III-2b

Pediatric Surgery (Part 2) Dr. Rubi Ann Claire Chan October 24, 2011

o ABDOMINAL WALL DEFECTS Gastroschisis Greek Word: Belly Cleft An abdominal wall defect lateral to the umbilicus usually contains small bowel and has no surrounding membrane Defect almost always to the right of intact umbilical cord, and in some cases separated from the cord by an intact skin bridge Anomaly probably results from defect that occurs at the site where the second umbilical vein involutes. Embryonic Origin Embryology of abdominal wall defect: Previously believed to be a result of failure closure of the abdominal wall A result of a vascular accident during embryogenesis (intrauterine occlusion of right omphalo-mesenteric artery) Failure of the formation of the umbilical coelom Congenital weakness of the right side of the umbilical cord - - Rupture Epidemiology Most common congenital abdominal wall defect More common than omphalocoele (1.5 to 2:1) Both sexes are equally affected No genetic, geographic or racial predilection Associated with: o young mothers (<20 years of age) o low socioeconomic status and social instability o Aspirin, Ibuprofen and Pseudoephedrine use o Alcohol, cigarette and recreational drug use 40-48% of patients are either premature or SGA All babies have some degree of malrotation 10-15% incidence of Intestinal Atresia Chromosomal and other associated anomalies (Rare) Liver almost never eviscerated; the stomach, small bowel and large intestine usually reside outside the defect. Occasionally, ovaries and fallopian tube in girls and undescended testis in boys are found outside the defect Bowel may be perforated in approximately 5% of patients. Viscera may be congested, thickened, matted, edematous and foreshortened May have fibrinous peel A result of the irritating effects of amniotic fluid (fetal urine and various growth factors) Compromised venous return and the drying effect of air Diagnosis Prenatal Fetal Ultrasound: o Allows antenatal diagnosis (2nd Trimester) o Shows a dilated bowel free in amniotic fluid adjacent to the umbilical insertion Maternal Alpha Feto-Protein (AFP): o 2nd trimester

o Management Mode of delivery (controversial): o Elective C-section vs. normal spontaneous vaginal delivery Surgical Repair: o Primary Closure ! Fascial-Skin closure ! Skin closure (ventral hernia) o Delayed Primary Closure ! Silo closure Pre-operative Care Gastric decompression and Rectal irrigation (Respiratory) Endotracheal intubation (may be needed) Fluid, electrolyte, and heat losses must be minimized and corrected The baby should be placed under radiant heater Exposed intestines should be wrapped with moist dressing then covered with plastic wrap and supported to minimize traction on the mesentery As an alternative, the baby's lower torso may be placed in a bowel bag A urinary catheter is inserted to monitor urine output A broad spectrum antibiotic therapy is administered to prevent infection. A central venous line is placed to provide parenteral nutrition and thus minimize catabolic protein loss during the period of gastrointestinal dysfunction Intra-operative Reduction Post-Surgical Repair Silastic Prosthesis Serves as an extra-abdominal compartment Infants whose intestine has become thickened and edematous making primary surgical closure of abdominal wall impossible Post-operative Complications Abdominal compartment syndrome o impeding venous return compromising cardiac output and decreasing renal blood flow o renal vein thrombosis and renal failure may ensue Necrotizing Enterocolitis (NEC) o diminished mesenteric blood flow Intestinal Dysfunction Infection/Sepsis Intestinal Obstruction o Intestinal atresia o malrotation

Used both for gastroschisis and omphalocoele (higher values for gastroschisis) Elevated in 77-100% of cases

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Omphalocoele ! Abnormality that occurs before birth as a fetus is forming in its mother's uterus. ! First trimester of pregnancy ! Some of the abdominal organs protrude through an opening in the abdominal wall in the area of the umbilical cord covered by a translucent membrane (sac) What causes an omphalocoele? ! 6th - 10th week of pregnancy: ! the intestines actually project into the umbilical cord as they are growing. ! 11th week of gestation: ! the intestines should return to the abdomen ! the abdominal muscles should then meet in the middle and grow together and thus closing off this opening ! Omphalocele occurs when the abdominal organs do not return to the abdominal cavity as they should. Clinical Findings in infants with Abdominal Defects Factor Omphalocele Gastroschisis Location Umbilical ring Lateral to cord Defect Size Large (2-10 cm) Small (2-4 cm) Cord Inserts in sac Normal insertion Sac +/Contents Liver and Bowel Bowel and Gonads GI function Normal Prolonged ileus Associated Anomalies Common (30-70%) Rare Syndromes Associated with Omphalocoele: ! Beckwith-Wiedemann Syndrome " Macroglossia " Hyperinsulinism " Omphalocoele ! Cantrells Pentalogy " Epigastric omphalocele " Cleft sternum " Anterior diaphragmatic hernia (Morgagni) " Cardiac defects (eg, ectopia cordis,VSD) " Absent pericardium Karyotype Anomalies: " 30% of affected infants " More common in those with small defects " Trisomies 13 & 18 " Trisomy 21 (less frequently)

