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Introduction: Ureterocele is a rare urogenital malformation. The treatment is variable and complicated as it depends on several factors. The aim of this study was to evaluate the management and outcomes of children with ureterocele and to compare single system and duplex system ureteroceles. Materials and Methods: A retrospective study was conducted and all patients with ureterocele operated from January 1992 to December 2018 were included. The records of those included were assessed, and a detailed case record sheet was filled. The outcome parameters assessed were the persistence of symptoms and additional surgical procedure performed. Results: Forty-seven patients (28 boys and 19 girls) with a median age of presentation of 21 months were included. Four patients had bilateral ureterocele. Overall, 51 renal units with ureterocele were studied. Twenty renal units of the 31 renal units with duplex system underwent cystoscopic decompression, and of these, 8 (40%) needed a second procedure. Fourteen renal units of the remaining 20 renal units with single system underwent cystoscopy and decompression, and of these, 1 (7%) required another procedure (P = 0.024). Sixteen renal units had ectopic ureterocele, of which 9 (56%) underwent heminephrectomy/nephrectomy. Intravesical ureterocele was present in 35 renal units, of which only 2 (5.7%) underwent nephrectomy or heminephrectomy (P < 0.001). Conclusion: Duplex system ureteroceles are more likely to require a second procedure following an endoscopic puncture. Units with ectopic ureterocele were more likely to need nephrectomy.

Aim: The aim of this study was to review the compliance to the provisional date of elective surgery allotted to pediatric surgery outpatients at our center. Patients and Methods: This retrospective study was conducted in the department of pediatric surgery at our center. The children who were brought to the outpatient department with a previously allotted date of admission for elective surgery (DAES) and the final operation theater (OT) lists of elective surgery between July 2007 and September 2018 under one senior consultant (VB), were reviewed. Results: A total of 2409 cases were allotted a DAES during the study period. Out of these, 12% (288/2409) were defaulters with a male gender preponderance (82%). Most of the defaulters (>49%) belonged to the day-care surgery group, followed by simple reconstructive (32%), major reconstructive (18%), and infectious sequelae groups (

Adenomyoma of the stomach is a benign tumor with a very low incidence. Clinical presentation and imaging modalities are usually nonspecific and variable. A rare case of gastric adenomyoma in a 12-year-old child is being reported who presented with gastric outlet obstruction. The diagnosis could only be established after an excision biopsy performed after multiple diagnostic modalities failed to clinch the diagnosis. The case is being reported in view of the rarity of this entity in the pediatric age group as a cause of gastric outlet obstruction.

Aim: The aim was to study the correlation of hepatic artery resistive index (HARI) with the portal pressure (PP) and its surrogate marker serum nitric oxide (NO) levels and to determine the validity of HARI as noninvasive indirect marker of PP in extrahepatic portal venous obstruction (EHPVO) pre- and postoperatively. Methods: A prospective study was conducted on 19 patients with EHPVO undergoing proximal lienorenal (LR) shunt or devascularization from February 2016 to January 2018. HARI, calculated from Doppler sonography, and NO were measured preoperatively and 14, 30 and 90 days postoperatively. Intraoperatively, PP was measured before splenectomy, and both PP and NO were measured postshunt. Results: Mean age was 10.58 ± 2.85 years, and male:female ratio was 15:4. LR shunt was done in 16 while three patients required devascularization. There was a significant fall in the HARI (0.06 ± 0.02, P = 0.02), NO (14.31 ± 2.66 μmol/l, P < 0.001), and PP (11.81 ± 1.03 mmHg, P < 0.001) following shunt surgery. However, fall in HARI did not correlate with fall in PP. Preoperative HARI also did not correlate with preshunt/devascularization PP nor with preoperative NO. Postoperatively, HARI did not correlate with NO at 14-, 30-, and 90-day follow-up. Conclusion: HARI bears no correlation with PP or NO. Hence, it cannot be used as an indirect marker of PP.

Highlights • Prelicensure trials of ROTAVAC® not powered to assess risk of intussusception. • During ROTAVAC® rollout we assessed risk of intussusception in infants in 3 states. • No increased risk of intussusception within 21 days of 1st and 2nd dose. • No increased risk of intussusception within 21 days of any single dose or all 3 doses., Background Pre-licensure trials of ROTAVAC® were not adequately powered to assess risk of intussusception, a rare adverse event associated with other rotavirus vaccines in some settings. We examined the risk of intussusception after ROTAVAC® vaccination among Indian infants during pilot rollout of the vaccine in the public health system in three states - Himachal Pradesh, Maharashtra and Tamil Nadu. Methods Passive surveillance for intussusception was set up in 35 sentinel health facilities covering 26.3 million population in the three states under monitoring of an Interministerial-Interagency Steering Committee. Clinical and immunization data were collected by independent teams. An expert committee blinded to vaccination status, classified intussusception cases using Brighton criteria. The self-controlled case-series method was used to estimate risk of intussusception (Brighton Level 1) after ROTAVAC® vaccination. Results 151 intussusception cases were included in the analysis. The relative incidence (incidence during the risk period compared to the control period) 1–21 days after doses 1 and 2 combined was 1.56 (95% CI, 0.0–5.28) and that for three doses combined was 1.88 (95% CI, 0.76–4.30). Attributable risk 1–21 days after doses 1 and 2 combined was 0.11 (95% CI, 0.0–0.25) and that for 3 doses combined was 0.42 (95% CI, 0.0–0.70) per 100,000 doses. Conclusions No increased risk of intussusception within 21 days of receipt of the first two doses combined or all 3 doses combined of ROTAVAC® was detected.

