Congenital and acquired mesocolic hernias presenting with small bowel obstruction in childhood and adolescence.

OBJECTIVE: The objective was to present a case series of pediatric patients presenting with small bowel obstruction secondary to both congenital and acquired internal mesocolic hernias, and the use of imaging technology in the management of this condition. METHODS: A retrospective review of patients treated at the Yale-New Haven Children's Hospital for small bowel obstruction from 1998 to 2008 (n = 6) who presented with acute small bowel obstruction secondary to internal mesocolic hernias was performed. RESULTS: We present 6 patients with small bowel obstruction caused by congenital (n = 4) and acquired (n = 2) mesocolic hernias after previous surgery. The median age at presentation was 13 years. Small bowel obstruction with a mesocolic hernia was identified by preoperative abdominal computerized tomography in 3 patients (50%) and at operation in the others. The mean length of stay was 6 days, with no recurrent episodes in the follow-up period. CONCLUSION: Small bowel obstruction secondary to mesocolic hernias, although rare, may be considered in the differential diagnosis of patients with history of malrotation or abdominal wall defects owing to their association with congenital mesenteric anomalies. This condition requires special attention from the clinician because of its catastrophic consequences. Imaging studies are an important asset because of the difficulty in making an accurate clinical diagnosis and the rarity of internal hernias.

Pulmonary squamous cell carcinoma associated with repaired congenital tracheoesophageal fistula and esophageal atresia.

We report a 19-year-old man with pulmonary squamous cell carcinoma (SCC) who had a history of vertebral, anal, cardiac, tracheal, esophageal, renal, and radial limb defects (VACTERL) association and tracheoesophageal fistula (TEF) + esophageal atresia (EA) repair as an infant. Children that undergo TEF + EA repair may have an increased risk for developing cancer as they reach adulthood.

Outcomes after a Ladd procedure for intestinal malrotation with heterotaxia.

PURPOSE: Intestinal rotation abnormalities and complex congenital heart disease associated with heterotaxia coexist. Despite the risk for midgut volvulus, performing a Ladd procedure for asymptomatic malrotation with heterotaxia remains to be controversial because the presumed risk for postoperative complications is thought to exceed the benefits of the operation. The purpose of this study was to review the incidence of complications after a Ladd procedure in asymptomatic patients with heterotaxia to guide recommendations for this patient population. METHODS: The medical records of all patients with heterotaxia who underwent a Ladd procedure for asymptomatic malrotation between 1984 and 2004 were reviewed. Type of cardiac disease, postoperative complications, and survival were recorded. RESULTS: Twenty-two patients (9 boys and 13 girls) with heterotaxia underwent an elective Ladd procedure after their medical stabilization or surgical correction or palliation of their cardiac anomaly. Of these patients, 19 were younger than 1 month at the time of the operation. The remaining 3 patients underwent the operation when they were between 2 and 5 months old. Three of the 22 patients (14%) developed postoperative intestinal obstruction: lysis of adhesions was performed in 1 patient; another patient required a staged bowel resection for a closed loop obstruction; and yet another patient had recurrent midgut volvulus 4 years after an incomplete initial Ladd procedure. All patients survived the initial and secondary procedures. Four deaths, all more than 1 month after the surgery, occurred as sequelae of the underlying cardiac anomaly. Length of follow-up ranged from 1 to 17 years. CONCLUSIONS: We report on a 14% risk of postoperative bowel obstruction after an elective Ladd procedure, as compared with a small but significant incidence of midgut volvulus in patients with malrotation in the setting of complex congenital heart disease. Our results support the conclusion that an elective Ladd procedure at a time of relative cardiac stability for selected patients with heterotaxia has an acceptably low morbidity and should be considered to prevent midgut volvulus.

Postoperative outcomes of extremely low birth-weight infants with necrotizing enterocolitis or isolated intestinal perforation: a prospective cohort study by the NICHD Neonatal Research Network.

