinci M, Tunckran A, et al.
lithotripsy has been very successful in treating stones Management of symptomatic calculi complicating
in the upper urinary tract in the pregnant patient pregnancy. Urology. 2012;80:10111014. P
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PRIAPISM
Hunter Wessells, MD, FACS
Brad Figler, MD
316
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PRIAPISM
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r No data are available on the optimal PSA value at 5. Alfarone A, Panebianco V, Schillaci O, et al.
which to initiate treatment ONGOING CARE Comparative analysis of multiparametric magnetic
ADT is potentially detrimental to cognitive resonance and PET-CT in the management of local
function, quality of life, sexual health, PROGNOSIS recurrence after radical prostatectomy for prostate
r Prognosis is dictated by several variables including:
cardiovascular risk, and bone integrity cancer. Crit Rev Oncol Hematol. 2012;84(1):
Timing of initiation of ADT should be based on a Preoperative PSA 109121.
patients risk of metastatic progression and risk of Pathologic Gleason score 6. Crook JM, OCallaghan CJ, Duncan G, et al.
death from disease as opposed to absolute PSA Pathologic tumor stage Intermittent androgen suppression for rising PSA
levels Time to biochemical recurrence level after radiotherapy. N Engl J Med. 2012;
When ADT is initiated, in the absence of PSADT: <36 mo associated with the 367(10):895903.
metastases, intermittent ADT is preferred to development of metastatic disease 7. Stephenson AJ, Bolla M, Briganti A, et al.
continuous ADT given the side effects of this r For those patients that progress after biochemical
Postoperative radiation therapy for pathologically
treatment and the non-inferior overall survival of recurrence, in general, metastatic progression and advanced prostate cancer after radical
intermittent ADT (6) prostate cancer specific mortality occur at a median prostatectomy. Eur Urol. 2012;61(3):443451.
With the benefits that have been observed with of 8 and 13 yr after biochemical recurrence (4)
adjuvant/salvage radiation, the role of ADT as
COMPLICATIONS
monotherapy may be somewhat limited r Patient anxiety ADDITIONAL READING
Second Line r Complications are dictated by adjuvant and salvage r NCCN Practice Guidelines Version 1.2014
See Prostate Cancer, metastatic and Prostate therapy offered
Cancer, rising PSA following androgen ablation for http://www.nccn.org./ (Accessed January 6, 2014).
Either radiation or ADT r Thompson IM, Valicenti RK, Albertsen P, et al.
more information on additional drug classes
FOLLOW-UP Adjuvant and salvage radiotherapy after
SURGERY/OTHER PROCEDURES Patient Monitoring prostatectomy: AUA/ASTRO Guideline. J Urol.
r Scrotal orchiectomy r Monitoring of serum PSA is necessary to diagnose 2013;190(2):441449.
Can be used instead of ADT biochemical recurrence and the response to
Is more cost effective than ADT See Also (Topic, Algorithm, Media)
treatment when/if it is offered r Prostate Cancer, General
Does not allow for intermittent androgen r For patients on ADT, baseline and periodic r Prostate Cancer, Locally Advanced (Pathologic T3,
deprivation
assessment of bone density is recommended T4)
Quickly achieves castrate levels of testosterone
If PSA begins to rise on ADT, serum testosterone r Prostate Cancer, Metastatic (Clinical and Pathologic
(<50 ng/mL) within 24 hr
should be assessed to ensure a castrate level
Subcapsular scrotal orchiectomy can also be N+, M+)
offered in lieu of ADT Patient Resources r PSA elevation, General Considerations
May help avoid the psychological consequences r NCCN. http://www.nccn.org/patients/ r PSA, General Considerations
of an empty scrotum patient guidelines/prostate/index.html r Reference Tables: TNM: Prostate Cancer
r American Cancer Society. http://www.cancer.org/
ADDITIONAL TREATMENT cancer/prostatecancer/detailedguide/prostate-
Radiation Therapy
r Patients with demonstrable disease in the prostate cancer-treating-recurrence CODES
bed or those with suspected local recurrence may be
considered for adjuvant/salvage radiation REFERENCES ICD9
r 185 Malignant neoplasm of prostate
Favorable responses to adjuvant/salvage radiation
are associated with low PSA levels prior to 1. Roberts WB, Han M. Clinical significance and r 790.93 Elevated prostate specific antigen [PSA]
radiation(<1 ng/mL), long PSADTs, lower Gleason treatment of biochemical recurrence after definitive r V45.77 Acquired absence of organ, genital organs
scores, longer time to biochemical recurrence and therapy for localized prostate cancer. Surg Oncol.
positive surgical margins 2009;18(3):268274. ICD10
r C61 Malignant neoplasm of prostate
Adjuvant or early salvage radiation (based on a 2. Moul JW. Prostate specific antigen only progression
r R97.2 Elevated prostate specific antigen [PSA]
lower PSA trigger value) should be considered for of prostate cancer. J Urol. 2000;163(6):
patients with positive margins, extraprostatic 16321642. r Z90.79 Acquired absence of other genital organ(s)
extension, and seminal vesicle invasion (3) 3. Thompson IM, Valicenti RK, Albertsen P, et al.
Such therapy, compared to radical Adjuvant and salvage radiotherapy after
prostatectomy only, reduces the risk of prostatectomy: ASTRO/AUA guideline. J Urol. CLINICAL/SURGICAL
biochemical recurrence, local recurrence, and 2013;190(2):441449. PEARLS
clinical progression of cancer (3,7) 4. Pound CR, Partin AW, Eisenberger MA, et al. r Distinguishing local from systemic disease
The impact on subsequent metastases, Natural history of progression after PSA elevation
prostate-cancer specific mortality, and overall following radical prostatectomy. JAMA. 1999; recurrence dictates adjuvant/salvage therapy
survival is less clear (3,7). The survival benefit 281(17):15911597. options.
r Adjuvant/early salvage radiation is supported by
was established in one randomized trial but was
refuted in another randomized trial, which was randomized trials for patients with adverse
not powered to look at this outcome (3,7) pathology at the time of prostatectomy and is
Adjuvant/early salvage radiation is well endorsed by AUA/ASTRO Guidelines.
supported by randomized trials for patients with
adverse pathology at the time of prostatectomy
and is endorsed by AUA/ASTRO Guidelines
Additional Therapies
Clinical trials should be available and offered
Complementary & Alternative
Therapies
N/A P
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Diagnostic Procedures/Surgery
BASICS DIAGNOSIS r Prostate biopsy (3)[C]
Diagnosis is based on histologic exam
DESCRIPTION HISTORY Transrectal ultrasound guided (TRUS) transrectal
r Biopsy-proven adenocarcinoma of the prostate, r LCaP is rarely symptomatic
or transperineal needle biopsy
clinically confined to the prostate gland r Unintentional weight loss or new-onset skeletal pain Laterally directed, 18G, 1012 cores
r Clinical T1 or T2, N0, M0 suggests nonlocalized disease Increases detection rate
r LUTS Periprostatic local anesthetic injection
EPIDEMIOLOGY More commonly attributed to BPH Antibiotic prophylaxis is always recommended
Incidence Infection rate: 0.51% (see Prostate biopsy,
r Prostate cancer (CaP) (American Cancer Society PHYSICAL EXAM
r Digital rectal exam (DRE) Infections and Complications)
Data)
Estimated 233,000 new cases and 29,480 deaths No palpable nodule (cT1) Pathologic Findings
Nodule confined to prostate gland (cT2) r Gleason score (See Gleason Grading/Scoring
in 2014 in US
r Localized prostate cancer (LCaP) Ablation of lateral sulcus or palpable seminal System)
vesicles suggests more advanced disease than T2 r Proportion of biopsies positive for carcinoma
81% of newly diagnosed CaP
r Presence of extraprostatic extension
Prevalence DIAGNOSTIC TESTS & INTERPRETATION
r High-grade PIN and perineural invasion is usually
r Age dependent Lab
CaP cumulative prevalence in US men r Prostate-specific antigen (PSA) reported
Age 5060: 44%; Age 7080: 83% Produced by prostatic epithelium DIFFERENTIAL DIAGNOSIS
r 2035% worldwide Half-life of 23 days; Not specific to CaP r Abnormal DRE: Granulomatous prostatitis prostatic
A continuous parameter cyst, calcifications, cancer
RISK FACTORS The higher the value, the more likely the r Elevated PSA: UTI, BPH, acute or chronic prostatitis,
r Age
r Family history of CaP with highest risk in 1st-degree existence of CaP (1)[A] recent prostatic instrumentation
r Routine PSA screening is controversial
relative
2013 AUA Guidelines (2):
Suggestion that familial breast cancer increasies
Under age 40 yr: Do not screen [C] TREATMENT
prostate cancer risk
r African American race 4054 yr, average risk: Do not screen [C]
GENERAL MEASURES
<55 yr at higher risk: Individualized decision [C]
40% increased risk of disease r Assess life expectancy, overall health status, and
2.4 times risk of mortality 5569 yr: Shared decision-making if
tumor characteristics prior to treatment decisions
considering PSA screening [B] (4)[A]
Genetics
See Prostate cancer, general considerations Greatest benefit of screening in men ages r Review risk and benefits of all treatments and
5569 yr engage patient in informed decision making process.
PATHOPHYSIOLOGY Routine screening interval: 2 yr [C] r Treatment recommendations based on cancer
r Genetic predisposition
>70 yr and <1015-yr life expectancy: Do not
r Chronic inflammatory states biology, patient overall health, life expectancy, and
screen [C]
r Oxidative stress r Other PSA parameters preferences
r Gleason score and tumor stage are predictive of
ASSOCIATED CONDITIONS PSA velocity/doubling time
cancer outcomes
r Benign prostatic hypertrophy (BPH) Limited use in diagnosis (1)[A] r Risk strata are used to develop treatment
r Lower urinary tract symptoms (LUTS) (unrelated to Velocity >2 ng/mL: Increased risk for death recommendations (4)[A]
cancer) from CaP
r Obesity Low risk: PSA 10 and a Gleason score of 6 or less
% free PSA <10 and clinical stage T1c or T2a
GENERAL PREVENTION Increased (56%) risk of cancer (1)[A] Intermediate risk: PSA >1020 or a Gleason
r See also Prostate Cancer, prevention score of 7 or clinical stage T2b
PSA density
r There are unfortunately no approved agents for the 0.15 mg/mL/g suggests CaP High risk: PSA >20 or a Gleason score of 8 to 10
chemoprevention of CaP. Major clinical trials include: r CaP antigen 3 (PCA3) (see PCA3 Prostate Cancer or clinical stage T2c
r 5-reductase inhibitors
Gene 3 urine assay) MEDICATION
Finasteride (PCPT trial, 2003) Limited clinical use in diagnosis First Line
25% CaP risk reduction r Primary androgen deprivation therapy (ADT)
May help in decision to a repeat biopsy in men
Dutasteride (REDUCE trial, 2009) Rarely indicated for LCaP (4)[C]
27% CaP risk reduction with a negative 1st biopsy (1)[A]
Palliation of symptomatic patients
r Vitamin E and selenium (SELECT trial, 2011) Imaging Extensive or poorly differentiated tumors
r 2014 NCCN Guidelines for LCaP:
Increases the risk of CaP by 17% Short life expectancy
No imaging if low risk Not recommended by 2014 NCCN Guidelines
Bone scan: PSA >10 ng/mL or Gleason 8 r Neoadjuvant ADT for surgical treatment
Pelvic CT or MRI
Lymph node involvement risk >10% Not recommended (1)[A]
r ProstaScint imaging: Not indicated for LCaP r Neoadjuvant/concurrent androgen deprivation for
r No imaging modality can accurately estimate the 6 mo3 yr with XRT increases survival vs. XRT alone
extent of tumor and location within or surrounding or hormonal therapy alone (select intermediate and
the prostate all high-risk patients)
Second Line
N/A
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Pathologic Findings
BASICS DIAGNOSIS TRUS biopsy can confirm T3 disease with biopsy of the
capsule or SVs
DESCRIPTION HISTORY
r Clinical stage T3 prostate cancer (CaP) refers to r Family history of CaP DIFFERENTIAL DIAGNOSIS
r Voiding symptoms: Obstructive/irritative, hematuria r PSA can be elevated in the setting of prostatic
disease that is thought to extend outside the
prostate and may be palpable or seen on imaging and/or urethral instrumentation, infection, or benign
r Pathologic stage T3 CaP refers to extracapsular PHYSICAL EXAM prostatic hypertrophy (BPH)
Digital rectal exam (DRE): Note palpable abnormalities, r Lesions of the prostate seen on imaging can be
extension (T3a), or tumor invading seminal vesicles unilateral vs. bilateral, whether prostate is fixed, any
(SVs) (T3b) based on final surgical pathology areas of infarct, prostatitis, or tumor
r Clinical stage T4 CaP refers to palpable tumor that
extension of mass into adjacent structures r No single diagnostic modality can accurately predict
is fixed and/or invading adjacent structures DIAGNOSTIC TESTS & INTERPRETATION pathologic stage of CaP; incorporating multiple
r Pathologic stage T4 CaP refers to tumor that is Lab clinical parameters helps to best determine disease
r PSA extent
invading the bladder neck, external sphincter,
rectum, or levator muscle, and/or is fixed to the Positive predictive value (PPV): 1232% for PSA
pelvic sidewall based on final surgical pathology levels 410, 6080% for PSA levels >10, higher TREATMENT
r Transrectal ultrasound (TRUS) of the prostate, PSA increases risk for capsular penetration and SV
magnetic resonance imaging (MRI), and computed invasion GENERAL MEASURES
Value is decreased by 5-ARIs, elevated with r Currently no consensus regarding optimal
tomography (CT) can supplement physical exam
findings to assist with diagnosing clinical stage T3 instrumentation, infection, larger prostate volume management of locally advanced CaP
and T4 disease Lab variability: 2025% (7)[A] r Compared to clinically localized, low-grade CaP,
r PSA density: Serum PSA/prostate volume locally advanced CaP has a higher risk of recurrence
EPIDEMIOLOGY after any single treatment modality (up to 30%),
Incidence >0.15 is associated with CaP, >0.35 is associated
with 66% risk of extraprostatic extension and should be treated unless life expectancy is 5 yr
Declining incidence of locally advanced CaP: 1015% r PSA velocity
in 19891990, 15% in 20012003 (1,2) MEDICATION
Rate of rise of >0.75/yr is a specific marker for First Line
Prevalence CaP r Androgen deprivation therapy (ADT): Option for
N/A Should be calculated over 18 mo with 3 PSA patients unable or unwilling to undergo surgery or
RISK FACTORS measurements radiation therapy (RT)
r Higher Gleason score, elevated prostate-specific r Free PSA (fPSA) Orchiectomy, luteinizing hormone-releasing
antigen (PSA), and increased tumor volume on Lower fPSA is found in CaP; <10% free correlates hormone (LHRH) agonist or antagonist
biopsy predict increased likelihood of locally with 56% probability of CaP Can be administered continuously or
advanced disease PSA velocity, density, and fPSA do not correlate intermittently; optimal timing of administration
r Older males (75) are more likely to present with with Gleason score or CaP stage with respect to quality of life and survival benefit
high-risk prostate cancer (3) r PCA3 is debated
r African Americans and men with diabetes tend to RNA overexpressed in CaP, higher values indicate More effective when combined in neoadjuvant or
have more aggressive disease and higher stage at higher risk of CaP adjuvant fashion with RT
presentation (4) Collected via post-DRE urine sample Second Line
Sensitivity 48%, specificity 79%, not currently Antiandrogen monotherapy is less effective and not
Genetics used for routine screening
Research is ongoing to determine genetic markers that usually recommended; however, side effects are more
May correlate with Gleason score (8) tolerable overall
differentiate organ confined from locally advanced
CaP Imaging SURGERY/OTHER PROCEDURES
r TRUS r Radical prostatectomy (RP) with pelvic lymph node
PATHOPHYSIOLOGY Low sensitivity (2366%), specificity (4686%),
r Extension beyond prostatic capsule occurs when dissection (PLND)
and PPV (5062%) for predicting extracapsular Select patients can benefit from local control
tumor develops biologic ability to degrade physical
extension (9)[A] Complete excision/cure is possible
barriers to cancer cell movement, such as the
prostatic capsule and/or fascial investments of the Useful in guiding widespread sampling of prostate Clinical overstaging of T3 disease can occur in up
prostate and SVs tissue during biopsy to 727% of patients, thus exclusion from surgery
Intermediate-grade (Gleason 7) and high-grade r MRI on the basis of clinical staging may be
(Gleason 810) CaPs more commonly extend May delineate T2 from T3 disease inappropriate
outside the prostate than low-grade (Gleason 6) r Neoadjuvant ADT does not confer cancer specific or
Specificity for T3 disease is 95% when PSA >10,
CaP. abnormal DRE and >3 cores positive on TRUS overall survival (OS) benefit
biopsy (10)[A] r Adjuvant RT 5570 Gy improves local control and
ASSOCIATED CONDITIONS
Locally invasive prostate cancer can present with r CT reduces the risk of biochemical recurrence for T3
resulting symptoms such as hematuria, obstructive Consider for staging when PSA >20 or Gleason disease; it may also confer OS benefit (11)[A]
voiding symptoms and changes in bowel habits score 8 Indications include T3 disease, positive surgical
r Bone scan margins, Gleason score of 810
GENERAL PREVENTION Should be administered within 1 yr of surgery
r 5-Reductase inhibitors (5-ARIs): Finasteride and Indicated when PSA 20 to evaluate for skeletal
metastases and after operative side effects (eg, urinary
dutasteride continence) have improved/stabilized
r ProstaScint
Discuss with patient risks/benefits of 5-ARI use
May be useful in ruling out metastatic disease in
for CaP prevention (5)[A]
patients with locally advanced CaP
r Vitamin E, selenium, vitamin C:
Diagnostic Procedures/Surgery
No beneficial effect; should not be used r TRUS-guided prostate biopsy
r Statins, green tea, lycopene
Should be performed for abnormal DRE or PSA
Insufficient evidence to advocate these elevation
supplements for CaP prevention (6)
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ADDITIONAL TREATMENT Patient Resources 13. Chin JL, Ng CK, Touma NJ, et al. Randomized trial
r AUA Prostate Cancer Guide: http://www. comparing cryoablation and external beam
Radiation Therapy
r 7580 Gy to prostate, SVs, and pelvic lymph nodes, auanet.org/content/media/pc08.pdf radiotherapy for T2c-T3b prostate cancer. Prostate
or 4050 Gy and brachytherapy for cT3/T4 disease r NCCN Patient Guidelines for Prostate Cancer: Cancer Prostatic Dis. 2008;11:4045.
NCCN guidelines recommend 23 yr http://www.nccn.org/patients/patient guidelines/ 14. Stephenson AJ, Shariat SF, Zelefsky MJ, et al.
neoadjuvant/concomitant/adjuvant ADT for prostate/index.html Salvage radiotherapy for recurrent prostate cancer
higher disease free and OS in men with T3 and after radical prostatectomy. JAMA. 2004;291:
T4 disease (12)[A] 13251332.
r Advances in 3D conformal, intensity-modulated and
REFERENCES
image-guided RT are designed to deliver higher 1. Greene KL, Cowan JE, Cooperberg MR, et al.;
doses directly to prostate, avoiding adjacent Cancer of the Prostate Strategic Urologic Research
ADDITIONAL READING
structures and thus limiting side effects Endeavor (CaPSURE) Investigators. Who is the Thompson IM, Valicenti RK, Albertsen P, et al.
Additional Therapies average patient presenting with prostate cancer? Adjuvant and salvage radiotherapy after
r Cryoablation of the prostate Urology. 2005;66(5 suppl):7682. prostatectomy: AUA/ASTRO Guideline. J Urol.
2. Cooperberg MR, Lubeck DP, Mehta SS, et al. Time 2013;190(2):441449.
Higher rates of biochemical failure than RT (87% trends in clinical risk stratification for prostate
vs. 53%) for T2cT3 CaP (13)[A] cancer: Implications for outcomes (data from See Also (Topic, Algorithm, Media)
r High-intensity focused ultrasound (HIFU) r Prostate Cancer, General
CaPSURE). J Urol. 2003;170(6):2127. r Prostate Cancer, Locally Advanced (Clinical T3, T4)
Currently investigational, may prove to be an 3. Bechis SK, Carroll PR, Cooperberg MR. Impact of
option in combination with ADT for intermediate- age at diagnosis on prostate cancer treatment Images
r Prostate Cancer, Locally Advanced (Pathologic T3,
and high-risk CaP and survival. J Clin Oncol. 2011;29(2):235241.
4. Mitin T, Chen MH, Zhang Y, et al. Diabetes T4)
Complementary & Alternative r Prostate Cancer, Positive Margin Following Radical
Therapies mellitus, race, and odds of high grade prostate
cancer in men treated with radiation therapy. Prostatectomy
Clinical trials should be considered r PSA Elevation, General Considerations
J Urol. 2011;186(6):22332237.
r Reference Tables: TNM: Prostate Cancer
5. Kramer BS, Hagerty KL, Justman S, et al. Use of
ONGOING CARE 5--reductase inhibitors for prostate cancer
PROGNOSIS chemoprevention: American Society of Clinical
r Significant risk of progression and disease-specific Oncology/American Urological Association 2008 CODES
death if locally advanced prostate cancer is left Clinical Practice Guideline. J Clin Oncol.
untreated 2009;27(9):15021516. ICD9
r 185 Malignant neoplasm of prostate
OS without intervention ranges from 10 to 92% at 6. Stephenson AJ, Abouassaly R, Klein EA.
Chemoprevention of prostate cancer. Urol Clin r 198.1 Secondary malignant neoplasm of other
5 yr and 1478% at 10 yr for high-grade/stage
CaP North Am. 2010;37(1):1121. urinary organs
r RP for T3 CaP: OS 6496% at 5 yr, 1372% at 7. AUA PSA Best Practice Statement. Available at r 198.82 Secondary malignant neoplasm of genital
10 yr http://www.auanet.org/content/media/psa09.pdf. organs
Cancer-specific survival (CSS): 8592% at 5 yr, (Accessed May 21, 2014)
8. Artibani W. Landmarks in prostate cancer ICD10
7982% at 10 yr r C61 Malignant neoplasm of prostate
r RT monotherapy: OS 6070% at 5 yr and <50% at diagnosis: The biomarkers. BJU Int. 2012;110: r C79.11 Secondary malignant neoplasm of bladder
10 yr 813.
r C79.82 Secondary malignant neoplasm of genital
RT with ADT: OS 7287% at 5 yr 9. Hsu CY, Joniau S, Oyen R, et al. Detection of
clinical unilateral T3a prostate cancer-by digital organs
COMPLICATIONS rectal exam or transrectal ultrasonography. BJU
r Therapy complications are similar to those for
Int. 2006;98(5):982985.
localized CaP CLINICAL/SURGICAL
r Untreated T3T4 CaP may lead to hematuria, 10. Cornud F, Flam T, Chauveinc L, et al.
Extraprostatic spread of clinically localized PEARLS
obstruction at the level of the prostate requiring prostate cancer: Factors predictive of pT3 tumor r Risk assessment requires incorporating serum PSA,
indwelling catheter or transurethral resection of the and of positive endorectal MR imaging
prostate, or ureterovesical junction obstruction clinical stage, Gleason score, tumor volume, and
examination results. Radiology. 2002;224(1): patient age and comorbidities to best provide
requiring ureteral stents or percutaneous 203210.
nephrostomy counseling and treatment recommendations.
11. Thompson IM, Tangen CM, Paradelo J, et al. r RP should include PLND for locally advanced CaP,
FOLLOW-UP Adjuvant radiotherapy for pathologic T3N0M0 and adjuvant RT should be offered for T3T4
Patient Monitoring prostate cancer significantly reduces risk of disease, particularly if surgical pathology
r Periodic PSA measurements, initially at 3-mo metastases and improves survival: Long-term demonstrates positive margins.
intervals posttherapy and then gradually increasing followup of a randomized clinical trial. J Urol. r RT should be administered in conjunction with ADT
to annually 2009;181:956962.
for locally advanced CaP to maximize survival
r Failure of PSA to nadir or consecutive rises in PSA 12. NCCN Prostate Cancer Guidelines. Available at benefit with support from randomized clinical trials.
should prompt further evaluation with bone scan http://www.nccn.org/professionals/physician gls/
and/or CT pdf/prostate.pdf (Accessed May 21, 2014)
Risk of biochemical recurrence increased in:
Gleason score 810, PSA doubling time <10 mo
(14)
ProstaScint scan may be helpful in identifying local
recurrence if bone scan and CT scan are negative
Consider additional treatment options such as
ADT, adjuvant RT, and/or chemotherapy and
participation in clinical trials
P
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r A German trial of 395 patients demonstrated a 5. Wiegel T, Bottke D, Steiner U, et al. Phase III
TREATMENT biochemical progression-free survival benefit (72% postoperative adjuvant radiotherapy after radical
vs. 54%) to early radiation therapy in this pT3 prostatectomy compared with radical prostatectomy
GENERAL MEASURES group. The benefit of therapy was observed with or alone in pT3 prostate cancer with postoperative
r The American Society of Therapeutic Radiation without positive margins (5) undetectable prostate-specific antigen: ARO96-02/
Oncology (ASTRO) and the American Urologic r Genomic assays may allow more rationale decision AUO AP 09/95. J Clin Onc. 2009;27(28):
Association (AUA) have a joint guideline on the use making for post operative radiation following radical 29242930.
of adjuvant and salvage radiotherapy after prostatectomy (7) 6. Stephenson AJ, Shariat SF, Zelefsky MJ, et al.
prostatectomy Salvage radiotherapy for recurrent prostate cancer
Additional Therapies
They note clinical benefit of adjuvant radiotherapy after radical prostatectomy. JAMA. 2004;291:
Proton beam external radiotherapy is offered at
in reducing clinical progression in high-risk 13251332.
selected major US centers. However, there is no Level I
patients (seminal vesicle involvement, positive 7. Den RB, Feng FY, Showalter TN, et al. Genomic
evidence that it is superior to photon-based
surgical margins, extra prostatic extension) while prostate cancer classifier predicts biochemical
radiotherapy using an intensity modulated method.
the impact on future development of metastasis failure and metastases in patients after
and survival remains less clear Complementary & Alternative postoperative radiation therapy. Int J Radiat Oncol
It also emphasizes the value of early intervention Therapies Biol Phys. 2014;89(5):10381046.
with a threshold PSA 0.2 ng/mL with a 2nd No level I evidence of benefit
confirmatory after surgery to confirm relapse (2)
r Therapy is considered adjuvant if the postoperative
ONGOING CARE ADDITIONAL READING
PSA level is undetectable (<0.2 ng/mL)
r Therapy is considered salvage if there is a PROGNOSIS NCCN Prostate Cancer Guidelines: http://www.nccn.
biochemical/PSA recurrence r With salvage EBRT and persistent or increasing PSA org/professionals/physician gls/pdf/prostate.pdf
r Lymph node metastasis is an indication for ADT, levels after surgery 45% of patients were free of (Accessed May 21, 2014)
pelvic RT, or observation disease at 4 yr after salvage EBRT See Also (Topic, Algorithm, Media)
Patients with no adverse risk features achieved a r Prostate Cancer, General
MEDICATION 4-yr progression-free probability of 77%. A r Prostate Cancer, Locally Advanced (Clinical T3, T4)
First Line nomogram based on established risk factors to r Prostate Cancer, Locally Advanced (Pathlogic T3, T4)
r Androgen deprivation therapy (ADT) using an LHRH
more accurately identify patient-specific risks to
agonist or GNRH antagonist with or without an oral Images
assist in clinical decision-making is available (6) r PSA Elevation, General Considerations
antiandrogen is sometimes added to adjuvant or
salvage EBRT although there is not yet level I COMPLICATIONS PSA, General Considerations
r Morbidity from pelvic RT include radiation proctitis, r Reference Tables: TNM: Prostate Cancer
evidence from RCTs in the adjuvant/salvage setting
for pathologic T3/T4 disease cystitis, incontinence, ED, lymphedema, and stricture r Prostate Cancer, Positive Margin Following RP
r Potential benefits of ADT must be balanced against disease
r Morbidity from hormone therapy include hot flushes,
the side-effect profile
breast tenderness, osteoporosis, diabetes mellitus, CODES
Second Line depression, and cardiac disease
Newer generation oral hormonal therapies
(abiraterone acetate, enzalutamide) may have future FOLLOW-UP ICD9
role in adjuvant and salvage therapy Patient Monitoring r 185 Malignant neoplasm of prostate
For pT3/T4 after RP; patients are generally followed r 198.1 Secondary malignant neoplasm of other
SURGERY/OTHER PROCEDURES
r In the setting of clinical T3/T4 disease, extended every 3 mo for the 1st yr, every 6 mo for the next urinary organs
25 yr and yearly thereafter depending on risk. r 198.82 Secondary malignant neoplasm of genital
lymph node dissection is gaining support in these
patients (obturator, external iliac, internal iliac, and Patient Resources organs
presacral nodes) r AUA Prostate Cancer Guide: http://www.auanet.org/
r Neoadjuvant ADT with leuprolide and flutamide for
ICD10
content/media/pc08.pdf r C61 Malignant neoplasm of prostate
r NCCN Patient Guidelines for Prostate Cancer:
3 mo prior to RP has shown to reduce positive r C79.11 Secondary malignant neoplasm of bladder
margins, but has no impact on lymph node http://www.nccn.org/patients/patient guidelines/ r C79.82 Secondary malignant neoplasm of genital
metastasis or 5-yr biochemical recurrence rate. It prostate/index.html
organs
may be used in selected cases for technical
downsizing
r Chemo hormonal downstaging trials are ongoing REFERENCES CLINICAL/SURGICAL
before RP for clinical T3/T4 disease 1. Cooke EW, Shrieve DC, Tward JD, et al. Clinical PEARLS
ADDITIONAL TREATMENT versus pathologic staging for prostate
r Pathologic T3/T4 prostate cancer includes
Radiation Therapy adenocarcinoma: How do they correlate? Am J Clin
r 3 randomized trials have been completed demons- Oncol. 2012;35(4):364368. extracapsular cancer than extends beyond the
2. Thompson IM, Valicenti RK, Albertsen P, et al. gland, without distant metastasis.
trating a benefit to early adjuvant radiation therapy r High clinical stage, PSA levels, and Gleason score
r Treatment is optimally offered after continence is Adjuvant and salvage radiotherapy after
prostatectomy: AUA/ASTRO guideline. J Urol. (from biopsy cores) predict more advanced
restored, to allow healing to take place after surgery
r With a median follow-up of 12 yr, (Southwest 2013;190(2):144149. pathologic staging.
r Level I evidence from 3 RCTs support adjuvant EBRT
Oncology Group [SWOG] 8794), confirmed a 3. Thompson IM, Tangen CM, Paradelo J, et al.
Adjuvant radiotherapy for pathological T3N0M0 for high-risk pT3/T4 prostate cancer after RP.
significant improvement in the risk of metastasis r For patients who do not receive adjuvant EBRT, close
(43% vs. 54%) and overall survival (41% vs. 52%) prostate cancer signicantly reduces risk of
among patients randomized to adjuvant radiation (3) metastases and improves survival: Long-term follow-up with early salvage EBRT is very commonly
r The largest trial included 1,000 patients with T3 follow-up of a randomized clinical trial. J Urol. practiced.
2009;181:956962. r There is no level I evidence for adding ADT to EBRT
disease randomly assigned to 60 Gy of radiation vs.