Purpose: To identify complications, their incidence and risk factors for their occurrence in patients of esophageal atresia (EA) in the 1st year after discharge following surgery. Materials and Methods: Cases of EA discharged after surgical intervention in the period of July 2011–July 2013 were considered a cohort. All data regarding demographics, investigations, surgical procedure, outcome, and follow-up were recorded. Results: Seventy-six such patients were discharged in the study period, six of whom were lost to follow-up, and hence, seventy patients were included in the study. Of these 70, 48 (69%) had esophageal continuity restored (46 EA + tracheoesophageal fistula [TEF]; 2 pure EA), while 22 (31%) had been diverted (3 pure EA; 8 EA + TEF following major leak; 11 long gap EA + TEF). Risk of developing any complication (except death) was 48/70 (68%; 95% confidence interval [CI] = 57.4–79.7). Twenty-six of 48 patients with esophageal continuity restored, demonstrated narrowing on contrast study (54%; 95% CI = 39.5–68.7) but only 18 of these 48 (37.5%) had dysphagia. Thirty-one of seventy had an episode of lower respiratory tract infection (LRTI) (44.2%; 95% CI = 32.3%–56.2%). Poor weight gain was observed in 27/70 (37%), and this was significantly common in diverted patients (63% vs. 25%; P = 0.009). Twenty-one of total 70 (30%) patients died within the 1st year following discharge. Conclusions: Sixty-eight percent of cases developed some complication, while 30% succumbed within the 1st year of life following discharge. The common complications were stricture, LRTI, and poor weight gain. All of these were common in diverted patients.

Aim To assess the long-term urologic outcomes in follow-up of patients of sacrococcygeal teratoma (SCT) using urodynamic study (UDS) in addition to clinical and radiologic evaluation. Methods A prospective study of clinical, radiological and urodynamic evaluation in patients with SCT who underwent resection between January 2002–June 2015 and were followed up till January 2016 was conducted. Results Total 57 patients, 42 (73.7%) females and 15 (26.3%) males with 35 (62.4%) following treatment for benign and 22 (38.5%) for malignant disease were included. Twenty-eight of 57 (49.12%) had urological problems. Clinical complaints in 21 (36.8%) patients included stress urinary incontinence-14 (66.7%), enuresis-9 (42.9%), and poor stream or dribbling of urine-6 (28.6%). Eight of 51 patients (15.7%) had abnormal ultrasound findings, which included contracted, trabeculated thick walled bladder (3), bilateral hydronephrosis (3) and significant post void residue (PVR) (6). Seven of 57 underwent micturating cystourethrogram (MCU), 5 had an abnormal report[significant PVR (4), small trabeculated bladder (3), reflux (2) and large capacity bladder (1)]. Urodynamic study was done in 27 patients, 18/27 (66.7%) had abnormalities. Six patients without any clinical or ultrasonographic abnormalities had abnormal UDS. Total 28 (49.12%) had urological comorbidities. Three patients had overactive bladder, five dysfunctional voiding, one underactive bladder and one had giggle incontinence. Children were managed by behaviour therapy and pharmacotherapy. Conclusion Urodynamic evaluation could detect abnormalities in patients who had no urinary complaints or abnormality on ultrasound. The abnormalities have a potential for progressive upper tract damage. Urodynamics should be an integral part of urological surveillance in patients operated for SCT. Type of study Prognostic study. Level of Evidence Level II (Prospective cohort study).

