Sie sind auf Seite 1von 6

11/20/12

Cloacal Malformations

Medscape Reference Reference

Ne s Reference Education MEDLINE

Cloacal Malformations
Author: Marc A Levitt, MD; Chief Editor: Marleta Reynolds, MD more... Updated: Apr 17, 2012

Background
A persistent cloaca is defined as a confluence of the rectum, vagina, and urethra into a single common channel. This defect is one of the most formidable technical challenges in pediatric surgery. The goals of treatment include an anatomic reconstruction and achieving bowel and urinary control, as well as normal sexual function. Cloacal anomalies occur in 1 per 20,000 live births. They occur exclusively in girls and comprise the most complex defect in the spectrum of anorectal malformations. Diverse treatments for the management of persistent cloaca have been proposed in the past. The most common approach consisted of repairing the rectal component of the malformation without repairing the urogenital sinus or planning its repair in a second stage. A combined abdominal, perineal,- vaginal, and simultaneous rectal pullthrough was also used. Some treatments were adequate for certain cloacae but not for others. The perineal approach to urogenital sinus repair may be useful in patients with low defects, but not in those with higher defects. Similarly, the abdominal approach is required for some anomalies but not for others. Traditional approaches were limited in their exposure and, thus, could not clearly define the complex anatomy of the defect, and the urinary sphincter and anorectal sphincter were matters of speculation.

Histor Of The Procedure


In 1982, the posterior sagittal approach, which was used to repair an imperforate anus, was first performed for a cloacal malformation.[1] It was ideally suited for the more complex repair of cloacae and led to the operation described as the posterior sagittal anorectovaginourethroplasty (PSARVUP). This approach allowed for direct exposure to the complex anatomy and an excellent visualization of the voluntary muscles of urinary and fecal continence.

Problem
Families and physicians dealing with a newborn baby girl who has a cloacal malformation have 3 main concerns: (1) urinary control, (2) bowel control, and (3) sexual function (menstruation, intercourse, obstetric issues).

Epidemiolog
Frequenc
The literature reports that the incidence rate of cloacal malformations is approximately 1 per 20,000-25,000 live births. However, the literature often contains reports of rectovaginal fistula, which are most likely cloacal
emedicine.medscape.com/article/933717-o er ie 1/6

11/20/12

Cloacal Malformations

malformations in which the rectal problem was corrected but the urogenital sinus was not. Therefore, cloacal malformations are probably more common than previously thought.

Etiolog
The etiology of persistent cloaca is unknown.

Presentation
Persistent cloaca is clinically diagnosed. The presence of a single perineal orifice provides clinical evidence of persistent cloaca. The external genitalia often appear small. Examination of the abdomen may reveal an abdominal mass, which likely represents a distended vagina (hydrocolpos) and is present in 40% of patients with persistent cloaca. The goal of early management is to detect associated anomalies, achieve satisfactory diversion of the gastrointestinal tract, manage a distended vagina, and divert the urinary tract, when indicated. Postoperatively, if the patient does not gain weight and has frequent episodes of sepsis or urinary tract infections, the urinary tract probably has not been totally drained. Diversion of the fecal stream with a colostomy placed in the descending colon and with a mucous fistula is recommended. The defunctionalized colon is used for the future rectal pullthrough; thus, adequate length must be ensured when the colostomy location is chosen. Also, access to this distal colon is vital for radiologic evaluation. Total diversion of the fecal stream is necessary to prevent urinary infections; thus, the stomas must be separated to prevent spillage. Unfortunately, a common error in diagnosis may occur during the perineal inspection. Upon examination, the physician may believe that the patient has imperforate anus with a rectovaginal fistula. However, in actuality, the urinary tract, vagina, and rectum may all meet in a common channel, and the baby may have a persistent cloaca. This error could lead to incorrect treatment; the surgeon may repair only the rectum and leave a urogenital sinus. In addition, failure to identify persistent cloaca may mean that associated urologic problems (some potentially life threatening) go unnoticed. In addition, the clinician must be able to identify imperforate anus with rectovestibular fistula (see Imperforate Anus), which has its own clinical implications. Correctly diagnosing persistent cloaca is vital because 90% of babies with this malformation have an associated urologic problem, and 40% have hydrocolpos. The urinary tract and the distended vagina may both need to be managed within the newborn period to avoid serious complications. The distended vagina is a common cause of an obstructed urinary tract because of its pressure on the trigone; therefore, once the vagina is decompressed, the urinary tract may no longer be obstructed. If the hydrocolpos is not drained during the newborn period, it can become infected and can lead to vaginal scarring. Once the baby recovers from the colostomy, the main repair is planned. This procedure, called anorectovaginourethroplasty, simultaneously repairs the rectal, vaginal, and urethral anomalies. The repair can usually be performed using only the posterior sagittal approach. For more complex anomalies, an abdominal approach is added to mobilize (or replace) a very high vagina or gain length on a very high rectum. Cloacal exstrophy is the most severe cloacal anomaly. It involves an anterior abdominal wall defect in which 2 hemibladders are visible and are separated by a midline intestinal plate, an omphalocele, and an imperforate anus. Cloacal exstrophy occurs in 1 per 100,000 live births. Surgical treatment in the newborn period involves closing the omphalocele and the bladder, or bringing the bladder plates together with removal and tubularization of the cecal plate in between. This ensures adequate urinary flow via vesicostomy and preserves as much colon (the distal hindgut) as possible when creating a colostomy for fecal diversion. Management of this rare anomaly can be considered in a discussion of anterior abdominal wall defects. Significant urologic and anorectal issues are involved. Sex assignment is based on chromosomes. Surgical treatment and long-term follow-up are beyond the scope of this article.[2]