4. Bolla M, Collette L, Blank L, et al. Long-term results as part of adjuvant or salvage EBRT, but selected
observation. At 5-yr, progression-free survival was
significantly improved (74% vs. 53%), with no with immediate androgen suppression and external high-risk patients may benefit. P
demonstration of an overall survival benefit (4) irradiation in patients with locally advanced
A subsequent update suggested that the benefit prostate cancer (an EORTC study): A phase III
might be limited to patients with positive surgical randomized trial. Lancet. 2002;360:103106.
margins
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SURGERY/OTHER PROCEDURES
r Resection of solitary metastases is not generally
COMPLICATIONS
r ADT: Hot flashes, loss of sexual function and libido,
ADDITIONAL READING
performed with curative intent. loss of muscle mass, decreased in bone mineral r Antonarakis ES, Eisenberger MA. Expanding
r Decompression of epidural metastatic CaP can result density, weight gain, diabetes, lipid profile changes, treatment options for metastatic prostate cancer.
in stabilization of the spinal cord and neurologic and neurocognitive dysfunction. N Engl J Med. 2011;364:20552058.
symptoms. Best results are obtained if the procedure r In a metaanalysis of 8 randomized controlled trials, r Hussain M, Tangen CM, Berry DL, et al. Intermittent
is performed within 24 hr of the onset of symptoms. long-term ADT was not associated with an increased versus continuous androgen deprivation in prostate
r Stabilization of weight-bearing bones (femur and risk of death from cardiovascular causes, however. cancer. N Engl J Med. 2012;368(14):13141325.
hip) by internal fixation or replacement of the joint r Antiandrogens: Increased liver function test. r Loblaw DA, Virgo KS, Nam R, et al. Initial hormonal
prophylactically may prevent fracture. r Skeletal-related events: Defined by pathologic management of androgen-sensitive metastatic,
r In one randomized trial, immediate ADT was fracture, spinal compression/vertebral body collapse, recurrent, or progressive prostate cancer: 2006
osteonecrosis of the jaw, radiation or surgery to update of an American Society of Clinical Oncology
associated with improved overall and
bone, or change in antineoplastic therapy. Androgen practice guideline. J Clin Oncol. 2007;25(12):
disease-specific survival among men who had
blockade can cause osteopenia/osteoporosis; 15961605.
pathologically positive lymph nodes after RP (2)[A]. r Nguyen PL, Je Y, Schutz FA, et al. Association of
However, this study was limited by sample size and bisphosphonate/RANK ligand therapy can limit
lack of central pathologic review. In a randomized reductions in bone mineral density. androgen deprivation therapy with cardiovascular
EORTC study with clinically node-positive disease, Zoledronic acid: Renal insufficiency, adjust based death in patients with prostate cancer: A
early ADT did not have any survival benefit. Because on creatinine. meta-analysis of randomized trials. JAMA.
of the gradual natural history of CaP (median Osteonecrosis of the jaw can result from 2011;306(21):23592366.
survival after development of metastasis after RP = bisphosphonates and RANK ligand inhibitors; See Also (Topic, Algorithm, Media)
5 yr (3)[C]) and because the oncologic benefit of avoid major dental work (extractions) on therapy; r Antiandrogen Withdrawal Syndrome (Flutamide
early ADT is uncertain, many consider it reasonable perform oral exam before starting. Withdrawal Syndrome)
to delay ADT until symptoms or measurable disease FOLLOW-UP r Prostate Cancer, General
on imaging are present, to minimize systemic Patient Monitoring r Prostate Cancer, Metastatic (Clinical and Pathologic
adverse effects of ADT. r Monitoring patients is controversial. With start of N+, M+) Images
androgen blockade: Check PSA every 3 mo. Confirm r Prostate Cancer, Rising PSA Following Androgen
ADDITIONAL TREATMENT
castrate testosterone (<50 ng/dL) periodically and Ablation (Castration-resistant Prostate Cancer,)
Radiation Therapy r PSA Elevation, General
r Radiation can be used to palliate solitary painful upon demonstration of rising PSA while on LHRH
therapy. r Reference Tables: TNM: Prostate Cancer
bony metastases. r At PSA progression, antiandrogen should be
r Strontium89 and samarium153 (-emitters) can
withdrawn, and if the patient continues to progress,
palliate bone pain and are most useful for diffuse
metastasis.
a CT scan of the abdomen and pelvis should be CODES
r Radium-223 (-emitter) can also be used in the obtained.
r Timing of PSA testing during chemotherapy is
setting of symptomatic bone metastasis ICD9
controversial; usually every 3 wk. r 185 Malignant neoplasm of prostate
Additional Therapies r Serum creatinine should be monitored for patients r 196.5 Secondary and unspecified malignant
r Bone health
on bisphosphonates. Zoledronic acid should not be neoplasm of lymph nodes of inguinal region and
Patients should receive calcium (1,200 mg/d) and given with a Cr >2 mg/dL. lower limb
vitamin D (8001,000 IU/d) supplements. r Monitoring for osteoporosis, obesity, insulin r 198.5 Secondary malignant neoplasm of bone and
With ADT, consider zoledronate 4 mg IV yearly, resistance, lipid alteration, and the concern of
alendronate 70 mg PO weekly, denosumab (RANK bone marrow
increased risk of diabetes and cardiovascular
ligand inhibitors Prolia 60 mg q6mo SQ for men on diseases in men on ADT should be considered. ICD10
ADT or Xgeva with bone mets 120 mg SQ q4wk). r C61 Malignant neoplasm of prostate
r Metastatic CRPC: Zoledronate 4 mg adjusted to Patient Resources r C77.4 Sec and unsp malig neoplasm of inguinal and
renal function every 34 wk IV or denosumab American Cancer Society. http://www.cancer.org/
cancer/prostatecancer/index?sitearea=%26dt=10 lower limb nodes
(Xgeva) 120 mg SQ q4wk to prevent skeletal-related r C79.51 Secondary malignant neoplasm of bone
events.
Complementary & Alternative REFERENCES
Therapies CLINICAL/SURGICAL
1. NCCN Practice Guidelines Version 1.2014.
Weight-bearing exercise and stopping smoking
http://www.nccn.org./ Accessed January 6, 2014.
PEARLS
benefits osteoporosis.
2. Messing, EM, Manola J, Sarosdy M, et al. r ADT is the 1st-line treatment for metastatic CaP.
Immediate hormonal therapy compared with r There are several antiandrogen and
ONGOING CARE observation after radical prostatectomy and pelvic chemo-immunotherapeutic options for men with
lymphadenectomy in men with node-positive ADT-refractory metastatic CaP.
PROGNOSIS prostate cancer. N Engl J Med. 1999;341: r Bone-related issues should be considered in this
r The estimated 5-yr survival of metastatic CaP is
17811788. population.
28% (3)[C]. 3. Pound CR, Partin AW, Eisenberger MA, et al.
r For patients with lymph nodepositive disease
Natural history of progression after PSA elevation
post-RP, adjuvant hormone ablation following RP following radical prostatectomy. JAMA. 1999;
increases overall survival by 2.6 yr vs. RP alone 281(17):15911597.
(13.9 vs. 11.3 yr) and reduces mortality risk by 4. NIH-funded study shows increased survival in men
46%. with metastatic prostate cancer who receive
r With metastatic CaP starting androgen blockade,
chemotherapy when starting hormone therapy
the median time to progression is 1824 mo. The http://www.cancer.gov/newscenter/newsfromnci/
median survival once patients progress on androgen 2013/E3805 (Posted December 5, 2013).
blockade is 1219 mo. P
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PROSTATE CANCER, RISING PSA FOLLOWING ANDROGEN ABLATION (CASTRATION-RESISTANT PROSTATE CANCER)
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PATHOPHYSIOLOGY Imaging
BASICS r A combination of genetic, hormonal, and r Transrectal ultrasound is used to guide
environmental factors underlies the development of template-based prostate biopsies.
DESCRIPTION PCa. r A bone scan and/or cross-sectional imaging are
r The National Comprehensive Cancer Network r >95% of all tumors are adenocarcinoma. unnecessary due to the exceedingly low risk of
(NCCN) (1) defines verylow-risk prostate cancer Other histologic types include transitional cell, metastatic disease.
(PCa) with the following criteria: Clinical stage T1c, small cell, and sarcoma. r Magnetic resonance imaging (MRI) of the prostate is
prostate-specific antigen (PSA) <10 ng/mL, PSA NCCN risk categories and AS only pertain to currently under investigation for its utility in
density <0.15 ng/mL/g, biopsy Gleason score 6, adenocarcinoma. evaluating tumor extent and the presence of
2 positive biopsy cores, and 50% cancer in any r High-grade prostate intraepithelial neoplasia is felt high-grade disease.
1 core. to be a precursor to adenocarcinoma.
This definition is based on the work of Epstein r Early-stage adenocarcinoma is androgen Diagnostic Procedures/Surgery
r PCa is definitively diagnosed with prostate biopsy.
et al. (2) which identified parameters associated dependent. As PCa becomes more advanced,
with low-volume organ-confined (ie, insignificant) Most commonly performed in the office setting
tumors dedifferentiate and lose this dependency. using transrectal ultrasound.
PCa at the time of radical prostatectomy. r 70% of PCa arise from the peripheral zone of the
r Active surveillance (AS) aims to spare men with A 1012-core biopsy should be performed to
prostate. ensure adequate sampling of the prostate.
insignificant tumors the side effects of treatment r Tumors are often multifocal. Requires only local lidocaine for analgesia.
while maintaining the ability to intervene with
A hypoechoic lesion may represent an area of
curative intent upon the detection of disease ASSOCIATED CONDITIONS
progression. r Many men with PCa also have benign prostatic cancer but is not a sensitive finding.
The goals of AS are accomplished by carefully Transperineal biopsy is typically reserved for men
hyperplasia (BPH)/lower urinary tract symptoms.
following men with serial PSA measurements, with several negative biopsies in whom PCa is still
BPH is not a precursor to PCa.
digital rectal exams (DREs), and prostate biopsies. suspected.
r AS is most appropriate for men with verylow-risk GENERAL PREVENTION Allows for systematic saturation biopsies using a
r The 5-reductase inhibitors (5-ARIs) finasteride and grid-based approach.
PCa and a life expectancy of <20 yr, or for those dutasteride have been shown in 2 randomized Requires general anesthesia.
with low-risk PCa (defined by the NCCN as clinical clinical trials (PCPT and REDUCE) to decrease the Image fusion biopsy combining ultrasound/MRI
stage T1 to T2a, biopsy Gleason score 6, and PSA incidence of PCa. images are being explored.
<10 ng/mL) and a life expectancy of <10 yr (1). These medications, however, failed to receive FDA
r The exact criteria for AS enrollment vary by Pathologic Findings
approval for PCa prevention due to their r Biopsy Gleason score 6 and 2 positive cores
institution. suggested association with increased risk of
Triggers for intervention while on AS also vary by with 50% cancer in each core are required for the
high-grade tumors (controversial). definition of verylow-risk PCa.
center, but typically include violation of the r The SELECT trial showed that daily selenium and/or r Evolving use of genomic assays based on the
enrollment criteria or a rapid rise in PSA. vitamin in E do not prevent PCa and should not be
Dahabreh et al. (3) have reviewed the prostate biopsy to determine risk of progression on
recommended to patients. AS (Oncotype DX and Prolaris).
enrollment and progression criteria used at
number of different institutions. DIFFERENTIAL DIAGNOSIS
EPIDEMIOLOGY DIAGNOSIS r Localized PCa:
Incidence BPH, prostatitis (granulomatous, acute, or
r 240,000 new cases of PCa are diagnosed HISTORY chronic), recent instrumentation,
r Uniformly asymptomatic.
annually in the United States. nonadenocarcinoma prostate malignancy
Typically detected on prostate biopsy performed
r >90% of new cases will be clinically localized. (sarcoma, urothelial carcinoma)
for an elevated PSA and/or finding of a prostate
r Up to 40% cases would have remained clinically nodule on DRE.
insignificant had they not been detected on routine Occasionally detected at the time of transurethral TREATMENT
screening. resection of the prostate for BPH.
Prevalence GENERAL MEASURES
PHYSICAL EXAM r Adequate patient evaluation and review of all
An estimated 2.6 million men in the United States Verylow-risk PCa has no findings on DRE (clinical
carry a diagnosis of PCa. treatment options is essential
stage T1c). r Identification of patients who may be an
RISK FACTORS DIAGNOSTIC TESTS & INTERPRETATION appropriate candidate for AS
r Age
Lab MEDICATION
r Family history/genetics
PSA <10 ng/mL and PSA density <0.15 ng/mL/g are
r African American race required for the definition of verylow-risk PCa. First Line
r No role for chemotherapy or androgen deprivation
r Possibly obesity and a Western diet
for men who are candidates for AS.
Genetics r 5-ARIs do not appear to prevent disease progression
Mutations in a number of genes have been linked to of men on AS.
PCa including BRCA1, BRCA2, HOXB13, and HPC1.
Second Line
N/A
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SURGERY/OTHER PROCEDURES
r Radical prostatectomy may be offered to men who
FOLLOW-UP ADDITIONAL READING
Patient Monitoring r Heidenreicha A, Bellmunt J, Bolla M, et al. EAU
desire treatment. r The optimal protocol for monitoring men on AS is
A pelvic lymph node dissection may be omitted unknown. guidelines on prostate cancer. Part 1: Screening,
given the low risk of lymph node metastases in Most advocate for biannual PSA measurements diagnosis, and treatment of clinically localised
this population. with DRE and annual 1214-core prostate biopsy. disease. Eur Urol. 2011;59:6171.
Comparing open to robotic or laparoscopic PSA kinetics do not appear to be helpful in r Thompson I, Thrasher JB, Aus G, et al. Guideline for
surgery, outcomes appear to be equivalent predicting disease progression (5). the management of clinically localized prostate
between surgical approaches (4). r Monitoring for disease progression is not indicated cancer: 2007 update. J Urol. 2007;177:21062131.
Major side effects of surgery include urinary after age 75 of when life expectancy is <10 yr.
incontinence and erectile dysfunction.
See Also (Topic, Algorithm, Media)
r Prostate Cancer, General Considerations
Patient Resources
ADDITIONAL TREATMENT r The NCCN Guidelines for Patients: Prostate Cancer. r Prostate Cancer, Genomic Markers
Radiation Therapy (http://www.nccn.org/patients/guidelines/prostate/) r Prostate Cancer, Localized (T1, T2)
r External beam radiation therapy or brachytherapy r What You Need to Know About Prostate Cancer r PSA Elevation, General Considerations
are acceptable alternatives to AS and surgery. from the National Cancer Institute. (http://www.
Adjuvant hormonal therapy is not indicated with cancer.gov/cancertopics/wyntk/prostate/
either approach in this group of men. prostate.pdf). CODES
When external beam radiation therapy is
performed, treatment with either intensity-
REFERENCES ICD9
modulated or 3-dimensional conformal radiation r 185 Malignant neoplasm of prostate
therapy should be utilized to limit toxicity to r 790.93 Elevated prostate specific antigen [PSA]
surrounding organs. 1. NCCN Practice Guidelines Version 1. 2014.
http://www.nccn.org./ Accessed January 6, 2014. r V76.44 Screening for malignant neoplasms of
Brachytherapy should be avoided in men with
symptoms of bladder outlet obstruction (high 2. Epstein JI, Walsh PC, Carmichael M, et al. prostate
International Prostate Symptom Score) due to the Pathologic and clinical findings to predict tumor
extent of nonpalpable (stage T1c) prostate cancer. ICD10
risk of worsening lower urinary tract symptoms. r C61 Malignant neoplasm of prostate
Major side effects of radiation include urinary JAMA. 1994;271:368374.
r R97.2 Elevated prostate specific antigen [PSA]
incontinence, irritative voiding symptoms, erectile 3. Dahabreh IJ, Chung M, Balk EM, et al. Active
r Z12.5 Encounter for screening for malignant
dysfunction, radiation induced proctitis, surveillance in men with localized prostate cancer:
hemorrhagic cystits and secondary malignancies A systematic review. Ann Intern Med. 2012; neoplasm of prostate
most commonly of the bladder and rectum. 156:582590.
Additional Therapies 4. Magheli A, Gonzalgo ML, Su LM, et al. Impact of CLINICAL/SURGICAL
r Technologies for focal and hemi- ablation are surgical technique (open vs laparoscopic vs
robotic-assisted) on pathological and biochemical PEARLS
currently in the early phases of investigation.
Modalities include cryotherapy, high intensity outcomes following radical prostatectomy: An r A large percentage of older men with
focused ultrasound, interstitial laser and analysis using propensity score matching. BJU Int.
screen-detected PCa will have insignificant tumors
electroporation. 2011;107:19561962.
and, therefore, not benefit from intervention.
5. Ross AE, Loeb S, Landis P, et al. Prostate-specific r To avoid the potential morbidity associated with
Complementary & Alternative antigen kinetics during follow-up are an unreliable
Therapies treatment, AS should be offered to men with
trigger for intervention in a prostate cancer
Low fat diet appropriate recommendation verylow-risk PCa and a life expectancy of <20 yr.
surveillance program. J Clin Oncol. 2010;28: r The optimal follow-up protocol is not well defined
28102816.
but typically includes biannual PSA measurements
ONGOING CARE with DRE and annual 1214-core prostate biopsy.
r PSA kinetics are less useful than biopsy findings for
PROGNOSIS
r Up to 30% of men enrolled in AS will be reclassified accurately reclassifying men while on AS.
r It is unknown if men reclassified on AS would have
and go onto require some form of treatment.
It remains unknown if these men would have had been better served with immediate treatment;
a superior oncologic outcome had they undergone however, long-term data from a number of centers
immediate treatment. suggest good oncologic outcomes with this
management strategy.
COMPLICATIONS
r AS spares men with insignificant tumors the side
effects of unnecessary treatment.
r The major risk of AS is missing the opportunity to
intervene when cure is still possible.
r A small percentage of men will experience an
infectious or bleeding complication related to a
prostate biopsy.
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PROSTATE, ABSCESS
Garjae D. Lavien, MD
Michael J. Naslund, MD
r With severe immunocompromise, such as HIV; more DIAGNOSTIC TESTS & INTERPRETATION
BASICS unusual organisms such as TB, Cryptococcus, Lab
histoplasmosis, and Candida should be considered. r Complete blood count with differential
DESCRIPTION r Melioidosis is an infection (usually abscesses in r Urinalysis, urine/blood cultures
r Prostate abscess is an infection of the prostate with
many sites including the prostate). r Gram stain/culture of prostatic fluid once drained
focal accumulation of pus within the prostate gland Caused by the gram-negative Burkholderia r AFB or mycobacterium-specific PCR if TB suspected
r Difficult to initially clinically distinguish from acute pseudomallei. r Prostate-specific antigen should not be obtained in
bacterial prostatitis Usually associated with diabetes.
r Usually as a result of ineffective antibiotic therapy this setting as it will usually be elevated due to the
Very high prevalence in East Asia and Northern
inflammatory process
for acute prostatitis Australia.
Rare in nonhospitalized patients Imaging
ASSOCIATED CONDITIONS r Transrectal ultrasound (TRUS)
EPIDEMIOLOGY r Any disease process that causes
Will reveal hypoechoic zones with irregular
Incidence immunocompromise: internal echoes, septations, and indirect borders
Decreasing with widespread use of antibiotics Cancer with the surrounding prostate;
Chronic renal failure, hemodialysis Must be performed cautiously.
Prevalence Cirrhosis
Diagnosed in 0.2% of patients with urologic May guide drainage and aspiration
Diabetes The presence of gas suggests EPA
symptoms, 0.52.5% of patients hospitalized for HIV/AIDS
prostatic symptoms (1) r Color Doppler sonography
GENERAL PREVENTION Will show an increase in vascularity around the
RISK FACTORS r Aimed at preventing and treating sexually abscess, due to hyperemia stemming from the
r Bladder outlet obstruction, history of bacterial
transmitted infections surrounding inflammation
prostatitis r Relieving/improving signs/symptoms of bladder r Computed tomography (CT)
r Chronic hemodialysis
r Compromised immune system (eg, HIV/AIDS, outlet obstruction Can determine penetration of the abscess into the
r Diabetic glycemic control periprostatic tissues and identify gas within the
diabetes, etc.) r Appropriate treatment of patients with acute prostate
r Indwelling catheters
prostatitis CT findings include nonenhancing fluid-density
r Lower urinary tract instrumentation collections that can be multiseptated or
r Sexually transmitted infections rim-enhancing lesions.
DIAGNOSIS r MRI
Genetics
N/A MRI may not be feasible in patients who are
HISTORY critically ill and require acute management
r Fevers, chills
PATHOPHYSIOLOGY
r Usually an ascending infection in association with r Urinary symptoms with attention paid to voiding Diagnostic Procedures/Surgery
r TRUS with aspiration
poor bladder emptying. patterns prior to acute presentation
r Urethral infection combined with intraprostatic r Transperineal ultrasound with aspiration
Dysuria, urinary urgency and frequency are almost
universal symptoms r Transurethral unroofing of prostate abscess
reflux of infected urine causes acute prostatitis.
r Acute prostatitis can, in patients with Suprapubic or subpubic pain, Pathologic Findings
immunosuppression or other risk factors, progress to Severe perineal pain r Purulent material will be expressed from prostate
abscess. Rectal tenesmus during surgical drainage procedure:
r Hematogenous dissemination, especially with r Acute urinary retention
Gram stain and culture of material will give
r Sexual history/social history (IV drug use, etc.) causative agent, most commonly bacterial
Staphylococcus sp. seen in immunocompromised
patients/IV drug users. r Associated medical comorbidities
DIFFERENTIAL DIAGNOSIS
r Most common etiologic agent is Escherichia coli r Clinically can be hard to distinguish from urinary
ALERT
(2)[B]. Where prostate abscess or acute prostatitis is tract infection (UTI), acute prostatitis, or any other
r With the advent of antibiotics, the incidence of lower UTI.
suspected, rectal exam may be contraindicated. r Should have clinical suspicion, which can be
Neisseria gonorrhea as causative agent has
decreased significantly. PHYSICAL EXAM confirmed with imaging.
r Most common etiologic agent seen in r Perineal pain, tenderness
r Urethral discharge
emphysematous prostate abscess (EPA) is
Klebsiella pneumoniae (3)[B]. r Digital rectal exam can reveal exquisitely tender and
warm prostate with fluctuance or simply an
enlarged prostate
r Signs of other medical comorbidities (ie, new cardiac
murmur, ascites, cough, track marks, etc.)
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PROSTATE, ABSCESS
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DIFFERENTIAL DIAGNOSIS r Open simple prostatectomy usually for glands 2. Bushman W. Etiology, epidemiology and natural
r Obstructive symptoms: Detrusor sphincter >100 g: history of benign prostatic hyperplasia. Urol Clin
dyssynergia, foreign body, meatal stenosis, Suprapubic prostatectomy: Enucleation of North Am. 2009;36:403415.
neurogenic bladder, pelvic floor dysfunction, adenoma through bladder; useful with coexisting 3. McConnel JD, Roehrborn CG, Bautista OM, et al.
prostate cancer, prostatic abscess, prostatitis problems such as very large bladder calculi or to The long-term effect of doxazosin, finasteride, and
syndrome, urethral obstruction (stricture, condyloma) repair diverticulum combination therapy on the clinical progression of
r Irritative/storage symptoms: Bladder cancer, Retropubic simple prostatectomy: Enucleation of benign prostatic hyperplasia. N Engl J Med.
detrusor hyperreflexia/OAB, interstitial cystitis, adenoma through incision in anterior prostate 2003;349:23872398.
polyuria/polydipsia, prostatitis syndromes commissure 4. Roehrborn CG, Boyle P, Gould AL. Waldstreicher J
r Many minimally invasive alternative surgical Serum prostate-specific antigen as a predictor of
procedures: Microwave- and water-induced prostate volume in men with benign prostatic
TREATMENT hyperthermia, transurethral needle ablation, laser hyperplasia. Urology. 1999;53(3):581589.
vaporization (contact, noncontact, interstitial, 5. Cantrell MA, Baye J, Vouri SM. Tadalafil: A
GENERAL MEASURES diode), laser prostatectomy (holmium, KTP)
r Directed at QOL unless evidence of significant phosphodiesterase-5 inhibitor for benign prostatic
damage to urinary tract from obstruction ADDITIONAL TREATMENT hyperplasia. Pharmacotherapy. 2013;33(6):
(hydronephrosis, bladder calculi, recurrent Radiation Therapy 639649.
infections) N/A 6. McNicholas TA, Woo HH, Chin PT, et al. Minimally
r Guidelines suggest watchful waiting for men with invasive prostatic urethral lift: surgical technique
Additional Therapies and multinational experience. Eur Urol. 2013;64(2):
mild symptoms IPSS 7 or for more severe r Prostatic stents; best if need TURP but poor surgical
symptoms if they are not bothersome to the patient. 292299.
risk
Simple behavior modification (fluid restriction, r Prostatic urethral lift (UroLift TM) mechanically
decreased alcohol/caffeine) may help
r Medical therapy considered 1st-line by most, but opens the prostatic urethra with UroLift implants ADDITIONAL READING
that are placed transurethrally under cystoscopic
usually requires continuous therapy to maintain visualization, thereby separating the encroaching r AUA Practice Guidelines Committee. AUA guideline
benefit prostatic lobes (6) on management of benign prostatic hyperplasia
r -Blocker and 5-ARIs often prescribed together
(2003). Chapter 1: Diagnosis and treatment
Complementary & Alternative
MEDICATION recommendations. J Urol. 2003;170:530547.
Therapies r McVary KT, Roehrborn CG, Avins AL, et al. Update
First Line Phytotherapy (plant extracts) includes saw palmetto,
r -Blockers (reduce muscle tone in prostate/bladder Pygeum africanum; -sitosterols have limited support on AUA guideline on the management of benign
neck): in the literature prostatic hyperplasia. J Urol. 2011;185:17931803.
Terazosin (start 1 mg/d to max 20 mg) See Also (Topic, Algorithm, Media)
Doxazosin (start 1 mg/d to max 8 mg; XL form r Bladder Outlet Obstruction (BOO)
28 g/d) ONGOING CARE r Lower Urinary Tract Symptoms (LUTS)
Tamsulosin (start 0.4 mg to max 0.8 mg) PROGNOSIS r Prostate, Benign Hyperplasia/Hypertrophy (BPH)
Alfuzosin (10 mg/d) r Symptoms usually well managed by medications Image
Silodosin (8 mg/d) r Progression of disease, when risk factors identified, r Prostate, Stents (UroLume and Spanner)
Dizziness, orthostatic hypotension, and r Reference Tables: AUA Symptom Index/International
can be well managed
ejaculatory dysfunction are most common side
effects COMPLICATIONS Prostate Symptom Score (I-PSS)
r 5-ARIs (block intracellular DHT conversion; generally r Generally accepted sequelae of untreated,
best for larger glands, may take 612 mo for undertreated, or progressive BPH
improvement): r Historically, many men typically had complications of CODES
Finasteride (5 mg/d) BPH including UTIs, hematuria, bladder calculi,
Dutasteride (0.5 mg/d) bladder decompensation, incontinence, and upper ICD9
r -Blocker (tamsulosin 0.4 mg) combined with 5-ARI tract deterioration. With modern awareness and r 600.00 Hypertrophy (benign) of prostate without
(dutasteride 0.5 mg) management techniques, this is less common. urinary obstruction and other lower urinary tract
r The most commonly used endpoints in medical trials symptom (LUTS)
Second Line r 600.01 Hypertrophy (benign) of prostate with
r Antimuscarinic agents may help with bladder are symptom deterioration, BPH-related surgery, and
AUR. AUR continues to be the most widely accepted urinary obstruction and other lower urinary tract
overactivity: symptoms (LUTS)
and most scientific endpoint, although the exact
Various agents including oxybutynin (5 mg TID), r 600.10 Nodular prostate without urinary obstruction
pathophysiologic mechanism is not completely
tolterodine (24 mg/d), solifenacin (510 mg/d),
understood.
others ICD10
r Phosphodiesterase-5 inhibitor FOLLOW-UP r N40.0 Enlarged prostate without lower urinary tract
Tadalafil (2.55 mg/d) Patient Monitoring symptoms
FDA approved for LUTS secondary to BPH r Periodic monitoring depending on severity of r N40.1 Enlarged prostate with lower urinary tract
Contraindicated in patients on nitrates, symptoms symptoms
nonselective -blockers, and CYP 450 inhibitors Monitor response to therapy with history, AUA SS, r N40.2 Nodular prostate without lower urinary tract
Side effects include back pain, dizziness, PVR, flow rate symptoms
headache, and dyspepsia (5) Upper tract imaging and measure of renal
SURGERY/OTHER PROCEDURES function if elevated PVR (>300 cc)
r Often considered 2nd-line after failure of medical Patient Resources CLINICAL/SURGICAL
therapy; may be 1st-line in retention or if very large Urology Care Foundation. www.urologyhealth.org PEARLS
prostate. r 5-ARIs (such as finasteride and dutasteride)
r TURP represents gold standard against which all
REFERENCES contraindicated if prostate enlargement absent
other therapies are compared.
1. Roehrborn C. Male lower urinary tract symptoms
(<25 g). P
r Goal of treatment is improving symptoms/QOL.
(LUTS) and benign prostatic hyperplasia. Med Clin
North Am. 2011;95:87100.