Purpose: The purpose of the study is to evaluate the outcome of children with giant mediastinal germ cell tumors (GCTs). Materials and Methods: A retrospective study of children diagnosed with GCTs treated at our hospital from 1998 to 2014 was performed. They were evaluated for their tumor size, malignancy, treatment, complications, and outcome. Results: Twelve giant mediastinal GCT patients were included in the study. Age ranged from 7 to 144 months (median 12 months) and all except one were males. The average tumor size was 10.4 cm (range 6 cm × 5 cm–16 cm × 13 cm) and in four patients, they were large enough to occupy nearly the entire hemithorax. Nine children had benign tumors, and these were resected upfront. The remaining three cases with malignant disease received neoadjuvant chemotherapy. No significant reduction in size was noticed in these patients, but alpha-fetoprotein levels decreased in all the three, and they were later resected. Eight (67%) were resected through posterolateral thoracotomy and 4 (33%) through median sternotomy approach. One patient had a dumbbell-shaped thoracoabdominal tumor extending through a Bochdalek hernia. He required additional laparotomy as well as diaphragmatic repair. There were no postoperative complications. The malignant GCTs received total four courses of PEB. All patients were alive and asymptomatic at a mean follow-up of 55.4 months (range 10–146 months). Conclusions: Mediastinal GCTs have bimodal age distribution and show male preponderance. Malignant mediastinal GCTs responded well to neoadjuvant chemotherapy through a reduction in size was not noticed. Complete excision often in coordination with cardiothoracic-vascular surgeons can lead to long-term symptom-free survival even in giant tumors.

Purpose To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation. Material and methods Children with Type 2 Abernethy who had had been operated between 2013 and 2017 were included in the study. The diagnosis had been confirmed on ultrasonography, CECT or angiography. All patients underwent laparotomy. The shunt was identified, clamped and the bowel congestion was noted. The shunt was ligated if the bowel congestion was not significant or had improved. Relevant follow-up investigations were done to document the resolution or amelioration of symptoms and the patency of the shunt. Results Five patients were included in the study with a median age of 6 years. Hepatopulmonary syndrome was the presentation in 4 patients while one patient presented with liver tumor. Ultrasonography and CECT were able to diagnose Type 2 malformation in 4 patients whereas in 1 patient the distal portal vein was not seen. The postoperative period was complicated in 3 patients. At the median follow up at 14 months, good intrahepatic portal flow in all patients. All patients demonstrated improvement/ resolution of symptoms. Conclusion Abernethy is rare malformation which can have a varied presentation. Additional investigations may be needed to confirm the diagnosis of Type 2 variety. Most patients have gradual improvement of symptoms. Level of evidence Level IV/ Treatment study.

Background Teratoma of the hepatoduodenal ligament is a rare entity. Only 17 cases of hepatoduodenal ligament teratoma exist in the literature. Case presentation A girl with antenatally detected intraabdominal lesion was brought to us at the age of 3 years for the prime concern of progressive abdominal distension. Evaluation showed that the mass was a teratoma arising likely from the hepatoduodenal ligament. During the excision, there was an inadvertent pin-point perforation of the common bile duct. Primary repair of the common bile duct was performed. The child had an uneventful recovery. Conclusions The significance of anticipating complications and taking adequate measures while dissecting the porta has been emphasized in this kind of rare location of a teratoma.

Children with neuroblastoma (NB) and opsoclonus-myoclonus-ataxia syndrome (OMAS) have a favorable oncologic outcome and overall survival. In contrast, despite intensive multidrug immunomodulation, the neurologic outcome is complicated by the relapsing nature of the neurologic symptoms and long-term neurobehavioral sequelae. Being associated with low-risk NB, there exists an ambiguity in the current literature regarding the administration of chemotherapy in these children. We reviewed our archives for children with NB-OMAS over a 22-year (January 1996 to January 2018) period. Eighteen children (10 female) with a median age at diagnosis of 23 months had NB-OMAS and were included. They had stage 1 (9/18; 50%), 2 (1/18; 5.5%), 3 (7/18; 39%), and 4 (1/18; 5.5%) disease according to the International Neuroblastoma Staging System. Multimodality therapy included surgery (16/18; 89%), chemotherapy (11/18; 61%), and immunomodulatory therapy (10/18; 55%). Complete oncologic remission was achieved in all children. Relapse of OMAS and presence of neurologic sequelae were observed in 1 (5.5%) and 5 (28%) cases, respectively. Presence of neurologic sequelae was significantly associated with low-tumor stage (P=0.036) and treatment without chemotherapy (P=0.003). Chemotherapy administration was the only variable significantly predicting a favorable neurologic outcome (95% confidence interval: 0.26-1.40, P=0.01). To conclude, our study including a limited cohort of patients highlights a favorable neurologic outcome associated with chemotherapy administration in children with NB-OMAS. However, further studies with larger sample size need to be conducted before drawing any definite conclusions.