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Can be used as a guide to intervention in pregnancy. Its greatest advantage is that it can be quickly and frequently repeated as required with minimal risk on the patient and the fetus A definitive diagnosis of omphalocele is possible only beyond 12 weeks' gestation (first trimester) The mass consists of abdominal contents that have herniated through a midline central defect at the base of umbilical cord insertion. Mass has a smooth surface and contains abdominal viscera, usually the liver including the bowel and stomach

Maternal Serum alpha-fetoprotein (MSAFP) " Prenatal diagnosis of abdominal wall defects can be made by detection of an elevation in MSAFP " MSAFP levels are greater in gastroschisis than in omphalocele " MSAFP also is increased in spina bifida, which additionally increases the ratio of acetylcholinesterase and pseudocholinesterase

Surgical Treatment: ! SMALL omphalocele (<2 cm): " shortly after birth, an operation is done to return the organs to the abdomen and close the opening in the abdominal wall. ! Medium-sized defects: " careful removal of the sac at its base with suture ligation of the umbilical vessels and urachus. LARGE omphalocele: " the repair is done in "stages" and may include the following: " Because the abdomen may be small and underdeveloped, it may not be able to hold all of the organs at once. Therefore, the exposed organs are gradually moved back into the abdomen over several days or weeks. " The abdominal wall is closed surgically once the organs have been returned to the abdominal cavity.

Other associated anomalies: " Cardiac (most common) " Musculo-skeletal " Gastro-intestinal " Genito-urinary Risk factors ! Maternal illness and infection ! Drug use, smoking, and alcohol ! Genetic abnormalities ! Folic acid deficiency, hypoxia, and salicylates (experimental rats) ! 40 years or older ! Diagnostics ! Prenatal UTZ: " Inexpensive, safe and noninvasive " Done in real-time and is widely available. " Remains the imaging modality of choice for the prenatal assessment of the fetus. " In experienced hands, UTZ is highly accurate in the diagnosis of most complications associated with pregnancy.

Defect >10 cm: with suspected anomalies " Topical therapy with escharotic agent (silver sulfadiazine, 0.5% silver nitrate, betadine) " If the sac ruptures: homograft (amnion)/heterograft (pigskin) " Closure using synthetic material ! Closure with an alloderm patch ! 2 months after implantation -> epithelialization of the Alloderm patch Prognosis ! Mortality is related directly to the severity of coexisting defects and presence of chromosomal conditions inconsistent with survival. (Cantrells Pentalogy: 75% mortality) ! Groin Hernias

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Inguinal Hernia An indirect inguinal hernia protrudes through the internal inguinal ring supero-lateral to the inferior epigastric vessels, within the cremasteric fascia and extends down the spermatic cord for varying distances A direct inguinal hernia protrudes through the posterior wall of the inguinal canal infero-medial to the inferior epigastric vessels, destroying or stretching the transversalis fascia The typical patient: o Presents with an intermittent lump or bulge in the groin which may extend towards the scrotum or labia o Most notably noticed at times of increased intra-abdominal pressure Are almost exclusively indirect type (3-10%) Direct inguinal hernia in children are rare: o Usually associated with an indirect inguinal hernia o Caused by: ! Previous surgery secondary to floor disruption ! Connective tissue disease (Marfans, EhlersDanlos and Hunter-Hurler syndromes) ! Increased intra-abdominal pressure Male: Female (8:1 to 10:1); Right sided (2x more often) High risk: o Prematurity o Low Birth Weight Management Elective Herniotomy: o at the earliest convenient time o simple high ligation of the sac required Bilateral Exploration (Controversial): o Female (4-6 y.o) and Male (<1y.o) o Left sided inguinal hernia o Current indication is a history of incarceration Umbilical Hernia ! Small defect in the abdominal fascial wall in which fluid or abdominal contents protrude through the umbilical ring ! The actual size of the umbilical hernia is measured by physical examination of the defect in the rectus abdominis muscle. ! Related to an incomplete contraction of the umbilical ring ! The herniation is a result of the growing alimentary tract that is unable to fit within the abdominal cavity. ! Most umbilical hernias are asymptomatic Management ! Observation: ! most umbilical hernias spontaneously close by age two ! Surgical repair: ! recommended if the hernia has not closed by 5 to 6 ! Cosmetic: ! repair of umbilical hernia in the first year of life is largely cosmetic ! The procedure is done as outpatient surgery under general anesthetic. GASTROINTESTINAL DISEASES Neonatal Period Imperforate anus Hirchprungs disease Esophageal atresia with or without tracheo-esophageal fistula Intestinal atresias Malrotation Necrotizing enterocolitis Infancy and Older Children Acute appendicitis Typhoid ileitis (perforated) Intussusception Post op adhesions Meckels diverticulitis Abdominal trauma

