Clinical variables as prognostic tools in pediatric-onset ulcerative colitis: a retrospective cohort study.

BACKGROUND: Clinical variables may identify a subset of patients with pediatric-onset ulcerative colitis (UC) (≤18 years at diagnosis) at risk for adverse outcomes. We postulated that routinely measured clinical variables measured at diagnosis would predict colectomy in patients with pediatric-onset UC. METHODS: We conducted a chart review of patients with pediatric-onset UC at a single center over a 10-year period. We compared patients with and without colectomy across several variables, used proportional hazards regression to adjust for potential confounders, and assessed the ability of a UC risk score to predict colectomy. RESULTS: Among 470 patients with inflammatory bowel disease ICD9-coded encounters, 155 patients had UC and 135 were eligible for analysis. The 1- and 3-year colectomy rates were 16.7% (95% confidence interval [CI]: 11.0%-24.8%) and 35.6% (26.7%-45.4%). White blood cell (WBC) count and hematocrit measured at diagnosis were associated with colectomy at 3 years, even after correcting for potential confounding variables. A UC Risk Score derived from the WBC count and hematocrit was strongly associated with colectomy risk, with a high negative predictive value (NPV) for colectomy at 1 and 3 years (NPV = 0.95 and 0.89, respectively), but low positive predictive value (PPV = 0.22 and 0.38, respectively). CONCLUSIONS: A risk score calculated from WBC and hematocrit measured at diagnosis was associated with, but incompletely predictive of, colectomy in pediatric-onset UC. These data suggest 1) routinely measured clinical variables may have a prognostic role in risk stratification, and 2) multicenter prospective studies are needed to optimize risk stratification in pediatric UC. Our findings have impact on the design of such studies.

Reflux esophageal stricture--a review of 30 years' experience in children.

PURPOSE: Strictures of the esophagus in children may have multiple etiologies including congenital, inflammatory, infectious, caustic ingestion, and gastroesophageal reflux (peptic stricture [PS]). Current literature lacks good data documenting long-term outcomes in children. This makes it difficult to counsel some patients about realistic treatment expectations. The objective of this study is to evaluate our institutional experience and define the natural history and treatment outcomes. METHODS: A retrospective review of clinical data obtained from children who underwent dilation for PS was performed. RESULTS: Over the past 30 years, 114 children and adolescents received 486 dilations. The most common indications for stricture dilation were PS (42%) and esophageal atresia (38%). Other lesser indications included congenital, foreign body, corrosive, cancer, radiation, allergic, and infectious. This review focuses on the 48 children with PS. Of the children with PS, a congenital anomaly was identified in 23 children; and 12 had neurologic impairment. Average age at presentation was 10.2 years (range, 0.5-18.3 years). Most patients had had symptoms for many months before diagnosis. Peptic stricture was most common in the lower esophagus (n = 39). However, middle (n = 8) and upper (n = 1) strictures were occasionally identified. Noncompliance with medical therapy was a challenge in 12% (n = 5) of children. Children with a PS received a median of 3 dilations, but a subset of 5 patients with severe strictures underwent up to 48 dilations (range, 1-48). Repeated dilations were required for a median of 20 months (range, 1-242 months). Among patients receiving esophageal dilation for PS, 94% required an antireflux procedure (19% required a second antireflux surgery). A subgroup of patients (n = 10) was identified who required extended dilations, multiple surgeries, and esophageal resection. This subgroup had a significantly longer period of symptomatic disease and increased risk of esophageal resection compared with those patients requiring fewer dilations. Surgical resection of the esophageal stricture was ultimately required in 3 children with PS after failure of more conservative measures. CONCLUSION: Children and adolescents presenting with reflux esophageal stricture (PS) frequently require antireflux surgery, redo antireflux surgery, and multiple dilations for recurrent symptoms. We hope that these data will be of use to the clinician attempting to counsel patients and parents about treatment expectations in this challenging patient population.

The split abdominal wall muscle flap repair for large congenital diaphragmatic hernias on extracorporeal membrane oxygenation.

BACKGROUND: Numerous techniques exist for repairing large congenital diaphragmatic hernias (CDHs) including prosthetic patches, tissue-engineered grafts, and various muscle flaps. A split abdominal wall muscle flap is a simple, durable way to repair a large diaphragmatic hernia. This technique has not gained widespread use, and some have suggested that it would be inappropriate in the setting of extracorporeal membrane oxygenation (ECMO) because of bleeding risk. We present our series of diaphragmatic hernias with a focus on those repaired with the split abdominal wall technique while on ECMO. METHODS: A retrospective, single-institution chart review was performed on all patients who underwent surgical repair for CDH over 6 years beginning in August 2000. RESULTS: Seventy-five patients underwent repair. Sixteen were performed with patients on ECMO. Of these, 4 were closed primarily, 7 used a prosthetic patch, and 5 used a split abdominal wall muscle flap. Two patients in the prosthetic group developed a recurrent hernia, and 2 required reoperation for bleeding while on ECMO. No reoperations for bleeding were required in the abdominal muscle flap group. CONCLUSIONS: The split abdominal wall muscle flap can be safely performed on anticoagulated patients. We believe it is a practical option for repairing large CDHs.