Background: Recent SIOPEL studies have shown cisplatin monotherapy to be equally effective in management of Standard risk Hepatoblastoma (SRHB) as compared to PLADO. Aim: To study the chemotherapy, response and outcomes in children with SRHB in a Resource Challenged Nation (RCN). Material and Methods: A retrospective study was conducted and all children with SRHB who were treated by us from June 2007 to December 2017 were included. All patients with standard risk hepatoblastoma who had received at least 2 courses of chemotherapy were included. Data regarding the demographics, PRETEXT stage, chemotherapy, response to chemotherapy and outcomes were recorded. Kaplan Meier survival analysis was performed to calculate 5-year overall survival (OS) and event free survival (EFS). Results: Thirty-two children were included in the study. Nineteen children (59.4%) received Cisplatin monotherapy and of these 6 patients (all PRETEXT III) had poor response and were upgraded to PLADO. The remaining 13 (40.6%) received upfront PLADO. The 5-year OS and EFS was 100% in the monotherapy group (n=13), 92% and 69% in the upfront PLADO group (n=13), and 62% and 22% in the upgraded to PLADO group (n=6). Patients in upgraded to PLADO group had significantly lower 5-year EFS (70% vs 22%; p= 0.036) compared to upfront PLADO group. Conclusion: Two thirds of SRHB patients with PRETEXT stage III who received cisplatin monotherapy showed poor response and were upgraded to PLADO chemotherapy. These patients had a significantly poorer outcome compared to the rest of the cohort. PRETEXT stage III standard-risk hepatoblastoma may benefit from PLADO chemotherapy instead of cisplatin monotherapy.

Background: A number of techniques are described for correction of pectus excavatum (PE). This article describes the experience with an innovative procedure which combines features from the Ravitch and Nuss procedures without using prosthetic material. Methods: This cross-sectional study included 12 cases of PE from January 2000 to March 2017 managed by excision of deformed costal cartilages and support to the thoracic cage using an autologous free rib graft as a strut. Indication for surgery was Haller's Index above 3.2 with or without respiratory distress. Noncontrast computed tomography scans were done at 6 months after surgery to document the position of the strut and to see the final correction and new Haller's Index, respectively. Results: The male-to-female ratio was 2:1. Preoperative Haller's Index in all cases was >3.2 (range 3.25–14). The average age at surgery was 5 years and 8 months (range: 7 months–15 years). Mean duration of hospital stay was 11 days (range 5–16 days).The 11th rib was used commonly although in two cases, the 10th rib was used as the 11th rib was considered relatively short. Pericardial effusion requiring strut removal was seen in one case; in another case, removal of the rib was needed because of nonhealing of a delayed dehisced surgical wound. Others had an uneventful postoperative period. The mean postoperative Haller's Index was 2.75 (range 2.0–7). Conclusion: This modified procedure using an autologous rib strut is technically feasible and reproducible even with limited facilities and gives excellent results.

Peutz–Jeghers syndrome (PJS) is inherited as an autosomal dominant disorder presenting as hamartomatous polyps in the small bowel, mucocutaneous pigmentation and with a predisposition to develop cancer. We report a case of PJS, with an adenomatous giant gastric polyp. The purpose is to highlight that adenomatous giant gastric polyp may be an extremely rare presentation of PJS. Awareness of this possibility will help us in not missing out these atypical cases of PJS.

Introduction Esophageal atresia with or without tracheoesophageal fistula (EA with or without TEF) is one of the neonatal surgical emergencies requiring surgical intervention in the early neonatal period, influencing the developmental outcome in the operated children. This study was aimed to assess the developmental status of children operated for EA with or without TEF along with maternal stress, their quality of life (QOL), and coping abilities. Materials and Methods A descriptive cross-sectional survey was conducted on 51 children aged up to 5 years after EA with or without TEF repair and their mothers' in a tertiary care facility. The tools used were, namely, demographic datasheet of child and mother, anthropometry assessment, Developmental Assessment Scale for Indian Infants, and modified Vineland Social Maturity Scale for the developmental evaluation, Child Behavior Checklist (1.5–5 years) caregiver report form, Parental Stress Scale, WHOQOL BREF, and Coping Strategies Checklist. Results The majority of children had low weight (47.1%) and height (31.4%), for reference age. The overall and social developmental delay was observed in 40.7 and 37.5% of children, respectively, and few children (7.4%) had behavioral problems in the borderline range. Among the mothers, 47% had moderate stress and relatively poor QOL in environmental (60.1 ± 18.9) and psychological (60.8 ± 18.8) domains. The most commonly used coping strategy by the mothers was an emotional outlet (29.4%). Conclusion Developmental delay was present in children operated for EA with or without TEF caused significant stress among mothers, affecting their QOL for which the emotional outlet was the most commonly used coping strategy.