Diagnosis: ! Invertogram (Rice-Wangensteen) or Prone Cross-table lateral x-ray: >18 hrs of life Types: ! ! High type (> 1cm from anal dimple) = Colostomy Low type (< 1cm from anal dimple) = Anoplasty

Associated Anomalies: ! VACTERL Syndrome o V = Vertebral anomalies; and o A = Anal atresia (no hole at the bottom end of the intestine); o C = Cardiac defect, most often ventricular septal defect; o TE = TracheoEsophageal fistula (communication between the esophagus and trachea) with esophageal atresia (part of the esophagus is not hollow); o R = Renal (kidney) abnormalities; and o L = Limb abnormalities, most often radial dysplasia (abnormal formation of the thumb or the radius bone in the forearm). Definitive Tx: o Posterior sagital anorectoplasty (PSARP) o Cutback o Anal transposition Persistent Cloaca ! In females, when the urethra, vagina, and anus all go out through one opening

Other treatmnents: ! Distal colonogram ! Colostomy Hirschsprungs Disease Absence of ganglion cells in the colon Presentation: ! Acute/chronic obstruction: ! Vomiting ! Obstipation/Constipation ! Abdominal Distention ! No passage of meconium (24-48hrs of life) ! Rectal exam shows sudden gush of air and feces 1:5000 live births Absent ganglion cells ! ineffective peristalsis ! functional bowel obstruction Limited to rectosigmoid in majority Failure to pass meconium within 48 hrs Non-specific sxs in older infant and child: ! Episodic abdominal distention, overflow diarrhea, severe constipation Diagnosis: Contrast enema ! narrowed rectum with proximal bowel dilation Transition zone may be absent in infants due to underdeveloped haustra Retained colonic barium at 24 hrs is pathognomonic Definitive Diagnosis: Genetic markers exist as research tools in familial cases; not for general use Anorectal manometry possible: "resting pressure, absent anorectal inhibitory reflex Pathologic confirmation necessary: ! Suction mucosal or full thickness rectal biopsy ! Absent ganglion cells, thickened nerve trunks, + ACE

Imperforate Anus ! No anal opening ! With/without fistula (meconium coming out)

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Operative Management: Traditional: ! Temporary colostomy above aganglionic segment at time of dx ! Second stage pullthrough procedure (6 to 12 mos later) Contemporary: ! Trend to avoid colostomy; Repair within days to weeks of dx Key: ! Evacuate colon with rectal dilations, tube decompression, saline washouts

Malrotation ! Must consider in every infant with bilious emesis ! Many subtle variations of malrotation and nonfixation ! 30% present within first week of life ! 50% within first month ! Midgut volvulus with necrosis disasterous Clinical Presentation ! Sudden onset of bilious emesis in 95% ! Abdominal distention common; tenderness variable ! Stool guiaic + ! Bloody vomitus or diarrhea in 30% ! If baby in extremis, shock or peritoneal signs emergent surgical exploration Diagnosis ! Plain abdomen: ! Double-bubble sign ! Gasless abdomen ! Gastric outlet or Small bowel obstruction ! UGIS: ! Location of ligament of treitz and jejunum ! Barium enema: ! High lying cecum or located to the left ! Abdominal ultrasonography r ! Reversed position of SMA:SMV ! Study MUST be Expeditious Pre-operative preparation: (Malrotation with Volvulus) ! Clock ticking until bowel detorted ! Mortality remains as high as 28% ! Preoperative preparation ?? NONE... GO TO OR. QUICKLY Operative Correction: ! Ladds procedure ! Position of corrected malrotation ! Small bowel descends on Right ! Large bowel on Left ! Appendix potentially in LUQ ! Removed ! Role of second look operation (midgut volvulus) Atypical presentation of Malrotation ! Later presentation can occur in children through aged adults ! Only 10-14% present with acute volvulus ! Vague chronic symptoms in majority: ! 30% intermittent vomiting, colicky pain, recurrent SBO, malabsorption, etc. Intestinal Atresias Etiology ! Doudenal (Tandlers theory) ! Jejuno-ileal (Vascular accident) ! Colonic (Vascular accident) Types ! ! ! ! ! 1: Continuity but with membranous web 2: Atresia but separated by a fibrous cord 3A: Atresia with complete mesenteric defect 3B: Apple-peel deformity 4: Multiple atresias

Trans-anal pull-through Swensons pull-through Endorectal pull-through

Contemporary Surgical Options Open neonatal pullthrough via lower midline incision Laparoscopic assisted pullthrough with varied port site incisions Transanal pullthrough... Incision-less procedure (endorectal/fullthickness) All procedures: ! reduces need for multiple operations ! Reduces morbidity ! Reduces operative cost of stoma Earlier postoperative feeding / hospital discharge