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PROSTATE, CALCULI
Christopher Amling, MD, FACS
Nicholas Cowan, MD
PATHOPHYSIOLOGY
BASICS r Urinary intraprostatic reflux implicated in stone DIAGNOSIS
formation
DESCRIPTION r Intraprostatic calculi presumed to form by the HISTORY
r Prostatic calculi are extremely common and rarely r Typically stones are asymptomatic
precipitation of prostatic secretions and calcification r Evaluate for history of lower urinary tract symptoms
symptomatic of the corpora amylacea under inflammatory
r Most stones are discovered incidentally conditions (LUTS) (2)
r Treatment typically reserved for severely Inspissation of prostatic secretions within the Patients should complete the international
symptomatic men prostatic ducts prostate symptom score (IPSS)
r Stones within the prostatic urethra rare and likely Concentric layering of calcium phosphate and Presence of large calculi associated with moderate
due to bladder or upper tract stones that become calcium carbonate on inspissated core result in LUTS
r 2547% of men with chronic pelvic pain have
trapped in the prostatic urethra growth
r Reports of calculi in the prostatic urethra following Stone elements may contain constituents found significant prostatic calcifications
transurethral resection of the prostate only in urine and not in prostatic secretions Correlation seen with stone size, not number
r Stones may harbor bacteria and serve as source for r Prostatitis history
EPIDEMIOLOGY relapsing UTI r With prostatic utricle stones patients typically
Incidence r For prostatic utricle stones, prostatic utricle distends present with chronic UTI, hematuria, urethral
r 7% in pathologic specimens discharge, epididymitis, and voiding dysfunction.
during voiding and then passively drains.
r 20% in autopsies Often a history of hypospadias is present
Impaired emptying results in urinary stasis stone
r 30% in radiologic studies, with higher percentages formation. Patients present clinically with chronic PHYSICAL EXAM
in ultrasound scan exams UTI, hematuria, urethral discharge, epididymitis, r Genitourinary exam including DRE
Prevalence and voiding dysfunction. DRE unlikely to localize stones
r Small areas of microcalcification can be seen in 2nd r Presence of hypospadias
ASSOCIATED CONDITIONS
and 3rd decades of life r Chronic pelvic pain syndrome
r Almost all men (99%) have some degree of r Prostatitis DIAGNOSTIC TESTS & INTERPRETATION
prostatic calcification noted at autopsy r No association between prostate calculi and risk of Lab
r Urine culture
Stone burden and size typically increase as a man prostate cancer
ages r Hypospadias (enlargement of the prostatic utricle, a Escherichia Coli, enterococci, and Klebsiella spp.
more common
RISK FACTORS (1) Mullerian duct remnant) r Expressed prostatic secretions
r Intraprostatic calculi:
GENERAL PREVENTION May see increased leukocytes
Recurrent urinary tract infections (UTIs) No known preventative strategies r PSA optional
Pelvic radiation (for prostate cancer) PSA levels not influenced by presence or volume
Studies are mixed on role of inflammation in stone of prostatic calculi, but infection related to chronic
development nidus may falsely elevate PSA (3)
r Prostatic urethral calculi
Urolithiasis Imaging
r Stones often identified incidentally
Enlarged prostatic utricle
r Transrectal ultrasound (TRUS)
History of transurethral resection of the prostate
Highly sensitive for large calculi
Genetics r Sometimes seen on plain film
N/A
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PROSTATE, NODULE
Gurdarshan S. Sandhu, MD
Gerald L. Andriole, MD, FACS
Genetics r BPH
BASICS See Prostate Cancer, General Considerations Prostate gland can be variably enlarged (size does
not correlate with extent of voiding symptoms)
DESCRIPTION PATHOPHYSIOLOGY
r Normal prostate has a soft, uniform consistency. Rubbery consistency
r A prostatenadule is usually described as a palpable r Infectious lesions
r Prostate enlarges with age.
lesion detected on digital rectal exam (DRE) raise Prostatitis
r Microscopically, nodular prostatic hyperplasia
concern for prostate cancer (CaP) Warm, tender prostate
r Nodules can be described as soft, rubbery, firm, consists of nodules of glands and intervening Can be fluctuant or feel boggy
hard, or rock hard stroma. May occasionally form benign palpable Prostate abscess
r Nodules can be well circumscribed or irregular and nodules. Localized, fluctuant tender region in prostate
r Nodule can be subjectively graded by degree of r Calculus can present as hard, small nodule
diffuse
r A normal prostate is about the size of a chestnut firmness/hardness (grades 13)
r CaP has to have a volume of 0.2 mL or larger to be DIAGNOSTIC TESTS & INTERPRETATION
and has a consistency similar to that of the
contracted thenar eminence of the thumb. detected by DRE. Lab
r PSA
This can be simulated by opposing the thumb to ASSOCIATED CONDITIONS
the little finger and palpating the contracted r Prostate adenocarcinoma Serum levels vary with age, race, and prostate
muscle r Benign prostatic hyperplasia (BPH) volume
r Consistency of nodule can denote underlying Improves the positive predictive value of DRE for
r History of intravesical BCG for bladder cancer
cancer
pathology.
r Rapidity of appearance and changes in size and GENERAL PREVENTION No cut-off value below which the absence of
None prostate cancer can be guaranteed
consistency can infer malignant potential. Risk of prostate cancer is continuous as PSA
r Nodule detection by DRE is recommended as part of
increases (3)
prostate cancer detection programs. DIAGNOSIS See PSA Elevation, General Considerations for
Current recommendations from the American further specifics on PSA
Cancer Society are, if men decides to be tested for HISTORY r Urinalysis
prostate cancer, they should have the PSA blood r History of lower urinary tract symptoms
Variable findings in men with abnormal DRE;
test with or without a rectal exam. Irritative voiding symptoms
sterile pyuria in granulomatous prostatitis
Obstructive voiding symptoms
EPIDEMIOLOGY Generally normal in men with prostate cancer
Fever
Incidence without urinary tract infection
Previous prostate biopsy or surgery such as TURP
r Prostate nodule as an isolated finding with normal Urine culture can be positive for gram-negative
Previous pelvic external beam radiation or
PSA is found in <10% of cases of prostate cancer in bacteria in acute and chronic bacterial prostatitis
prostate brachytherapy
the US Urine cytology can be positive in urothelial cancer
History of prostatitis, abscess, or exposure to TB
r Increasingly men are being diagnosed with prostate Systemic granulomatous disease (Wegners, etc.) Imaging
cancer based on an elevated serum prostate-specific Family history of genitourinary malignancies r Transrectal ultrasound (TRUS)
antigen (PSA) and not an abnormal DRE (50% of Classic appearance of prostate adenocarcinoma is
diagnoses in 2002) (1) ALERT a round or oval hypoechoic lesion located in the
r 94% of men diagnosed with prostate cancer in Where prostate abscess or acute prostatitis is peripheral zone
20042005 have localized disease (cT1 or cT2) (2) suspected, rectal exam may be contraindicated. Not very sensitive as 39% of tumors can be
isoechoic
Prevalence Also nonspecific as granulomatous lesions can
510% of men in screening programs have PHYSICAL EXAM
r DRE be hypoechoic
abnormal/suspicious DRE
Carcinoma (prostatic or urothelial cell carcinoma) BPH can have variable appearance
RISK FACTORS Firm, indurated nodules within the prostate Distinguishable from prostate cancer only by
r Prostate cancer biopsy
gland
Nodule that changes in consistency and size over Prostate cancer most often arises in the r Abdominal computed tomography (CT) or magnetic
time posterior peripheral region of the prostate resonance imaging (MRI)
Elevated serum PSA (>2.54 ng/mL) Advanced prostate cancer can make the entire Primarily used for staging purposes in patients
Positive family history gland firm and cause obliteration of the medial with high-risk prostate cancer
r Benign nodule r Bone scan to detect bone metastases
and lateral sulci
No significant change over time Advanced cancer can also extend into the Also primarily used for staging purposes in
Nodule may be softer seminal vesicles or toward the side wall laterally patients with high-risk prostate cancer
Prior episodes of prostatitis, biopsy, or prostate
surgery (transurethral resection)
Prior therapy with intravesical Bacillus
CalmetteGuerin (BCG)
Granulomatous nodules can be due to infectious
causes (eg, tuberculosis [TB]) or systemic
granulomatous diseases
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PROSTATITIS, CHRONIC NONBACTERIAL, INFLAMMATORY AND NONINFLAMMATORY (NIH CP/CPPS III A AND B)
r Pentosan polysulfate 100 mg TID 3. Shoskes DA, Landis JR, Wang Y, et al; Chronic
TREATMENT r Gabapentanoids Prostatitis Collaborative Research Network Study
Pregabalin 150600 mg daily Group. Impact of post-ejaculatory pain in men with
GENERAL MEASURES r Muscle relaxants category III chronic prostatitis/chronic pelvic pain
r As the pathogenesis of CP/CPPS category III is syndrome (CPPS). J Urol. 2004;172:542547.
Baclofen
considered multifactorial, effective treatment for Diazepam 4. Barbalias GA, Meares EM Jr, Sant GR.
CP/CPPS III often requires multimodal therapy Prostatodynia: Clinical and urodynamic
r A meta-analysis comparing -Blockers, antibiotics Second Line
characteristics. J Urol. 1983;130:514517.
See above
and anti-inflammatory/immune modulating 5. Thakkinstian A, Attia J, Anothaisintawee T, et al.
therapies found a combination of -blockers and SURGERY/OTHER PROCEDURES -blockers, antibiotics and anti-inflammatories
antibiotics to be superior to -blockers, antibiotics, r Not recommended. Last resort unless other have a role in the management of chronic
or anti-inflammatory/immune modulating therapies indications are discovered during the workup prostatitis/chronic pelvic pain syndrome. BJU Int.
alone in the reduction of NIH-CPSI scores (5) Transurethral microwave thermotherapy 2012;110:10141022.
r Treatment should also be targeted to the etiologic
ADDITIONAL TREATMENT
mechanisms using the UPOINT system
r Focus of therapy should be on symptom relief Radiation Therapy
N/A
ADDITIONAL READING
r Conservative measures such as diet modification,
r Nickel JC. Prostatitis and related conditions, orchitis,
myofascial physical therapy, phytotherapies, Additional Therapies
See below and epididymitis. In: Wein AJ, Kavoussi LR, Novick
acupuncture should be considered as part of the
AC, et al. eds. Campbell-Walsh Urology. 10th ed.
1st-line therapy Complementary & Alternative
r Symptoms should be followed with NIH-CPSI Philadelphia, PA: Elsevier; 2011.
Therapies r Schaeffer AJ. Chronic Prostatitis and the chronic
questionnaires and voiding diaries r Dietary and lifestyle modification
r Phytotherapy pelvic pain syndrome. N Eng J Med. 2005;355:
MEDICATION 16901698.
Pollen extract
First Line See Also (Topic, Algorithm, Media)
r The choice of agents in the 1st- or 2nd-line setting is Quercetin
r NIH-CPSI Questionnaires
Saw palmetto
practitioner dependent with no specific agent r Acupuncture r Prostatitis, Acute Bacterial (NIH I)
approved specifically for this condition r Myofascial physical therapy r Prostatitis, Asymptomatic Inflammatory (NIH IV)
r -Blockers: Multiple randomized, placebo-controlled
r Stress management/cognitive-behavioral therapy r Prostatitis, Chronic Bacterial (NIH II)
trials have demonstrated a duration of at least 3 mo r Prostatitis, Chronic Nonbacterial, Inflammatory and
r Frequent ejaculation
or longer may be needed before assessment can be
made of treatment failure or success. Noninflammatory (NIH CP/CPPS III A and B)
Side effects of -blockers include hypotension, Image
dizziness, fatigue, and retrograde ejaculation ONGOING CARE r Prostatitis, General
Alfuzosin 10 mg BID for 12 wk; contraindicated r Stamey Test (3-glass test, 4-glass tests,
PROGNOSIS
with moderate hepatic insufficiency or with Remissions and flare-ups common over the long term MearesStamey Test)
cytochrome P450 3A4 inhibitors
Doxazosin 14 mg daily for 12 wk; escalate COMPLICATIONS
dose until symptom relief obtained None known CODES
Tamsulosin 0.4 mg daily for 12 wk FOLLOW-UP
Terazosin 15 mg daily; escalate dose until ICD9
Patient Monitoring
symptom relief obtained r 338.4 Chronic pain syndrome
Silodosin (8 mg/d) Long-term supportive care
r 601.1 Chronic prostatitis
r Antibiotic therapy: Data conflicting on the benefit Patient Resources r 789.09 Abdominal pain, other specified site
r Urology Care. Foundation. http://www.
and therapeutic benefit should be reassessed after 2
to 4 wk of initiating therapy urologyhealth.org/urology/index.cfm?article=15 ICD10
r http://www.prostatitis.org r G89.29 Other chronic pain
Can be considered in antibiotic-nave patients
Fluoroquinolones: Side effects include dizziness, r N41.1 Chronic prostatitis
restlessness, headache, nausea, rash r R10.2 Pelvic and perineal pain
Ciprofloxacin, levofloxacin 500 mg daily for REFERENCES
4 wk (some concern over growing resistance to 1. Suskind AM, Berry SH, Ewing BA, et al. The
this class of drugs) prevalence and overlap of interstitial CLINICAL/SURGICAL
Trimethoprimsulfamethoxazole 160/80 mg BID
for 4 wk: Side effects include anorexia, nausea,
cystitis/bladder pain syndrome and chronic PEARLS
prostatitis/chronic pelvic pain syndrome in men:
vomiting, rash, urticaria Results of the RAND Interstitial Cystitis Multimodal therapy is oftentimes required because of
r 5-Reductase inhibitor: the varied pathologies associated with this condition.
Epidemiology Male Study. J Urol. 2013;189:
Finasteride 5 mg daily or 141145.
Dutasteride 0.5 mg daily 2. Shoskes DA, Nickel JC, Kattan MW. Phenotypically
r Anti-inflammatory agents
directed multimodal therapy for chronic
Rofecoxib 2550 mg daily: Symptom relief at prostatitis/chronic pelvic pain syndrome: A
higher doses, but not recommended because of prospective study using UPOINT. Urology.
cardiovascular risk 2010;75:12491253.
Oral prednisolone
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r A modified MearesStamey test (2-glass test) can Second Line Patient Resources
also be performed that is considered more r Anti-inflammatories (ibuprofen) for symptoms Urology Care Foundation. http://www.urologyhealth.
convenient and practical r -Blockers (doxazosin, tamsulosin, alfuzosin, org/urology/index.cfm?article=15
After cleansing the glans, obtain 10 mL of a silodosin) may help with LUTS
midstream urine for culture (preprostate
massage). Represents bladder flora. Should also SURGERY/OTHER PROCEDURES REFERENCES
r Not generally recommended
be dipped for white cells r TURP 1. Schaeffer AJ. Chronic prostatitis and the chronic
Perform prostate massage and obtain 10 mL of pelvic pain syndrome. N Engl J Med. 2006;
urine (postprostate massage) for culture and Can be considered in select cases of refractory
prostatitis with infected calculi and/or obstruction 355:16901698.
microscopic exam. Represents prostate and
2. Schaeffer AJ, Knauss JS, Landis JR, et al. Leukocyte
bladder flora. If white cells are present, they may ADDITIONAL TREATMENT and bacterial counts do not correlate with severity
represent bacterial prostatitis or NIH type IIIA Radiation Therapy of symptoms in men with chronic prostatitis: The
If postmassage colony counts are 10 higher N/A National Institutes of Health Chronic Prostatitis
than premassage sample, bacterial prostatitis is
Additional Therapies Cohort Study. J Urol. 2002;168:10481053.
present. If both cultures have similar counts,
r Frequent ejaculation (in patients with enlarged, 3. Naber KJ. Management of chronic bacterial
cystitis is present
r Semen culture is of limited use, demonstrating low symptomatically congested glands) prostatitis: Whats New? BJU Intern. 2008;101(S3):
r Dietary modifications of common comestibles found 710.
sensitivity but high specificity compared to Stamey
test to irritate the lower urinary tract 4. Wagenlehner FM, Weidner W, Sorgel F, et al. The
r Uroflowmetry may demonstrate diminished flow r Moist heat with Sitz baths or heating pad for role of antibiotics in chronic bacterial prostatitis. Int
symptomatic relief J Antimicrob Agents. 2005;26:17.
with intermittency
r Elevated PVR may be present 5. Shoskes DA. Phytotherapy in chronic prostatitis.
Complementary & Alternative Urology. 2002;60:3537.
Pathologic Findings Therapies
r Prostate massage (very controversial):
N/A
May work by stimulating hibernating bacterial ADDITIONAL READING
DIFFERENTIAL DIAGNOSIS biofilms (making them more susceptible to
r Acute bacterial prostatitis/prostatic abscess
antimicrobials), draining the obstructed inflamed Krieger JN, Nyberg L Jr, Nickel JC. NIH consensus
r Bladder outlet obstruction/BPH ducts (allowing for better antimicrobial definition and classification of prostatitis. JAMA.
r Chronic nonbacterial prostatitis (NIH IIIA/B) penetration), and stimulating blood supply to the 1999;282(3):236237.
r Cystitis area.
r Interstitial cystitis r Avoid bicycling or other activities that cause perineal See Also (Topic, Algorithm, Media)
r Prostatitis, Acute, Bacterial (NIH I)
r Prostatic cyst pressure r Prostatitis, Asymptomatic Inflammatory (NIH IV)
r Seminal vesiculitis r Zinc supplements: Unproven benefit
r Prostatitis, Chronic, Bacterial (NIH II)
r STDs r Phytotherapy: Plant extracts and herbal medications
r Prostatitis, Chronic, Nonbacterial, Inflammatory
r Tuberculous/granulomatous prostatitis (ie, saw palmetto) popular but may only be as
effective as placebo (5) (NIH CP/CPPS IIIA)
r Urethritis or urethral pathology (stricture) r Prostatitis, Chronic, Nonbacterial, Noninflammatory
(NIH CP/CPPS IIIB)
ONGOING CARE r Prostatitis, General
TREATMENT r Prostatitis, Granulomatous
PROGNOSIS r Stamey Test (3-Glass Test, 4-Glass Tests,
GENERAL MEASURES r Fluoroquinolones have improved the ability to clear
r Antibiotic course normally extends for 68 wk and MearesStamey Test)
the infection (6090% cure reported).
sometimes longer with refractory infections. Goal is r Variable course with flare-ups possible. If
to eradicate the nidus of infection in the prostate.
culture-positive infection persists, consider longer
Follow culture results (3,4).
course of therapy (36 mo) with a lower daily dose. CODES
r Avoid alcohol, spicy foods, perineal pressure for
r Treating underlying obstruction or prostatic calculi if
extended times (sitting or bicycle riding), acidic ICD9
necessary may prevent further infections. r 041.49 Other and unspecified Escherichia coli [E.
beverages.
r Continue to engage in safe protected sexual activity, COMPLICATIONS coli]
as this is thought to reduce prostatic congestion. r Recurrent cystitis, epididymitis, urethritis r 131.03 Trichomonal prostatitis
r CPPS r 601.1 Chronic prostatitis
MEDICATION r Infertility (effect on semen quality debatable)
First Line r Primarily affects QOL ICD10
r Current sensitivity patterns are showing increased r A59.02 Trichomonal prostatitis
r Unknown if predisposes to prostate cancer
resistance to quinolones, TMX/SULFA, ampicillin. In r B96.20 Unsp Escherichia coli as the cause of
choosing antibiotic coverage prior to culture FOLLOW-UP diseases classd elswhr
sensitivity reports, know local resistance patterns Patient Monitoring r N41.1 Chronic prostatitis
based on your hospital antibiogram r Document clearing of positive culture
r Fluoroquinolones still preferred r Following prostate cancer screening guidelines is
No difference in bacterial eradication between recommended. Do not obtain PSA for at least 6 wk CLINICAL/SURGICAL
levofloxacin and ciprofloxacin, although prostatic
fluid concentration of levofloxacin is higher than
after culture clears. PEARLS
ciprofloxacin. Levofloxacin has daily dosing and Inadequately treated acute bacterial prostatitis may
may have better prostatic penetration increase risk for developing chronic prostatitis
Ciprofloxacin 500 mg PO BID for 46 w; syndromes including chronic bacterial prostatitis.
Levofloxacin 500 mg/d for at least 68 wk
r Trimethoprim 60 mgsulfamethoxazole 80 mg BID
for at least 68 wk
P
r Tetracycline derivatives (eg, doxycycline) only if
Chlamydia or Mycoplasma suspected
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Ryan S. Levey, MD
Justin D. Ellett, MD, PhD
r Phimosis
BASICS r Urethral stricture, distal DIAGNOSIS
r BPH
DESCRIPTION r Prostatic calculi HISTORY
r Prostatitis is a general term that refers to r Acute bacterial prostatitis (NIH I)
r Transurethral surgery/instrumentation
inflammation of the prostate. Fever, chills, malaise
r Transrectal prostate biopsy
r Traditionally classified as acute bacterial prostatitis, Perineal, suprapubic pain
r Unprotected anal sex Irritative voiding symptoms: Urgency, frequency,
chronic bacterial prostatitis, nonbacterial prostatitis, r UTI
and prostatodynia today the definitions used are dysuria
much more precise and based on the NIH system (1). Obstructive voiding symptoms: Hesitancy,
Genetics
r Revised 1995 NIH classification of prostatitis is intermittent stream, acute urinary retention
N/A
standard nomenclature: Rare sepsis
NIH Class I: Acute bacterial prostatitis; infection of
PATHOPHYSIOLOGY 5% will develop CP
r Extension of UTI r Chronic bacterial prostatitis (NIH II)
prostate, sudden onset, often associated with UTI r Manipulation of urinary tract or prostate
>10 WBC/HPF in 1st 10 mL voided urine and Recurrent UTIs
r Bacterial: Asymptomatic or CPPS (see below)
midstream catch
Positive culture in 1st 10 mL voided urine and Ascending infection through urethra r CP/CPPS (NIH IIIA/B)
midstream catch Refluxing urine into prostate ducts Pain in perineum, suprapubic region, penis,
NIH Class II: Chronic bacterial prostatitis; insidious Direct extension or lymphatic spread from rectum testicles, groin, low back
onset, relapsing, recurrent UTI Hematogenous spread Pain especially after or during ejaculation
>10 WBC/HPF Calculi serve as a nidus for infection Irritative/obstructive voiding symptoms lasting
Positive culture in expressed prostatic secretions Aerobic gram-negative bacteria >3 mo
(EPS) and 1st 10 mL of voided urine after EPS (Enterobacteriaceae [most common cause], ED, sexual disturbances, severe effect on quality of
NIH Class III: Chronic prostatitis (CP)/Chronic Escherichia coli [most common organism], life
pelvic pain syndrome (CPPS): Pseudomonas, Klebsiella, Proteus, Serratia), r NIH Class IV: None; usually only elevated PSA or
NIH Class IIIA: Inflammatory: Inflammatory cells in Neisseria gonorrhoeae, Burkholderia pseudomallei nodule that prompts biopsy
prostatic secretion, seminal fluid, postprostatic Miscellaneous: Chlamydia trachomatis
Gram-positive bacteria (Enterococcus, PHYSICAL EXAM
massage urine r Acute bacterial prostatitis (NIH I):
>10 WBC/HPF in EPS, 1st 10 mL of voided Streptococcus faecalis, Staphylococcus aureus)
r Organisms suspected, but unproven: Staphylococcus Suprapubic tenderness
urine after EPS, or semen Assess for acute urinary retention
NIH Class IIIB: Noninflammatory: Insignificant epidermidis, micrococci, nongroup D Streptococcus,
DRE: Hot, boggy, exquisite tenderness
inflammatory cells diphtheroids, Ureaplasma urealyticum,
Sepsis: Febrile, tachycardia
<10 WBC/HPF in EPS, 1st 10 mL voided urine Trichomonas vaginalis r Chronic bacterial prostatitis/CPPS (NIH II/IIIA/IIIB)
after EPS, or semen r Nonbacterial:
Suprapubic tenderness
NIH Class IV: Asymptomatic inflammatory Leading theory: Nonrelaxation of internal urinary
DRE: May be normal or soft/boggy, variable
prostatitis, incidental biopsy finding sphincter and pelvic floor muscles leading to
>10 WBC/HPF and/or bacteria in EPS, 1st amounts of pain, prostatic calculi
increased prostatic urethral pressure and
10 mL voided urine after EPS, semen, or intraprostatic urinary reflux
histologic specimens in asymptomatic patients r Uncommon: Mycobacterium tuberculosis, parasitic, ALERT
Do not perform massage or aggressive rectal exam
EPIDEMIOLOGY mycoses (blastomycosis, coccidioidomycosis,
Cryptococcus, histoplasmosis, candidiasis in the face of acute prostatitis or prostatic abscess.
Incidence paracoccidiomycosis)
r 2 million cases annually DIAGNOSTIC TESTS & INTERPRETATION
r 916% men have had diagnosis of prostatitis ASSOCIATED CONDITIONS Lab
r 312% male outpatient urology visits r Cystitis (secondary to bacterial prostatitis) r PSA may be elevated with prostatitis PSA should not
r Most common urologic diagnosis in men <50 yr, r Epididymitis
be checked in cases of acute bacterial prostatitis.
r Prostatic hypertrophy r Suspected acute bacterial prostatitis:
3rd most common >50 yr (2)
Overall incidence of acute prostatitis or prostatitis r STD Urinalysis, urine culture, CBC, blood culture
NOS 2.8/1,000 person-years (PY) over 70,166 PY r Urethritis/urethral stricture r Suspected CPB/CP/CPPS (NIH II/III)
of follow-up (3) r UTI Urinalysis, urine culture
3.2/1,000 PY in patients aged 2029 yr MearesStamey 4-glass test (gold standard)
3.6/1,000 PY in patients aged 3039 yr GENERAL PREVENTION 2-glass test more convenient: Pre/postprostatic
r Proper treatment of acute bacterial prostatitis may
5.4/1,000 PY in patients aged 7079 yr massage:
reduce chronic bacterial prostatitis (NIH II) Urine microscopy and culture of midstream urine
Prevalence r Safe sex practices
N/A specimen prior to prostate massage (Pre-M)
Urine microscopy and culture of 10-mL urine
RISK FACTORS postprostate massage (Post-M)
r Acute epididymitis With NIH II (chronic bacterial):
r Chronic catheterization (indwelling or condom) Pre-M: Urine WBC, culture
r Dysfunctional voiding Post-M: + Urine WBC, + culture
r Immunocompromised states NIH IIIA inflammatory CP/CPPS:
r Intraprostatic ductal reflux Pre-M: Urine WBC, culture
Post-M: + Urine WBC, culture
NIH IIIB noninflammatory CP/CPPS
Pre-M: Urine WBC, culture
Post-M: urine WBC, culture
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Brian Cox, MD
Christopher Amling, MD, FACS
EPIDEMIOLOGY Very rare, may suggest allergic etiology DIAGNOSTIC TESTS & INTERPRETATION
Incidence Associated with systemic condition (asthma,
Lab
N/A Wegener granulomatosis, ChurgStrauss r Urinalysis may be unremarkable
syndrome r Urine cultures
Prevalence r Autoimmune based:
r 0.81% of benign inflammatory prostatic specimens Are often sterile
r Reported up to 410% of prostatitis cases HLA-DR15linked T-cellmediated response r Elevated erythrocyte sedimentation rate (ESR), acid
against PSA
r Reported in 1.340% of post-Bacillus phosphatase, serum eosinophils may be present
CalmetteGuerin (post-BCG) patients ASSOCIATED CONDITIONS r PSA may rise transiently
r Prostate cancer can be coincident with r If evidence of TB/mycotic disease, appropriate
RISK FACTORS granulomatous prostatitis in 1014% of biopsy
r Age: Mean age 62 yr (range 1886 yr) (1) testing includes:
specimens (2) AFB stain of urine and semen
Typically 5070 yr of age r May be associated with systemic conditions
r Infections: TB cultures (may take up to 10 wk)
Asthma, Wegener granulomatosis, ChurgStrauss Polymerase chain reaction (PCR): Genomic
Bacterial, viral, fungal, parasitic, Mycobacterium, syndrome, sarcoidosis, rheumatoid arthritis, amplification of Mycobacterium Tuberculosis DNA
and sexually transmitted diseases (STDs) polyarteritis nodosa, malakoplakia High sensitivity/specificity
Human immunodeficiency virus (HIV) infection Rapid: Takes 48 hr
may increase risk for tuberculosis (TB) prostatitis GENERAL PREVENTION
Infectious etiologies make up 1520% of N/A Imaging
r TRUS
granulomatous prostatitis cases
r Iatrogenic causes: DIAGNOSIS Limited utility except to direct biopsy
Transurethral resection of prostate (TURP) Appears as focal hypoechoic area
BCG instillation for bladder cancerup to 40% HISTORY Difficult to discern granulomatous prostatitis and
r Often asymptomatic prostate cancer
may develop granulomatous prostatitis after BCG.
r Previous urinary tract infection (UTI) or STD: r MRI
Iatrogenic etiologies make up 75% of
granulomatous prostatitis cases Syphilis, TB, or other infectious etiology Limited utility
r Systemic granulomatous diseases: Often associated with UTI 23 m prior to onset of Difficult to discern granulomatous prostatitis from
Wegener granulomatosis, ChurgStrauss symptoms prostate cancer on MRI
syndrome, sarcoidosis, rheumatoid arthritis, r History of lower urinary tract symptoms (LUTS) Diagnostic Procedures/Surgery
polyarteritis nodosa, malakoplakia Voiding symptoms including urgency, frequency, TRUS-guided prostate biopsy is needed for pathologic
These make up a minority of granulomatous dysuria diagnosis
prostatitis cases Obstructive voiding symptoms, including acute
r Idiopathic: No specific cause identified Pathologic Findings
urinary retention r Histologically granulomatous prostatitis appears as
Theory: Ductal/acinar obstruction causes prostatic r Systemic granulomatous disease:
noncaseating granulomas, prominent macrophage
secretions to leak into the stroma and cause If associated with systemic vasculitis or infiltrates with occasional multinucleated giant cells
granulomatous reaction granulomatous disease, may have constitutional (Langerhan cells) which are characteristic of
Idiopathic etiologies make up a significant signs/symptoms granulomas
proportion of granulomatous prostatitis cases r History of prostate surgery or bladder cancer:
Immunohistochemistry for cytokeratin (CAM 5.2)
Genetics BCG or TURP can cause granulomatous prostatitis may stain glands positive but not the macrophage
r Fever, chills, or other constitutional signs:
N/A infiltrate
Suggest infectious, systemic etiology Macrophage infiltrate stains for macrophage
marker CD68
r Fibrosis replaces parenchyma
r BCG therapy related
Caseating or noncaseating granulomas located
next to benign prostatic glands (not engulfing
them)
Usually AFB negative
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PROSTATITIS, GRANULOMATOUS
DIFFERENTIAL DIAGNOSIS
r Nodular DRE (neoplasm/malignant):
SURGERY/OTHER PROCEDURES
r Majority of symptomatic cases of granulomatous
ADDITIONAL READING
r Humphrey PA. Prostate Pathology, ASCP Press:
Lymphoma, primary, and secondary prostatitis resolve spontaneously
Prostatic adenocarcinoma r Reserve TURP or prostatectomy for refractory cases Chicago. 2003;8795.
Sarcoma, small-cell carcinoma, and other rare r Warrick J, Humphrey PA. Nonspecific granulomatous
Reported in up to 10% of cases in some series
tumors and metastases prostatitis. J Urol. 2012;187:22092210.
Urothelial carcinoma ADDITIONAL TREATMENT
Radiation Therapy See Also (Topic, Algorithm, Media)
Granulomatous prostatitis r BCG Sepsis/BCGosis
Infectious, iatrogenic, etc. (See Risk Factors) N/A
r Prostate, Nodule
r Nodular DRE (benign): Additional Therapies r Prostatitis, General
Prostatic calculus/calcification Corticosteroids and antihistamines have been r Prostatitis, Granulomatous Image
Ejaculatory duct cyst recommended in idiopathic cases r Prostatitis, Tuberculosis
Scarring/fibrosis from prior surgery or infection
TURP, prostate biopsy Complementary & Alternative r Tuberculosis, Genitourinary, General Considerations
Therapies
Granulomatous prostatitis
r Rectal wall lesions (thrombosed hemorrhoid, N/A
carcinoma, etc.) CODES
ONGOING CARE
ICD9
TREATMENT PROGNOSIS r 135 Sarcoidosis
r Majority of symptomatic cases of granulomatous
r 446.4 Wegeners granulomatosis
GENERAL MEASURES prostatitis resolve spontaneously
r Majority of granulomatous prostatitis symptoms r DRE findings may persist for months/years r 601.8 Other specified inflammatory diseases of
resolve spontaneously including those that are BCG r PSA elevation may last up to 3 mo prostate
related (35)
r DRE changes and PSA elevation may persist COMPLICATIONS ICD10
r Acute urinary retention r D86.9 Sarcoidosis, unspecified
r Use antibiotics as indicated for UTI
r Possible transmission of infectious etiology to sexual r M31.30 Wegeners granulomatosis without renal
r Symptom control:
partner involvement
Sitz baths, fluids, anti-inflammatory -blockers, r N41.4 Granulomatous prostatitis
r Possible infertility
and other symptomatic medications
r Temporary transurethral urinary catheterization if r Possible undetected prostate cancer
acute urinary retention or severe symptoms are FOLLOW-UP CLINICAL/SURGICAL
present
r TRUS biopsy is indicated for: Patient Monitoring PEARLS
Rebiopsy may be indicated if DRE remains abnormal or r Majority of symptomatic cases of granulomatous
Differentiating granulomatous prostatitis from PSA remains elevated after treatment to avoid missing
prostate carcinoma coincident prostate cancer (reported in 1014% of prostatitis resolve spontaneously.
Consider rebiopsy if PSA remains elevated or DRE r Up to 40% of patients may develop granulomatous
cases)
remains abnormal several months after treating prostatitis after intravesical BCG.
symptomatic granulomatous prostatitis Patient Resources r DRE changes and PSA elevation may persist for
Prostatitis Foundation. http://www.prostatitis.org/
MEDICATION months.
r TRUS-guided prostate biopsy is needed for
First Line
r Antibiotics as indicated for documented UTI REFERENCES pathologic diagnosis.
r Anti-TB medications for TB prostatitis: r Rebiopsy may be indicated if DRE remains abnormal
1. Stillwell T, Engen DE, Farrow GM. The clinical
Use only if documented TB cause or PSA remains elevated after treatment to avoid
spectrum of granulomatous prostatitis: A report of
Isoniazid, rifampin, pyrazinamide, and either missing coincident prostate cancer (reported in
200 cases. J Urol. 1987;138:320323.
ethambutol or streptomycin for initial regimen, 1014% of cases).