OBJECTIVE: Purposes of this study were: 1) to compare mortality and postoperative morbidities (intra-abdominal abscess, wound dehiscence, and intestinal stricture) in extremely low birth weight (ELBW) infants who underwent initial laparotomy or drainage for necrotizing enterocolitis (NEC) or isolated intestinal perforation (IP); 2) to determine the ability to distinguish NEC from IP preoperatively and the importance of this distinction on outcome measures; and 3) to evaluate the association between extent of intestinal disease determined at operation and outcome measures. BACKGROUND: ELBW infants who undergo operation for NEC or IP have a postoperative, in-hospital mortality rate of approximately 50%. Whether to perform laparotomy or drainage initially is controversial. Also unknown is the importance of distinguishing NEC from IP and the current ability to make this distinction based on objective data available prior to operation. METHODS: A prospective, multicenter cohort study of 156 ELBW infants at 16 neonatal intensive care units (NICU) within the NICHD Neonatal Research Network. RESULTS: Among the 156 enrolled infants, 80 underwent initial peritoneal drainage and 76 initial laparotomy. Mortality rate was 49% (76 of 156). Ninety-six patients had a preoperative diagnosis of NEC and 60 had presumed IP. There was a high level of agreement between the presumed preoperative diagnosis and intraoperative diagnosis in patients undergoing initial laparotomy (kappa = 0.85). The relative risk for death with a preoperative diagnosis of NEC (versus IP) was 1.4 (95% confidence interval, 0.99-2.1, P = 0.052). The overall incidence of postoperative intestinal stricture was 10.3%, wound dehiscence 4.4%, and intra-abdominal abscess 5.8%, and did not significantly differ between groups undergoing initial laparotomy versus initial drainage. CONCLUSIONS: Survival to hospital discharge after operation for NEC or IP in ELBW neonates remains poor (51%). Patients with a preoperative diagnosis of NEC have a relative risk for death of 1.4 compared with those with a preoperative diagnosis of IP. A distinction can be made preoperatively between NEC and IP based on abdominal radiographic findings and the patient's age at operation. Future randomized trials that compare laparotomy versus drainage would likely benefit from stratification of treatment assignment based on preoperative diagnosis.

Thirty-five-year institutional experience with end-to-side repair for esophageal atresia.

HYPOTHESIS: End-to-side repair (ES) with ligation of the tracheoesophageal fistula (TEF) reduces the risks of stricture and gastroesophageal reflux disease requiring operation compared with the end-to-end repair of esophageal atresia and distal TEF. DESIGN: Case series with institutional and historical control subjects. SETTING: Referral children's hospital. PATIENTS: One hundred thirty-four infants diagnosed as having esophageal atresia and distal TEF between June 30, 1968, and July 1, 2003. INTERVENTIONS: Ninety-six infants having ES and 38 having end-to-end repair. MAIN OUTCOME MEASURES: Patients were studied for overall survival, surgical complications, and well-being during the first year of life. RESULTS: Survival was 95% vs 90% (patients undergoing ES vs end-to-end repair). Complications included anastomotic leak, 8% vs 13%; recurrent TEF, 7% vs 3%, with only 1 recurrence in the last 28 patients having ES; anastomotic stricture (requiring dilatation), 5% vs 13%; gastroesophageal reflux disease requiring operation, 6% vs 18%; and esophageal dysmotility, which was present following nearly all ES and end-to-end procedures. Tracheomalacia-related respiratory symptoms following ES decreased from 50% to 11% at 1 year of age. Age-appropriate diet following ES was achieved in 93% by 1 year; 5% experienced occasional dysphagia or choking episodes. CONCLUSIONS: The ES operation is accompanied by a reduced rate of stricture and gastroesophageal reflux disease requiring operation compared with end-to-end repair. Earlier concerns regarding an unacceptable risk of recurrent TEF were not substantiated.