Hirschsprungs Enterocolitis ! Major cause of morbidity/mortality ! Commonly misdiagnosed as gastroenteritis ! No known etiology or effective prevention ! Occurs in 1/3 of patients ! Presenting sx: 7.7% Postop: 21% ! Higher incidence of enterocolitis associated with: " early dx (especially first week of life) " definitive repair <4 mo of age Presentation: ! Abdominal distension - 83% ! Explosive foul-smelling diarrhea - 69% ! Bilious vomiting - 51% ! Fever - 34% ! Lethargy - 27% ! Colonic perforation/shock - 2.5% Diagnosis: ! Diffusely tender, distended abdomen ! Digital rectal exam results in explosive gas and stool ! KUB: " Cutoff sign pathognomonic " Absent distal air, proximal bowel dilation, serrated sawtooth appearance, pneumatosis intestinalis ! Contrast enemas discouraged because of risk of perforation ! Stool cultures not helpful ! Barium enema Treatment: ! Prompt recognition is ESSENTIAL ! Rectal tube decompression (straight cath) " Rectal irrigation ! Aggressive IV fluid resuscitation ! Broad spectrum antibiotics ! Rectal saline washouts every 4-6 hours ! Emergent ostomy: " Septic patient with long-segment HD ! Can be life-threatening if unrecognized

Intestinal Atresias: ! Doudenal atresia: " double-bubble sign ! Jejuno-ileal atresias: " Multiple air-fluid level ! Presentation: " Depends on level of atresia

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Associated with: " Prematurity " polyhydramios

Management ! Duodenal: " Duodenoduodenostomy " Excision of web ! Jejuno-ileal and colonic: " Resection or tapering of bulbous end " Bypass procedure (Duodeno-jejunostomy, jejunojejunostomy) Necrotizing Enterocolitis ! An ischemic disorder of the intestine in the newborn ! Although more common in premature infants, it can also be observed in term babies ! Typically occurs in the second to third week of life in the infant who is premature and has been formula fed Etiology ! No specific etiology of NEC has been identified ! Multiple factors are believed to contribute to its development ! The principal factors identified are: ! PREMATURITY ! EARLY ENTERAL FEEDING Clinical Presentation ! Abdominal distention ! Frank or occult blood in stools ! Vomiting ! Diarrhea ! Feeding intolerance ! Visible intestinal loops ! Palpable abdominal mass ! Erythema in the abdominal wall Diagnosis ! Leukopenia, thrombocytopenia, low hematocrit, low serum sodium levels, metabolic acidosis and coagulation defects ! Abnormal radiographs: ! Distended and edematous intestines; intramural air; portal vein gas; isolated persistent distended loop of bowel; free peritoneal air suggesting perforation ! Radiograph demonstrates multiple dilated loops in the large bowel and small bowel. Note pneumatosis intestinalis with bubbly and linear gas collections in the bowel wall ! Paracentesis may be performed for infants with ascites ! A positive finding on paracentesis with the free flow of at least 0.5 mL of brownish fluid that contains bacteria on Gram stain is highly specific for intestinal necrosis. Objective Staging Criteria (developed by Bell) ! Bell stage I: Suspected disease ! Stage IA: ! Mild nonspecific systemic signs such as apnea, bradycardia, and temperature instability are present. ! Mild intestinal signs such as increased gastric residuals and mild abdominal distention are present. ! Radiographic findings can be normal or can show some mild nonspecific distention. ! Treatment is NPO with antibiotics for 3 days. ! Stage IB: ! Diagnosis is the same as IA, with the addition of grossly bloody stool. ! Treatment is NPO with antibiotics for 3 days.

Bell stage II: Definite disease ! Stage IIA: ! Patient is mildly ill. ! Diagnostic signs include the mild systemic signs present in stage IA. ! Intestinal signs include all of the signs present in stage I, with the addition of absent bowel sounds and abdominal tenderness. ! Radiographic findings show ileus and/or pneumatosis intestinalis. This diagnosis is sometimes referred to colloquially as medical NEC. ! Treatment includes NPO and antibiotics for 7-10 days. ! Stage IIB: ! Patient is moderately ill. ! Diagnosis requires all of stage I signs plus the systemic signs of moderate illness, such as mild metabolic acidosis and mild thrombocytopenia. ! Abdominal examination reveals definite tenderness, perhaps some erythema or other discoloration, and/or right lower quadrant mass. ! Radiographs show portal venous gas with or without ascites. ! Treatment is NPO and antibiotics for 14 days. Bell stage III: Represents Advanced NEC with severe illness that has a high likelihood of progressing to surgical intervention ! Stage IIIA: ! Patient has severe NEC with an intact bowel. ! All of the above conditions with the addition of hypotension, bradycardia, respiratory failure, severe metabolic acidosis, coagulopathy and/or neutropenia. ! Marked distention with signs of generalized peritonitis. ! Radiographic examination reveals definitive evidence of ascites. ! Treatment involves NPO for 14 days, fluid resuscitation, inotropic support, ventilator support, and paracentesis. ! Stage IIIB ! This stage is reserved for the severely ill infant with perforated bowel observed on radiograph. ! Free air visible on abdominal radiograph indicates surgery