2. Oppenheimer J, Kahane H, Epstein JI.
then change based on TB isolate sensitivities Granulomatous prostatitis on needle biopsy. Arch
Pyridoxine (Vitamin B6) 2550 mg/d to prevent Pathol Lab Med. 1997;121:724729.
isoniazid neuropathy
3. Uzoh CC, Uff JS, Okeke AA. Granulomatous
Second Line Prostatitis. BJU Int. 2007;99(3):510512.
N/A 4. Eyre RC, Aaronson AG, Weinstein BJ. Palisading
granulomas of the prostate associated with prior
prostatic surgery. J Urol. 1986;136:121122
5. Lafontaine PD, Middleman BR, Graham SD Jr, et al.
Incidence of granulomatous prostatitis and
acid-fast bacilli after intravesicular BCG therapy.
Urology. 1997;49:363366.
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PROTEINURIA
Anthony J. Tracey, MD, MPH
Raju Thomas, MD, MHA, FACS
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PROTEINURIA
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r Template transperineal PB SURGERY/OTHER PROCEDURES 3. Pinsky PF, Crawford ED, Kramer BS, et al. Repeat
May allow better sampling of peripheral zone r The number of cores on repeat biopsy is debatable. prostate biopsy in the Prostate, Lung, Colorectal
Controversial if better cancer detection NCCN guidelines suggest performing a 2nd and Ovarian cancer screening trial. BJU Int.
r MRI and ultrasound fusion targeted biopsy extended biopsy and consider saturation biopsies 2007;99:775779.
Stored MRI is fused with real-time ultrasound only in with high risk of cancer after multiple 4. Auprich M, Augustin H, Budaus L, et al. A
using a digital overlay negative biopsies comparative performance analysis of total
Series report 41% vs. 18% compared to r Transurethral resection prostate biopsy prostate-specific antigen, percentage free
conventional ultrasound in detection of CaP in Once advocated for diagnosis of transition zone prostate-specific antigen velocity and urinary
men with prior negative biopsies cancers prostate cancer gene 3 in the first, second, and
Requires specialized training and equipment Less than 5% CaP are transitional zone CaP third repeat biopsy. BJU Int. 2011;109:16271635.
Pathologic Findings without concomitant peripheral zone tumors 5. Jones JS. Managing patients following negative
r Quality assurance in the initial biopsy is critical. Data Improved TRUS technique in sampling transitional prostate biopsy. Renal & Urology News.
suggests a core length of >10 mm and the presence zone; no definite value in performing transurethral 2011:10:23.
of glandular elements suggest an adequate sample resection PB
r High-grade prostatic intraepithelial neoplasia ADDITIONAL TREATMENT
(HGPIN) 024.6% on initial biopsy (median 4%) Radiation Therapy
ADDITIONAL READING
Considered by some to be premalignant lesion: N/A r Elshafei A, Li YH, Hatem A, et al. The utility of PSA
2335% risk of diagnosis cancer on subsequent velocity in prediction of prostate cancer and high
biopsy; however, EAU does not recommend repeat Additional Therapies
r The European Randomized Study of Screening for grade cancer after initially negative prostate biopsy.
biopsy with HGPIN Prostate. 2013;73(16):17961802.
r Atypical small acinar proliferation (ASAP) PCa (ERSPC)-based model has several calculators to
determine outcome after negative biopsy r Levy DA, Jones JS. Management of rising
Incidence 0.723.4%, median 4.4% prostate-specific antigen after a negative biopsy.
(http://www.prostatecancer-riskcalculator.com/)
Increased CaP risk on subsequent biopsy (up to r Genomic testing may help determine risk after Curr Urol Rep. 2011;12(3):197202.
40%) r Presti JC. Management of patients with persistently
negative biopsy
DIFFERENTIAL DIAGNOSIS Confirm MDxTM : Epigenetic assay to distinguish elevated PSA level and negative biopsy. AUA Update
r ASAP men who have a true-negative biopsy from those Series. 2012; Lesson 1, Volume 31.
r BPH with occult cancer r Scott JG, John G, Eric K, et al. Emotional
r HGPIN Identifies methylation signature in area near PCa consequences of persistently elevated PSA with
r Prostatitis location using recent prostate biopsy material negative prostate biopsy. Am J Cancer Prevention.
Complementary & Alternative 2013;1(1);48.
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Prevalence
BASICS US CaP approximately 2,106,499 men, or 1.5% all DIAGNOSIS
ages and races
DESCRIPTION HISTORY
r PSA is used for diagnosis and treatment of prostate RISK FACTORS r Difficulty with urination, such as hesitancy, straining,
r For elevated PSA
cancer (CaP). This section reviews the use of PSA in weak stream, or intermittency
the diagnosis of CaP recognizing that screening is Advancing age r Dysuria, frequency, or urgency
an area of controversy Benign prostatic hypertrophy (BPH) r Previous PSA levels or prostate biopsies
r CaP is only diagnosed through tissue biopsy and not CaP r Family history of prostate carcinoma
Infection, infarction r Medications, including herbals
by PSA alone
r Normal PSA level is controversial, and can be Recent instrumentation (TURP, cystoscopy,
r Markedly elevated PSA >20 ng/mL with bone, back,
catheterization, prostate biopsy)
elevated due to malignant or benign causes. r CaP (See chapter Prostate Cancer, General) or hip pain suggests metastatic CaP
Elevated PSA traditionally >4.0 ng/mL based on
the Baltimore Longitudinal Study of Aging Genetics PHYSICAL EXAM
r PSA associated with kallikrein genes family (long r DRE: Nodules, induration, asymmetry, bogginess,
Specificity 91%, sensitivity 21% (51% for
Gleason 8), PPV 30% arm of chromosome 19 region q13.2q13.4). tenderness (Note: American Cancer Society
Elevated PSA >2.5 ng/mL has support r PSA is also called human kallikrein 3 (hKLK3) recommends PSA screening with or without DRE)
r Age/race-specific proposed, but controversial: r Adenopathy, supraclavicular
PATHOPHYSIOLOGY r Bony pain, point tenderness with metastasis
r PSA: A serine protease produced by the prostatic
r Neurologic: Lower extremity strength/sensation
Range (yr) Asian Black White epithelium and periurethral glands that liquefies
4049 02.0 02.0 02.5 seminal coagulum DIAGNOSTIC TESTS & INTERPRETATION
r Seminal fluid has high PSA concentrations (mg/mL); Lab
5059 03.0 04.0 03.5 r Routine UA to rule out UTI/prostatitis (13)
PSA is much lower (ng/mL) in serum
6069 04.0 04.5 04.5 r Many forms of serum PSA: Free PSAs (nicked, intact, r Consider evaluation for prostatitis by modified
7079 05.0 05.5 06.5 several forms of proPSA) and complexed PSA StameyMeares test or exam of EPS (See Section I:
r Based on Prostate Cancer Prevention Trial (biopsy (bound to protease inhibitors 1-antichymotrypsin Prostatitis, Chronic, Bacterial [NIH II])
[ACT], 2-macroglobulin [MG], 1-protease r Consider %FPSA (FPSA/TPSA or F/T PSA)
regardless of PSA), can have CaP with low PSA.
inhibitors [API]); bound PSA forms stable complex With CaP lower FPSA; postulated that CaP
No lower cutoff or normal PSA to indicate absence
(no serum enzymatic activity) (2) produces more ACT
of cancer. PCPT data:
6090% complexed to ACT; free portion is also FPSA best with TPSA 4.010.0 ng/mL and
detected by assay, while that bound to MG is not prostates <50 g; not useful if TPSA >10 ng/mL
PSA CaP Rate PSA CaP Rate
detected by routine assay FPSA stratifies CaP risk on biopsy (table) (3)
00.5 6.6% 3.14.0 26.9 Complexed PSA: Hepatic clearance (1/2-life
0.61.0 10.1% 4.010.0 25% 2.2 days); FPSA cleared by glomerular filtration PSA ng/mL CaP Rate %FPSA CaP Prob
(1/2-life 23 hr)
1.12.0 17% >10.0 >50% r CaP PSA elevation is due to disrupted prostatic 02 1% 010 56%
2.13.0 23.9% architecture and compromised integrity of the basal 24 15% 1015 28%
r The challenge: Lower normal PSA to recommend layer or basement membrane 410 25% 1520 20%
CaP makes less PSA/g than benign tissue >10 >50% 2025 16%
biopsy where life-threatening cancer is present, but r Androgens influence PSA levels
not to point where overdetection of incidental r Sources of fluctuation in PSA: >25 8%
(autopsy/insignificant CaP) occurs
r PSA derivatives may overcome problem, but not No PSA analytic standard; can vary by lab and use r Consider PSAD: PSA TRUS volume:
same lab to compare serial values
absolute: Used in PSA range of 4.010 ng/mL: PSA Correlates PSA to TRUS prostatic size to
15% coefficient of variation in PSA assay
density (PSAD), PSA velocity (PSAV), newer PSA distinguish BPH from CaP:
Physiologic variation in PSA 1530% in the short
assays (free, molecular forms)
r PSA changes over time more useful than a single term; BPH can vary up to 30% PSA ng/mL
2637% with elevated PSA return to normal 1 yr PSAD =
PSA in screening for CaP Prostate Volume (cc)
r A single PSA of >1.3 ng/mL before age 50 predicts later, and 4555% normal within 4 yr
Seasonal variation: PSA is higher in summer Useful with PSA 410 ng/mL and a previous
increased lifetime CaP risk (1) Infection, infarction, trauma, ejaculation within negative biopsy
r PSA >10: More risk of advanced disease. Cutoff of 0.15 ng/mL/cm3 improves specificity by
24 hr, or prostate instrumentation or massage can
r PSA proportional to prostate volume; prostate produce elevations (not routine DRE) 50%, missed 2748% CaP
volume/mean PSA were as follows: 14 cm3 /1; Finasteride (5 mg BPH; 1 mg alopecia) and Cutoff 0.1 avoids 31% of biopsies, misses 10%
25 cm3 /1.13/52 cm3 1.45 in 1 study dutasteride are 5-reductase inhibitors; lower cancers; cutoff of 0.8 avoids 12% of biopsies,
PSA by 50% over 6 mo; correct PSA by misses 5% of cancers
ALERT doubling to maintain PSA utility r Consider PSAV:
PSA should not be done with acute prostatitis or Rate of PSA increase; PSA rises more rapidly if
ASSOCIATED CONDITIONS clinically significant CaP present:
within 34 wk of prostate instrumentation: r BPH
false-positive risk. r Acute and chronic bacterial prostatitis Minimum 18-mo interval with 3 repeat PSAs for
most accurate PSAV determination.
r Urinary retention
EPIDEMIOLOGY
PSA2 PSA1 PSA3 PSA2
Incidence GENERAL PREVENTION PSAV = 0.5 +
r Across all races, age >50, only 7.9% of men r None for CaP Time1 Time2
randomly screened have PSA >4.0 ng/mL r Avoid PSA measurement when false-positive PSA1 = 1st PSA (ng/mL)
r Median PSA: 4th decade, 0.7 ng/mL; 5th decade, elevation likely (See Risk Factors above) PSA2 = 2nd PSA (ng/mL)
0.9 ng/mL 6th decade, 1.3 ng/mL; 7th decade, r Use same lab/assay for serial measurements PSA3 = 3rd PSA (ng/mL)
1.7 ng/mL Time1 = time between PSA1 & PSA2 (yr)
Time2 = time between PSA2 & PSA3 (yr)
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Baltimore Longitudinal Study: 72% CaP had PSA r Numerous assays under study to help differentiate REFERENCES
rise >0.75 ng/mL/yr vs.10% with BPH benign form malignant PA elevation (See Section II:
PSAV > 0.35 predicts PCa death w/ PSA <4 PSA, General Considerations.) 1. Lilja H, Cronin AM, Dahlin A, et al. Prediction of
PSAV >0.75 90100% PCa sensitivity w/ PSA >4 significant prostate cancer diagnosed 20 to
MEDICATION 30 years later with a single measure of prostate-
PSA velocity >02 in year before dx predicts PCa
mortality First Line specific antigen at or before age 50. Cancer.
r Prostate Health Index or phi assay r Empiric antibiotics for elevated PSA is no longer
2011;117(6):12101219.
CaP low FPSA, increased % proPSA recommended by most sources 2. Shariat SF, Semjonow A, Lilja H, et al. Tumor
r With bacterial prostatitis, treat and repeat PSA 4 wk
Calculation ([2]proPSA/FPSA) TPSA markers in prostate cancer I: Blood-based markers.
phi 2755, CaP 9.850%, Gl 7 3.928.9%; phi after: Fluoroquinolone (eg, ciprofloxacin 500 mg Acta Oncol. 2011;50(suppl 1):6175.
27: 18.8% could be spared biopsy (low risk) BID.) or TMP-SMX (180/800 mg BID) 3. Catalona WJ, Beiser JA, Smith DS. Serum free
Imaging Second Line prostate specific antigen and prostate specific
r TRUS: Determine prostatic size; PSAD; most useful N/A antigen density measurements for predicting cancer
to guide systematic needle biopsy in men with prior negative biopsies. J Urol.
SURGERY/OTHER PROCEDURES 1997;158:21622167.
r Multiparametric MRI with/without endorectal coil: If patient has anorectal pathology, consider
Useful if CaP suspicion and negative biopsy. 4. Carter HB, Albertsen PC, Barry MJ, et al. Early
transperineal prostate biopsy for CaP diagnosis
Anterior tumors and other sites can be identified detection of prostate cancer: AUA Guideline. J Urol.
r CT or bone scan: No role in CaP screening ADDITIONAL TREATMENT 2013;190(2):419426.
r CaP risk calculators are available on the Internet to
5. Greene KL, Albertsen PC, Babaian RJ, et al.
Diagnostic Procedures/Surgery predict outcome of biopsy. Prostate specific antigen best practice statement:
r TRUS-guided prostate biopsy with 18G biopsy r PCA3 urine testing after attentive DRE; FDA 2009 update. J Urol. 2013;189(1 suppl):S2S11.
needle and local anesthesia: approved only after initial negative biopsy;
Systematic biopsy (12 cores) with laterally directed PCA3/TMPRSS2-ERG urine test investigational
samples is now standard for CaP.
Additional Therapies ADDITIONAL READING
Pathologic Findings Any PSA rise while on finasteride/dutasteride baseline
See Section I: Prostate Cancer, General. Heidenreich A, Abrahamsson PA, Artibani W, et al.
raises CaP risk Early detection of prostate cancer: European
DIFFERENTIAL DIAGNOSIS Complementary & Alternative Association of Urology recommendation. Eur Urol.
r Adenocarcinoma of the prostate (CaP) Therapies 2013;64(3):347354.
r BPH No evidence for herbals effect on PSA
r Prostatitis (usually bacterial infection) See Also (Topic, Algorithm, Media)
r Prostate Cancer Screening Guidelines
r Prostatic infarction: Idiopathic or after shock r Prostate Cancer, Biochemical Recurrence (Elevated
r Iatrogenic: Recent cystourethroscopy, Foley catheter
ONGOING CARE
PSA) Following Cryotherapy
placement, prostate biopsy PROGNOSIS r Prostate Cancer, Biochemical Recurrence (Elevated
r Prostatic massage (but not routine DRE) r With elevated PSA, positive biopsy rate is about
PSA) Following Radiation Therapy
r Trauma (cycling, extensive) 2530%; elevated PSA and nodule 1860% r Prostate Cancer, Biochemical Recurrence (Elevated
r Ejaculation within 24 hr of PSA test (rare) r Overall, if 2nd biopsy is performed after initial
PSA) Following Radical Prostatectomy
negative, detection rate is 1035%. r Prostate Cancer, General
COMPLICATIONS r PSA Elevation Following Negative Prostate Biopsy
TREATMENT Failure to diagnose cancer; patient anxiety over repeat r PSA, General Considerations
GENERAL MEASURES testing; risk of biopsy and drugs
r Shared decision making before PSA based CaP
FOLLOW-UP
screening in asymptomatic patients Patient Monitoring CODES
r Due to PSA fluctuations, confirm an elevated PSA r There is no single threshold PSA which should
with a 2nd reading before biopsy. Patient should not prompt prostate biopsy. Biopsy decision based on ICD9
ejaculate for 48 hr before test. PSA, DRE, and multiple factors (F/T PSA, age, PSA r 185 Malignant neoplasm of prostate
r Review serial PSA determinations for PSAV velocity, PSA density, family history, ethnicity, prior r 600.00 Hypertrophy (benign) of prostate without
r CaP screening recommendations: biopsy history, comorbidities, patient preferences) urinary obstruction and other lower urinary tract
See Appendix for ACS, ACP, EAU, NCCN, USPSTF (5). symptom (LUTS)
r AUA 2013 CaP Early Detection Guideline (4): r PSA <2.5 ng/mL, low PSAV: Annual DRE/PSA r 790.93 Elevated prostate specific antigen [PSA]
No PSA screening in men under age 40 yr r PSA 2.610 ng/mL, low PSAV:
Does not recommend routine screening between Consider biopsy or obtain FPSA ICD10
r C61 Malignant neoplasm of prostate
ages 40 and 54 yr at average risk F/T PSA >25%: Repeat PSA/DRE in 6 mo r N40.0 Enlarged prostate without lower urinary tract
Shared decision-making for men 5569 yr r Based on TRUS biopsy results:
considering PSA screening due to risks/benefits; Negative: Repeat DRE/PSA in 6 mo; consider F/T symptoms
proceed based on a mans values and preferences r R97.2 Elevated prostate specific antigen [PSA]
PSA as guide for another biopsy
To reduce harms, screening intervals of 2 yr HGPIN or ASAP:
preserves the benefits and reduces overdiagnosis Repeat biopsy (36 mo); consider transition
and false-positives zone sampling with any repeat biopsy.
CLINICAL/SURGICAL
No routine PSA screening in men age 70+ yr or Positive biopsy (CaP): Staging studies, discuss PEARLS
<1015-yr life expectancy; but some 70+ yr in treatment options r Routine DRE will not clinically significantly elevate
excellent health may benefit from screening r Persistent PSA elevation (PSA >10 ng/mL)
r Some published prostate biopsy indications: PSA.
Repeat biopsy; transition zone sampling; r PSA 1/2-life is 2.2 days; may remain elevated for up
Prostate nodule, regardless of PSA multiparametric MRI of TRUS/MRI fusion biopsy
PSA >10 ng/mL in the absence of prostatitis to 4 wk after instrumentation.
Patient Resources
PSA >4.0 ng/mL and PSAV >0.75 ng/mL/yr
PSA <4.0 ng/mL and PSAV >0.30.5 ng/mL/yr AUA Urology Care Foundation. http://www. P
PSA >2.5 ng/mL and PSAV >0.60 ng/mL/yr urologyhealth.org/urology/index.cfm?article=68
PSA 410 and F/T PSA<10%
F/T PSA <20% and PSAV >0.75 ng/mL/yr
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REFERENCES
DIAGNOSIS TREATMENT
1. Hughes IA, Houk C, Ahmed SF, et al. Consensus
HISTORY GENERAL MEASURES statement on management of intersex disorders.
r Family history: r Multidisciplinary team for evaluation of the patient Arch Dis Child. 2006;91:554563.
Genital abnormalities r Gender assignment early 2. Ahmed SF, Rodie M. Investigation and initial
Amenorrhea management of ambiguous genitalia. Best Pract
Sterility MEDICATION Res Clin Endocrinol Metab. 2010;24:197218.
Hirsutism First Line 3. Romao RL, Salle JL, Wherrett DK. Update on the
Early infant deaths (possible unrecognized CAH r Salt-wasting CAH
management of disorders of sex development.
with salt-wasting crisis) Fluid and electrolytes replacement Pediatr Clin North Am. 2012;59:853869.
r Maternal exposure to androgens Glucocorticoid and mineralocorticoid replacement
r History of maternal virilization (androgen-producing Hydrocortisone 10 mg/m2 /d
Fludrocortisone 0.10.2 mg/d
tumor)
Oral sodium chloride, 12 g/d added to formula or
ADDITIONAL READING
PHYSICAL EXAM breast milk r Auchus RJ, Chang AY. 46,XX DSD: The masculinized
r External genitalia
Second Line female. Best Pract Res Clin Endocrinol Metab.
Phallic structure (length, breadth, and amount of
N/A 2010;24:219242.
erectile tissue) r Barbaro M, Wedell A, Nordenstrom A. Disorders of
Position of urethral meatus SURGERY/OTHER PROCEDURES
Number of orifices in the perineum and their r Masculinizing genitoplasty (between the ages of 6 sex development. Semin Fetal Neonatal Med.
characteristics 2011;16:119127.
and 18 mo) (See Disorders of Sex Development r Barthold JS. Disorders of sex differentiation: A
Labioscrotal folds (separated or fused) chapter)
r Gonads pediatric urologists perspective of new terminology
r Feminizing genitoplasty (during the 1st 6 mo of and recommendations. J Urol. 2011;185:393400
Palpable gonads (testis, very rarely ovotestis) r http://www.ncbi.nlm.nih.gov/omim/ (Online
r Abdomen life) (3)[A] (See Disorders of Sex Development
Mass referable to enlarged uterus
chapter) Mendelian Inheritance in Man )
r Mullerian remnants (See Disorders of Sex
DIAGNOSTIC TESTS & INTERPRETATION See Also (Topic, Algorithm, Media)
Development chapter) r Congenital Adrenal Hyperplasia
Lab r Disorders of Sexual Development (DSD)
r Karyotype ADDITIONAL TREATMENT
r Hypospadias
r Serum levels of sodium, potassium, and Radiation Therapy
N/A r Micropenis (Microphallus)
17-hydroxyprogesterone r Mullerian Duct Remnants and Persistent Mullerian
r Androgens (testosterone, dihydrotesterone, Additional Therapies
androstenedione) N/A Duct Syndrome (PMDS)
r Cortisol, gonadotrophins, and AMH levels
Complementary & Alternative
r Stimulation test with human chorionic gonadotropin
Therapies CODES
(suspected defect of androgen production) Actually some patient groups strongly advocate to
Imaging delay any surgical procedures until patients are
r Abdominal/pelvic ultrasound (utero presence) competent to provide informed consent ICD9
r Cystogram/genitogram (visualization of vagina, 752.7 Indeterminate sex and pseudohermaphroditism
sinus) ICD10
r MRI ONGOING CARE r Q56.1 Male pseudohermaphroditism, not elsewhere
PROGNOSIS classified
Diagnostic Procedures/Surgery r Generally good with appropriate care r Q56.2 Female pseudohermaphroditism, not
r Laparoscopy to define internal anatomy
r Cysto/vaginoscopy to confirm anatomy and level of r Many patients have a good quality of life elsewhere classified
r Many patients remain fertile r Q56.3 Pseudohermaphroditism, unspecified
confluence of urogenital sinus
r Gonadal biopsy to analyze presence of ovarian
COMPLICATIONS
and/or testicular tissue See Disorders of Sex Development chapter CLINICAL/SURGICAL
r Skin biopsy to obtain cellular lines
FOLLOW-UP PEARLS
Pathologic Findings Patient Monitoring r Infants with a DSD presenting with truly ambiguous
See Disorders of Sex Development chapter r Lifelong psychosocial support mandatory
genitalia are a rare occurrence.
DIFFERENTIAL DIAGNOSIS r Monitoring for increased risk for developing r DSD should be regarded as a heterogeneous group
r Cryptorchidism malignancies of conditions with substantially different prognoses
r Inguinal hernia, hydrocele r Evaluation of sexual function
and treatment prospects.
r Hypospadias r DSDs represent a broad complex field that requires
Patient Resources
r Microphallus r http://www.congenitaladrenalhyperplasia.org the interaction of multiple disciplines with a diverse
r Gonadoblastoma r http://www.livingwithcah.com knowledge base.
r Menstruation disorders r http://rch.org.au/cah book/index.cfm?doc id=1375
r http://www.ahn.org.uk
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r Oral culture-directed antibiotics once clinically FOLLOW-UP See Also (Topic, Algorithm, Media)
improving and tolerating oral intake r Pyelonephritis, Acute, Pediatric Image
Patient Monitoring
Avoid nitrofurantoin due to minimal tissue r Current American Academy of Pediatrics guidelines r Pyonephrosis
penetration recommends RUS in children with febrile UTI r Urinary Tract Infection (UTI), Complicated, Pediatric
Parenteral antibiotics (daily ceftriaxone, IM) also Selective VCUG in patients with abnormal RUS or r Urinary Tract Infection (UTI), Pediatric
an option for outpatient therapy recurrent episodes (2)[C] r Vesicoureteral Reflux, Pediatric
r 714 days total duration of therapy (2)[B] Indications for radiographic imaging in children
with 1st episode of febrile UTI remain controversial
Second Line r Delayed DMSA scan to detect renal scarring
r Vancomycin if penicillin allergic CODES
r Aztreonam an alternate to aminoglycoside if renal Patient Resources
r National Kidney and Urologic Diseases Information ICD9
insufficiency r 041.49 Other and unspecified Escherichia coli [E.
Clearinghouse (NKUDIC)
SURGERY/OTHER PROCEDURES http://kidney.niddk.nih.gov/kudiseases/pubs/ coli]
r Urethral catheter if critically ill or poor bladder pyelonephritis/index.aspx r 590.10 Acute pyelonephritis without lesion of renal
emptying http://kidney.niddk.nih.gov/kudiseases/pubs/ medullary necrosis
r Surgery generally not indicated in acute treatment utichildren/ r 593.73 Other vesicoureteral reflux with reflux
r Ureteral stent or nephrostomy tube if obstruction
nephropathy NOS
r Percutaneous aspiration/drainage if progression to
renal abscess REFERENCES ICD10
r B96.20 Unsp Escherichia coli as the cause of
ADDITIONAL TREATMENT 1. Montini G, Tullus K, Hewitt I. Febrile urinary tract
diseases classd elswhr
Radiation Therapy infections in children. N Engl J Med. 2011; r N10 Acute tubulo-interstitial nephritis
N/A 365(3):239250. r N13.729 Vesicoureter-reflux w reflux nephropathy
2. Roberts KB. Urinary tract infection: Clinical practice
Additional Therapies guidelines for the diagnosis and management of w/o hydrourt, unsp
N/A the initial UTI in febrile infants and children 2 to
Complementary & Alternative 24 months. Pediatrics. 2011;128:595609. CLINICAL/SURGICAL
Therapies 3. Rushton HG, Majd M. Dimercaptosuccinic acid
Probiotics (experimental) renal scintigraphy for the evaluation of
PEARLS
pyelonephritis and scarring: A review of r Signs and symptoms are often nonspecific in infants
experimental and clinical studies. J Urol. and young children with pyelonephritis.
ONGOING CARE 1992;148(5 pt 2):17261732. r Culture of appropriately collected urine specimen
PROGNOSIS mandatory in patients with suspected UTI and in
Related to degree of renal injury from pyelonephritic infants with fever and no obvious source.
scarring ADDITIONAL READING r Acute imaging (RUS) recommended if critically ill or
r Juliano TM, Stephany HA, Clayton DB, et al. failure to respond to treatment.
COMPLICATIONS
r Pyelonephritic scarring, especially with recurrent Incidence of abnormal imaging and recurrent
episodes and delayed treatment pyelonephritis after first febrile urinary tract infection
r Pyonephrosis in children 2 to 24 months. J Urol. 2013;
r Renal abscess 190(4 suppl):15051510. doi: 10.1016/
r Xanthogranulomatous pyelonephritis j.juro.2013.01.049.
r Shaikh N, Ewing AL, Bhatnagar S, et al. Risk of renal
r Hypertension
scarring in children with a first urinary tract
infection: A systematic review. Pediatrics.
2010;126:10841091.
r Shortliffe LD. Infection and inflammation of the
pediatric genitourinary tract. In: Wein AJ,
Kavoussi LR, Novick AC, et al. Campbell-Walsh
Urology. 10th ed. Philadelphia, PA: Elsevier
Saunders; 2012.
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PYELONEPHRITIS, CHRONIC
Debra L. Fromer, MD
Drew A. Freilich, MD
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PYELONEPHRITIS, CHRONIC
DIFFERENTIAL DIAGNOSIS 3. Rivera JA, OHare AM, Harper GM. Update on the
r Analgesic nephropathy ONGOING CARE management of chronic kidney disease. Am Fam
r Diabetic nephropathy Physician. 2012;86(8):749754.
r Gouty nephritis PROGNOSIS 4. Nicolle LE, Bradley S, Colgan R, et al. Infectious
r 24-hr protein excretion may be an important
r Hypertensive renal disease Diseases Society of America guidelines for the
r Psoas and subdiaphragmatic abscess prognostic indicator of progressive deterioration of diagnosis and treatment of asymptomatic
r Renal artery stenosis renal function due to focal and segmental bacteriuria in adults. Clin Infect Dis. 2005;40(5):
glomerulosclerosis superimposed on 643654.
r Renal malakoplakia
tubulointerstitial disease 5. Dai B, Liu Y, Jia J, et al. Long-term antibiotics for
r Renal tuberculosis r Radionuclide renal scan can assess renal function
r Urolithiasis the prevention of recurrent urinary tract infection in
and scarring children: A systematic review and meta-analysis.
r Xanthogranulomatous pyelonephritis (XGP) (2)[B] COMPLICATIONS Arch Dis Child. 2010;95(7):499508.
r Emphysematous pyelonephritis
r End-stage renal disease (rare)
TREATMENT r Focal segmental glomerulosclerosis ADDITIONAL READING
r HTN
GENERAL MEASURES N/A
r Chronic pyelonephritis is difficult to manage as it is r Perinephric abscess: Requires surgical drainage
an irreversible process r Polyuria, nocturia from loss of tubular concentrating See Also (Topic, Algorithm, Media)
r Pyelonephritis, Acute
r With mild VUR, suppressive antibiotics are used ability r Pyelonephritis, Chronic Image
until resolution or puberty in children r Pregnancy-related miscarriages in women with
r Severe reflux may require reimplantation r Pyelonephritis, Emphysematous
chronic reflux r Pyelonephritis, Xanthogranulomatous
r Correct anatomic anomalies or stones if possible r Proteinuria
r Pyonephrosis r Vesicoureteral Reflux, Pediatric
MEDICATION r XGP
First Line
r Acute episodes of pyelonephritis should be treated FOLLOW-UP CODES
(See Section I: Pyelonephritis, Acute) (3)[B] Patient Monitoring
r Annual serum creatinine to monitor chronic kidney ICD9
r Suppressive antibiotics VUR in children has become
disease 590.00 Chronic pyelonephritis without lesion of renal
controversial: r Blood pressure monitoring (good control of BP may medullary necrosis
In children <36 mo, use low-dose amoxicillin or
limit renal damage over time)
cephalexin, cefazolin, or other 1st-generation ICD10
15% of patients with reflux nephropathy who r N11.1 Chronic obstructive pyelonephritis
cephalosporin can be considered
reach adulthood have HTN r N11.8 Other chronic tubulo-interstitial nephritis
In children >6 mo, switch to nitrofurantoin,
Some advocate screening renal scan or VCUG of r N11.9 Chronic tubulo-interstitial nephritis,
trimethoprim-sulfamethoxazole, or trimethoprim
siblings who have reflux
alone can be considered unspecified
r Hypertension is best treated by ACE inhibitors r Urine analysis to monitor for proteinuria and
(lisinopril, enalapril, ramipril) that may also protect bacteruria (4)[A]
the kidney from progressive renal failure r Selective long-term antibiotics to limit infection CLINICAL/SURGICAL
ACE inhibitors are contraindicated in pregnancy (5)[A] PEARLS
Second Line Patient Resources For best determination of renal function the bladder
Based upon urine culture sensitivities, prior treatment National Kidney and Urologic Diseases Information
should be empty and kidneys unobstructed (ie,
attempts and patient presenting symptoms Clearinghouse (NIH). http://kidney.niddk.nih.gov/
ureteral stent if large stones).