Indications
As discussed in Imperforate Anus, certain anorectal anomalies can be repaired in the newborn period. When discussing cloaca, fecal diversion prior to definitive repair is clearly the safest approach; thus, the operations are performed in stages. The newborn fecal diversion (and urinary and vaginal diversion, if necessary) is performed first; definitive repair is then performed at a later date, followed by the final operation of colostomy closure.
emedicine.medscape.com/article/933717-o er ie 2/6

11/20/12

Cloacal Malformations

Relevant Anatom
Genitourinar defects
More than 80% of all patients with a cloaca experience an associated urogenital anomaly. Persistent cloaca - 90% Rectobladderneck fistula - 84% Rectoprostatic urethral fistula - 63% Rectovestibular fistula - 47% Rectobulbar urethral fistula - 46% Rectoperineal fistula - 26% Anorectal malformation with no fistula - 31% All patients must be evaluated at birth for an associated urologic problems; the most valuable screening test is abdominal and pelvic ultrasonography. Urologic evaluation, either by a pediatric surgeon experienced with pediatric urology or in consultation with a pediatric urologist, is necessary before proceeding with colostomy. This provides the surgeon the necessary information needed to address the urologic problem at the time of the colostomy. In addition, drainage of a distended vagina (hydrocolpos) may be required.

Tethered cord
A tethered spinal cord refers to an intravertebral fixation of the phylum terminale. Tethered cord has a known association with anorectal malformations and is particularly common in patients with persistent cloaca. Motor and sensory disturbances of the lower extremities may result. Patients with anorectal malformations and tethered cord have a worse functional prognosis regarding bowel and urinary function. However, they also have higher anorectal defects, less-developed sacra, associated spinal problems, and less-developed perineal musculature; thus, the actual impact of tethered cord alone on the functional prognosis is unclear. Untethering of the cord to avoid motor and sensory problems is indicated in the neurosurgical literature. No definitive evidence suggests that this operation affects the functional prognosis of a patient with anorectal malformation. Some evidence in the urologic literature indicates that this operation may improve urodynamics. Anomalies of the sacrum, particularly hypodevelopment, sacral hemivertebrae, and hemisacra, are associated with tethered cord and most likely comprise the spectrum of caudal regression, of which spinal anomalies are a part. Spinal ultrasonography in the first 3 months of life and MRI thereafter are useful in diagnosis.

Sacrum and spine anomalies


The sacrum is the most frequently affected bony structure. Traditionally, to evaluate the degree of sacral deficiency, the number of sacral vertebral bodies was counted. A more objective assessment of the sacrum can be obtained by calculating a sacral ratio. The sacrum is measured, and its length is compared with bony parameters of the pelvis (see the image below).

Calculation of the sacral ratio.

The distance from the coccyx to the sacroiliac joint divided by the distance from the sacroiliac joint to the top of the pelvis provides the sacral ratio. Lateral radiography is more accurate than the anteroposterior view because its calculation is not affected by the tilt of the pelvis.
emedicine.medscape.com/article/933717-o er ie 3/6

11/20/12

Cloacal Malformations

The assessment of the hypodevelopment of the sacrum correlates with the patient's functional prognosis. Normal sacra have a ratio of greater than 0.7. Bowel control has rarely been observed in patients with ratios less than 0.3. A hemisacrum is almost always associated with a presacral mass, commonly teratomas, or anterior meningoceles. The Currarino triad, which includes an anorectal malformation, a hemisacrum, and a presacral mass, has been described. Hemivertebrae may also affect the lumbar and thoracic spine, leading to scoliosis. Patients may have spinal anomalies other than tethered cord, such as syringomyelia and myelomeningocele.