Introduction: A crossing lower polar vessel (CLPV) is found in 11%–20% of children of pelviureteric junction obstruction (PUJO). Preoperative imaging (Doppler or magnetic resonance angiography (MRA)) may help but does not form a part of routine diagnostic workup. An attempt has been made here to evaluate clinical and imaging features (ultrasound and renal dynamic scan [RDS]) in children of PUJO with CLPV and define variables that could provide a diagnostic clue to its presence. Materials and Methods: Records of children having PUJO with CLPV over 10 years (2006–2015) were reviewed retrospectively. Their demographic profile, clinical presentation, imaging features, management, and outcomes were evaluated. Results: Of 372 children with PUJO, 21 (5.6%) had a CLPV. Median age at presentation was 7 years (range 4 months–11 years). Presenting features included pain (66.6%), urinary tract infection (14.3%), antenatally diagnosed fetal hydronephrosis (14.3%) and lump (4.8%). All had an obstructive drainage on RDS and mean split renal function (SRF) of the affected kidney was 32.5% ± 15.65%. The majority had mild-moderate hydronephrosis with intrarenal pelvis. None of the ultrasound images suggested the presence of a CLPV. Diagnosis of a CLPV was made intraoperatively in all. Dismembered pyeloplasty anterior to the vessel was done in the majority (80.9%). Mean SRF on postoperative RDS improved to 36.6% ±17.76% with nonobstructive drainage. All were asymptomatic at a mean follow-up of 34.5 ± 17.5 months (range 2–56 months). Conclusions: Pain is the predominant presenting feature in these patients who present at an older age. Despite older age at presentation, these patients have well-preserved renal function and mild hydronephrosis. No specific RDS or ultrasound findings can predict the presence of a CLPV. As the incidence of CLPV is

Lingual leiomyomatous hamartoma (LLH) is a rare cause of midline tongue swelling. LLH with bifid tongue and ankyloglossia is still rarer, and so far only one case report in an adult exists in the literature. Here, we report a 5 months old male infant with a 1.5 cm × 0.5 cm LLH with bifid tongue and ankyloglossia.

Anomalies affecting the capillary and venous channels form the bulk of the spectrum of vascular anomalies. As per International Society for the Study of Vascular Anomalies (ISSVA) classification, these are referred to as hemangiomas and venous malformations respectively. The present article is a descriptive note of their management and outcomes. Retrospective records of patients over 17 y (January 2000 through December 2016) were reviewed for presentation, management and outcomes. Outcomes were graded into 3 subgroups based on subjective assessment of clinical images: Group A = near-total response (>90%); Group B = 50–90% and Group C =

Aims: The aim of this study is to evaluate hepatic artery resistive index (HARI) as a noninvasive prognostic predictor by correlating it with peripheral blood nitric oxide (NO) levels, portal pressure (PP) and histopathological changes in the liver in patients of biliary atresia (BA). Materials and Methods: Twenty-five patients were included in the study prospectively from November 2012 to June 2014. All patients underwent Doppler sonography to calculate the HARI preoperatively. Peripheral blood NO was also measured preoperatively. Biochemical liver function tests (LFTs) were measured preoperatively and at 1, 3, and 6 months postoperatively. The PP was measured intraoperatively, and a liver biopsy was taken in all patients. Disappearance of jaundice defined successful surgical treatment. Postoperatively, a hepatobiliary IminoDiacetic Acid scan (HIDA) was done to demonstrate a patent bilio-enteric pathway. Results: The mean preoperative HARI was 0.78 ± 0.105, and the median was 0.80 (range 0.60–1.0). The median HARI was used to correlate the other parameters; 13 (52%) patients had HARI ≥0.8. The mean PP was 24.96 ± 6.54 mmHg. The HARI had a strong correlation with PP (P = 0.0001) and (NO) (P = 0.0001); with every 0.1 increase in HARI, there was 5.2 mmHg increase in PP and 3.8 μmol/L increase in NO. The histological parameters which reached significance in relation to HARI were hepatocellular damage, bile duct inflammation, portal inflammation, and portal fibrosis. The postoperative improvement in LFT was significantly better in patients with HARI 0.8, elevated PP, and NO levels. Conclusions: Preoperative HARI was found to have a direct correlation with PP and peripheral blood NO as a measure of portal hypertension. A preoperative HARI ≥0.8 should be considered as a risk factor for poor outcomes in BA.

Introduction: Females with Krickenbeck low-type anorectal malformations - vestibular fistula (VF) and perineal fistula (PF) - are managed either by a primary definitive or conventional three-staged approach. Ultimate outcome in these children may be affected by wound dehiscence leading to healing by fibrosis. Most of the literature favors one approach over other based on retrospective analysis of their outcomes. Whether a statistically significant difference in wound dehiscence rates between these approaches exists needed to be seen. Materials and Methods: A randomized controlled trial for girls