SURGERY/OTHER PROCEDURES kudiseases/pubs/pyelonephritis/
r Correction of reflux may be necessary in children
with high-grade reflux (Grade 45). Low-grade REFERENCES
reflux (Grade 13) often resolves with time
r Nephrectomy for persistent/recurrent infection 1. Peters C, Rushton HG. Vesicoureteral reflux
unresponsive to systemic treatment, markedly associated renal damage: Congenital reflux
decreased function (ie, 10%), pain, or refractory nephropathy and acquired renal scarring. J Urol.
HTN, XGP 2010;184(1):265273.
2. Guzzo TJ, Bivalacqua TJ, Pierorazio PM, et al.
ADDITIONAL TREATMENT Xanthogranulomatous pyelonephritis: Presentation
Radiation Therapy and management in the era of laparoscopy. BJU
N/A Int. 2009;104(9):12651268.
Additional Therapies
N/A
Complementary & Alternative
Therapies
Little data to support
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PYELONEPHRITIS, EMPHYSEMATOUS
Jennifer E. Heckman, MD, MPH
Stephen Y. Nakada, MD, FACS
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PYELONEPHRITIS, EMPHYSEMATOUS
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PYELONEPHRITIS, XANTHOGRANULOMATOUS
Demetrius H. Bagley, MD, FACS
Kelly A. Healy, MD
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PYELONEPHRITIS, XANTHOGRANULOMATOUS
SURGERY/OTHER PROCEDURES
r Nephrectomy is the most common treatment
FOLLOW-UP ADDITIONAL READING
Patient Monitoring
Diffuse inflammatory process r Urinalysis and urine culture Goyal S, Gupta M, Goyal R. Xanthogranulomatous
Nonfunctioning kidney r Serum creatinine, CBC, liver enzymes repeated to pyelonephritis: A rare entity. N Am J Med Sci.
Concern for malignancy follow for normalization 2011;3(5):249250.
Inflammatory reaction, nephrectomy can be r Further radiographic studies depending upon the See Also (Topic, Algorithm, Media)
technically difficult histopathology of the kidney specimen r Pyelonephritis, Acute, Adult
r Partial nephrectomy in rare cases of segmental XGP r Pyelonephritis, Acute, Pediatric
r Mechanical and antibiotic bowel prep is performed Patient Resources
r Pyelonephritis, Xanthogranulomatous Image
since XGP may involve any adjacent organs or N/A
r Renal Mass
tissues r Urinary Tract Infection (UTI), Complicated, Adult
Drains should be placed in renal bed REFERENCES r Urolithiasis, Renal
r Laparoscopic nephrectomy has been shown to be
safe without increasing complications but, again 1. Kim SW, Yoon BI, Ha US, et al.
difficult (6) Xanthogranulomatous pyelonephritis. Clinical
experience with 21 cases. J Infect Chemother. CODES
ADDITIONAL TREATMENT 2013;19(6):12211224.
Radiation Therapy 2. Korkes F, Favoretto RL, Broglio M, et al. ICD9
N/A Xanthogranulomatous pyelonephritis. Clinical r 041.6 Proteus (mirabilis) (morganii) infection in
Additional Therapies experience with 41 cases. Urology. 2008;71: conditions classified elsewhere and of unspecified
Percutaneous drainage with antibiotics (7) 178180. site
3. Dwivedi US, Goyal NK, Saxena V, et al. r 590.00 Chronic pyelonephritis without lesion of
Complementary & Alternative
Xanthogranulomatous pyelonephritis: Our renal medullary necrosis
Therapies r 599.60 Urinary obstruction, unspecified
experience with review of published reports. ANZ J
N/A
Surg. 2006;76:10071009.
4. Loffroy R, Guiu B, Watfa J, et al. ICD10
r B96.4 Proteus (mirabilis) (morganii) causing dis
ONGOING CARE Xanthogranulomatous pyelonephritis in adults:
Clinical and radiological findings in diffuse and classd elswhr
PROGNOSIS r N11.8 Other chronic tubulo-interstitial nephritis
r Preservation of renal function related to the function focal forms. Clin Radiol. 2007;52:884890.
r N13.9 Obstructive and reflux uropathy, unspecified
5. Zorzos I, Moutzouris V, Korakianitis G, et al.
of the contralateral kidney
r Recurrence in the contralateral kidney is very rare Analysis of 39 cases of xanthogranulomatous
pyelonephritis with emphasis on CT findings. Scand
r Chance of recurrent stones is high
J Urol Nephrol. 2003;37:342347. CLINICAL/SURGICAL
COMPLICATIONS 6. Shah KJ, Ganpule AP, Kurien A, et al. Laparoscopic PEARLS
r Postoperative respiratory complications vs. open nephrectomy for xanthogranulomatous r Be suspicious in patients with fever, flank pain, and
r Wound infection pyelonephritis: An outcome analysis. Indian J Urol.
weight loss.
r An injury to adjacent organs can occur during 2011;27:470474. r Persistent UTI with adequate treatment is a warning.
nephrectomy 7. Ergun T, Akin A, Lakadamyali H. Stage III r CT scan for diagnosis and extent of disease.
r Major vascular injury related to the inflammatory xanthogranulomatous pyelonephritis treated with
r XGP is primarily a surgically managed disease
process antibiotic therapy and percutaneous drainage.
r Fistulas or abscesses postoperatively require JBR-BTR. 2011;94(4):209211. usually by nephrectomy with antibiotics critical to
the management of this condition.
drainage and antibiotic therapy r Usually unilateral and frequently confused clinically
r Renal insufficiency related to the function of the
and radiographically with renal cell carcinoma.
contralateral kidney
r Recurrent stone formation
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PYONEPHROSIS
Anthony J. Tracey, MD, MPH
Raju Thomas, MD, MHA, FACS
r Infectious agents (in decreasing order of incidence) DIAGNOSTIC TESTS & INTERPRETATION
BASICS (1) Lab
Escherichia coli r Pyuria
DESCRIPTION Enterococcus species r Elevated white count (less specific)
r Pyonephrosis is a collection of purulent material in Candida species and other fungal infections r Bacteriuria (less specific)
the renal collection system Enterobacter species r Urine culture of obstructed system
r Typically resulting from an underlying obstruction Klebsiella species r Elevated C-reactive protein
within the upper urinary tract (2) Proteus species
With concomitant urinary tract infection Pseudomonas species 1 study showed CRP levels >28 mg/L with flank
r Considered a surgical emergency with drainage of Bacteroides species pain a reliable indication for emergent
Staphylococcus species decompression (1)
obstructed collecting system necessary (1)
Methicillin-resistant Staphylococcus aureus Imaging
EPIDEMIOLOGY (MRSA) r CT scan with IV contrast (2)
Incidence Salmonella species Diagnostic criteria for pyonephrosis
r True incidence is unknown Increased wall thickness of the renal pelvis
Tuberculosis (causes both infection and strictures)
r Increased in patients with upper urinary tract (2) 2 mm
obstruction The presence of renal pelvic contents and debris
ASSOCIATED CONDITIONS Parenchymal and perirenal findings, such as
Prevalence r Nephrolithiasis (most common) (1)
See above r UPJO perirenal fat stranding (3)
r Ultrasonography (US)
RISK FACTORS r Urothelial carcinoma (UC) of the upper tracts
r Upper urinary tract obstruction r Pyelonephritis Sensitivity of renal US for differentiating
hydronephrosis from pyonephrosis is 90%, and
r History of prior urologic instrumentation r Emphysematous pyelonephritis/pyelitis
the specificity is 97% (1)
r Immunocompromised patient r Xanthogranulomatous pyelonephritis (XGP) Debris
r Diabetes mellitus r Ureteral stricture (2) Low-level echogenic foci
r Chronic UTIs Hydronephrosis
GENERAL PREVENTION r MRI
Genetics r Relief of underlying urologic obstruction
None r Proper medical management of immunosuppression Use increasing for inflammatory disorders of the
r Identification of any anatomic urologic abnormality GU tract
PATHOPHYSIOLOGY Diffusion MRI shows hyperintense collecting
r Etiologies of obstruction (1) (ie, horseshoe kidney) system for pyonephrosis and hypointense signal
Stones and staghorn calculi: In as many as 75% of for simple hydronephrosis
patients ALERT May be useful for patients with impaired renal
Mucinous adenocarcinoma of the renal pelvis Patients may rapidly decline clinically and become function (3)
Pregnancy septic. r Renal nuclear scan
Fungus balls Useful in assessing renal function after
Metastatic retroperitoneal fibrosiseg, renal decompression and to evaluate if involved renal
tumors, testicular cancer, colon cancer DIAGNOSIS unit is salvageable
Obstructing transitional cell carcinoma Not helpful in the immediate diagnostic period
Ureteropelvic junction obstruction (UPJO) HISTORY
r Fever Diagnostic Procedures/Surgery
Obstructing ureterocele r Once pyonephrosis has been diagnosed, there are
r Flank pain
Ureterovesical junction obstruction
Chronic stasis of urine and hydronephrosis r Clinical evidence of UTI two possible initial interventions:
secondary to neurogenic bladder Antegrade nephrostomy tube placement
PHYSICAL EXAM Retrograde ureteral stent placement
Ureteral strictures
CVA tenderness with or without palpable abdominal
Papillary necrosis Pathologic Findings
mass (hydronephrotic kidney) r Aspiration of obstructed system will usually show:
Tuberculosis
Duplicated kidneys with obstructive components WBCs
Ectopic ureter with ureterocele Bacteria or fungus
Neurogenic bladder Sloughed urothelial cells
DIFFERENTIAL DIAGNOSIS
r Nephrolithiasis/urolithiasis
r Xanthogranulomatous pyelonephritis
r Ureteropelvic junction obstruction (UPJO)
r Urothelial carcinoma (UC) of upper tracts
r Ureteral stricture
r Extrinsic obstruction with hydronephrosis
(malignancy, retroperitoneal fibrosis)
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PYONEPHROSIS
REFERENCES
TREATMENT ONGOING CARE
1. Peterson AC. Pyonephrosis Medscape Article
GENERAL MEASURES PROGNOSIS 2013; emedicine.medscape.com/article/440548
r Drainage of obstructed collecting system is the r Good in patients who receive prompt diagnosis and 2. Raynor MC, Carson CC. Urologic issues for the
mainstay of treatment therapy internist urinary infections in men. Med Clin North
Antegrade nephrostomy tube placement r Most patients will improve 2448 hr after drainage Am. 2011;95:4354.
Indicated in the clinically unstable patient of obstructed renal collecting system 3. Hammond NA, et al. Genitourinary imaging
Best for maximal decompression r Recovery of renal function is rapid infectious and inflammatory diseases of the kidney.
Retrograde ureteral stent placement Rad Clin North Am. 2012;50:259270.
Indicated in the stable patient able to tolerate COMPLICATIONS
r Sepsis is the most common complication of delayed
general anesthesia
Relatively contraindicated in setting of a large treatment
r Other complications of delayed treatment include: ADDITIONAL READING
upper tract stone that will eventually need
percutaneous therapy or fungus ball Rupture of pyonephrotic kidney resulting in: Schaeffer AJ, Schaeffer EM. Infections of the urinary
r Treatment of source obstruction: Generalized peritonitis tract. In: Wein AJ, et al. eds. Campbell-Walsh Urology.
Renocolic fistula 10th ed. Philadelphia: Saunders; 2012.
Once collecting system has been decompressed Renoduodenal fistula
and appropriate antibiotic/antifungal therapy has Renocutaneous fistula See Also (Topic, Algorithm, Media)
been given for 2 wk r Fungal Infections, Genitourinary
Splenic rupture
May include endoscopic, percutaneous, r Hydronephrosis/Hydroureteronephrosis, (Dilated
Rare complications:
transurethral, laparoscopic, robotic, Pneumoperitoneum Ureter/Renal Pelvis), Adult
extracorporeal, or open approaches Renal vein thrombosis r Pyonephrosis Image
Depends on the nature of obstruction (ie, stone, Psoas abscess r Ureter, Obstruction
stricture) (1) Perinephric abscess r Urosepsis
Clinical feasibility of intervention Rhabdomyolysis r Urolithiasis, Staghorn
MEDICATION Loss of renal function
First Line r Complications from nephrostomy tube:
r Broad spectrum intravenous antibiotics (ie, Blood transfusions CODES
piperacillin and tazobactam, gentamicin, and Hematoma
ampicillin) and antifungals if clinically indicated for Nephrostomy tube replacement/revision ICD9
funguria r Increased risk of infection if nephrectomy is not r 590.80 Pyelonephritis, unspecified
Antibiotics can be focused once cultures result performed when indicated. r 593.89 Other specified disorders of kidney and
Second Line FOLLOW-UP ureter
N/A Patient Monitoring r 599.0 Urinary tract infection, site not specified
SURGERY/OTHER PROCEDURES r Treatment of underlying obstruction (ie, calculus,
r Indication for nephrectomy is controversial stricture, malignancy) ICD10
r N13.6 Pyonephrosis
May be indicated if source of infection is not found r Treatment and control of any predisposition to
r N28.89 Other specified disorders of kidney and
Help to exclude malignant etiology of obstruction infection (ie, DM, HIV/AIDS, neurogenic bladder)
Lack of response to percutaneous drainage and IV ureter
Patient Resources r N39.0 Urinary tract infection, site not specified
antibiotics/antifungals r http://kidney.niddk.nih.gov/kudiseases/pubs/
Poorly functioning kidney stonesadults/
ADDITIONAL TREATMENT r http://www.medicinenet.com/kidney stone/ CLINICAL/SURGICAL
Radiation Therapy article.htm PEARLS
N/A r http://www.mayoclinic.com/health/
kidney-stones/DS00282 r Patients with pyonephrosis may be asymptomatic or
Additional Therapies present with a picture of an abscess with fever and
N/A
chills.
Complementary & Alternative r Urolithiasis, staghorn calculi, and fungus balls are
Therapies the most common clinical causes of pyonephrosis.
N/A
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PYURIA
Christina Carpenter, MD
Mark L. Jordan, MD, FACS
clavulanic acid
Presence of nitrites, blood, or protein suggests UTI
386
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PYURIA
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Imaging
BASICS DIAGNOSIS r Differentiation between early renal abscess and
acute pyelonephritis is difficult due to small size
DESCRIPTION HISTORY r Abdominal CT:
r Renal abscess/carbuncle: r Significant chronic or acute illnesses including
Diagnostic procedure of choice
Collection of purulent material confined to the diabetes, neurogenic bladder dysfunction, chronic Can often delineate the route of spread of
renal parenchyma renal failure, hemodialysis, and polycystic renal infection into surrounding tissues
r Perirenal abscess: disease Abscesses are characteristically well defined both
r Renal calculi
Results from extension of an acute cortical before and after contrast agent enhancement
abscess into the perinephric space; confined by r IV drug abuse
Acute findings include renal enlargement and
Gerota fascia Gram-positive source of infection 18 wk before focal, rounded areas of decreased attenuation
r Pararenal/perinephric abscess: the onset of urinary tract symptoms Chronic findings include obliteration of adjacent
Results from the rupture of a perinephric abscess Preceding infection can occur in any area of the tissue planes, thickening of Gerota (perinephric)
through Gerota fascia into the pararenal space body (eg, skin lesions, dental infections) fascia, a round or oval parenchymal mass of low
r Patients with UTI and abdominal or flank mass attenuation, and a surrounding inflammatory wall
EPIDEMIOLOGY r Persistent fever with suspected genitourinary source of slightly higher attenuation that forms a ring
Incidence after 35 days of antimicrobial therapy when the scan is enhanced with contrast material
Perinephric and renal abscesses are uncommon but (ring sign)
potentially lethal complications of UTI PHYSICAL EXAM See Figure 1, Renal Abscess
r Elevated temperature
Prevalence r IV urography (if performed)
r 2/3 of gram-negative abscesses are associated with r CVA or flank tenderness
r Abdominal and/or flank mass Abnormal in up to 80% of patients, although
renal calculi or kidneys with poor function findings often are nonspecific
r Pregnant women with untreated bacteriuria are r Distended or palpable bladder
Generalized enlargement of involved renal unit
r Look for skin carbuncles or dermatologic evidence of with distortion of renal contour and collecting
associated with a higher incidence of pyelonephritis
and subsequent diagnosis of abscess IV drug abuse system
r Renal infection is among the most common sites for r Heart murmurs Absence of psoas shadow on affected side
extrapulmonary disease in patients with TB Bubbles of extraluminal gas can be seen
DIAGNOSTIC TESTS & INTERPRETATION
surrounding the kidney in large perinephric
RISK FACTORS Lab abscesses
Diabetes mellitus, polycystic kidney disease, r Serum creatinine:
r Abdominal US:
hemodialysis, neurogenic bladder, IV drug users, Variable findings, dependent on concurrent
Quickest and least expensive diagnostic imaging
tuberculosis, recurrent urinary tract infection and/or obstruction and underlying renal dysfunction
r CBC: study
pyelonephritis, nephrolithiasis, vesicoureteral reflux, Common findings include an echo-free or
ureteropelvic junction obstruction or other source of Patients typically have marked leukocytosis low-echodensity space-occupying lesion with
obstruction, any immunocompromised state r Urine analysis:
increased transmission, which is poorly
Genetics Pyuria and bacteria often present, although marginated during the acute phase
N/A pyuria/bacteriuria may not be evident unless the Well-defined discrete lesion during chronic stages,
abscess communicates with the collecting system which is difficult to distinguish from a renal mass
PATHOPHYSIOLOGY Sterile pyuria often seen with TB
r Gram-negative organisms have been implicated in r Urine culture:
the majority of adults with renal abscesses
ALERT
When abscesses contain gram-negative Evidence of air within renal parenchyma tissue is
(Escherichia coli, Proteus mirabilis, and organisms, urine culture often demonstrates the
Staphylococcus aureus) account for the majority of diagnostic for emphysematous pyelonephritis which
same organism isolated from the abscess may require urgent surgical intervention. See
infections (in descending order of occurrence) (1) Since gram-positive organisms are most commonly
r Hematogenous renal seeding by gram-negative Section I: Emphysematous pyelonephritis.
blood borne, urine cultures in these cases typically
organisms may occur, but this is not likely to be the show no growth or a microorganism different
primary pathway for gram-negative abscess Diagnostic Procedures/Surgery
from that isolated from the abscess CT- or US-guided needle aspiration may be necessary
formation Catheterized urine collection recommended for
r Hematogenous renal seeding: Skin infection with to differentiate an abscess from a hypervascular
female patients tumor; aspirated material can be collected for culture
gram-positive organisms, IV drug abuse, r Blood cultures:
to guide appropriate antimicrobial therapy. A
immunocompromised status Gram-negative organisms are most commonly
r Ascending infection associated with tubular percutaneous drain may be left in place and clinical
cultured course can be evaluated.
obstruction from prior infections, vesicoureteral Gram-positive organisms are not routinely similar
reflux, or calculi appears to be the primary pathway to those cultured from abscess Pathologic Findings
for the establishment of gram-negative abscesses Abscess fluid will demonstrate neutrophils and gram
stain will reveal bacteria
ASSOCIATED CONDITIONS
See Risk Factors above
GENERAL PREVENTION
Increased clinical suspicion, prompt recognition, and
treatment of infection, especially in the face of
obstruction in high-risk patients
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RENAL ANGIOMYOLIPOMA
Casey Allison Seideman, MD
Ganesh V. Raj, MD, PhD, FACS
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RENAL ANGIOMYOLIPOMA
R
SURGERY/OTHER PROCEDURES FOLLOW-UP r Rakowski SK, Winterkorn EB, Paul E, et al. Renal
r Indications: Diagnostic uncertainty, hemorrhage manifestations of tuberous sclerosis complex:
Patient Monitoring
causing significant symptoms, pain, hematuria, risk r Controversial in patients with newly diagnosed Incidence, prognosis, and predictive factors. Kidney
of rupture AML; screen for TS Int. 2006;70(10):17771782.
r Asymptomatic AML <4 cm: r Conservative management: Serial imaging (usually r Roy C, Tuchmann C, Lindner V, et al. Renal cell
Observation with serial imaging at 12-mo intervals with CT or US) every 612 mo carcinoma with a fatty component mimicking
r Asymptomatic AML >4 cm: r Growth rate typically 5% per yr for solitary AML angiomyolipoma on CT. Br J Radiol. 1998;71:
Treatment should be considered; observation with r TSC patients and those with multicentric AMLs have 977979.
serial imaging r Steiner MS, Goldman SM, Fishman EK, et al. The
growth rate of 20% per yr
The risk of spontaneous hemorrhage appears natural history of renal angiomyolipoma. J Urol.
greatest in masses >4 cm Patient Resources 1993;150:17821786.
r TS alliance www.tsalliance.com
Women of childbearing age may consider
r LAM Foundation www.theLAMfoundation.com See Also (Topic, Algorithm, Media)
proactive treatment r Renal Angiomyolipoma Image
r Symptomatic AML/lesion >4 cm:
r Renal Cell Carcinoma, General
Selective arterial embolization or nephron-sparing
surgery
REFERENCES r Renal Mass
r Acute hemorrhage: r Retroperitoneal Hematoma
1. Kennelly MJ, Grossman HB, Cho KJ. Outcome r Tuberous sclerosis
Initially treated with embolization (stabilizes analysis of 42 cases of renal angiomyolipoma.
patient and often eliminates need for more J Urol. 1994;152:19881991.
intervention) 2. Margulis V, Matin SF, Wood CG. Chapter 51:
If explored emergently, total nephrectomy usually Benign Renal Tumors. In: Campbell-Walsh Urology.
CODES
necessary Philadelphia, PA: Elsevier; 2010:14921505.
3. Nelson CP, Sanda MG. Contemporary diagnosis
ICD9
ADDITIONAL TREATMENT r 223.0 Benign neoplasm of kidney, except pelvis
Radiation Therapy and management of renal angiomyolipoma. J Urol. r 593.81 Vascular disorders of kidney
N/A 2002;168:13151325.
r 759.5 Tuberous sclerosis
4. Dabora SL, Franz DN, Aswal S, et al. Multicenter
Additional Therapies phase 2 trial of sirolimus for tuberous sclerosis:
r Limited reports of treatment using cryoablation and ICD10
Kidney angiomyolipomas and other tumors regress r D17.71 Benign lipomatous neoplasm of kidney
radiofrequency ablation and VEGF-D levels decrease. PLoS One. 2011;6(9):
r In patients with LAM or TS, mTOR inhibitors such as r N28.89 Other specified disorders of kidney and
e23379.
sirolimus/temsirolimus have been shown to decrease ureter
mass size by 30% r Q85.1 Tuberous sclerosis
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r Hemangiopericytoma
BASICS DIAGNOSIS Solid, encapsulated mass
Microscopically, varied cell shapes and sizes with
DESCRIPTION HISTORY morphologic variability
r Predominantly mesenchymal neoplasms arising from r Usually asymptomatic or discovered incidentally
Immunostaining positive for vimentin, BCL2,
the renal capsule encompassing a wide variety of r May present with hematuria or flank pain
CD99 and negative for S100, cytokeratins, and
cell progenitors r Weight loss, anorexia, malaise, or bone pain may HMB-45 (2)[C]
Tumors can be composed of fibrous, smooth signify metastatic disease r Lymphangioma
muscle, vascular, adipose, nerve, or other tissue Well-encapsulated, multilocular cystic mass
PHYSICAL EXAM
differentiation r Usually normal Microscopically, communicating cysts seen with
Encompasses benign and malignant neoplasms flattened endothelial cells
Rarely, flank mass may be palpable
EPIDEMIOLOGY Immunostaining positive for D2-40
DIAGNOSTIC TESTS & INTERPRETATION Labels lymphatic endothelium (1,3)[C]
Incidence r Solitary fibrous tumor
r Very rare tumors Lab
r Urinalysis Well-encapsulated firm mass without necrosis,
r Represent up to 1.5% of all surgically treated
Microscopic hematuria may be identified, but cysts, or hemorrhage
benign renal masses (1) usually normal
r Incidentally found at autopsy in up to 5% of Microscopically, usually shows areas of spindle
r CBC cells intermixed with hypocellular areas of fibrous
cases (1) Anemia may be present with advanced disease or tissue
r Similar gender preference
bleeding mass Immunostaining strongly positive for CD34
Prevalence r Serum chemistries usually normal May also stain positive for CD99 and BCL2 and
Unknown, due to rarity of tumor can be misclassified as hemangiopericytoma
Imaging
r CT or MRI with and without contrast (1,2)[C]
RISK FACTORS r Leiomyosarcoma
r None known May show enhancing mass arising from the
kidney Usually large circumscribed mass with areas of
Increased cross-sectional imaging use may identify
Indistinguishable from renal cell carcinoma in necrosis
incidental mass
most cases Microscopically, spindle cells with haphazard
Genetics Presence of fat may signify angiomyolipoma, growth pattern, nuclear pleomorphism, mitoses,
r No recognized genetic predisposition
lipoma, or liposarcoma and necrosis
r Some common genetic alterations seen in soft tissue Immunostaining positive for SMA, desmin, and
r Chest x-ray
sarcomas calponin (1)[C]
Evaluate for metastatic disease r Fibrosarcoma
No current clinical application for genetic
alterations Diagnostic Procedures/Surgery Large encapsulated mass
r Core needle biopsy
PATHOPHYSIOLOGY Microscopically, elongated spindle cells
r Benign May be used in cases of suspected renal Herringbone pattern
malignancy or if active surveillance considered Immunostaining positive for vimentin
Leiomyoma, hemangiopericytoma, hemangioma, r Angiography Differentiates fibrosarcoma from sarcomatoid
lymphangioma, myxoma, schwannoma, solitary
May be utilized for bleeding lesions RCC and leiomyosarcoma (1)[C]
fibrous tumor, paraganglioma, lipoma, fibroma, r Malignant fibrous histiocytoma
Benign lesions usually hypovascular
myolipoma Except hemangiopericytoma, which is highly
r Malignant Solid, well-encapsulated mass
vascular Microscopically, proliferation of fibrohistiocytes
Leiomyosarcoma, malignant fibrous histiocytoma,
fibromyxoid sarcoma, hemangiosarcoma, Pathologic Findings Immunostaining positive for 1-antitrypsin and
r Leiomyoma vimentin
liposarcoma, fibrosarcoma
Firm, well-circumscribed, exophytic mass
ASSOCIATED CONDITIONS Microscopically, composed of spindle cells
Some renal hemangiomas may be associated with arranged in fascicles typical of smooth muscle
SturgeWeber or KlippelTrenaunay syndromes (1) Immunostaining positive for desmin, smooth
GENERAL PREVENTION muscle actin, and usually HMB-45 (1)[C]
No preventive strategies identified
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DIFFERENTIAL DIAGNOSIS
BASICS DIAGNOSIS r Renal masses:
Angiomyolipoma (fat-poor)
DESCRIPTION HISTORY Collecting-duct tumors
r Advanced stage of renal cell carcinoma (RCC) r Usually no symptoms in early stage but incidental
Cystic nephromas
N1: Metastasis in regional lymph node(s) diagnosis of kidney mass. Metastasis may or may Cysts (hemorrhagic, infected)
M1: Distant metastasis not cause symptoms. Focal pyelonephritis
Nodal involvement is now simplified to N0 vs. N1 r Gross hematuria
Hemangioma
r Other changes for staging from the AJCC Cancer r Flank or abdominal pain Inflammatory masses (xanthogranulomatous
Staging Manual (7th edition): r Symptoms related to involved organ(s) pyelonephritis, abscess)
Ipsilateral adrenal involvement is reclassified as T4 r Constitutional symptoms: Leiomyoma
if contiguous invasion and M1 if not contiguous Fatigue, weight loss, fever, or lower-extremity Metanephric adenoma
Renal vein involvement is reclassified as T3a edema Metastasis from other primary tumor
Oncocytoma
EPIDEMIOLOGY PHYSICAL EXAM
r With attention to abdominal mass, adenopathy, Pseudotumors (column of Bertin, others)
Incidence RCC
r Renal cancer increased by 2% per year for the past lower-extremity edema Renal lymphoma
65 yr. Increased by 3.1% per year from 20052009 r Varicocele (classically on left)
Renal medullary carcinoma
primarily due to an increase in early-stage disease. Sarcomas
Renal cancer accounts for 23% of all malignancies. DIAGNOSTIC TESTS & INTERPRETATION
Lab Reninoma (Juxtaglomerular tumors)
Median age of diagnosis is 65 yr, median age of r CBC: Anemia of chronic disease in up to 30% Urothelial carcinoma
death is 70 yr. Wilms tumor (nephroblastoma)
r Urinalysis: Hematuria
1/3 of patients with RCC present M+/N+ r Lymphadenopathy:
r 4050% will develop metastatic disease after initial r Complete metabolic panel
r Paraneoplastic syndromes (found in 20% of Inflammatory related to RCC
diagnosis. Male > Female (ratio is 3:2). RCC
r Death rates for kidney cancer decreased by 0.5% patients): Infectious/inflammatory:
per year from 20052009 Hypercalcemia Granulomatous: TB, sarcoidosis, histoplasmosis,
Hypertension lymphogranuloma venereum, Castleman
Prevalence Erythrocytosis
N/A disease, etc.