Contraindications
Patients with cloaca have no contraindications to definitive surgery when future fecal or urinary incontinence is a concern. Even for patients with incontinence, a bowel management program (see Bowel Management) is almost always successful in keeping a patient clean and dry. In patients in whom bowel management is unsuccessful (< 3%), a colostomy may be the best option to ensure good quality of life. In patients with urinary incontinence, many options are available for keeping a patient clean. Urinary diversions, such as the Mitrofanoff procedure and the use of intermittent catheterization, are usually successful in keeping the patient dry of urine.

Contributor Information and Disclosures


Author Marc A Levitt, MD Professor of Surgery, University of Cincinnati; Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center Marc A Levitt, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Surgeons, and Society of American Gastrointestinal and Endoscopic Surgeons Disclosure: Nothing to disclose. Coauthor(s) Alberto Pena, MD Founding Director, Colorectal Center for Children, Department of Pediatric Surgery, Cincinnati Children's Hospital Medical Center Alberto Pena, MD, is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, American Society of Colon and Rectal Surgeons, Pacific Association of Pediatric Surgery, and Royal College of Surgeons of England Disclosure: Nothing to disclose. Specialty Editor Board Aviva L Kat , MD Assistant Professor of Surgery, University of Pittsburgh School of Medicine; Consulting Staff, Division of General and Thoracic Surgery, Children's Hospital of Pittsburgh Aviva L Katz, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association of Women Surgeons, Physicians for Social Responsibility, and Wilderness Medical Society Disclosure: Nothing to disclose. Mar L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference Disclosure: Nothing to disclose. Gail E Besner, MD John E Fisher Endowed Chair in Neonatal Reseach, Director, Pediatric Surgical Research, Department of Surgery, Nationwide Children's Hospital; Professor of Surgery and Pediatrics, Department of Surgery, Ohio State University College of Medicine Gail E Besner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Burn Association, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Medical Women's Association, American Pediatric
emedicine.medscape.com/article/933717-o er ie 4/6

11/20/12

Cloacal Malformations

Surgical Association, Association for Academic Surgery, Federation of American Societies for Experimental Biology, Society of Critical Care Medicine, Society of Surgical Oncology, and Society of University Surgeons Disclosure: Nothing to disclose. H Biemann Othersen Jr, MD Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association Disclosure: Nothing to disclose. Chief Editor Marleta Re nolds, MD Professor of Surgery, Northwestern University, The Feinberg School of Medicine; Head, Department of Surgery and Surgeon in Chief, Head, Division of Pediatric Surgery, Children's Memorial Hospital of Chicago Marleta Reynolds, MD is a member of the following medical societies: American Pediatric Surgical Association Disclosure: Nothing to disclose.

References
1. Hendren WH, Oesch IL, Tschaeppeler H, Bettex MC. Repair of cloacal malformation using combined posterior sagittal and abdominal perineal approaches. Z Kinderchir. Apr 1987;42(2):115-9. [Medline]. 2. Levitt MA, Mak GA, Falcone RA, Pea A. Cloacal exstrophy pull through or permanent stoma? A review of 53 patients. J Pediatr Surg. Jan 2008;43:164-170. [Medline]. 3. Bischoff A, Levitt MA, Breech L, Louden E, Pea A. Hydrocolpos in cloacal malformations. J Pediatr Surg. Jun 2010;45:1241-5. [Medline]. 4. Bischoff A, Levitt MA, Lim FY, Guimares C, Pea A. Prenatal diagnosis of cloacal malformations. Pediatr Surg Int. Nov 2010;26:1071-5. [Medline]. 5. Breech L. Gynecological concerns in patients with anorectal malformations. Semin Pediatr Surg. May 2010;19:139-45. [Medline]. 6. Calvo-Garcia MA, Kline-Fath BM, Levitt MA, Lim FY, Linam LE, Patel MN, et al. Fetal MRI clues to diagnose cloacal malformations. Pediatr Radiol. Sep 2011;41:1117-28. [Medline]. 7. Ebert AK, Reutter H, Ludwig M, Rsch WH. The exstrophy-epispadias complex. Orphanet J Rare Dis. Oct 30 2009;4:23. [Medline]. 8. Hendren WH. Cloaca, the most severe degree of imperforate anus: experience with 195 cases. Ann Surg. Sep 1998;228(3):331-46. [Medline]. 9. Hendren WH. Cloacal malformations: experience with 105 cases. J Pediatr Surg. Jul 1992;27(7):890-901. [Medline]. 10. Hendren WH. Further experience in reconstructive surgery for cloacal anomalies. J Pediatr Surg. Dec 1982;17(6):695-717. [Medline]. 11. Hendren WH. Management of cloacal malformations. Semin Pediatr Surg. Nov 1997;6(4):217-27. [Medline]. 12. Hendren WH. Urological aspects of cloacal malformations. J Urol. Nov 1988;140(5 Pt 2):1207-13.
emedicine.medscape.com/article/933717-o er ie 5/6