Indications for Operative Intervention ! The most compelling predictor of intestinal necrosis indicating a need for operative intervention is pneumoperitoneum ! Erythema and sclerema in the abdominal wall, positive paracentesis, persistent bowel loop on plain film, persistent abdominal mass on a particular location ! Abdominal tenderness Contraindications ! Patients with stage I or stage II disease, for whom nonoperative medical therapy is the treatment of choice ! Critically ill newborns with a relative contraindication to formal operative exploration may be treated with the placement of a peritoneal drain ! Peritoneal drain placement may be the treatment of choice for extremely small (<600 g) premature newborns ! Such premature, critically ill infants cannot tolerate formal exploration, and drain placement may be preferred and definitive ! Many infants whose condition is too unstable for formal exploration do not survive regardless of intervention Acute Appendicitis ! Most common cause of surgical abdomen ! S/S less typical than in adults ! Abdominal pain Anorexia/vomiting- Fever

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Diagnosis: " PE findings (KEY Factor) " Differential Diagnosis Delay in Dx: " Perforation Complications: " Peritonitis & Abcess Treatment: " Correction of fluid and electrolyte deficits " Appendectomy Acute congestive appendicitis Acute suppurative appendicitis

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Most common form of congenital abnormality of the small intestine Results from an incomplete obliteration of the vitelline duct It has been reported in as many as 97% of the vitelline duct anomalies Meckel diverticulum is a true diverticulum because it contains all layers of the intestinal wall. Heterotopic mucosa is likely to be gastric in origin in 80% of cases of Meckel diverticulum

Demography ! 1-2% of the Population ! No gender predominance (Incidental Diverticula) ! 3-4:1 Male:Female (Symptomatic Diverticula) ! Associated with: ! Esophageal Atresia (12%) ! Imperforate Anus (11%) ! Minor Omphalocele (25%) Clinical Presentation ! Rule of 2s: ! 2% of the Population ! < 2 feet from the ileocecal valve ! 2 types of heterotopic mucosa (Gastric and Pancreatic) ! < 2 years old ! 2 inches long ! Symptoms: ! Variety of clinical signs ranging from no symptoms to acute abdominal pain 3 Most Common Presentations: ! gastrointestinal bleeding ! intestinal obstruction ! inflammation of the diverticulum (diverticulitis)

Intussusception Telescoping of a segment of intestine thru itself ! Age: " " ! 3-8 months well-nourished child

Diagnosis: " Episodic colicky pain with vomiting " sausaged-shaped mass " currant-jelly stools ! ! Barium Enema: Diagnostic and Therapeutic U.S findings: Target or donut hole sign

Treatment: ! Barium or Air reduction ! Surgery

Meckels Diverticulum Vitelline (Omphalomesenteric) Duct Anomalies ! Meckels Diverticulum ! Patent Omphalomesenteric Duct ! Cord-like Remnant ! Vitelline Cyst ! Vitelline Sinus Tract

Complications ! Obstruction: ! Most frequently encountered ! Usually present at a younger age ! Bleeding: ! Most frequently encountered ! Usually present at a younger age ! Inflammation ! Others: ! Foreign body impaction, origin of neoplasia, site of parasitic infection Bleeding ! < 5 years old ! Painless Melena (Massive and Episodic) ! Radionuclide Scanning (Indication) ! Pertechnetate Ions with Technetium 99m (secreted by Gastric Mucosal Cells)

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! 90% Accuracy ! 85% Sensitivity ! 95% Specificity In children, painless gastrointestinal bleeding is the most common presenting symptom: ! Prevalence of different types of bleeding: ! dark red (maroon): 40% ! bright red: 35% ! bright red with dark red: 12% ! dark red and tarry: 6% ! tarry: 7% ! For rapid bleeding, stools are bright red or have an appearance like currant jelly stools ! When slow bleeding occurs, the stools are black and tarry

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Wedge resection of the intestinal wall containing the diverticulum with suture closure Segmental resection of the intestine, including the diverticulum and end-to-end anastomosis Division of the fibrous band with or without diverticulectomy

Incidental Meckels Diverticulum ! Resection is recommended for: ! patients younger than 40 years ! diverticula longer than 2 cm ! diverticula with narrow necks ! diverticula with fibrous bands ! suspected ectopic gastric tissue ! inflamed, thickened diverticula Pediatric Cancer United States: Constitutes less than 1% of adult malignancies 2nd leading cause of death in children <15y/o Philippines : 4% relative frequency to adult malignancy 7th leading cause of death Differs markedly from adult cancer: Nature Distribution Prognosis Cancer in Children