Elevated erythrocyte sedimentation rate (ESR) Nongranulomatous: Viral, bacterial (if abscess in
RISK FACTORS Nonmetastatic hepatic dysfunction (Stauffer local areas), sinus histiocytosis
r Smoking syndrome) Primary lymphatic malignancy: Lymphoma
r Obesity r Urine cytology: R/O urothelial carcinoma
(non-Hodgkin and Hodgkin, others)
r Hypertension and chronic renal failure Other metastatic malignancies:
Imaging
Chemicals or radiation exposure slightly increases r MRI or CT of abdomen and pelvis (85% of Gastrointestinal (GI) (carcinoid, colorectal,
risk. enhancing renal masses are RCC) lymphoma), urothelial, prostate, melanoma,
r Rare hereditary conditions Most enlarged lymph nodes by imaging are penile, germ cell, cervical, ovarian, uterine
Von HippelLindau (VHL) disease inflammatory rather than neoplastic r Pulmonary nodules:
Hereditary papillary RCC r CNS imaging: In patients with symptoms or Benign hamartoma/AVM, nonspecific granuloma,
Genetics radiographic evidence of advanced disease infectious granuloma (aspergillosis,
r Majority of cases are sporadic r Positron emission tomography (PET) has poor coccidioidomycosis, cryptococcosis,
r VHL tumor suppressor gene (on 3p2526) sensitivity and not commonly used histoplasmosis, tuberculosis, atypical
associated with clear cell RCC mycobacterial infections), septic emboli, abscesses
Diagnostic Procedures/Surgery Malignancy: Lung primary, metastasis
r Chromosome 7q31 is associated with papillary RCC, r Needle biopsy to the metastatic site or
(choriocarcinoma, RCC, melanoma, thyroid
type 1 associated with mutations in c-MET metastasectomy usually required to establish
r Fumarate hydratase (FH) associated with papillary carcinoma, Kaposis sarcoma), non-Hodgkin
diagnosis lymphoma
RCC type 2 r Needle biopsy is generally safe and warranted if any
r BirtHoggDube (BHD) syndrome associated with uncertainty for M1 disease
chromophobic RCC Pathologic Findings TREATMENT
r Xp11.2 translocation: Patients under 45 yr r Histologic subtypes:
r BRCA1-associated protein-1 (BAP1) loss associated GENERAL MEASURES
Clear cell: 7080% r Stage IV disease may also benefit from cytoreductive
with high tumor grade Papillary: 1015% surgery
PATHOPHYSIOLOGY Chromophobe: 35% r Minimal regional adenopathy does not preclude
r RCCs derived from renal tubular epithelium Collecting duct/medullary cancer <1%
surgery
r Mode of spread is via direct extension, propagation Translocation Xp11.2: Clear and/or eosinophilic, r Potential candidate for nephrectomy and/or surgical
into renal vein, or hematogenous voluminous cytoplasm
r Sarcomatoid variants all histologic subtypes: metastasectomy:
r Rare reports of spontaneous regressions, usually Resectable primary RCC and a solitary resectable
pulmonary, following nephrectomy Represent poorly differentiated regions
metastasis
Portends a much more aggressive biology with
ASSOCIATED CONDITIONS A solitary recurrence after prolonged disease-free
recurrence/resistance to therapy
r VHL syndrome interval from nephrectomy
r Chronic renal failure r Tends to be resistant to both traditional
r Lymphoma chemotherapy and radiation therapy
GENERAL PREVENTION
Smoking cessation helps prevent primary tumor
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DIFFERENTIAL DIAGNOSIS
BASICS DIAGNOSIS r Benign renal mass in children:
Choledochal cyst, intestinal duplication cyst
DESCRIPTION HISTORY Congenital mesoblastic nephroma
r Gross hematuria (40%), flank pain, abdominal
Renal cell carcinoma (RCC) is a very rare tumor in Hydronephrosis
childhood arising from the renal tubular epithelium distension (2) Mesenteric cyst
r Nausea, vomiting, malaise common
EPIDEMIOLOGY Multicystic dysplastic kidney
r Pain in up to 50% Polycystic kidney
Incidence r 30% found incidentally (2) Renal abscess
r 26% of all pediatric renal tumors
r Only 4 cases of pediatric RCC per year Splenomegaly
PHYSICAL EXAM r Malignant renal masses in children:
r Estimated at <0.3% of all pediatric tumors r Palpable abdominal mass (40%) (2)
r Triad of hematuria, flank pain, and palpable mass Hepatoblastoma
r Just over 350 cases reported in the literature
Lymphoma
r Mean age of presentation between 8 and 10 yr vs. found in <6% of children (3)
Lymphosarcoma
<3 yr for Wilms tumor DIAGNOSTIC TESTS & INTERPRETATION Neuroblastoma
r Equal male:female RCC
Lab
r Urinalysis: Hematuria found in >40% of patients (3) Rhabdomyosarcoma
Prevalence
N/A r CBC: Polycythemia is rare Wilms tumor
r Liver and renal function tests: Baseline prior to
RISK FACTORS
r Von HippelLindau syndrome treatment TREATMENT
r Tuberous sclerosis Imaging
r Abdominal x-ray may show tumor calcifications GENERAL MEASURES
Genetics (25%) vs. Wilms tumor (5%) (3) Management is primarily surgical excision by either
r Translocation type of RCC, which forms a distinct radical nephrectomy or partial nephrectomy
r US demonstrates solid or cystic renal mass.
category has recently emerged as the predominant r CT or MRI with and without contrast reveal MEDICATION
type of RCC in children and adolescents, whereas it
is rarely diagnosed in adults. enhancing renal mass. First Line
r Pediatric RCCs typically present as large, r The use of chemotherapy, immunotherapy, or
r Chromosomal translocations in Xp11.3 region
heterogeneous masses, commonly hemorrhage and tyrosine kinase inhibitors is not adequately
involving TFE3 gene (1).
r Less frequently, 6p21 translocation. contain internal calcifications. described in pediatric population.
r IVP can demonstrate renal mass by displacement of r The use of tyrosine kinase inhibitors should be
r If seen with Von HippelLindau, more likely to be
the collecting system. considered in the pediatric patient with
bilateral. r Chest x-ray or chest CT scan for workup of unresectable, metastatic, or advanced-stage RCC.
PATHOPHYSIOLOGY metastatic disease. r Small series of patients treated with neoadjuvant
r Thought to arise from renal tubular epithelium r Radionuclide bone scan is indicated based on chemotherapy according to Wilms tumor protocol.
r Most frequently papillary subtype with Xp11 concern for mets. Second Line
translocation N/A
Diagnostic Procedures/Surgery
Role of translocation at Xp11.2 region involving
Biopsy is not indicated SURGERY/OTHER PROCEDURES
TFE3 gene unknown (1)
r Lung and bone are the most common distant Pathologic Findings r Radical nephrectomy (4)
r Predominately papillary histologic features in Removal of entire kidney and portion of the ureter
metastases.
children vs. clear cell features in adults Common approaches in children include flank and
ASSOCIATED CONDITIONS r Pathologic staging based on modified Robson abdominal incisions
r Tuberous sclerosis, chronic renal failure, r Partial nephrectomy (5)
staging system
neuroblastoma, and teratoma with chemotherapy r Up to 25% pediatric RCC cannot be clearly classified r Laparoscopic and robotic-assisted radical or partial
r Rarely associated with adult familial RCC
due to atypical features nephrectomy for RCC in children are described in
GENERAL PREVENTION r Pathologic parameters typically associated with poor select cases (6,7)
N/A outcome in adults (metastasis/high tumor stage,
high Fuhrman nuclear grade, angiolymphatic
invasion, tumor necrosis), do not appear to have
similar implications in pediatric patients
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RENAL COLIC
Scott G. Hubosky, MD
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RENAL COLIC
R
DIFFERENTIAL DIAGNOSIS r UPJ obstruction 3. Chauhan V, Eskin B, Allegra JR, et al. Effect of
r Ureteral calculus Pyeloplasty (open, laparoscopic, robotic) season, age and gender on renal colic incidence.
r Ureteral stricture Endopyelotomy (retrograde, antegrade) Am J Emerg Med. 2004;22:560563.
r UPJ obstruction r Upper tract neoplasm 4. Stamatelou KK, Francis ME, Jones CA, et al. Time
r Upper tract urothelial neoplasm Ureteroscopic ablation trends in reported prevalence of kidney stones in
Upper tract urothelial carcinoma Nephroureterectomy (open or laparoscopic) the United States: 19761994. Kidney Int.
Fibroepithelial polyps 2003;63:18171823.
ADDITIONAL TREATMENT
r Iatrogenic ureteral obstruction 5. Wolf JS. Medscape practice essentials:
Radiation Therapy Nephrolithiasis. Available online at
Ureteral ligation after hysterectomy or colon N/A
resection http://emedicine.medscape.com/article/437096-
Obstructing residual stone fragments after Additional Therapies overview. Accessed January 5, 2014.
r Obtain adequate drainage if necessary, especially if
lithotripsy
Obstructing blood clots following upper tract the patient appears septic
urologic procedure Ureteral stenting is usually a good 1st choice ADDITIONAL READING
r Upper urinary tract infection Chronically obstructed patients often have
r Malo C, Audette-Cote JS, Emond M, et al.
Pyelonephritis significant ureteral tortuosity making retrograde
access challenging Tamsulosin for treatment of unilateral distal
Pyonephrosis ureterolithiasis: A systematic review and
Renal abscess Percutaneous drainage
Can be performed with conscious sedation meta-analysis. CJEM. 2013;15(0):114.
Obstructing fungus ball r Moore CL, Bomann S, Daniels B, et al. Derivation
r Renal vascular etiology Optimal in cases of significant extrinsic ureteral
compression and validation of a clinical prediction rule for
Renal artery embolus/renal infarction uncomplicated ureteral stonethe STONE score:
Renal vein thrombosis Complementary & Alternative
retrospective and prospective observational cohort
Therapies
studies. BMJ. 2014;348:g2191.
N/A
TREATMENT See Also (Topic, Algorithm, Media)
r Flank Pain, General
GENERAL MEASURES
r Rule out sepsis
ONGOING CARE r Pyonephrosis
r Treat infection PROGNOSIS r Pyelonephritis, Acute, Adult
r Control pain Depends on underlying etiology but usually good once r Renal Colic Image
r Alleviate obstruction, if present obstruction is relieved and infection treated, if present r Urolithiasis, Renal
r Urolithiasis, Ureteral
COMPLICATIONS
MEDICATION r Persistent obstruction if left untreated
First Line Renal cortical loss
r Analgesia based on degree of discomfort
Could lead to nonfunctioning kidney CODES
Narcotic analgesics for more severe pain Serious infection
Given PO or IV
FOLLOW-UP ICD9
Morphine sulfate, oxycodone/APAP, r 599.69 Urinary obstruction, not elsewhere classified
hydrocodone/APAP, meperidine, nalbuphine Patient Monitoring r 787.01 Nausea with vomiting
Ketorolac Renal/bladder ultrasound after treatment to ensure no r 788.0 Renal colic
IV acetaminophen evidence of silent hydronephrosis or recurrent
Less dizziness and hypotension than morphine obstruction ICD10
in one study Patient Resources r N13.8 Other obstructive and reflux uropathy
r Antiemetics (metoclopramide, ondansetron) r N23 Unspecified renal colic
Urology Care Foundation http://www.urologyhealth.
Second Line org/urology/index.cfm?article=148 r R11.2 Nausea with vomiting, unspecified
r -blockers: Tamsulosin, alfuzosin, silodosin
Given to relieve ureteral smooth muscle spasm
patients with ureteral stones
REFERENCES CLINICAL/SURGICAL
Off-label use in cases of urolithiasis 1. Silen W. The colics. In: Copes Early Diagnosis of PEARLS
Alfuzosin (10 mg/d) the Acute Abdomen. 22nd ed. New York, NY:
Silodosin (8 mg/d) r Vast majority of patients with renal colic will have
Oxford University Press; 2010.
Tamsulosin (start 0.4 mg to max 0.8 mg); most calculi.
2. Brown J. Diagnostic and treatment patterns for r Young patients with hydronephrosis and no
reported data renal colic in US emergency departments. Int Uro
Nephrol. 2006;38:8792. evidence of calculus likely have congenital UPJ
SURGERY/OTHER PROCEDURES obstruction or other upper tract narrowing.
r Initial stent placement for significant obstruction
r Support patient with medicines to alleviate the
r Lithotripsy for nephrolithiasis
acute pain of renal colic.
Ureteroscopy with laser lithotripsy r Treat infection if present.
ESWL (extracorporeal shock wave lithotripsy)
PCNL (Percutaneous nephrolithotomy)
r Ureteral stricture treatment
Balloon dilation, laser incision
Open or laparoscopic reconstruction
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Other genetic cystic diseases: Juvenile nephronoph- (Hounsfield units (HU) ranging from 10 to
BASICS thisis, medullary cystic kidney disease, glomerulo- +20); no enhancement with IV contrast
cystic kidney disease, Von HippelLindau syndrome CT diagnosis of a simple cyst is almost 100% if
DESCRIPTION (VHL), tuberous sclerosis, BirtHoggDube study performed properly (bi- or triphasic)
r Renal cycts are fluid-filled renal structures not syndrome Enhancement is defined as an increase in HU by
continuous with the nephron or collecting system at least 1520
r Simple cyst PATHOPHYSIOLOGY If cyst does not meet criteria for being simple,
r Simple cysts
Arise from the renal parenchyma further workup of the lesion is necessary
Development of discrete fibrous saccules of clear
Size varies, often <2 cm but may be significantly Parapelvic cyst
fluid lined with cuboidal epithelium Appears on US as a medially located cystic mass
larger
Estimated growth rate: 2.18 mm/yr
Typically asymptomatic incidentally detected on with surrounding echogenic walls (located
Some will involute and disappear over time
CT or US within the fatty renal sinus)
although most will not On US or even CT can be confused with
Can be single, multiple, and/or bilateral
It is controversial if renal cysts are causative
If large, may impinge on the renal pelvis causing hydronephrosis. An excretory phase CT is most
agents of HTN
obstruction r Parapelvic cysts helpful in distinguishing a parapelvic cyst from a
Diagnostic US findings include a mass that is free simple parcehncymal renal cyst.
of internal echos (anechoic), through transmission Found on <2% of kidneys at autopsy r MRI:
with posterior acoustic enhancement Can be confused with hydronephrosis
Bosniak criteria can be applied to MRI (exception:
r Complex cyst ASSOCIATED CONDITIONS Calcifications may not be well seen)
Features not consistent with simple cyst; raise the r ADPKD (Autosomal dominant polycystic kidney Low signal T1, high signal T2 is consistent with
possibility of malignancy disease) benign simple cyst
Increased fluid density, internal thick-walled r ARPKD (Autosomal recessive polycystic kidney Hyperdense cysts can be high signal on T1 and
septations, thickened wall, nodular projections disease) low on T2 but appearance depends on
into the lumen, calcifications, and contrast r BirtHoggDube syndrome hemoglobin breakdown
enhancement r ESRD (end stage renal disease) MRI may have a role in a subset of patients (VHL,
r Pyogenic cysts are infected cysts r Tuberous sclerosis multiple renal masses) if concern exists regarding
r Parapelvic cyst (aka peripelvic, parapelvic lymphatic, excessive radiation due to multiple long-term
50% have multiple renal angiomyolipomas
parapelvic lymphangiectasia, and renal sinus cysts); imaging studies
2025% of have renal cysts
arise from the renal sinus r VHL disease May be superior in characterization of internal cyst
contents (blood, mucin)
Individuals develop cysts in multiple organs r Bi- or triphasic CT represents the gold standard for
ALERT
(kidney, pancreas, liver, epididymis)
Parapelvic cysts may be confused with distinguishing renal cysts:
Increased risk of clear cell renal cell carcinoma
hydronephrosis given their central location. Discriminate between cysts and collecting system
(RCC) in cyst wall
r Acquired cyst on excretory phase
GENERAL PREVENTION Particularly important in assessing hydronephrotic
Associated with chronic hemodialysis
Family members of patients with ADPKD and VHL systems
Occasionally regress spontaneously US may be misleading/difficult to interpret
r Bosniak classification used to classify cysts based on should be screened
r Bosniak classification system of cystic renal masses
CT complexity and likelihood of malignancy
DIAGNOSIS originally based on CT (image) (1):
EPIDEMIOLOGY Category I: Benign simple cysts; thin wall without
Incidence HISTORY septa, calcifications, or solid components, water
r 0.22% from birth to 18 yr r Patients may present with an abdominal mass, pain, density, and no contrast enhancement; No further
r 20% by age 40 hematuria, or HTN but most are radiographically imaging needed
r 33% by age 60 incidental Nearly all are benign
r In autopsy series, 50% of patients >50 have 1 r Family member with polycystic kidney disease or Category II: Benign cysts with a few thin septae;
other inherited cystic disease the wall or septa may contain fine calcification,
simple renal cysts
r Acquired cystic renal disease is more common sharp margins, nonenhancing
PHYSICAL EXAM Category IIF: Well marginated and may have thin
among men r Abdominal/flank mass (rare)
r Bilateral simple cysts infrequent <50 yr r Often a benign exam septae or minimal smooth thickening of the septa
or wall, which may contain calcification that may
Prevalence DIAGNOSTIC TESTS & INTERPRETATION also be thick and nodular; no contrast
N/A Lab enhancement; includes totally intrarenal
r Urinalysis most often normal unless concurrent nonenhancing complex lesions >3 cm
RISK FACTORS These require follow-up (designated by the F
r Age, a known risk factor for simple renal cysts medical renal disease present
r Increasing age (7-fold increase from 4th8th decade r Renal function testscalculate eGFR and stage designation)
520% of Bosniak II/IIF cysts contain
or an increased incidence from 536%) chronic kidney disease (CKD)
malignancy in wall
r Polycystic kidney disease (autosomal dominant and Imaging Category III: Indeterminate cysts with thickened
recessive types) r Ultrasound irregular or smooth walls or septae; enhancement
r Hemodialysis Simple cyst present
In ESRD, cysts in 813% prior to hemodialysis (HD) No internal echoes, distinct walls with defined 4060% of these are malignant (cystic RCC and
1020% have acquired cystic renal disease after margins, spherical shape with no internal echoes multiloculated cystic RCC)
3 yr of dialysis, 4060% after 5 yr, and >90% Other class III lesions are benign (infected cysts
after 10 yr ALERT and multiloculated cystic nephroma)
Complex renal cysts including hyperdense cysts may Category IV: Characteristics of category III cysts
Genetics
r ARPKD: PKHD1 gene, chromosome 6, protein mimic solid renal masses. Doppler US is helpful. plus they contain contrast-enhancing soft tissue
r CT components that are adjacent to and independent
product fibrocystin
r ADPKD: PKD1 & PKD2 genes, chromosome 16, Simple cysts of the wall or septum
Have sharp walls with smooth margins, Risk of malignancy is 85100%
protein products polycystin-1,-2 spherical shape, homogenous throughout
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Vani S. Menon, MD
Derek Matoka, MD
PATHOPHYSIOLOGY
BASICS r Failure of ascent DIAGNOSIS
Anomalous vasculature impeding ascent; possibly
DESCRIPTION and abnormally situated umbilical artery HISTORY
r Renal ectopia describes a kidney that is located r UTIs (30%), vague abdominal pain or renal colic
Thought to occur at the 4th8th wk of gestation
outside of the normal orthotopic position within the r Incidentally during pre- or postnatal screening
Normal kidney ascent to the level of L2 at the end
renal fossa of the 8th wk of gestation r Abdominal mass, hypertension, hematuria,
r Positions for ectopia: r Abnormality of the ureteric bud or metanephric incontinence, renal insufficiency
Pelvic kidney: Below aortic bifurcation; this is the blastema PHYSICAL EXAM
most common ectopic position r Fusion abnormalities occur early in embryogenesis r Usually normal
Lumbar: Near sacral promontory Horseshoe kidney is the most common fusion r May find abdominal mass or flank tenderness
Abdominal: Above iliac crest anomaly r Genitourinary abnormalities
Cephalad: Seen in conjuncture with omphalocele Two renal moieties joined at lower pole in 90% of
when intra-abdominal organs herniate into the cases DIAGNOSTIC TESTS & INTERPRETATION
defect and cranial ascent of kidney is limited by r Anatomic considerations: Lab
the diaphragm Orthotopically located adrenal gland r Urine analysis and culture
Thoracic: Above the diaphragm with vasculature Ureter inserts into bladder in orthotopic position r BUN/Cr
arising from a cranial source Renal pelvis of ectopic kidney is usually anterior to
r Crossed fused renal ectopia Imaging
the parenchyma secondary to malrotation r If kidney absent on ultrasound (US), radionucleotide
Fusion occurs in up to 90% of cases Failure of development of fascial layers in the
Left to right crossing and more common in males imaging should be performed to evaluate for an
flanks on the side not occupied by renal tissue
Solitary and bilateral crossed varieties less r Malrotation of the ectopic kidney almost always ectopic kidney
common Average differential function of ectopic kidney is
occurs 35% (1)[C]
Type of anomaly is descriptive of the fusion
ASSOCIATED CONDITIONS r Diuretic renography if moderate-to-severe
anomaly
(inferior, lump, S-shaped [aka sigmoid], r Vesicoureteral reflux: Estimated incidence between pelvicalyceal dilation or progressive dilation found to
L-shaped, disc, or pancake) 20 and 30% evaluate for obstructive process
Fused unit usually caudal to the orthotopic renal Contralateral kidney demonstrates reflux in r Voiding cystourethrogram for febrile UTI and/or
moiety approximately 50% of cases pelvicalyceal dilation
r Horseshoe kidney is a noncross-fused ectopia Bilateral renal ectopia carries highest risk for r If kidney is nonfunctional, computed tomography
reflux>70% (1)[C] scan or abdominal US for localization
EPIDEMIOLOGY r Hydronephrosis: Seen in over 50% r Recent use of magnetic resonance urogram for
Incidence Half of these cases are due to either ureteropelvic small, poorly functioning kidneys can be utilized
r 1 in 500 to 1 in 1,290 in postmortem studies
junction obstruction (UPJO) or ureterovesical
Incidence is higher in autopsy series than in junction obstruction (UVJO) Diagnostic Procedures/Surgery
clinical studies, suggesting many clinically 25% of the hydronephrotic cases are secondary to N/A
insignificant and not recognized reflux and the remaining 25% due to malrotation Pathologic Findings
Left side favored over right (2)[C] N/A
r Pelvic kidney 1 in 2,200 and 1 in 3,000 r Genital anomalies: Estimated incidence between
r Crossed renal ectopia: Extremely rare DIFFERENTIAL DIAGNOSIS
15 and 45% r Horseshoe kidney
Prevalence 1020% of males will have cryptorchidism, r Malrotated kidney
N/A hypospadias, or duplicated urethras r Ptosis of orthotopically located kidney
2066% of females will have uterine or vaginal r Supernumerary kidney:
RISK FACTORS anomalies
Potential relationship with maternal r Cloacal anomalies: 14% of these patients will have Usually caudad to orthotopic kidney
illnesses/teratogenic exposure an ectopic kidney
Genetics r Nephrolithiasis
N/A r Recurrent urinary tract infections (UTIs)
GENERAL PREVENTION
N/A
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R
3. Gupta M, Lee MW. Treatment of stones associated
TREATMENT ONGOING CARE with complex or anomalous renal anatomy. Urol
Clinic N Am. 2007;34(3):431441.
GENERAL MEASURES PROGNOSIS 4. van den Bosch CM, van Wijk JA, Beckers GM, et al.
r Outcomes for treatment of nephrolithiasis and UPJO
Specific treatment for renal ectopia itself is not Urological and nephrological findings of renal
indicated. However, special considerations for are comparable to management of these entities in ectopia. J Urol. 2010;183:15741578.
associated conditions may be necessary. the orthotopic-positioned kidney
r Current literature suggests no adverse effects on
MEDICATION
First Line blood pressure or kidney function (4)[B] ADDITIONAL READING
Antibiotic prophylaxis for reflux based on clinical need r No evidence for increased risk of malignancy
Cinman NM, Okeke Z, Smith AD. Pelvic kidney:
Second Line COMPLICATIONS Associated diseases and treatment. J Endourol.
N/A r Vesicoureteral reflux 2007;21(8):836842.
r Nephrolithiasis
SURGERY/OTHER PROCEDURES See Also (Topic, Algorithm, Media)
r Nephrolithiasis Most likely due to urinary stasis r Horseshoe Kidney
r UPJO/UVJO r Malrotated Kidney/Renal Malrotation
Shock-wave lithotripsy, ureteroscopy,
percutaneous nephrolithotomy, laparoscopic r UTIs r Renal Dysplasia, Hypodysplasia and Hypoplasia
nephrolithotomy (3)[C] r Bowel laxity in the region of the empty renal fossa r Renal Ectopia Image
r UPJO r Traumatic injury to renal unit due to poor protection r Renal Fusion Anomalies
<15% are due to an aberrant crossing vessel in ectopic location r UPJO
Goal of management is to achieve dependent r UTI, Complicated, Pediatric
FOLLOW-UP
pelvic drainage r Urolithiasis, Pediatric, General Considerations
Dismembered pyeloplasty: Open and minimally Patient Monitoring
r Nephrolithiasis
invasive
Ureterocalicostomy Imaging by renal US and/or CT scans
Endopylotomy could be a consideration for failed
r Vesicoureteral reflux CODES
pyeloplasty but is rarely indicated as the initial VCUG and/or DMSA
surgical intervention r Hydronephrosis ICD9
r Vesicoureteral reflux Renal US and/or nuclear scans 753.3 Other specified anomalies of kidney
Open vs. endoscopic repair for clinically significant r Yearly blood pressure measurements
ICD10
reflux r Yearly BUN/Cr measurements r Q63.2 Ectopic kidney
Patient Resources r Q63.1 Lobulated, fused and horseshoe kidney
ADDITIONAL TREATMENT
r Urology Care Foundation: Ectopic Kidneys
Radiation Therapy
N/A http://www.urologyhealth.org/urology/
index.cfm?article=22 CLINICAL/SURGICAL
Additional Therapies PEARLS
N/A
REFERENCES r Renal ectopia carries an increased risk of urologic
Complementary & Alternative
Therapies abnormalities such as reflux, hydronephrosis, and
1. Guarino N, Tadini B, Camardi P, et al. The incidence genital abnormalities.
N/A r Over half the cases of reflux occur in the orthotopic
of associated urological abnormalities in children
with renal ectopia. J Urol. 2004;172:17571759. kidney.
2. Shapiro E, Bauer S, Chow J. Anomalies of the upper r >80% of ectopic kidneys will have differential
urinary tract. In: Campbell-Walsh Urology. 10th ed. function of approximately 35%.
Philadelphia, PA: Elsevier Saunders; 2012. r An anterior renal pelvis and anomalous vasculature
must be a consideration prior to surgical
intervention.
r Surgical interventions for nephrolithiasis have
similar success rates as for orthotopic kidneys.
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Matthew A. Meissner, MD
Ganesh V. Raj, MD, PhD, FACS
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R
Pathologic Findings SURGERY/OTHER PROCEDURES REFERENCES
r Acutely, histology demonstrates apoptosis of r Surgical intervention is not considered a primary
glomerular and renal tubular epithelial cells treatment for thromboembolic renal infarction. 1. Fergany A, Novick AC. Renovascular hypertension
r Chronic changes include necrosis and nuclear loss in Exceptions include: and ischemic nephropathy Chapter 39. In: Wein AJ,
glomeruli and tubules Young patients diagnosed within 6 hr of the ed. Campbell-Walsh Urology. 10th ed.
infarct. Philadelphia, PA: Elselvier/Saunders; 2012:
DIFFERENTIAL DIAGNOSIS Bilateral infarcts or infarcts in a solitary kidney. 10471083.
r Acute abdominal processes (acute mesenteric
r For traumatic injuries leading to renal infarction 2. Rajeev TP, Kohler TS, Ryndin I, et al. Case report:
ischemia, appendicitis, bowel obstruction)
r Cystic renal disease (avulsion of the renal pedicle), open surgical repair Renal infarct mimicking renal mass: Further
r Pyelonephritis may be attempted during exploration for other rationale for minimally invasive management.
injuries. J Endourol. 2007;21:10651068.
r Renal artery stenosis
ADDITIONAL TREATMENT 3. Lang EK, Sullivan J, Frentz G. Renal trauma:
r Renal calculi
Radiologic studies. Comparison of urographic,
r Renal tumor Radiation Therapy computerized tomography, angiography, and
r Renal vein thrombosis N/A
radionuclide studies. Radiology. 1985;154(1):16.
Additional Therapies
Percutaneous angioplasty of the renal artery or
TREATMENT thrombectomy ADDITIONAL READING
GENERAL MEASURES Complementary & Alternative Saeed K. Renal Infarction. Int J Nephrol Renovasc Dis.
r The optimal therapy for renal infarction is not clear Therapies 2012;5:119123.
r Initial therapy includes supportive measures with IV N/A
fluids and pain control See Also (Topic, Algorithm, Media)
r Since thromboembolic disease is the most common r Flank Pain
ONGOING CARE r Renal Colic
cause of renal infarction, primary anticoagulation is
r Renal Infarction Image
considered 1st line PROGNOSIS
r Other acute treatment options include thrombolysis, r The duration of renal ischemia is the critical factor in r Renal Trauma, Adult
endovascular stenting, and thrombectomy determining prognosis r Renal Trauma, Pediatric
r Prognosis also depends on the cause of the infarct r Renal Artery Stenosis/Renovascular Hypertension
MEDICATION r Sickle Cell Disease, Urologic Considerations
and the amount of parenchyma affected
First Line r Patients often die of illness related to the comorbid
r Antihypertensives to control hypertension
r Anticoagulation therapy: medical conditions causing the infarct
Heparin: Start with a bolus of 80 U/kg followed by COMPLICATIONS CODES
r Chronic renal insufficiency
a continuous infusion titrated to a therapeutic PTT
Begin warfarin therapy concurrently, goal INR of r Renal atrophy ICD9
r Hypertension r 440.1 Atherosclerosis of renal artery
23
r 584.5 Acute kidney failure with lesion of tubular
Continue in patients with known causes of
thromboembolic disease
FOLLOW-UP necrosis
Patient Monitoring r 593.81 Vascular disorders of kidney
Second Line r Regular blood pressure monitoring to assess for
r Thrombolytic therapy may be used, especially in
new-onset hypertension following infarct ICD10
unstable patients r Follow-up imaging to monitor the progression or r I70.1 Atherosclerosis of renal artery
Direct intra-arterial infusion to limit systemic side remission of an infarct r N17.0 Acute kidney failure with tubular necrosis
effects r Medical therapy for underlying condition that led to r N28.0 Ischemia and infarction of kidney
Many contraindications: Cerebral malignancy or
the infarct
AVM, history of cerebral hemorrhage, GI bleed, r Monitoring of serum creatinine
active hemorrhage, or aortic dissection CLINICAL/SURGICAL
Relative contraindications: Pregnancy, major Patient Resources
surgery within the last 3 wk, uncontrolled N/A PEARLS
hypertension r Renal infarction is most commonly due to
thromboembolic disease from underlying medical
conditions.
r Prompt diagnosis is paramount for preserving renal
parenchyma and function.
r Signs and symptoms may mimic more common GU
or intra-abdominal pathology.
r Renal infarction should be in the initial differential
diagnosis of nephrolithiasis and pyelonephritis.
r Anticoagulation therapy is the primary form of
treatment for renal infarction.
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R
r Nuclear medicine for split functional differentiation COMPLICATIONS
rarely needed if Cr <1.5 and kidney size and shape TREATMENT Surgical complications include hematoma,
similar to contralateral side pneumothorax, infection, adjacent organ injury (liver,
r Brain/bone imaging for mets if symptomatic GENERAL MEASURES spleen, pancreas, duodenum, and bowel), urinary
r Renal lesions suspicious for RCC are treated leak, myocardial infarction, thromboembolism,
Diagnostic Procedures/Surgery
surgically (laparoscopically or open) usually with positive surgical margins
Biopsy necessary for metastatic disease prior to
radical nephrectomy or partial nephrectomy (PNx).
initiation of systemic therapy and if concern for mets FOLLOW-UP
PNx use dependent on surgeon experience and
to the kidney. Indeterminate biopsy occurs 1020%. Patient Monitoring
location of tumor to hilum; most <34 cm (1). r AML: Renal US every 612 mo
Biopsy not routinely needed due to high positive r PNx decreases long-term risk of CVD mortality and
predictive value of enhanced imaging but has role in r Poor surgical candidate with SRM imaging every
ablation. ESRD vs. nephrectomy
r Most recommend surgery for Bosniak III (50% 6 mo alternating with renal US and CT scan with
DIFFERENTIAL DIAGNOSIS yearly CXR or chest CT
malignant) and IV cysts (100%) r Stage 13 RCC, 2030% relapse, lung most
r Adults: A solid primary renal mass in adult is most r TCC of the renal pelvis: By endoscopic ablation for
likely to be a RCC, although UCC or metastatic common (5060%), median relapse 12 yr,
small superficial lesions (low-grade Ta), or radical
disease is an important consideration evaluate every 6 mo for 2 yr, then annually.
nephroureterectomy
AML: Fat in a renal mass strongly suggests AML; r Asymptomatic AML >3.5 cm or small symptomatic Stage 4 RCC f/u dependent on primary treatment
fat-poor AML may resemble RCC and provider dependent.
lesions are treated by embolization, partial
Carcinoid tumors Patient Resources
nephrectomy, or nephrectomy
Collecting duct tumor (Bellini) r Painful simple renal cysts and infected cysts: Kidney Cancer Association www.kidneycancer.org/
Cystic nephromas
Cysts (simple, hemorrhagic, infected) Percutaneous aspiration and sclerotherapy
r Although cytoreductive nephrectomy is debated in
Focal pyelonephritis REFERENCE
Hemangioma post-IL-2 era, FDA approval of TKI & mTOR
Inflammatory masses (xanthogranulomatous inhibitors based on studies where almost half 1. Buethe DD, Spiess PE. Current management
pyelonephritis, abscess) patients had received a nephrectomy considerations for the incidentally detected small
Leiomyoma: Usually in renal capsule renal mass. Cancer Control. 2013;20(3):211221.