11/20/12

Cloacal Malformations

[Medline]. 13. Levitt MA, Bischoff A, Pea A. Pitfalls and challenges of cloaca repair; how to reduce the need for reoperations. J Pediatr Surg. Jun 2011;46:1250-5. [Medline]. 14. Levitt MA, Pena A. Pitfalls in the management of newborn cloacas. Pediatr Surg Int. Apr 2005;21(4):2649. [Medline]. 15. Levitt MA, Pea A. Cloacal malformations: lessons learned from 490 cases. Semin Pediatr Surg. May 2010;19:128-38. [Medline]. 16. Levitt MA, Pea A. Anorectal malformations. In: Coran AG, Adzick NS, Krumel TM, Laberge JM, Shamberger RC, Caldamone AA, eds. Pediatric Surger . 7th ed. Philadelphia: Elsevier Saunders; 2012:1289-1309. 17. Levitt MA, Stein DM, Pena A. Gynecologic concerns in the treatment of teenagers with cloaca. J Pediatr Surg. Feb 1998;33(2):188-93. [Medline]. 18. Lund DP, Hendren WH. Cloacal exstrophy: a 25-year experience with 50 cases. J Pediatr Surg. Jan 2001;36(1):68-75. [Medline]. 19. Lund DP, Hendren WH. Cloacal exstrophy: experience with 20 cases. J Pediatr Surg. Oct 1993;28(10):1360-8; discussion 1368-9. [Medline]. 20. Moore SW, Alexander A, Sidler D, Alves J, Hadley GP, Numanoglu A, et al. The spectrum of anorectal malformations in Africa. Pediatr Surg Int. Jun 2008;24(6):677-83. [Medline]. 21. Patel MN, Racadio JM, Levitt MA, Bischoff A, Racadio JM, Pea A. Complex cloacal malformations: use of rotational fluoroscopy and 3D reconstruction in diagnosis and surgical planning. Pediatr Radiol. Mar 2012;42:355-63. [Medline]. 22. Pena A. Management of anorectal malformations during the newborn period. World J Surg. May-Jun 1993;17(3):385-92. [Medline]. 23. Pena A. Total urogenital mobilization--an easier way to repair cloacas. J Pediatr Surg. Feb 1997;32(2):263-7; discussion 267-8. [Medline]. 24. Pena A, Grasshoff S, Levitt MA. Reoperations in anorectal malformations. J Pediatr Surg. Feb 2007;42(2):318-25. [Medline]. 25. Pena A, Levitt M. Cloaca. In: Coran, Spitz eds. Operative Pediatric Surger . 6th. Hodder Arnold; 2006. 26. Pena A, Levitt MA. Neurogenic bladder and anorectal malformations. In: Esposito C, Guys JM, Gough D, Savanelli A, eds. Pediatric Neurogenic Bladder D sfunction. Berlin: Springer; 2006:85-88. 27. Pena A, Migotto-Krieger M, Levitt MA. Colostomy in anorectal malformations: a procedure with serious but preventable complications. J Pediatr Surg. Apr 2006;41(4):748-56; discussion 748-56. [Medline]. 28. Rangel SJ, de Blaauw I. Advances in pediatric colorectal surgical techniques. Semin Pediatr Surg. May 2010;19:86-95. [Medline]. 29. Rich MA, Brock WA, Pena A. Spectrum of genitourinary malformations in patients with imperforate anus. Pediatric Surg Intl. 1988;(3):110-113. 30. Rintala RJ, Pakarinen MP. Outcome of anorectal malformations and Hirschsprung's disease beyond childhood. Semin Pediatr Surg. May 2010;19(2):160-7. [Medline]. 31. Soffer SZ, Rosen NG, Hong AR, Alexianu M, Pena A. Cloacal exstrophy: a unified management plan. J Pediatr Surg. Jun 2000;35(6):932-7. [Medline]. Medscape Reference 2011 WebMD, LLC

emedicine.medscape.com/article/933717-o er ie

6/6

Das könnte Ihnen auch gefallen