Context and Aim:The aim of this study is to highlight the rarity of this disorder and its associated anomalies and our objective was to review our experience in the management of colonic atresia (CA) with respect to staged surgery versus one-step procedure for a better outcome of the disease.Settings and Design:A randomized, controlled, single-center study conducted over a period of 4 years from October 2013 to 2017.Subjects and Methods:Nine cases were operated for CA of which three underwent primary anastomosis and six underwent initial colostomy followed by definitive surgery. Age, sex, body weight, clinical presentation, type of atresia, site, time interval of operation, associated anomalies, initial procedure, postoperative complications, final procedure, biopsy, hospital stay, and outcome were noted.Statistical Analysis Used:Microsoft Excel was used for statistical analysis.Results:Out of 9 patients, 6 were males and 3 were females, 7 patients survived and 2 patients died (mortality 22.2%), of which one patient with primary anastomosis had leak and sepsis and one patient with primary anastomosis had associated Hirschsprung's disease (HD). Mean time gap for operation was 12.8 h and mean hospital stay was 3.5 days for initial colostomy and 21 days for primary anastomosis. Wound dehiscence occurred in 4 patients. Malrotation was found in 3 patients, HD in 2 patients, cardiac anomaly in 2, Meckel's diverticulum in 1, and cleft lip in 1 patient. Type IIIa atresia was found in in 6 patients, Type I in 2, and Type II in 1 patient. The most common site was transverse colon (n = 5).Conclusion:Diagnosis and management of CA is a challenge. Early presentation and diagnosis should be prompt. Staged procedure with initial colostomy followed by definitive procedure is the preferred choice. Associated anomaly HD must be ruled out.

Background: Enhanced recovery after surgery (ERAS) protocols after colorectal surgery focused on reduced bowel preparation, standardized feeding schedule, earlier return of bowel function, and earlier resumption of normal activities. ERAS in pediatric surgical practice is not well established. The present study aims to present the results of two colonic anastomosis techniques of interrupted single-layered closure: Halsted (Horizontal Mattress) and Matheson (serosubmucosal or appositional extramucosal) along with two different methods of colostomy wound closure and their influence on the adoption of ERAS protocol of early feeding and early discharge. Materials and Methods: This single institute-based randomized control study was conducted in a tertiary care facility in Kolkata for 2.4 years. Patients were chosen randomly for serosubmucosal (Group I) and full-thickness (Group II) anastomosis. Results: Among total of 91 patients (Group I–43 and Group II–48), Return of bowel sounds and passage of bowel averaged 1.51 ± 0.51 and 1.91 ± 0.55 days in Group I and 1.91 ± 0.57 and 3.9 ± 0.66 days in Group II, respectively. Postoperative hospital stay averaged 5.88 ± 1.12 days in Group I and 8.9 ± 1.17 days in Group II. Overall 15 (16.48%) patients had complications among which SSI (Suprficial surgical site infection) and minor leaks (Group I–3 and 1 and Group II–5 and 3, respectively) which were treated conservatively (Clavien–Dindo Grade-I) and three major leaks under Group II requiring surgical intervention (Clavien–Dindo Grade-III). Conclusion: This study concludes that the technique of colostomy closure in the form serosubmucosal closure helps in the implementation of ERAS protocol by producing early bowel movement, early initiation of food, and less postoperative complications.

Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.