MEDICATION
Metanephric adenoma First Line
Metastasis: Lung, gastric, breast cancers most r Usually used for advanced mRCC
common; melanoma and others r Sunitinib or bevacizumab + IFN-: 1st line in
ADDITIONAL READING
Oncocytoma: Benign; cannot be reliably
low/intermediate risk r 2012 EUA Guidelines: http://www.uroweb.org/
differentiated from RCC on imaging studies r Temsirolimus: 1st line in high risk gls/pdf/10 Renal Cell Carcinoma LRV2.pdf
Pseudotumors (column of Bertin, others) r Pazopanib: 1st line and after cytokine failure r NCCN Guidelines: http://www.nccn.org/
Renal capsule neoplasm
r Interleukin-2 has more side effects than INF-. professionals/physician gls/pdf/kidney.pdf
RCC
Renal cortical adenoma: Controversial entity; High-dose IL-2 gives durable complete responders in See Also (Topic, Algorithm, Media)
cannot be distinguished from RCC on imaging: a limited number of patients. IL-2 can be r RCC, General Considerations
<2 cm, metastasis exceedingly rare monotherapy in selected good prognosis. r Renal Capsule Neoplasm
Renal lymphoma Second Line r Renal Mass, Algorithm
Renal medullary carcinoma r Sorafenib: 2nd line after cytokine failure r Renal Mass Image
Renal sarcomas: 12% of all renal masses r Everolimus: 2nd line after TKI r Renal Mass, Intraoperative Consultation
(leiomyosarcomas, fibrosarcomas, malignant r Renal Masses, Benign WHO, Classification
fibrous histiocytomas, anaplastic sarcoma) ADDITIONAL TREATMENT
r Renal Pseudotumors
Reninoma (JG apparatus tumors) Radiation Therapy
r Radiation used in pediatric tumors: Wilms stage r Renal Sarcoma, Adult and Pediatric
Urothelial carcinoma upper tract (UTUC)
r Benign renal mass in children: 34, all clear cell and rhabdoid stages r Reference Tables: TNM: Kidney Cancer
Choledochal cyst, intestinal duplication cyst r Metastatic RCC for pain/CNS mets in adults
Congenital mesoblastic nephroma Additional Therapies
Crossed-fused ectopia r RCC: Embolization prior to nephrectomy not CODES
Cystic nephroma (multiloculated cystic nephroma) beneficial but can be palliative for pain and bleeding
Hydronephrosis if nonsurgical candidate ICD9
Mesenteric cyst r Ablation (cryotherapy or radio frequency) of smaller r 189.0 Malignant neoplasm of kidney, except pelvis
MCDKinvoluted nonfunctional kidney r 593.9 Unspecified disorder of kidney and ureter
renal masses (<3 cm) may be considered in selected
Polycystic kidney disease
cases (elderly, poor surgical risk) (1)
Renal abscess ICD10
Splenomegaly Complementary & Alternative r C64.9 Malignant neoplasm of unsp kidney, except
r Malignant renal masses in children: Therapies renal pelvis
Lymphoma, lymphosarcoma N/A r N28.89 Other specified disorders of kidney and
Neuroblastoma (actually adrenal origin) ureter
Ossifying renal tumor of infancy ONGOING CARE
RCC (rare in children)
Sarcomas (clear cell, rhabdomyosarcoma) PROGNOSIS
CLINICAL/SURGICAL
WT (nephroblastoma): Renal mass in a child is WT r MSKCC (Motzer) criteria to predict survival of PEARLS
until proven otherwise patients with advanced RCC; for RCC, Kattan and r Much less than 10% of patients with RCC present
UCLA nomograms popular (based on 2002 TNM)
r RCC 5-yr cancer-specific survival (2010) is with the classic triad of hematuria, flank pain,
and a renal mass.
88100% stage T1T2, T3 4575%, T4 <530%; r A solid renal mass in childhood is WT until proven
+LN 030%, or +mets 010%
r Wilms: 4-yr survival stage 14 favorable histology otherwise.
(FH): 9098%, stage 5 FH 5687%; stage 15
unfavorable histology 66%; clear cell RCC any
stage 75%; rhabdoid sarcoma any stage 25%
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Gillian Stearns, MD
Oleg Shapiro, MD, FACS
Pathological-Findings (3,4)
BASICS DIAGNOSIS r The term oncocytoma is a general descriptor of an
epithelial tumor that consists of oncocytes.
DESCRIPTION HISTORY Oncocytes are large eosinophilic cells with small,
r Renal oncocytoma is the most common benign solid r Most patients asymptomatic
round, benign-appearing nuclei without nucleoli.
renal tumor in adults r Incidentally detected
Oncocytomas can arise in a number of different
r Usually asymptomatic at time of presentation r Gross hematuria/flank pain/flank mass rare organs
r Frequently detected incidentally at time of CT scan r Family history of renal tumors, fibrofolliculomas, r Renal oncocytoma: Gross findings
lung cysts/pneumothoraxrule out BHD Well-circumscribed mass, mahogany brown, often
EPIDEMIOLOGY
PHYSICAL EXAM with pseudocapsule
Incidence r No specific findings for sporadic oncocytoma Average size 67 cm
r 37% of all renal masses
r Palpable flank mass rare 33% with central stellate scar
r Epidemiology similar to renal cell carcinoma (RCC)
r Dermatologic exam if suspected BHD 20% demonstrate extension into perinephric fat
r Male > female (2:1) Calcifications and necrosis rarely seen
r Median age of diagnosis 62 yr DIAGNOSTIC TESTS & INTERPRETATION r Renal oncocytoma: Microscopic findings
r 6% occur bilaterally Round to polygonal eosinophilic cells
Lab
r 17% multifocal r No lab test can identify renal tumor as oncocytoma Abundance of mitochondria seen on electron
r Lab panel as with any newly diagnosed renal mass microscopy
Prevalence
CBC, chemistry panel, LFTs Mitotic figures rare
N/A
Regular nuclei
RISK FACTORS Imaging Cells arranged in distinct nests
r Familial renal oncocytoma syndrome r Cannot be used to reliably distinguish oncocytoma
May be difficult to distinguish from chromophobe
Described rarely to date from RCC RCC
r BirtHoggDube (BHD) r CT with and without IV contrast Colloidal iron stain positive in chromophobe
Diagnostic test of choice for solid renal mass RCC but not oncocytoma
Genetics Central scar within mass often seen in Chromophobe RCC is vimentin, cytokeratin 7
r Most frequent abnormalities (1,2):
oncocytoma, but this can be confused with positive
Loss of chromosome 1p necrosis, commonly seen with RCC CD82 and epithelial-related antigen (MOC31)
Loss of chromosome Y (males) r MRI may be helpful in the distinction between
r Less frequent translocations
Solid enhancing renal mass central scar chromophobe RCC and renal oncocytoma
Breakpoint region on 11q13 Test of choice with IV contrast allergy, renal Gene expression differences are being explored
Region encoding mDNA insufficiency The World Health Organization (2004) renal
Loss of heterozygosity at chromosomes 1,14,21 r Renal US tumor classification indicates renal oncocytomas
May reflect progression of oncocytoma to
Not typically helpful for identification of mass as are benign neoplasms. In the past some renal
chromophobe RCC oncocytoma oncocytomas were classified as malignant.
PATHOPHYSIOLOGY r Renal angiogram This may have resulted from confusion with
r Arise from intercalated cells in collecting duct of Spoke wheel pattern of feeding vessels clear cell renal carcinomas with eosinophilic
kidney (like chromophobe RCC) Not definitive for diagnosis component or due to eosinophilic chromophobe
r Occasionally oncocytoma and RCC may be found in Usually not helpful in evaluation of renal mass RCC (low metastatic potential)
the same kidney r Metastatic evaluation for solid renal mass
Chest x-ray vs. chest CT ALERT
ASSOCIATED CONDITIONS r Additional studies may be indicated clinically Although oncocytoma has Classic Findings on
r Most usually sporadic
r BHD imaging, no radiographic studies exist to
Diagnostic Procedures/Surgery differentiate benign oncocytoma from malignant
Autosomal dominant r Percutaneous biopsy
RCC.
Mutation in gene for folliculin May be useful to exclude metastasis to kidney
Renal tumors based on patient history
Chromophobe/oncocytoma May guide management of poor surgical DIFFERENTIAL DIAGNOSIS
r Adrenal mass
Spontaneous pneumothorax, lung cysts candidates
r Pitfalls of biopsy r Angiomyolipoma
Fibrofolliculomas, especially on face
r Carcinoid tumor
Difficult to distinguish oncocytoma from
GENERAL PREVENTION r Collecting duct tumor
chromophobe RCC
No preventative strategies have been described. r Cystic nephroma
Coexistence of RCC and oncocytoma in up to
Relatives of those with genetic syndromes may be r Cysts
10% cases
screened.
r Focal pyelonephritis
r Hemangioma
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R
r Inflammatory masses (xanthogranulomatous 5. Breen DJ, Bryant TJ, Abbas A, et al. Percutaneous
pyelonephritis (XGP), abscess) ONGOING CARE cryoablation of renal tumours: Outcomes from 171
r Leiomyoma tumours in 147 patients. BJU Int. 2013;112(6):
r Metanephric adenoma PROGNOSIS 758765.
r Oncocytoma is uniformly considered a benign tumor
r Metastasis 6. Romis L, Cindolo L, Patard JJ, et al. Frequency,
r Pseudotumor (column of Bertin) and surgical removal is curative clinical presentation, and evolution of renal
r Multiple series report no metastases or death from
r RCC oncocytomas: Multicentric experience from a
r Renal cortical adenoma oncocytoma on long-term follow-up european database. Eur Urol. 2004;45:5357.
r Older, rare reports of metastases may represent
r Renal lymphoma 7. Van der Kwast T, Perez-Ordonez B. Renal
unrecognized, low-grade RCC (6,7) oncocytoma, yet another tumour that does not fit
r Renal medullary carcinoma r Risk of metachronous oncocytoma 46% in the dualistic benign/malignant paradigm. J Clin
r Renal sarcoma
Pathol. 2007;60(6):585586.
r Reninoma COMPLICATIONS
r Perioperative for partial/radical nephrectomy
r Urothelial carcinoma
r Wilms tumor Bleeding, infection, urine leak
r Long term after nephrectomy depends on renal ADDITIONAL READING
reserve r Chen YT, Tu JJ, Kao J, et al. Messenger RNA
TREATMENT Chronic renal insufficiency/dialysis expression ratios among 4 genes predict subtypes of
FOLLOW-UP renal cell carcinoma and distinguish oncocytoma
GENERAL MEASURES from carcinoma. Clin Cancer Res. 2005;11:
Mainstay of treatment is surgical Patient Monitoring
r Patient monitoring 65586566.
r Dechet CB, Bostwick DG, Blute ML, et al. Renal
MEDICATION Long-term surveillance of renal units
First Line recommended annually to semiannually oncocytoma: Multifocality, bilateralism,
No medical treatment exists Metachronous ipsilateral and bilateral metachronous tumor development and coexistent
oncocytomas have been reported renal cell carcinoma. J Urol. 1999;162:4042.
Second Line r Renal US preferred modality r Linehan WM, Walther MM, Zbar B. The genetic
N/A basis of cancer of the kidney. J Urol. 2003;
Minimizes radiation exposure
SURGERY/OTHER PROCEDURES No need for IV contrast 170:21632172.
r Establishes diagnosis of oncocytoma r Urinalysis
r Partial nephrectomy whenever feasible based on See Also (Topic, Algorithm, Media)
Hematuria/proteinuria r BHD
size and location r Serum creatinine r Reference Tables: TNM: Kidney Cancer
r Radical nephrectomy rarely indicated unless very r Renal Cell Carcinoma, Chromophobe
large, uncertain diagnosis or partial not possible Patient Resources
r Kidney Cancer Association r Renal Cell Carcinoma, General Considerations
ADDITIONAL TREATMENT www.kidneycancer.org/ r Renal Mass, Algorithm
Radiation Therapy r Renal Mass, Intraoperative Consultation
No role r Renal Masses, Benign WHO, Classification
Additional Therapies
REFERENCES r Renal Oncocytoma Image
N/A r Renal Pseudotumors
1. Al-Saleem T, Cairns P, Dulaimi EA, et al. The
Complementary & Alternative genetics of renal oncocytosis: A possible model for
Therapies neoplastic progression. Cancer Genet Cytogenet.
r Renal cryotherapy and radiofrequency ablation (5) 2004;152:2328. CODES
Being studied as treatment option 2. Boris RS, Benhammou J, Merino M, et al. The
Unable to differentiate between oncocytoma and impact of germline BHD mutation on histological ICD9
RCC due to lack of tissue for histology concordance and clinical treatment of patients with 223.0 Benign neoplasm of kidney, except pelvis
Primarily for smaller lesions (<3 cm) bilateral renal masses and known unilateral ICD10
May be done laparoscopically or percutaneously oncocytoma. J Urol. 2011;185(6):20502055. r D30.00 Benign neoplasm of unspecified kidney
No long-term follow-up exists 3. Gudbjartsson T, Hardarson S, Petursdottir V, et al. r D30.01 Benign neoplasm of right kidney
Usually considered for poor surgical candidates Renal oncocytoma: A clinicopathological analysis of r D30.02 Benign neoplasm of left kidney
r Active surveillance 45 consecutive cases. BJU Int. 2005;96:
Good for select patients with solid renal masses, 12751279.
but no size can reliably differentiate between 4. Kuroda N, Tanaka A, Ohe C, et al. Review of renal CLINICAL/SURGICAL
benign and malignant processes oncocytosis (multiple oncocytic lesions) with focus
3 cm usually used as cutoff in BHD on clinical and pathobiological aspects. Histol
PEARLS
Histopathol. 2012;27(11):14071412. r Oncocytoma is a benign solid renal lesion.
r No imaging study reliably differentiates oncocytoma
from RCC. The CT finding of a central scar, previously
felt to be specific for oncocytoma, has been found
with RCCs, and this finding is not specific.
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PATHOPHYSIOLOGY r CT
BASICS r Kidneys are well protected in the retroperitoneum Contrast enhanced is best
Deceleration can lead to intimal tearing of renal Delayed films to evaluate urine leak and collecting
DESCRIPTION artery and thrombosis system
r Renal injuries can occur by either blunt or Medial urine extravasation
penetrating trauma ASSOCIATED CONDITIONS r IV urography (IVP)
r Rib fractures
Renal contusions, renal laceration, and renal r Injury to other organ systems While mostly replaced by CT scan, single shot
vascular injury are the general categories intraoperative IVP when pre-op imaging is not
r Renal injury classification: Based on American GENERAL PREVENTION available before abdominal exploration in the OR
Association for the Surgery of Trauma (AAST) renal r General trauma preventative measures with a film under patient on OR table
injury grading system (1) Restraints in motor vehicles Single plain film 10 min after 2 mL/kg of IV
Grade I: Subcapsular hematoma contrast (max 150 mL)
Grade II: Laceration <1 cm deep into cortex, small
hematoma with Gerotas fascia DIAGNOSIS Diagnostic Procedures/Surgery
Angiography may be performed if embolization is
Grade III: Laceration >1 cm into medulla, no HISTORY being considered
collecting system injury r Ample trauma history:
Grade IV: Laceration into collecting system, Allergies DIFFERENTIAL DIAGNOSIS
vascular segmental vein or artery injury, renal r Spontaneous hemorrhage in patients with renal
Medications
pelvis laceration and/or complete ureteral pelvic Past medical history mass: Traumatic or atraumatic
disruption Last meal Renal angiomyolipoma
Grade V: Main renal artery or vein injury or Event Renal cell carcinoma
thrombosis r Contrast allergy, previous renal surgeries, stones, Wunderlich syndrome: Atraumatic renal
Substratification of grade IV injuries (2)[B] hemorrhage
Grade IVb: Higher risk of intervention trauma, cancer r Injury to other organs
(angioembolization or exploration) if 2 or more PHYSICAL EXAM
Active vascular extravasation r Tachycardia and hypotension suggest major bleeding
Perinephric hematoma >3.5 cm r Primary survey TREATMENT
Medial/complex laceration
Flank contusion
Abdominal tenderness GENERAL MEASURES
EPIDEMIOLOGY r Supportive care
Incidence r Assessment of associated injuries
r 13% of all traumatic injuries ALERT
Degree of hematuria does not correlate with degree r Decision for nonoperative or operative management
r Most commonly injured GU organ of renal injuries (for operative management see
of injury.
Prevalence Surgery/Other Procedures below)
r Nonoperative management: Blunt trauma
Estimated 245,000 cases of traumatic renal injuries DIAGNOSTIC TESTS & INTERPRETATION
per year, world wide Hemodynamically stable patients with well-staged
Lab
r Urinalysis renal injury may be managed nonoperatively
RISK FACTORS
r Blunt trauma Hematuria >90% of renal injuries 97% blunt renal injuries can be managed
Rapid deceleration Hematuria absent in 36% renal vasculature nonoperatively (1)[B]
Motor vehicle injuries Monitor with serial Hct and imaging
Falls Hematuria with hypotension predictor for major Consider angiography and embolization as
Direct strike to abdomen or flank (sports injury renal injury alternative to renal exploration
related, bicycle accident, pedestrian in motor r Basic labs: Hgb, Hct, Cr, electrolytes
Large perinephric hematoma and extravasation
vehicle accident [MVA]) Imaging of contrast predictive for need of angiographic
r Penetrating trauma r Indications for imaging embolization (3)[B]
Upper abdominal Blunt trauma
Stab, gunshot, or industrial injury Isolated renal injuries
Hypotension (SBP < 90) and hematuria Most managed nonoperatively except for grade
r Iatrogenic injury Gross hematuria
V pedicle avulsion
Laparoscopic, endourologic, renal biopsy, Clinical indicators of renal injury from Consider placement of ureteral stent for
percutaneous procedures mechanism or associated injury persistent urine extravasation
Penetrating trauma
Any degree of hematuria
r US
Focused abdominal sonography for trauma (FAST)
used in some centers for detection of
hemoperitoneum
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r CT:
BASICS DIAGNOSIS Currently the most commonly used imaging
modality in these patients
DESCRIPTION HISTORY Triphasic abdominal and pelvic CT (ideally):
r Traumatic injury overall is the leading cause of r Mechanism of injury: Degree of actual traumatic
Clinically stable patients; most sensitive method
childhood death in the United States. injury may not correlate with the mechanism for diagnosing and classifying GU trauma,
r Pediatric renal trauma is subdivided into blunt and Blunt: Falls, automobile collision, sporting injuries, precontrast phase, nephrogram phase after
penetrating mechanisms of injury. etc. injection of contrast, and delayed images at
r The pediatric kidney is believed to be more Penetrating: Gunshot wound, stabbing, etc. 15 min. The downside of this is radiation exposure.
r Vital signs in the field:
susceptible to trauma vs. the adult kidney. Single-phase CT: Clinically labile patients; allows
r Over the past 2 decades, the management of Hypotension: Children will often have a normal BP for determination of renal perfusion and major
pediatric renal trauma has shifted from operative despite a significant blood loss. renal fractures. This can be followed by a KUB to
r Hematuria: Unlike adults, an unreliable indicator of assess renal integrity.
intervention to conservative management.
underlying renal injury in children: Delayed CT: Obtained postoperatively after patient
EPIDEMIOLOGY Up to 70% of children with grade II or higher is stabilized or after patient is resuscitated in the
Incidence renal injury may have neither gross nor ICU for full trauma evaluation; used to assess
r 1020% of all abdominal blunt trauma involves a microscopic hematuria. grade 35 renal injuries 23 days posttrauma to
renal injury. r Medical history: Any acute or chronic medical assess for baseline hematoma or urinoma
r 90% of GU injuries are from blunt trauma. conditions and any previous GU abnormality r Focused assessment with sonography for trauma
r Nearly 90% of patients with GU injuries have r Surgical history: Previous urologic procedure for (FAST):
coexisting injuries to the thorax, spine, pelvis, or reflux, stone, hypospadias, etc. Often combined with serial physical exams as a
intra-abdominal organs. r Iodine or latex allergy screening modality after blunt trauma
r Loss of consciousness Sensitivity ranges from 7085% and specificity
Prevalence
N/A ranges from 93100%; operator dependent
PHYSICAL EXAM Option in areas with limited radiologic resources
r Vital signs and ABCD of resuscitation to stabilize r Arteriography:
RISK FACTORS
r Pre-existing GU abnormalities (ie, ureteropelvic patient Used for diagnosis of arteriovenous fistula in the
junction obstruction horseshoe kidney vs. pelvic r BP is often normal in severely hypovolemic children
setting of delayed hemorrhage following renal
kidney): r Exposure: Observe for obvious signs of abdominal/ trauma
35-fold more common in pediatric patients flank/thoracic trauma, abdominal/flank tenderness, r Retrograde pyelography:
undergoing CT for trauma flank ecchymosis, gross hematuria, pelvic instability Rule out presence of partial/total ureteral
Classically presents with a history of hematuria r DRE: Observe for perineal ecchymosis disruption
disproportionate to the severity of trauma r If blood at the urethral meatus, do not insert Management of symptomatic urinoma with
r Decrease in physical renal protective mechanisms:
catheter placement of ureteral stent
More pliable thoracic cage and weaker abdominal r Single-shot IVP:
muscles ALERT Increasingly limited role
Less renal fat Degree of hematuria does not correlate with degree Done after patient is hemodynamically stable
Position of the kidney within the abdomen of injury. following trauma-exploratory laparotomy; allows
Genetics for visualization of functioning contralateral
Disorders that lead to an increase in GU anomalies DIAGNOSTIC TESTS & INTERPRETATION kidney when considering unilateral nephrectomy
have a greater risk for traumatic injury r DMSA scan:
Lab
r CBC, basic metabolic profile, coagulation profile Allows for quantification of renal function for
PATHOPHYSIOLOGY r Urinalysis
r Tissue or organ injury from external source of energy grade 35 injuries; obtain at least 1 wk after
r Grading system: Unreliable in determining the extent of GU trauma traumatic injury, also indicated
r Follow-up imaging:
Grade I: Subcapsular hematoma, microscopic or Up to 70% of children with grade II renal trauma
will have neither gross nor microscopic hematuria. Triphasic CT is indicated for patients with
gross hematuria, normal radiographic studies
persistent fever, worsening flank pain, or gross
Grade II: Nonexpanding perirenal hematoma or
ALERT hematuria >72 hr after injury
cortical laceration <1 cm deep
Grade III: Laceration >1 cm in parenchyma The patients hemodynamic status determines when Diagnostic Procedures/Surgery
without collecting system rupture or urine and what type of imaging modality is indicated. N/A
extravasation Pathologic Findings
Grade IV: Parenchymal laceration through renal Imaging N/A
cortex, medulla, collecting system; contained main r Indications for radiographic imaging: All penetrating
renal artery or vein hemorrhage abdominal trauma or blunt trauma victims with 1 of DIFFERENTIAL DIAGNOSIS
Grade V (shattered kidney): Renal pedicle the following criteria (1): Injury to other major abdominal viscera in the setting
avulsion, multiple parenchymal lacerations, major Significant deceleration or high-velocity injury: of acute trauma
injury to the renal vessels, urinary extravasation MVA, fall from >15 ft
Trauma resulting in fracture of the thoracic cage,
ASSOCIATED CONDITIONS spine, pelvis, or femur, or bruising of the
Injury to other organ systems
torso/perineum
GENERAL PREVENTION Acute peritonitis
Measures that decrease traumatic injury in general, Gross hematuria
such as seat belts, air bags Microscopic hematuria (>50 RBC/HPF) associated
with shock (SBP <90 mm Hg)
Delayed hemorrhage following renal trauma
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ASSOCIATED CONDITIONS
BASICS r Acquired RTA type I: DIAGNOSIS
Autoimmune disease: Systemic lupus
DESCRIPTION erythematosus (SLE), Sjogren syndrome, primary HISTORY
r Renal tubular acidosis (RTA) is a metabolic condition r Failure to thrive, rickets, and osteomalacia in children
biliary cirrhosis, chronic active hepatitis
characterized by abnormal urinary acidification due r Anorexia, nausea, vomiting
Chronic pyelonephritis
to a defect in renal tubules, resulting in Diseases causing nephrocalcinosis r Weakness and polyuria due to potassium loss
hyperchloremic nonanion gap metabolic acidosis, Drugs (amphotericin B, lithium, analgesics) r Constipation
increased pH of the urine EhlersDanlos syndrome r Polydipsia
r 4 major types of RTA are now considered to be Fabry disease r History of hematuria, urinary tract infections (UTIs),
only 3: Glycogenosis type III passage of stones in urine
Type I (distal): Defective distal tubular H+ Hepatic cirrhosis r History of recurrent, familial, or childhood renal
secretion Hypercalciuria stone disease
Type II (proximal): Defective proximal tubular Hypergammaglobulinemic syndrome r Ask about systemic diseases causing RTA
bicarbonate reabsorption Leprosy
Type III (mixed): No longer considered as a distinct Malnutrition PHYSICAL EXAM
entity Medullary cystic disease r Urologic exam of genitalia, suprapubic area for
Type IV: Aldosterone deficiency/resistance Obstructive uropathy swelling and tenderness
Sickle cell disease, hereditary elliptocytosis r Exam for osteomalacia, hypokalemic muscle
EPIDEMIOLOGY
Toxins (toluene, glue) weakness, and growth retardation
Incidence Vitamin D intoxication r Exam for other systemic diseases
r RTA I: More common in adults (2/3 adults, 1/3
Wilson disease
children) and women; endemic in certain regions of r Acquired RTA type II: DIAGNOSTIC TESTS & INTERPRETATION
Thailand Fanconi syndrome due to toxin-related or Lab
r RTA II: Usually more predominant in males r Renal function test usually normal
immunologic nephrotoxic damage
associated with Fanconi syndrome; urinary loss of Tubular toxicity causing acute tubular necrosis: r Electrolytes and blood gas reveal hyperchloremic,
glucose, amino acids, uric acid, phosphate, and Sepsis nonanion gap metabolic acidosis:
bicarbonates Rhabdomyolysis Hypokalemia or normokalemia in type I and II
r Most RTAs are sporadic occurring at any age. Hypotension Hyperkalemia in type IV
Familial RTA are rare and usually occurs in childhood. Nephrotoxins: Intravenous (IV) contrast, r Urine pH (fasting, under oil, pH meter):
RISK FACTORS aminoglycoside antibiotics pH > 5.5: Complete type I RTA
r Genetic disorders Interstitial renal disease: pH > 5.5, but systemic acidosis mild or absent:
r Secondary to systemic disease. See Commonly Multiple myeloma Ammonium chloride loading test and measure
Heavy metal poisoning (cadmium, lead, urinary bicarbonates; failure of urine pH to go
Associated Conditions.
mercury) below 5.5 is diagnostic of RTA (1)[C]
Genetics Medications (methicillin, cisplatin, adefovir, r RTA II is diagnosed by bicarbonate loading test:
r Familial RTA I (1):
tenofovir, COX-2 inhibitors, cimetidine, After IV bicarbonate infusion, fractional excretion of
Autosomal dominant (AD) form is associated with acetazolamide, sulfanilamide, ifosfamide, bicarbonate >15% is diagnostic (1)[C]
mutation in anion exchanger 1 gene tetracycline, Topamax) r Urine calcium: High in type I, normal in type II
Autosomal recessive (AR) form is due to a Infections: Leptospirosis, corynebacterium, r Phosphaturia, glycosuria, and aminoaciduria in
mutation in the B1 or H+ -ATPase (V-ATPase) gene diptheria, polyomavirus, cytomegalovirus Fanconis syndrome
and associated with sensorineural deafness Autoimmune disease: SLE, Sjogrens syndrome,
r Familial RTA II: sarcoidosis Imaging
r Plain x-ray and computed tomography (CT)
AD form is rare Amyloidosis
AR form associated with ocular abnormalities and r Acquired RTA type IV: urogram:
mental retardation Addison disease Likely to demonstrate nephrocalcinosis and
AR form associated with osteopetrosis and Diabetic nephropathy nephrolithiasis
cerebral calcification Hypertension Pathologic Findings
AR form associated with Fanconi syndrome Lupus nephropathy r Nephrocalcinosis
r Familial RTA IV: Associated with Obstructive nephropathy r Nephrolithiasis
pseudohypoaldosteronism type 1 Tubulointerstitial nephropathies r Osteomalacia
r Gordon syndrome
PATHOPHYSIOLOGY
r Type I (distal) tubular acidosis: Secondary to r Sickle cell nephropathy
impaired ability to secrete hydrogen ions into the GENERAL PREVENTION
distal tubule or collecting duct. Urine pH > 5.5. N/A
r Type II (proximal) tubular acidosis: Impaired
bicarbonate absorption in the proximal tubule. Urine
pH may be <5.5.
r Type IV: Presence of aldosterone resistance or
deficiency leading to hyperkalemia (not seen in type
I and II) along with acidosis. Urine pH may be <5.5.
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Second Line
N/A
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r Adult:
BASICS Abdominal tumors, especially renal cell carcinoma DIAGNOSIS
Endothelial damage
DESCRIPTION Intrinsic hypercoagulability (eg, Factor V Leiden HISTORY
r Renal vein thrombosis (RVT) is an acute or chronic r Newborn/infant:
deficiency)
thrombosis in the renal vein leading to a reduction Nephrotic syndrome: Risk factors: Mothers history, birth, and early
in venous drainage of the kidney. Membranous nephropathy: Lesion most frequently postnatal course
r In infants, RVT typically presents as a severe illness, associated with nephrotic syndromerelated RVT; Gross hematuria
r Adult:
occasionally with colicky pain: also reported in many other nephropathies.
60% have enlarged kidneys on physical exam; Use of oral contraceptives, steroids Sudden onset of hematuria and flank pain should
gross hematuria and microangiopathic hemolytic Renal transplantation, particularly in those taking raise the question of RVT, as should a history of
anemia and thrombocytopenia can also be present OKT-3 and cyclosporine for immunosupression nephrotic syndrome in the presence of hematuria
r In the adult, RVT presentation depends on onset of Shock, sepsis, dehydration PHYSICAL EXAM
RVT: Trauma r Newborn/infant:
Acute RVT: Triad of sudden flank pain Use of IV contrast agents Unilateral, or often bilateral, flank masses
Costovertebral angle tenderness and gross Vasculitis Evidence of dehydration
hematuria only present in minority of cases. Compression from aortic aneurysm, Evidence of cyanotic heart disease
Chronic RVT: Generally asymptomatic; lymphadenopathy, retroperitoneal fibrosis (2) If the thrombosis is bilateral, oliguria may be
Can have proteinuria and microscopic or gross Genetics present; urine output may be normal with a
hematuria. Unknown unilateral thrombus (4)
r Adult:
EPIDEMIOLOGY PATHOPHYSIOLOGY
Incidence r Newborn/infant: Evidence of blunt trauma, abdominal mass
r Newborns and infants: Diminished intrarenal blood flow due to
Edema or anasarca suggestive of nephrotic
Commonly associated with hypoxia, dehydration, syndrome
hypovolemia (sepsis, dehydration, diarrhea)
shock, and/or sepsis Creates prothrombotic state DIAGNOSTIC TESTS & INTERPRETATION
Usually acute and unilateral (more common on Clot can then propagate in antegrade and/or Lab
the left side); although 30% bilateral retrograde manner, resulting in RVT r Newborn/infant:
Male-to-female ratio 2:1 in the neonate, with no May become bilateral, produce vena caval Thrombocytopenia, leukocytosis, hemolytic anemia
sex predilection beyond age 1 occlusion and/or renal artery thrombosis Consumptive coagulopathy (prolonged clotting
Accounts for approximately 10% of venous 65% in neonates, 30% beyond 1 yr age time, elevated fibrinogen and fibrin split products)
thrombosis in newborns Associated with adrenal hemorrhage in 15% of Proteinuria
Most common form of thrombosis not associated cases Elevated BUN and creatinine
with a vascular catheter (1) r Adults: Most often unilateral; acute and chronic r Adult:
r Adults: forms described: Proteinuria and microscopic hematuria
Associated with nephrotic syndrome (reported Acute RVT: Severe dehydration, sudden Hemolytic anemia, consumptive coagulopathy,
incidence of RVT in patients with nephrotic hypercoagulability, renal vein obstruction from and thrombocytopenia may be present
syndrome ranges from 562%), renal cell tumor or transplant rejection Elevated BUN and creatinine
carcinoma, or renal transplantation Chronic RVT: Hypoalbuminemia
More often chronic and unilateral Nephrotic syndrome leads to alterations in Marked elevation in LDH with normal
Prevalence coagulation pathway that creates prothrombotic transaminases
N/A conditions (3)
RVT is a result of nephrotic syndrome and not Imaging
r Newborn/infant:
RISK FACTORS the cause
r Newborn/infant: Slow onset allows the development of collateral The extent of thrombus may be assessed by
Acute hypoxia venous kidney drainage duplex ultrasonography, and only rarely will CT,
Birth trauma MRI, or renal venography be required for
Cyanotic congenital heart disease with ASSOCIATED CONDITIONS confirmation of the diagnosis or determination of
r DVT in patients with nephrotic syndrome the extent of thrombus
polycythemia
r Pulmonary embolus Ultrasonography: Enlarged and echogenic kidneys
Cytomegalovirus infection
Dehydration from diarrhea/vomiting (90%) with attenuation or loss of corticomedullary
GENERAL PREVENTION
Maternal diabetes, polyhydramnios r Adults: Long-term anticoagulation is appropriate if differentiation
Hyperosmolar state form angiocardiography Doppler studies may detect increased resistance
RVT has recurred when patients discontinued
Preterm (<36 wk) infants at greater risk or absence of flow in renal venous branches;
anticoagulation
Sickle cell disease r Treatment of underlying cause increased resistance in the renal artery may be
present
Doppler ultrasonography is the primary modality
for the detection of RVT; however, its utility is
operator specific (3)
IVP: Delayed opacification and renomegaly
A renal scan may be obtained to assess the
function of the involved kidney.