Summary Background Glucose requirement in neonates during surgery and the impact of glucose supplementation on neonatal metabolism remain unclear. Aim This study was designed to identify an appropriate perioperative fluid regimen in neonates which maintains carbohydrate and lipid homeostasis. Methods Forty-five neonates undergoing primary repair of a trachea-esophageal fistula were randomly allocated into three groups. During surgery, the neonates received either 1% dextrose in Ringer lactate (RL) (group D1) at 10 ml·kg−1·h−1, or 2% dextrose in RL (group D2) at 10 ml·kg−1·h−1, or 10% dextrose in N/5 saline at 4 ml·kg−1·h−1 and replacement fluid with 6 ml·kg−1·h−1 of RL (group D4). Glucose homeostasis, electrolyte balance, acid–base status, and endocrine and metabolic parameters were compared among the groups during the perioperative period. Results Blood glucose increased in all the three groups at the end of surgery, with no significant difference in blood glucose and incidence of hyperglycemia (BG > 150 mg·dl−1) among them. At 24 h after surgery, blood glucose and incidence of hyperglycemia was significantly higher in Group D1 compared to Group D4. Base excess, bicarbonate, lactate, and pH showed a significant fall in Group D1. There was no significant difference in serum-free fatty acids, serum beta-hydroxy butyrate, and serum cortisol in three groups. At the end of surgery, serum insulin was significantly lower and glucagon : insulin (G : I) ratio was higher in Group D1 compared to Group D4. Conclusions All three solutions, when infused at 10 ml·kg−1·h−1, are equally effective in maintaining glucose homeostasis, but 1% dextrose-containing fluid promotes catabolism, insulin resistance, rebound hyperglycemia, and acidosis. Therefore, 2–4% dextrose-containing fluids is more suitable compared to 1% dextrose-containing fluids for use during major neonatal surgeries requiring average fluid infusion rate of 10 ml·kg−1·h−1.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.

In today's era of improved antenatal care and screening modalities (3D high-resolution fetal ultrasound, fetal magnetic resonance imaging, fetal echocardiography, maternal serum markers and fetal blood sampling), an early diagnosis of surgical fetal abnormalities is routinely possible. A thorough knowledge about the incidence of such defects, the means of detection and evaluation, the scope of fetal intervention, postnatal management and long-term outcomes of common surgical conditions diagnosed antenatally is essential. This knowledge would not only help in proper patient care and management but also to ensure appropriate counselling of the expectant parents. This article highlights the fetal anomalies which are amenable to some form of fetal intervention from a pediatric surgical perspective as also those which can be treated after birth.

The aim of the study was to evaluate the diagnostic value of fluorine-18 fluorodeoxyglucose (18F-FDG) PET/computed tomography (CT) in paediatric patients with neuroblastoma (NB) and compare the results with iodine-131 metaiodobenzylguanidine (131I-MIBG) scintigraphy.Data on 40 paediatric patients (age, 5.5 ± 5.6 years; male, 32; female, eight) with histopathologically proven NB who underwent 18F-FDG PET/CT (staging, 21 patients; restaging/response monitoring, 19 patients) were retrospectively evaluated. I-MIBG scintigraphy data were available for 28/40 patients (median interval, 15 days; staging, 20 patients; restaging/response monitoring, eight patients). 131I-MIBG scintigraphy and 18F-FDG PET/CT images were evaluated by two nuclear medicine physicians in consensus and in separate sessions. Histopathology (n = 50 lesions) and/or clinical/imaging follow-up (n = 90 lesions) data were taken as the reference standard.Patient-wise sensitivity, specificity, positive-predictive value, negative-predictive value and accuracy of 18F-FDG PET/CT were 100, 50, 91.89, 100 and 92.50%, respectively. A total of 140 lesions (primary, 37; lymph node, 31; bone, 50; bone marrow, 15; and others, seven) were detected on PET/CT. In 28 patients undergoing both imaging studies, the sensitivity, specificity, positive-predictive value, negative-predictive value and accuracy of 18F-FDG PET/CT were 100, 60, 92, 100 and 92.80%, respectively, and those of 131I-MIBG were 95.65, 60, 91.67, 75 and 89.20%, respectively. In these 28 patients, PET/CT detected 107 lesions (primary, 25; lymph node, 22; bone/bone marrow, 56; and others, four) and 131I-MIBG scintigraphy detected 74 lesions (primary, 24; lymph node, five; and bone/bone marrow, 45). On a patient-based comparison there was no significant difference between 18F-FDG PET/CT and 131I-MIBG (P = 1.000), but 18F-FDG PET/CT was superior to 131I-MIBG on a lesion-based comparison (P0.0001). Although no difference was noted for primary lesions (P = 1.000), PET/CT was superior to 131I-MIBG scintigraphy for the detection of lymph nodal (P = 0.001) and bone/bone marrow lesions (P = 0.007).18F-FDG PET/CT shows high accuracy in paediatric patients with NB and demonstrates more lesions as compared with 131I-MIBG scintigraphy.