Obstruction ! Triad: " Abdominal Distention " Vomiting " Crampy Abdominal Pain ! Secondary to: " Intussusception (More Common) " Volvulus Inflammation: ! Rarely diagnosed pre-operatively ! Clinically similar to Appendicitis ! Perforation (Complication 2ndary to Peptic Ulceration) Complications: Meckels diverticulitis Intussusceptions on meckels diverticulum Perforated Meckels diverticulitis Torsion of giant meckel Persistent omphalo duct (Mekels diverticuum is attached to the umbilicus) Diagnostic Procedures asymptomatic ! Blood test: ! to check for anemia ! Stool Occult Blood: ! To check for minimal bleeding in stool ! Barium enema and/or small bowel series: ! A procedure performed to examine the small and large intestine for abnormalities Diagnostic Procedures symptomatic ! Meckel's scan: ! A substance called technetium is injected into your child's bloodstream though an intravenous (IV) line ! Ectopic Pancreatic and Gastric Tissues ! Superior Mesenteric Angiography: ! helpful in patients presenting with acute GI bleeding ! effective when blood loss exceeds 0.5 mL/min. Treatment ! Symptomatic Diverticulum: ! Surgical (Segmental Resection) ! Incidental Diverticulum: ! Controversial ! Morbidity/Mortality vs. Chance of becoming symptomatic ! Dependent on age, sex and physical characteristics of the diverticulum ! 4-6% lifetime risk of becoming symptomatic

Childhood Cancer " Recently, marked increase in survival and better outcome: " Better diagnostic imaging techniques " New chemotherapeutic agents " Collaborative and multi-disciplinary approaches " Multi-institutional studies and treatment protocols " Future, will lead to more improvement and much better outcome: " Molecular biology " Immunology " Pharmacology " Tumor biology " Genetics Wilms Tumor " One of the great successes of modern pediatric oncology " Was considered once a lethal malignancy

Surgical Approach ! Four possible surgical procedures: ! Diverticulectomy with suture closure of the base

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Recently, achieves a cure rate of >90% National Wilms Tumor Study (NWTS) Group and the International Society of Pediatric Oncology (ISOP) Developed a multimodal therapeutic regimen

Incidence Expensive but may later find an increasing role " Most common 1o malignant renal tumor in children " 1 to 5 years old (78% of cases) Function of contra-lateral kidney " 3 years old (peak incidence) " Develops in patients with other congenital anomalies (12-15% of Staging of Wilms Tumor cases) " Essential for appropriate treatment " Hereditary (15-20% of cases) " Determined by: " Bilaterality (5% of cases) " Extent of the disease " Resectability of the primary tumor Associated Congenital Disorders " Status of perirenal, capsular and renal involvement " Local invasion of adjacent organs and structures Disorder and Incidence " Metastasis Hamartomas, Hemangiomas, Multiple Nevi, Caf-au-lait spots " Contralateral kidney involvement 7.9% " Defined at the time of exploration G-U anomalies, Horseshoe Kidneys, Dysplasia, Cystic disease, Hypospadias, Cryptorchidism, Duplication anomaly 4.4% NWTS Group Staging Congenital hemihypertrophy - 2.9% " Stage 1: " Limited to kidney; completely excised; intact capsule; not Musculoskeletal anomaly, Clubfoot, Rib fusion, Hip dislocation, distal ruptured nor biopsy sampled (except FNAB); no vessel phocomelia 2.9% involvement; no residual tumor at or beyond margin of Congenital aniridia - 1.1% resection " Stage 2: Genetic Aspect of Wilms Tumor " Extends beyond kidney but completely excised; may have " WT1 and WT2: specific Wilms tumor suppressor genes regional extension; may have vessel involvement; biopsy " Located in chromosome 11: performed; tumor spillage confined to flank; no evidence of " WT1 (band 11p13) tumor at or beyond margins of resection " WT2 (band 11p15) " Stage 3: " A result of mutation and/or deletion " With residual non-hematogenous tumor confined to " Explains the hereditary and sporadic cases abdomen; with anyone of the following: lymph nodes within abdomino-pelvic, penetrated or implants on peritoneal Clinical Signs and Symptoms surface, gross/microscopic residual tumor, incomplete resection; tumor spillage not confined to the flank Symptoms and Frequency " Stage 4: Palpable mass (Flank/Upper Abdomen) 60% " Hematogenous metastasis or lymph node mestastasis not Hypertension 25% confined to abdomino-pelvic area Hematuria 15% " Stage 5: Obstipation 4% " Bilateral involvement Weight loss 4% " Stage 1 to 5, Anaplasia (Unfavorable Histology) UTI 3% Diarrhea 3% Local Spread/Extension: Previous Trauma 3% " Renal sinus Others: Bleeding diathesis, Polycythemia, Nausea/Vomiting 8% " Intrarenal blood and lymphatic vessels Differential Diagnosis Neuroblastoma Soft-tissue sarcoma Intra-renal Neoplasms: o Congenital Mesoblastic Nephroma (Benign) o Clear Cell Sarcoma (Malignant) o Rhabdoid Tumor of the Kidney (Malignant) " " Through renal capsule Sites of Metastasis: " Lungs " Regional lymph nodes " Liver