MRI is more expensive and requires sedation in
pediatric patients (6)
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RETROGRADE EJACULATION
Pravin K. Rao, MD
PATHOPHYSIOLOGY
BASICS r Normal ejaculation requires: DIAGNOSIS
Seminal emission
DESCRIPTION Bladder neck closure HISTORY
r Expulsion of semen from posterior urethra into r Absence or presence of orgasm
Antegrade expulsion from urethra
bladder with low- or zero-volume antegrade r Neurologic control: r Presence of erectile dysfunction
ejaculate. r Cloudy urine after sex/orgasm
Central control in multiple brain regions
r Suspected in men with symptoms or semen analysis Can promote or inhibit ejaculation r Symptoms of hypogonadism
findings suggesting low or absent ejaculate volume. Sympathetic (T12L3): r Medical history (see risk factors)
r Primary implications are for infertility. Hypogastric nerve (thoracolumbar) r Surgical history (see risk factors)
Also: Sexual function/satisfaction. Seminal emission into posterior urethra by r Medications (see risk factors)
r No other known medical health effects. contraction of epididymis, vas deferens/ampulla, r Accuracy of semen analysis findings:
r Men with failure of emissions are often labeled as seminal vesicle (SV), and prostate smooth Low measured volume may be due to spillage
having retrograde ejaculation. muscle Some patients report subjective low volume only
Failed deposition of ejaculate contents into Bladder neck closure preventing retrograde
at time of sample collection
posterior urethra before expulsion. ejaculation Anxiety due to lab/atmosphere
Parasympathetic (S2S4): Anxiety related to medical condition
EPIDEMIOLOGY Pelvic nerve
Incidence Gland secretions of prostate SV PHYSICAL EXAM
r Usually normal physical exam findings
r 7478% incidence after transurethral prostate Somatic (S2S4):
Pudendal nerve r Absent vasa suggests congenital absence of the vas
surgery (1)[B]
r 426% incidence with -blocker tamsulosin Efferents from sacral cord deferens
Contraction of bulbocavernosal and r Small testes may suggest hypogonadism
(2,3)[A] r Seminal vesical (SV) dilation may suggest
r As low as 14% incidence after bilateral ischiocavernosal muscles
Relaxation of external urethral sphincter ejaculatory duct obstruction (EDO)
nerve-sparing retroperitoneal lymph node Projectile expulsion of ejaculate r Muscle weakness or focal neurologic deficit may
dissection (RPLND) (4)[B] Sensory suggest primary neurologic cause
Prevalence Pudendal nerve
N/A Tactile stimulation of penis can activate DIAGNOSTIC TESTS & INTERPRETATION
ejaculatory reflex Lab
RISK FACTORS r Retrograde ejaculation occurs from impaired bladder r If ejaculate volume <1.5 cc, consider
r Bladder neck/prostate procedures
neck closure due to various causes postejaculatory urinalysis (PEU)
Transurethral resection of the prostate r PEU
Transurethral incision of the prostate (TUIP) Poor coaptation of bladder neck
Medication side effect >1015 sperm/HPF is diagnostic for retrograde
Bladder neck incision (BNI)
r Surgical/traumatic/congenital neuropathy Prostate surgery ejaculation
Idiopathic Small number of sperm may be normal
Retroperitoneal surgery
eg, RPLND Neural disruption Technique:
Spinal cord injury (SCI) Abstain from ejaculation 23 days
Pelvic surgery Diabetes mellitus (DM) Empty bladder
eg, abdominoperineal resection
Retroperitoneal lymphnode dissection (RPLND) Collect/attempt antegrade ejaculate
Spinal cord injury (SCI)/surgery Pelvic surgery Collect urine by void or catheter
Spina bifida/myelomeningocele r Endocrine evaluation if clinical suspicion for
r Medical neuropathy ASSOCIATED CONDITIONS
Diabetes mellitus (DM) r See risks factors hypogonadism
Multiple sclerosis (MS) r Benign prostatic hyperplasia (BPH) Imaging
r Iatrogenic from medications r Bladder neck dysfunction Only performed for concurrent medical conditions
-blockers r Diabetes mellitus (DM) Diagnostic Procedures/Surgery
Reduce bladder neck muscle tone r Multiple sclerosis (MS) N/A
Reduce seminal emissions r Rectal cancer
Tamsulosin, terazosin, doxazosin Pathologic Findings
r Testicular cancer N/A
Antipsychotic and psychotropic medications
Risperidone GENERAL PREVENTION
Antidepressants r Avoidance of iatrogenic causes
Selective serotonin reuptake inhibitors r Nerve sparing during RPLND
Genetics
N/A
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RETROGRADE EJACULATION
R
DIFFERENTIAL DIAGNOSIS SURGERY/OTHER PROCEDURES 3. Lepor H. Long-term evaluation of tamsulosin in
r Anejaculation r Sperm retrieval benign prostatic hyperplasia: Placebo-controlled,
r Anorgasmia (inability to reach orgasm) For use with assisted reproductive techniques double-blind extension of phase III trial. Tamsulosin
r Aspermia due to failure of emission IUI (intrauterine insemination) Investigator Group. Urology. 1998;51:901906.
r Congenital bilateral/unilateral absence of the vas IVF In vitro fertilization 4. Steiner H, Zangerl F, Stohr B, et al. Results of
ICSI Intracytoplasmic sperm injection bilateral nerve sparing laparoscopic retroperitoneal
deferens
r EDO r Sperm retrieval technique lymph node dissection for testicular cancer. J Urol.
r Erectile dysfunction Prior to collection, alkalinize urine to pH 7 2008;180(4):13481352.
Sodium bicarbonate 650 mg QID or 5. van der Linden PJ, Nan PM, te Velde ER, et al.
Failure to reach orgasm
Poor expulsion of ejaculate through flaccid penile 13 tablespoons of baking soda, 1248 hr Retrograde ejaculation: Successful treatment with
urethra before collection artificial insemination. Obstet Gynecol. 1992;
r Hypogonadism Catheterize or void for collection 79:126128.
r Sperm retrieval from the testis and epididymis, an
r Semen spillage in lab
r Poor semen collection technique option for unsuccessful retrograde collection
Adoption and use of donor sperm can prevent the ADDITIONAL READING
need for IVF/ICSI
Ohl DA, Quallich SA, Snksen J, et al. Anejaculation
TREATMENT ADDITIONAL TREATMENT and retrograde ejaculation. Urol Clin North Am.
Radiation Therapy 2008;35:211220.
GENERAL MEASURES N/A
r Treatment typically reserved for fertility purposes See Also (Topic, Algorithm, Media)
r Treat reversible causes Additional Therapies r Anorgasmia/Dysorgasmia
r Modify causative medications N/A r Ejaculatory Disturbances
Change or discontinue causative medications Complementary & Alternative r Infertility, Urologic Considerations
Some clinicians favor alfuzosin for BPH (possibly Therapies r Semen Analysis, Abnormal Findings and Terminology
less RE than other -blockers) N/A r Semen Analysis, Technical and Normal Value
MEDICATION
First Line ONGOING CARE
r -adrenergic agents CODES
Dosing structure highly variable: PROGNOSIS
r Success rates with assisted reproductive techniques
Pseudoephedrine 60 mg ICD9
Ephedrine 2550 mg largely dependent on female factors r 355.9 Mononeuritis of unspecified site
r 44% pregnancy rates with intrauterine insemination r 608.87 Retrograde ejaculation
Imipramine 2550 mg (may cause dizziness and
nausea) (5)[C] r 606.9 Male infertility, unspecified
Frequency ranges from QD to QID COMPLICATIONS
Duration ranges from 214 days r Main issue is infertility ICD10
Side effects: HTN, tachycardia r G62.9 Polyneuropathy, unspecified
r Emotional distress
r Author recommendation: Pseudoephedrine 60 mg r N46.8 Other male infertility
QID 27 days prior to ejaculation (titrate to FOLLOW-UP r N53.14 Retrograde ejaculation
effectiveness) Patient Monitoring
r Medical therapy less likely to be effective after Follow up semen analysis to determine effectiveness
bladder neck injury or surgery of medical therapy CLINICAL/SURGICAL
Second Line Patient Resources PEARLS
N/A MedlinePlus: Retrograde Ejaculation http://www.nlm.
In men with spinal cord injury (SCI) or history of
nih.gov/medlineplus/ency/article/001282.htm
retroperitoneal surgery, men may have failure of
seminal emission, so sperm retrieval from the bladder
REFERENCES may not be feasible.
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RETROPERITONEAL ABSCESS
Jessica H. Hannick, MD
Ahmer V. Farooq, DO
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RETROPERITONEAL ABSCESS
R
r MRI: SURGERY/OTHER PROCEDURES
r Early percutaneous drainage (5)
REFERENCES
Thick purulent collections have high-intensity
signal on T1-weighted images Essential in lesions >3 cm 1. Solomkin JS. Peritonitis, pancreatitis and
Edema in surrounding fat seen as high signal on May consider antibiotics only in abscesses <3 cm intra-abdominal abscesses. In: Cohen J, Powderly
T2-weighted images r Surgical drainage must be considered if: WG, eds. Cohen & Powderly: Infectious Diseases.
High soft tissue contrast resolution sensitive for Safe percutaneous drainage not possible 2nd ed. Philadelphia, PA: Mosby; 2004:517527.
detecting psoas muscle pathology and Percutaneous drainage has failed 2. Anaya DA, Dellinger EP. Surgical infections and
intervertebral disc involvement Multiple abscesses choice of antibiotics. In: Townsend CM Jr,
May not show calcifications or gas collections Multiloculated abscesses Beauchamp RD, Evers M, et al., eds. Sabiston
r US: Can reveal gas/fluid collections Purulent material too thick to be drained Textbook of Surgery. 18th ed. Philadelphia, PA:
r KUB: May show psoas shadow, loss of renal outline, If patient is persistently febrile after 4872 hr of Saunders Elsevier; 2008.
displacement of organs, gas, or urolithiasis appropriate antibiotics 3. Brook I, Frazier EH. Aerobic and anaerobic
r Gallium67 citrate and indium111 chloride scanning If primary cause must be addressed surgically (ie, microbiology of retroperitoneal abscesses. Clin
can be helpful: xanthogranulomatous pyelonephritis (XGP), Infect Dis. 1998;26:938941.
False positives: Pyelonephritis, acute tubular malignancy, urolithiasis) 4. Crepps JT, Welch JP, Orlando R 3rd. Management
necrosis, vasculitis, and neoplasms r Surgical approach should be retroperitoneal unless and outcome of retroperitoneal abscesses. Ann
r Chest x-ray may show elevation of hemidiaphragm, pancreatic pathology is present: Surg. 1987;205:276281.
pleural effusions, secondary pneumonia Obtain cultures 5. Tunuguntla A, Raza R, Hudgins L. Diagnostic and
Irrigate abscess cavity aggressively therapeutic difficulties in retroperitoneal abscess.
Diagnostic Procedures/Surgery Use drains liberally
r CT, MRI, or US-guided aspiration and drainage of South Med J. 2004;97:11071109.
abscess cavity ADDITIONAL TREATMENT
r Specimens must be sent for both aerobic and Radiation Therapy
anaerobic cultures No role ADDITIONAL READING
r Consider sending for AFB culture r Brook I. Microbiology and management of
Additional Therapies
Pathologic Findings N/A abdominal infections. Dig Dis Sci. 2008;53:
Coagulation necrosis 25852591.
Complementary & Alternative r Heller MT, Haarer KA, Thomas E, et al. Acute
DIFFERENTIAL DIAGNOSIS Therapies
r Malignancy N/A conditions affecting the perinephric space: Imaging
r Necrotizing fasciitis anatomy, pathways of disease spread, and
differential diagnosis. Emerg Radiol. 2012;19:
r Osteomyelitis ONGOING CARE 245254.
r Pancreatitis r Negus S, Sidhu PS. MRI of retroperitoneal
r Perforated viscus (ie, duodenal ulcer) PROGNOSIS collections: A comparison with CT. Br J Radiol.
r Mortality is considerable (550%) despite modern
r Perinephric aneurysm/pseudoaneurysm 2000;73:907912.
r Perinephric/perirenal abscess management that combines antibiotics, drainage,
and intensive care support. See Also (Topic, Algorithm, Media)
r Psoas abscess r High success with antibiotics and percutaneous r Psoas Abscess, Urologic Considerations
r Pyelonephritis r Renal and Perirenal Abscess
drainage (>80%):
r Ruptured aortic aneurysm r Only 14% recurrence with percutaneous drainage r Retroperitoneal Abscess Image
r TB r If abscess is not drained and only antibiotics are r Retroperitoneal Hematoma
r Trauma/retroperitoneal hematoma r Retroperitoneal Mass and Cysts
used, mortality approaches 100%
r Urinoma
COMPLICATIONS
r Abscess crossing the midline to opposite side or
TREATMENT tracking into the ipsilateral thigh CODES
r DVT
GENERAL MEASURES r GI bleed ICD9
r Supportive care r 567.31 Psoas muscle abscess
r Organ failure
r DVT prophylaxis r 567.38 Other retroperitoneal abscess
r Pneumonia
r Secondary infections: Osteomyelitis, involvement of r 998.59 Other postoperative infection
MEDICATION
First Line psoas muscle, fistulization to the skin ICD10
r Broad-spectrum antibiotics to empirically cover most r K68.11 Postprocedural retroperitoneal abscess
FOLLOW-UP
likely pathogens (ampicillin, gentamicin, and r K68.12 Psoas muscle abscess
metronidazole) (4) Patient Monitoring
r Reimaging necessary r K68.19 Other retroperitoneal abscess
Refine antibiotic coverage based on culture results
Tailor duration of treatment to clinical progress CT or MRI (MRI avoids radiation)
Timing depends on clinical progress
Second Line r Drains: CLINICAL/SURGICAL
N/A Must be monitored carefully and irrigated PEARLS
appropriately r Nonspecific signs and symptoms frequently lead to a
r Can be removed when:
delay in diagnosis and treatment.
Patient is clinically improved r Early percutaneous drainage is essential in lesions
Drainage stops (<10 mL/d) or becomes serous
Abscess cavity involution is documented on >3 cm.
imaging
Patient Resources
N/A
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RHABDOMYOLYSIS
Sanjay S. Kasturi, MD
Leonard G. Gomella, MD, FACS
PATHOPHYSIOLOGY
BASICS r Muscle cell destruction DIAGNOSIS
Pressure or crush
DESCRIPTION Cellular hypoxia HISTORY
r Rhabdomyolysis is muscle necrosis resulting in the r Reperfusion injury results in large quantities of r Trauma
egress of cellular muscle particles (namely r Sepsis
potassium, phosphate, myoglobin, CK, and urate
myoglobin, potassium, creatine kinase [CK], and r New medication
leak into the circulation
lactic acid dehydrogenase [LDH]) into the blood Electrolyte alterations further impact cellular r Toxin exposure, drug use or infection
stream. integrity r Excessive muscle use
r In particular myoglobin is harmful to the kidney and r Normal plasma myoglobin is very low r Electrolyte or endocrine disorder
often causes acute kidney injury. (00.003 mg/dL) r Prolonged operating room timemost common
Up to 15% of patients who have rhabdomyolysis With >100 g of skeletal muscle damaged, serum reason in urology (2,3,4)
can have renal failure. haptoglobin binding capacity becomes saturated Most commonly reported in the laparoscopic
r There are many causes for rhabdomyolysis; this At this point circulating myoglobin becomes nephrectomy data when patients are in flank or
section primarily focuses on operative causes. free and is filtered by the glomeruli modified flank position for >6 hr but also seen in
EPIDEMIOLOGY Myoglobin precipitates in the kidney and cause exaggerated lithotomy and steep Trendelenburg
renal tubular obstruction, potentially leading to Male sex predominates
Incidence acute kidney injury
r 2,600 cases per year (likely underreported) Elevated BMI, reported mean BMI of 33.2 in
r Myoglobin levels return to normal values in 16 hr review of the laparoscopic nephrectomy data
r Overall incidence of 04.9% in the laparoscopic
after injury due to hepatic metabolism and renal
nephrectomy data excretion
PHYSICAL EXAM
r Generalized fatigue, nausea, fevers
RISK FACTORS r Up to 12 L of fluid may be sequestered in the
r Trauma and immobilization (1) r Mental status changes
necrotic muscle tissues r Skin discoloration
Lying unconscious on hard surface under the This relative hypovolemia is an additional cause of
r Muscular pain and swelling (symptoms may be out
influence of alcohol or drugs renal failure in rhabdomyolysis
Crush injury Free iron, which catalyses free radical production of proportion with exam)
Prolonged compression as seen in excessive further enhances ischemic renal tubule damage r Muscle weakness
operating times (esp. males) r Compartment syndrome r Symptoms may be absent in 50% of patients
Laparoscopic, robotic, and open Caused by insufficient blood supply to muscles due r Reddish-brown urine
Dorsal lithotomy positioning to increased pressure within a body compartment r Classic triad of muscle pain, weakness, and dark
r Elevated BMI (esp. muscle mass) (arm, leg, any enclosed space within the body) urine
r Sepsis and shock 6 Ps associated with compartment syndrome:
r Toxins (spider and snake venom [mostly in South Pain out of proportion based on exam, DIAGNOSTIC TESTS & INTERPRETATION
America, Asia, Africa]) paresthesia, pallor, paralysis, pulselessness, and Lab
r Myoglobinuria:
r Medications (cocaine, Ecstasy [MDMA] LSD, pressure
For most prolonged operative cases the Myoglobin only appears in the urine when serum
amphetamines, statins [especially in combination
paraspinous muscles and the extremities are at level >1.5 mg/dL
with fibrate-derived lipid-lowering agents such as Red-brown urine when urine levels >100 mg/dL
niacin], cyclosporin, itraconazole, colchicine, risk for compartment syndrome
Positive for blood on urine dipstick but no RBCs
steroids) ASSOCIATED CONDITIONS
r Infections (HIV, influenza) r Acidosis suggests myoglobinuria (5)
Short half-life only 23 hr, so it may return to
r Excessive muscle use (status epilepticus, prolonged r Cardiac arrest (hyperkalemia)
normal if muscle damage is limited
exercise) r Compartment syndrome r Elevated CK levels (5 upper limit of normal which
r Electrolyte and endocrine abnormalities r Disseminated intravascular coagulation (DIC) is about >1,000 U/L)
(hyponatremia, hyperthyroidism, ketoacidosis) Activation of the coagulation cascade by the CK half-life of 26 hr and remains elevated longer
r Electrical shock injury, lightning strike substances released from damaged muscle cells than myoglobin, peaks at 13 days and declines
r 3rd-degree burns r Hepatic dysfunction at 35 days after all muscle injury has stopped
r High body temperature, heat stroke, malignant r Myoglobinuric acute renal failure r Basic metabolic panel
hyperthermia Monitor for acute renal failure and hyperkalemia
r Myopathy (eg, Duchenne muscular dystrophy) GENERAL PREVENTION r Calcium level:
r Avoid immobilization or prolonged operating times
Genetics r Appropriate patient padding in the operating room Can be hypocalcemic early, then hypercalcemic
r Suspect a genetic disorder with recurrent r Some reports of using pulse oximetry monitoring of later
r Uric acid:
rhabdomyolysis after minimal to moderate exertion lower extremity to monitor for compartment
or after viral infections starting in childhood Conversion of purines from lysed muscle cells
syndrome r CBC/clotting studies (for DIC)
Glycogen and lipid disorders (McArdle disease, r Monitor for malignant hyperthermia
carnitine deficiency, others) r LFTs, ABG
Duchenne muscular dystrophy r Microscopic urine: Pigmented casts, dysmorphic red
cells
Imaging
r Often unnecessary
r MRI with gadolinium best modality for muscle
injury
Sensitivity of 100% vs. 42% for US and 62% for
CT scan
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RHABDOMYOLYSIS
R
Diagnostic Procedures/Surgery SURGERY/OTHER PROCEDURES 4. Reisiger KE, Landman J, Kibel A, et al. Laparoscopic
r Muscle biopsy is unnecessary r Fasciotomy for compartment syndrome. renal surgery and the risk of rhabdomyolysis:
r Forearm ischemic test to differentiate genetic causes For the extremity such as the leg Diagnosis and treatment. Urology. 2005;66(suppl
of rhabdomyolysis (6) recommendations for fasciotomy vary. In general 5):2935.
Relies on forearm compression with exercise and an intracompartmental pressure of >30 mmHg or 5. Bagley WH, Yang H, Shah KH. Rhabdomyolysis.
determination of ammonia and lactate levels a >30 mm Hg difference between diastolic blood Intern Emerg Med. 2007;2(3):210218.
pressure and the compartment pressure. 6. Sauret JM, Marinides G, Wang GK.
Pathologic Findings r Dialysis for hyperkalemia, acidosis, and/or fluid
r Though muscle biopsy is not needed, one would see Rhabdomyolysis. Am Fam Physician. 2002;65(5):
noninflammatory loss of the nucleus and muscular overload. 907913.
stria. In general, only 4% of patients require dialysis, but 7. Huerta-Alardn AL, Varon J, Marik PE.
r Renal biopsy: Myoglobin precipitates and forms up to 55% in the laparoscopic nephrectomy data Bench-to-bedside review: Rhabdomyolysisan
obstructive casts. ADDITIONAL TREATMENT overview for clinicians. Crit Care. 2005;9(2):
Radiation Therapy 158169.
DIFFERENTIAL DIAGNOSIS
r Acidotic states N/A
r Compartment syndrome Additional Therapies ADDITIONAL READING
r DIC Monitor osteoporosis
r Nephritic syndromes Complementary & Alternative Wen T, Deibert CM, Siringo FS, et al.
r Rhabdomyolysis can be the result of another process Positioning-related complications of minimally invasive
Therapies
radical prostatectomies. J Endourol.
(ie, sepsis) and result in other complications (ie, N/A
2014;28(6):660667.
compartment syndrome, DIC)
See Also (Topic, Algorithm, Media)
ONGOING CARE r Acute Kidney Injury, Adult
TREATMENT PROGNOSIS
r Acute Tubular Necrosis (ATN)
r Overall survival rate of 78.6% at 14 yr r Compartment Syndrome, Urologic Considerations
GENERAL MEASURES r Myoglobin Nephrotoxicity
r Prevention of acute renal failure (ARF) r Initial mortality rate as high as 8%
r Recognition of compartment syndrome r ARF develops in 33% of patients r Myoglobinuria
r Elderly patients and those with comorbidities should In review of the laparoscopic nephrectomy data, r Urine, Abnormal Color
be treated in an intensive care unit patients who developed ARF had a higher peak
CK than those who did not (46,780 U/L vs.
MEDICATION 25,650 U/L) CODES
First Line (6,7)
r Aggressive hydration initially with normal saline COMPLICATIONS
r Long-term weakness, pain, and numbness; may ICD9
Urine output should be maintained at (goal of r 728.88 Rhabdomyolysis
2 mg/kg/h of urine output) until myoglobinuria have permanent disability
r Morbidity associated with dialysis and fasciotomy r 929.9 Crushing injury of unspecified site
has ceased r 958.5 Traumatic anuria
High rates of IV fluid administration should be FOLLOW-UP
used at least until the CK level decreases to or Patient Monitoring ICD10
below 1,000 U/L r Check for return of renal function r M62.82 Rhabdomyolysis
r Consider mannitol for osmotic diuresis to purge r Physical therapy as needed r T79.5XXA Traumatic anuria, initial encounter
nephrotoxic agents r T79.6XXA Traumatic ischemia of muscle, initial
r Diuretics should not be used as they may worsen the Patient Resources
r Medline Plus: Rhabdomyolysis http://www.nlm. encounter
condition
r Alkalinize urine to prevent ARF: nih.gov/medlineplus/ency/article/000473.htm
Bicarbonate: 1 ampule in 1 L of normal saline CLINICAL/SURGICAL
Goal: Urine pH >6.5 and serum pH 7.47.45 REFERENCES PEARLS
r There are retrospective data to suggest aggressive
r The classic triad of muscle pain, weakness, and dark
hydration is sufficient for treatment and that 1. Khan FY. Rhabdomyolysis: A review of the
mannitol and bicarbonate are not needed literature. Neth J Med. 2009;67(9):272283. urine suggests rhabdomyolysis.
r Avoid correction of early hypocalcemia as r Limit operative times especially in obese and
2. Glassman DT, Merriam WG, Trabulsi EJ, et al.
hypercalcemia can develop later Rhabdomyolysis after laparoscopic nephrectomy. muscular patients. Consider high BMI as a risk factor
JSLS. 2007;11(4):432437. for intraoperative rhabdomyolysis.
Second Line r Some advocate not using a kidney rest/bar during
May need to correct acidosis, DIC, and hyperkalemia, 3. Wolf JS Jr, Marcovich R, Gill IS, et al. Survey of
neuromuscular injuries to the patient and surgeon laparoscopic surgery to help prevent rhabdomyolysis.
if present r Appropriately pad all pressure points in the
during urologic laparoscopic surgery. Urology.
2000;55(6):831836. operating room.
r Early recognition and aggressive hydration (better
outcomes within the 1st 6 hr of presentation).
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Pathologic Findings
BASICS DIAGNOSIS r Embryonal:
Most common subtype
DESCRIPTION HISTORY Accounts for majority of GU RMS
r Rhabdomyosarcoma (RMS) (sarcoma botryoides) is a r Family history of malignancy or genetic syndromes
Embryonal variants associated with excellent
malignancy arising from embryonal mesenchyme (LiFraumeni, neurofibromatosis) prognosis:
that tends to occur mostly in children (Sometimes r Bladder/prostate: Sarcoma botryoides
also called Embryonal Rhabdomyosarcoma) Urinary frequency Spindle cell/leiomyomatous
r Most common soft tissue sarcoma in children Stranguria r Alveolar:
r Sarcoma botryoides describes a polypoid variant of Urinary retention Less common in GU RMS
RMS originating in a hollow viscus (vagina, bladder) Hematuria More common in trunk/extremity RMS
r Of all types of pediatric RMS1520% involve GU r Paratesticular:
Higher rates of local recurrence, LN spread, and
system: Scrotal swelling or pain distant metastasis
Paratesticular Back pain r Pleomorphic:
r Vaginal/uterine:
Bladder Undifferentiated/anaplastic variant
Prostate Vaginal discharge/bleeding Poor prognosis
Uterus PHYSICAL EXAM DIFFERENTIAL DIAGNOSIS
Vagina r Bladder/prostate r Bladder/prostate
EPIDEMIOLOGY Abdominal mass TCC of bladder
Incidence Bladder distention Inflammatory pseudotumor of bladder
r 0.50.7 cases per million children <15 yr Firm prostate or mass on rectal exam Nephrogenic adenoma of bladder
r Bimodal age distribution: r Paratesticular Fibroepithelial polyps of prostatic urethra
Scrotal mass r Testis
1st peak: 24 yr r Vagina/uterine
2nd peak: 1519 yr Primary testicular tumor
r 3rd most common solid tumor in children (behind Vaginal mass (may be prolapsing) Benign adnexal mass
Abdominal mass r Vagina/uterine
neuroblastoma and Wilms tumor)
DIAGNOSTIC TESTS & INTERPRETATION Prolapse of ureterocele, urethra, vagina
Prevalence
N/A Lab
r Basic metabolic panel: BUN/Cr may be elevated with
RISK FACTORS TREATMENT
ureteral obstruction
See genetics r Complete blood count: May see anemia due to GENERAL MEASURES
Genetics vaginal bleeding or hematuria r Pre- and post-op staging and risk classification are
r LiFraumeni syndrome: r -HCG or AFP: Evaluate for testicular tumors critical in evaluation and treatment planning
Mutation of p53 tumor suppressor gene Preoperative staging: Intergroup
Higher incidence of RMS Imaging
r CT/MRI of abdomen/pelvis: Evaluate local extent of Rhabdomyosarcoma Study Group (IRSG) staging/
r Neurofibromatosis: classification system based on TNM and primary
tumor, pelvic or retroperitoneal LN involvement,
Higher incidence of RMS location
r Cytogenetic abnormalities: distant metastasis
r Chest x-ray/CT: Evaluate for pulmonary metastases Postoperative grouping: IRSG grouping based on
Alveolar histology subtype: r PET scan: Evaluate the metabolic activity of the primary resection
1;13 translocation (favorable prognosis) Risk classification: Combines stage, group, and
2;13 translocation (unfavorable prognosis) primary for future comparison after therapy, as well histologyhelps determine therapy and prognosis
Embryonal histology subtype: as assess for metastasis r Preoperative staging: TNM system
r Bone scan: Evaluate for osseous metastasis
Loss of heterozygosity on chromosome 11 T1: Confined to organ of origin
r Scrotal US: Characterize paratesticular mass (a) 5 cm in diameter
PATHOPHYSIOLOGY (b) >5 cm in diameter
r The Latin word botryoides refers to the polypoid Diagnostic Procedures/Surgery
r Bone marrow aspirate/biopsy: Evaluate for T2: Extension or fixed to surrounding tissue
or grape-like lesion appearance of the tumor (a) 5 cm in diameter
beneath the mucosa metastases for all primary sites of RMS
r Bladder/prostate (b) >5cm in diameter
Some sources refer to this as embryonal RMS
r Rapid growth with local invasion Cystoscopy: Transurethral resection/biopsy for N0: Regional LNs clinically negative
r Can spread by lymphatic and hematogenous routes pathologic diagnosis N1: Regional LNs clinically positive
Image-guided needle biopsy: Pathologic diagnosis Nx: Unknown
r Thought to arise from immature cells that are
r Paratesticular M0: No distant metastasis
destined to form striated skeletal muscle: M1: Metastasis present
However, may arise in locations where skeletal Radical inguinal orchiectomy: Diagnostic and r Preoperative staging: IRSG
muscle is not typically found, such as the bladder therapeutic
r Vagina/uterine Stage 1: Vaginal and paratesticular, any T, any N,
r Defect in regulatory mechanism that controls
M0
proliferation and differentiation of skeletal muscle Cystoscopy/vaginoscopy: Evaluate extent of tumor,
Stage 2: Bladder/prostate, T1/T2a, N0/Nx, M0
r Prognosis and pattern of spread depends on biopsy for pathologic diagnosis
Stage 3 Bladder/prostate, T1/T2a and N1, OR
histologic subtype and clinical staging T1b/T2b, any N, M0
r Lymph nodes (LNs) and lungs are the most common Stage 4: Any T, M1
sites of distant metastasis
ASSOCIATED CONDITIONS
See Genetics
GENERAL PREVENTION
None
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