Background: Though the outcomes in operated children with anorectal malformation (ARM) have greatly improved, postoperative soiling and constipation remain major issues. Among the various factors described for poor outcomes; misplaced bowel, hypoplastic sphincters and obtuse anorectal angle bear special mention. The aim of this study was to compare the stooling outcomes, type of anomalies and surgical procedure with postoperative pelvic magnetic resonance imaging (MRI). Materials and Methods: This was a cross-sectional study involving operated children of ARM who had at least 2 years of follow-up, and who were at least 3 years of age. The subtypes of ARM, surgical procedures, and functional outcomes were documented using the Krickenbeck classification. All children were subjected to a pelvic MRI. Results: Thirty-three eligible children were part of this study. Twenty-two patients underwent posterior sagittal anorectoplasty, seven patients underwent abdominoperineal pull-through (APPT) and four patients underwent perineal operations. Local abnormalities were present in 66% patients, and 34% had abnormalities of the spine detected on MRI. Poorer stooling outcomes were twice as common in children with local pelvic MRI abnormalities as compared to asymptomatic children. The highest incidence of local abnormalities were seen in patients treated with APPT ( P = 0.0001). No significant difference in the pelvic MRI was seen among children who were constipated and those who had soiling. Conclusion: MRI is a useful imaging modality in operated children of ARM with poor stooling outcomes. Local abnormalities were the most common in children undergoing abdominoperineal pull-through procedure.

Context: Stage IV Wilms tumor is associated with poor prognosis, and recent changes in management have been suggested based on genetic markers and response to chemotherapy in this subgroup of patients. Objective: The objective was to evaluate the outcomes of children with Stage IV Wilms tumor who were managed with the AIIMS-WT-99 protocol. Materials and Methods: All the children with Stage IV Wilms tumor who were managed by us from October 2000 to December 2012 were included in the study. All the patients who had received primary treatment elsewhere were excluded from the study. All patients were managed as per the AIIMS-WT-99 protocol. After appropriate investigations, tumors that were deemed resectable underwent an upfront surgery. Unresectable and inoperable tumors received chemotherapy after cytological confirmation of the diagnosis. Chemotherapy was administered as per the NWTS-5 study. Pulmonary and flank radiotherapy was advised to all patients. Patients with poor response to chemotherapy or with recurrence were managed with an alternative chemotherapy regimen. The outcomes that were assessed the 4-year overall survival (OS) and the 4-year event-free survival (EFS). Statistical Analysis Used: Kaplan–Meier survival estimates. Results: During the study period, 219 patients with Wilms tumor were treated. Of these, 36 (16.4%) had Stage IV disease, and they formed the study group. The 4-year OS was 48% with a mean survival time of 59 months limited to 115 months (95% confidence interval: 41.3–75.9 months). The 4-year EFS was 42.4%. Patients with liver metastases had a poor outcome, whereas patients with good response to chemotherapy had a good outcome. Conclusion: Stage IV Wilms had a poor prognosis, and the survival rates in the index study are lower than those quoted in the literature. Although the exact reason for this poor result eludes us, these patients may benefit from the intensification of chemotherapy.

Mediastinal pancreatic pseudocyst (MPP) is an infrequent complication of acute pancreatitis in children. A contrast-enhanced computed tomogram (CECT) of the chest and abdomen can aid in diagnosing pleural effusion and MPP. We describe a child with MPP in whom a transcutaneous computed tomogram-guided external drainage was curative. The case is being presented, and the relevant literature is highlighted in view of rarity of this entity.

Conservative management of giant omphalocele in the neonate period is a known strategy to allow tissue growth aiding in anatomical closure. However, rupture of the covering sac is considered an absolute contraindication for continuing conservative management. We report a case where a ruptured sac of giant omphalocele was ingeniously sutured to restore its integrity, and conservative management continued. The giant omphalocele later became a huge ventral hernia and was gradually reduced and primary closure was achieved with multiple surgeries over a period of 4 years.

Background: Nutritional depletion and growth stunting are present in patients with biliary atresia; “normal” nutrient and vitamin supplementation fail to correct these deficiencies. Children with this condition form the largest group for possible liver transplantation in the future; hence, stress should be laid on close attention to their nutrition. Methods: Twenty-five patients with biliary atresia as cases and 25 age-matched children as controls were enrolled in the study from November 2010 to June 2012. Preoperatively, patients underwent standard investigations and anthropometric measurement (weight, height, and head circumference) assessment. Nutritional status (assessed with standard growth chart) was compared with control population, and children were divided into poor nutritional status and good nutritional status. Kasai's portoenterostomy was performed in all patients, and comparison was done between preoperative nutritional status with postoperative status of children and also between hepatic iminodiacetic acid (HIDA) scan-positive (patent bilioenteric pathway) children with HIDA scan-negative children. Postoperatively, after 12 weeks, the same anthropometric measurements were taken again, growth velocity (GV) was assessed, and children were divided into poor, average, and good GV. Results: Nutritional status of children with biliary atresia was significantly poor than that of control group. Postoperatively, children had better nutritional status than preoperative nutritional status, especially in HIDA scan-positive children. GV was also significantly better in those children in whom postoperative HIDA scan was positive. Conclusion: Children with biliary atresia have poor nutritional status in comparison to normal population and require multifaceted approach to achieve adequate nutrition. Establishment of a patent bilioenteric pathway in these children improves their nutritional status and GV.