Chest: ! Chest radiography: ! MRI: ! Renal scan: ! o

Detects metastasis Detects metastasis

Treatment Surgical: " Primary treatment " Radical or Modified radical nephrectomy " Contralateral Kidney exploration

Diagnostics Ultrasonography: o Abdominal: ! Initial study selected Chemotherapy: ! Localizes tumor, may demonstrate extension " Pre-operative (Neo-adjuvant): (renal vein and vena cava) " Bilateral Wilms tumor CT-scan: " IVC extension (above hepatic veins) o Abdominal: " Unresectable on surgical exploration ! Outlines and localizes tumor " Bulky or massive tumors ! Determines extension as well as regional and " Bleeding diasthesis or clotting abnormality distant spread " Post-operative (Adjuvant): ! May identify nephrogenic rests " All stages (ipsilateral/contralateral kidney) ! Plan surgery

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Radiation Therapy: " Stages 3 to 4 (favorable histology) " Stages 2 to 4 (unfavorable histology) " Stage 1 to 4 (clear cell sarcoma and rhabdoid tumor) Prognosis " Histologic features (key determinant): " Favorable vs. Unfavorable Histology " Lymph node involvement: " Important predictor of treatment failure " Chromosomal markers: " An important predictor of prognosis " Loss of heterozygosity of the 16q and 1p markers Neuroblastoma " Most common abdominal malignancy in children " 6%-10% of childhood cancers " 15% pediatric cancer deaths (U.S.) Pathophysiology: Results from aberrant migration and differentiation of neural crest cells destined to form the sympathetic ganglia From brain to pelvis Adrenal gland (60% of cases) Incidence 80% present before 4 years old Majority less than 2 years old Male:Female (1.3:1) Majority have stage 3-4 disease at time of diagnosis (poor prognosis) Overall survival: 30% Favorable prognosis: o Patients less than 1 year old o Primary mass and metastasis (liver, bone and skin) o Spontaneous regression and maturation Clinical Signs and Symptoms Palpable abdominal mass (75%) Malaise, Fever Abdominal Distention, Anorexia, Weight loss, Abdominal pain Bowel or Bladder dysfunction Respiratory symptoms (dyspnea, wheezes, stridor) Neurologic symptoms (dumbbell lesions or intra-spinal extension) Locoregional spread and metastasis (2/3): o Bone marrow o Cortical bone o Liver o Skin Diagnostics " Ultrasonography: " No characteristic diagnostic appearance " CT Scan: " Greater anatomic detail " Stage disease " Metastasis " Plan surgery " MRI: " Thoracic or intra-spinal extension " Biopsy: " Needle " Incision " Excisional Para-diagnostics " Plain radiography and Bone scan: " Bone metastasis " Radiolabeled Metaiodobenzyl Guanidine (MIBG): " Recognizes receptors for peptide somatostatin

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Provide information on tumor viability, disease extent, recurrence and prognosis

Serologic Studies: " Lactate Dehydrogenase (LDH) " Ferritin " Neuron-specific Enolase (NSE) " Vanillymandelic Acid (VMA)

Neuroblastoma Staging (INSS) Stage Definition 1 complete gross excision of localized tumor; with/without microscopic residual disease; representative ipsilateral lymph nodes negative for tumor 2A incomplete gross excision of localized tumor; representative ipsilateral lymph nodes negative for tumor complete or incomplete gross excision of localized tumor; representative ipsilateral lymph nodes positive for tumor;representative contralateral lymph nodes negative for tumor Unresectable unilateral tumor infiltrating across midline with or without lymph node involvement; or localized unilateral tumor with contralateral regional lymph node involvement; or midline tumor with bilateral extension With dissemination to distant lymph nodes, bone, bone marrow, liver, skin and/or other organs Localized primary tumor with dissemination limited to skin, liver, and/or bone marrow; limited to infant < 1 year old

2B

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Treatment " Surgery: " " " " "

Early staged (Stage 1 or 2) Localized Resectable Goal: total or near-total excision

Chemotherapy: " Neo-adjuvant or Adjuvant " Locally advanced (Stage 2b or 3) " Distant metastasis (Stage 4) Radiotherapy or Myeloablation (TBI): " Residual disease " Progressive hepatomegaly (Stage 4S) " Prior to bone-marrow transplant Immunotherapy:

