Biliary Reconstruction by Isolated Jejunal Interposition Loop: Our Experience after Excision of Choledochal Cyst.

BACKGROUND: Choledochal cyst is a fairly common hepatobiliary condition in pediatric surgical practice. For the most common type (type I), it is well established that the total excision of the cyst with an wide biliary-enteric anastomosis is key for long-term good result. Multiple options remain for biliary-enteric reconstruction after excision. Jejunal interposition loop reconstruction is thought to be the most physiologic. MATERIALS AND METHODS: We have retrospectively reviewed the data of patients of type I choledochal cysts which were operated between January 2010 and September 2018 and undergone jejunal interposition loop reconstruction. Clinical presentation, investigations, operative procedure morbidity and complications were reviewed. RESULTS: There were 33 patients, with a male-to-female ratio of 1:3 and a mean age of 4.63 years (mean ± standard deviation [SD] = 4.63 ± 2.98 years). The follow-up period ranged from 3 to 81 months (mean ± SD = 36.30 ± 19.24 months). There was no stricture or cholangitis. Reoperation required in one due to leak at biliary-enteric anastomosis leading to biliary peritonitis. One each had pancreatitis and prolonged ileus. Two bile leaks stopped spontaneously. Operative time and postoperative hospital stay were 228.78 ± 40.43 min (mean ± SD) and 8.96 ± 3.63 days (mean ± SD), respectively. CONCLUSION: Jejunal interposition loop reconstruction is safe and reproducible with acceptable morbidity. However, if this procedure is abandoned, the long-term benefits may remain ever elusive.

Clinicopathological study of intestinal smooth muscles, interstitial cells of Cajal, and enteric neurons in neonatal jejuno-ileal atresia with special reference to muscle morphometry.

PURPOSE: To assess the thickness of the intestinal smooth muscle layer and analyze the distribution and density of interstitial cells of Cajal (ICC) and enteric neurons in the proximal and distal segments of neonatal jejuno-ileal atresia. METHODS: This is an observational study done over a period of one year in which fifteen cases of jejuno-ileal atresia were included. All the cases underwent laparotomy and resection of the atretic segment with variable portions of the dilated proximal segment and distal segment. Histopathological analysis was done on the sections taken from proximal segments (at 3 cm, 5 cm & 8 cm) and the distal segment (at 2 cm) from the atretic portion. The mean thickness of the inner circular muscle layer (ICML) and outer longitudinal muscle layer (OLML) was assessed in the above segments using image morphometry. In addition, we also analyzed the distribution and density of the ICCs and enteric neurons in the different segments using immunohistochemistry for c-kit and S-100, respectively. Controls included normal jejuno-ileal segments resected from postmortem cases (n=7) and other nonrelated surgeries (n=3). The findings were then compared with each-other and with normal controls. RESULTS: Mean thickness of ICML and OLML of the proximal segments at 8 cm was significantly lower than at 3 cm and 5 cm of ileal and jejunal atresias (p≪ 0.5). The mean thickness of ICML and OLML of distal segments at 2 cm was similar to the controls in all the atretic cases (p≫ 0.5). The mean ICML thickness at proximal 8 cm segment was similar to the distal segment of both ileal & jejunal atresias (p= 0.06 & 0.37 respectively). The mean thickness of the OLML of the proximal 8 cm segments was significantly more than that at the distal segment (p=0.008) in ileal atresias but was similar in cases of jejunal atresias (p=0.07). Both the proximal and distal segments of ileal as well as jejunal atresias showed reduction in distribution and density of ICCs, as compared to normal controls. The density of ICCs in proximal segments at 3 cm and 5 cm was similar in both ileal (p=0.33) and jejunal segments (p=0.41) but was significantly lower than the proximal 8 cm segments (p≪0.05).The distribution of ICCs in the proximal segment at 8 cm was similar to the distal segments (p≪0.05). S-100 staining showed dense expression of neurons and glial cells with presence of submucosal giant ganglia within the proximal dilated segments as compared to the distal segments and the controls, which were more marked at 3 cm and 5 cm levels than at 8 cm level. CONCLUSION: Muscle morphometry using image analysis is a simple technique to assess the thickness of the intestinal smooth muscle layers. There is significant smooth muscle hypertrophy along with marked alteration in density and distribution of ICCs and ENS in the dilated proximal segments up to 5 cm, and relatively milder changes at 8 cm levels, as compared to the distal segments and the controls. TYPE OF STUDY: Prognosis study. LEVEL OF EVIDENCE: Level II.

Synchronous Bilateral Wilms Tumor: Five-Year Single-Center Experience with Assessment of Quality of Life.

CONTEXT: Synchronous Bilateral Wilms tumor (sBWT). AIMS: This study aimed to assess the outcome of patients with sBWT treated on SIOP protocol. SETTINGS AND DESIGN: Retrospective and prospective randomized study. SUBJECTS AND METHODS: SIOP 93-01 protocol was used to study nine patients of sBWT in a single center and followed up over a period from 2 to 5 years. STATISTICAL ANALYSIS USED: Unpaired t-test and Mann-Whitney U-test were used for analysis. RESULTS: Of nine patients, six were included in the study as three patients lost to follow-up. Among the six patients, there were four girls and two boys with a median age of 2 years. Mean regression in the size of tumor was 87% in four out of six patients. Tumor with unfavorable histology showed 32% response (ratio of favorable: unfavorable histology 2:1). Event-free survival rate was 81.3% and overall survival was 90% over 2-5 years. Recurrence was seen in two patients of whom one had Denys-Drash syndrome. Mean DTPA glomerular filtration rate was 91.4/ml/min/1.73 m2 preoperatively and that of 3 months after completion of treatment was 84/ml/min/1.73 m2. Health-related quality of life (HRQOL) using Pediatric Quality of Life Inventory and Lansky Play Performance Scale revealed significant improvement results of all functioning domains such as physical, social, emotional, and school subscales with P < 0.05 and performance scale (P < 0.04). CONCLUSIONS: We suggest SIOP protocol for sBWT and bilateral nephron-sparing surgery in two stages. However, long-term follow-up is required to assess the ultimate renal function outcome. HRQOL is an essential guide in improving the conditions of pediatric cancer survivors.

Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2: Role of Surgery with Brief Review of Literature.

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.

Demographic Study and Management of Colonic Atresia: Single-Center Experience with Review of Literature.

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.

Cytodiagnosis of inflammatory myofibroblastic tumor: A report of three cases in infants.

Inflammatory myofibroblastic tumor is a tumor of intermediate grade with a low rate of metastasis. The tumor often mimics malignancy. There is sparse literature available describing the cytological findings of this rare condition. It often presents in infancy and childhood as an intra-abdominal mass. Here, we describe the cytological findings of three cases of inflammatory myofibroblastic tumor in infants aged 10 months, 4 months, and 15 months, respectively. While the first two cases are fine needle aspiration cytology (FNAC) smears, the third case is that of an intraoperative imprint cytology. All the three smears showed low cellularity with small clusters of bland spindle cells, along with sprinkling of inflammatory cells, suggestive of an inflammatory myofibroblastic tumor. The diagnosis was later confirmed on histopathology and positive immunostaining for ALK.