The split abdominal wall muscle flap--a simple, mesh-free approach to repair large diaphragmatic hernia.

PURPOSE: The authors present a technique to repair large diaphragmatic hernias that is simple, can be done primarily, and offers a durable closure with living tissue. METHODS: A review of congenital diaphragmatic hernias was performed for the period between January 1991 and August 2000. Repair types included primary repair, synthetic patch, or a split abdominal wall muscle flap. The split abdominal wall muscle flap is performed by downward rotation of the internal oblique and transversalis abdominal wall muscles. This repair requires that the initial subcostal incision be positioned at least 4 to 5 cm below the costal margin, low enough to insure an adequate length of muscle will be available to fill the defect. RESULTS: Eight of 158 children with congenital diaphragmatic hernia underwent repair by abdominal wall muscle flap. Five of 8 had complete agenesis. Five survived the newborn period. Of the survivors, 4 of 5 required an additional surgical procedure, but none have had a recurrent hernia. CONCLUSIONS: The split abdominal wall muscle flap is an effective technique to close large diaphragmatic hernias. The repair was carried out successfully in 8 patients with massive defects. In the surviving patients, the repair has proven to be a durable restoration of the diaphragm.

High-dose steroids, ursodeoxycholic acid, and chronic intravenous antibiotics improve bile flow after Kasai procedure in infants with biliary atresia.

BACKGROUND/PURPOSE: Early reports suggest that the use of steroids after Kasai portoenterostomy may improve bile flow and outcome in infants with biliary atresia. METHODS: Of 28 infants with biliary atresia, half received adjuvant high-dose steroids, and half received standard therapy. Infants in the steroid group (n = 14) received intravenous solumedrol (taper of 10, 8, 6, 5, 4, 3, 2 mg/kg/d), followed by 8 to 12 weeks of prednisone (2 mg/kg/d). The steroid protocol also included ursodeoxycholic acid indefinitely and intravenous antibiotics for 8 to 12 weeks followed by oral antibiotic prophylaxis. Infants in the standard therapy group (n = 14) received no steroids, occasional ursodeoxycholic acid, and perioperative intravenous antibiotics followed by oral antibiotic prophylaxis. The infants were not assigned randomly, but rather received standard therapy or adjuvant steroid therapy according to individual surgeon preference. RESULTS: Eleven of 14 (79%) in the steroid group and 3 of 14 (21%) in the standard therapy group had a conjugated bilirubin level less than 1.0 within 3 to 4 months of surgery (P <.001). Fewer patients in the steroid group (21% v 85%) required liver transplantation or died during the first year of life (P <.001). Infants in the steroid group did better despite the fact that this group included 5 infants with biliary atresia-polysplenia-heterotaxia syndrome, a subgroup that might have been expected to have a poor prognosis. Neither bile duct size nor liver histology was a reliable predictor of success or failure in either group. CONCLUSIONS: Adjuvant therapy using high-dose steroids, ursodeoxycholic acid, and intravenous antibiotics may accelerate the clearance of jaundice and decrease the need for early liver transplantation after Kasai portoenterostomy.

Biliary tract anomalies in thoraco-omphalopagus conjoined twins.

Thoraco-omphalopagus conjoined twins were separated urgently on the ninth day of life because of bowel obstruction and impending intestinal perforation. Preoperative studies clarified the cardiac, intestinal, and hepatic parenchymal anomalies, but the precise biliary anatomy remained obscure. Operative cholangiogram through the single gallbladder visualized only the extrahepatic biliary anatomy in twin A. At reoperation for abdominal patch removal, exploration in twin A found biliary atresia. Because of the unusual duodenal anatomy and extreme short bowel, the authors elected to treat the biliary atresia in twin A with an appendix portoenterostomy. Twin B had no gallbladder and a profoundly hypoplastic biliary tree. Both twins have normal bilirubin and liver function at 18 months of age. The anatomic details of 8 additional cases of complex biliary tract anomalies in thoraco-omphalopagus conjoined twins are reviewed. In all cases, the complex biliary anatomy has been associated with conjoined duodenum. In most cases the preoperative technetium 99m HIDA scan has been inconclusive or misleading.