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Prognosis " Shimada Pathologic Classification: Favorable Histology Unfavorable Histology Stroma rich: well differentiated; intermixed Nodular Stroma poor: Age <18 months Age 18-60 months Age >5 years old MKI* <200/5,000 MKI >100/5,000 None MKI <100/5,000 MKI >100/5,000 All

*MKI, Mitosis karyorrhexis index

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Neuroblastoma Biologic Markers Poor Prognosis Neuron-specific Enolase (NSE) >15ng/ml Lactate Dehydrogenase (LDH) >1,500IU/ml Ferritin >142ng/ml Vanillymandelic Acid/Homovanillic Acid ratio <1 N-myc Amplification >10 copies Chromosome 1 short arm (p) delete DNA Ploidy 1 CD44 Expression decreased Tyrosine Kinase A (TRKA) Expression increased Multi-drug Resistance Associated Protein (MDRP) increased Teratoma Gonadal: " Ovary: 27% " Testis: 5% " Extra-gonadal: " Sacrococcygeal (most common): 45% (60%) " Mediastinal: 6% " Central Nervous System (CNS): 5% " Retroperitoneal: 4% " Cervical: 3% " Head: 3% " Gastric, Hepatic, Pericardial, Umbilical cord: <1% " Malignancy in teratoma: " Yolk sac tumor or Endodermal sinus tumor: Most common " Neuroblastoma " Squamous cell carcinoma " Carcinoid " Others Malignancy at birth is uncommon, but increases with: " Age " Incomplete resection (residual tumor) Demography " Male:Female (1:3) " Most common tumor in the newborn " Incidence: 1 in 35,000-40,000 livebirths " Sacro-coccygeal (most common site) " Risk of malignancy: " testicular, mediastinal and sacrococcygeal " 10% at birth " 75% after 1 year of age " Isolated lesion (usually) but may present with associated anomalies

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Associated Anomalies " Anorectal malformation, sacral anomaly and presacral mass: " Well-recognized and most common " Urogenital anomalies: " Hypospadias, VUR, vaginal/uterine duplication Greek word: " Congenital dislocation of the hip: " Teratos : Monster " Klinefelters syndrome: " Onkoma : Swelling " Mediastinal teratoma (strongly associated) " Intracranial and retroperitoneal tumors Composed of multiple tissues foreign to the organ or site in " Histiocytosis: which they arise: " Mediastinal tumor " Endoderm " Hematologic malignancies and Hodgkins disease: " Mesoderm " Less common " Ectoderm

Clinical Presentation Embryology of Teratomas " Symptoms depend on location of the mass: " Theory A: " Sacrococcygeal: " Totipotent primordial germ cells (endodermal cells of yolk " Visible mass over sacro-coccygeal area sac) migrate to gonadal ridges (4th to 5th week) " Urinary retention/obstruction " Missing target destination gives rise to a teratoma (brain to " Constipation coccygeal area) " Abdomino-pelvic mass " Theory B: " Thoracic: " Arises from primitive streaks or nodes (3rd week) " Anterior mediastinum (most common site) " Gastrulation (gives rise to all 3 germ layers) " Respiratory distress (infants) " Theory C: " Incidental finding (older children) " A form of incomplete twinning (parasitic twin) " Abdominal/Retroperitoneal: " Suprarenal (most often) Tumors contain: " Head and Neck: " Skin elements " Neck, nasopharynx, oral cavity and orbit: " Neural tissue (immature elements) " Most common extracranial sites " Teeth Differential Diagnosis " Fat " Meningocoele: " Cartilage/Bone " Usually is proximal to the sacrum and covered by dura " Intestinal mucosa " Bulging fontanelle on gentle pressure of the mass " Small bowel " Ultrasonography, CT scan or MRI " Limbs " Lymphangioma " Lipoma " Not only is the overall tissue architecture variable but also the spectrum of " Tail-like remnants: cellular differentiation: " Mature cells: Diagnosis and Screening " Most benign teratomas " Plain Radiography " Immature cells: " Ultrasonography " 20-25% mostly neuroepithelium " CT-scan " MRI " Degree of histologic immaturity: " Serum Markers: " Has prognostic significance only in ovarian teratomas " Alphafetoprotein (AFP): " Considered normal and has no prognostic significance in neonatal " Yolk sac tumor and embryonal carcinoma teratoma

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Normally high in neonates and normalizes with age " Post-operative half-life is 6 days B-human chorionic gonadotropin (B-hcg): " Choriocarcinoma " May cause precocious puberty Carcino-embryonic antigen: " Rare "

Treatment " Surgical Excision: " Complete excision " Incomplete excision carries the risk of malignant transformation " Chemotherapy: " Adjuvant or neoadjuvant

DEANNA GUIILLEN In

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