Background: Children with biliary atresia (BA) have impaired metabolism of trace elements (TEs) (i.e., zinc, copper, selenium, and manganese), leading to an alteration in the serum levels. However, this alteration in serum level has any correlation with liver histopathological changes is not yet clear. Materials and Methods: This prospective study attempts to evaluate the preoperative serum levels of TE in comparison to controls and its correlation with liver histology in children with BA. Kasai portoenterostomy (KPE) and liver biopsy were performed in all cases. On liver histology, various parameters assessed and were graded according to predefined criteria. Serum levels of TE were determined again 12 weeks post-KPE and compared with the preoperative levels. Results: Mean(±standard deviation [SD]) preoperative serum Zn, Cu, Se, and Mn levels (in μg/dl) in BA patients were 41.6 ± 12.8, 130.6 ± 12.8, 50.0 ± 10.0, and 32.0 ± 20.0, respectively; in controls, these levels were 77.9 ± 13.7, 133.7 ± 13.7, 87.0 ± 13.0, and 8.0 ± 5.5, respectively. Mean postoperative levels in all patients were 68.5 ± 19.0, 91.7 ± 19.0, 79.0 ± 19.0, and 28.0 ± 12.0, respectively. Mean(±SD) postoperative serum Zn, Cu, Se, and Mn levels in BA patients with bile excretion were 73.8 ± 14.9, 83.6 ± 13.8, 85.0 ± 15.0, and 26.0 ± 10.0, respectively, whereas in those with no bile excretion, they were 40.6 ± 12.8, 134.0 ± 23.0, 49.0 ± 11.0, and 44.0 ± 16.0, respectively. In liver histology, specific parameters showed correlation with high Mn and low Zn levels. Conclusion: Serum TE levels are altered in children with BA and the establishment of successful biliary drainage may change the subsequent postoperative serum concentration. Serum Zn and Mn levels can signify specific histopathological liver changes and the extent of liver damage.

Background Inducible nitric oxide synthase (iNOS) has a significant role in ischemia reperfusion (I-R) injury. I-R injury impairs the healing at the intestinal anastomotic site. This study was designed to assess the role of aminoguanidine (AG, a selective inhibitor of iNOS), in healing at the colonic anastomotic site after intestinal I-R injury in rats. Methods Female Wistar rats (n = 60) were divided into three groups. Group I (n = 15): sham operation, Group II (n = 15): I-R injury and anastomosis, and Group III (n = 30): I-R injury + anastomosis + AG-50 mg/kg. On the 7th postoperative day, relaparotomy was done and 4 cm of the colon with an intact area of the anastomosis was resected. Bursting pressure and histology at the anastomotic site were assessed. Results The bursting pressure was significantly higher in Group III. In addition, bridging parameters (i.e., mucosal continuity, muscular continuity, re-epithelization, and granulation tissue), collagen pattern, and collagen density were significantly better in Group III. While the polymorphonuclear density was higher in Group II, suggestive of delayed healing. Conclusion AG, by decreasing inflammation and increasing collagen content in an organized pattern, helped in preventing I-R injury at the site of colonic anastomosis in rats.

Transverse testicular ectopia (TTE) is an uncommon anomaly of testicular descent. Herein, we describe a case of TTE with blindly ending vas and persistent Mόllerian duct syndrome in a 2-year-old child. Orchidopexy could be done through the normal orthotopic route after separating it from the Mόllerian structure and dividing the peritoneal fold just distal to the blindly ending vas. The report highlights that laparoscopy is useful for identifying subtle anomalies in addition to its therapeutic role.

Congenital diaphragmatic hernia (CDH) is a known cause of secondary gastric volvulus. It is also known that bronchopulmonary sequestration (BPS) may be associated with CDH. An extremely rare case of BPS associated with gastric volvulus in a girl with left sided CDH is being reported.

Aims: This study was undertaken to establish correlation between intracholedochal cystic pressure (ICCP) with biochemical changes in bile, liver histology, cyst wall histology, length of common channel, and cyst volume. Materials and Methods: In this cross-sectional study, ICCP was measured after surgical access before any mobilization by direct cyst cannulation. Bile was then aspirated for biochemical estimation (bilirubin, amylase, lipase, pH, and electrolytes). Common channel length and cyst volume were determined by preoperative magnetic resonance cholangiopancreatography (MRCP). Liver histology was assessed under parenchymal, bile duct, and portal parameters. Cyst wall was examined for ulceration, inflammation, fibrosis, and metaplasia. Results: ICCP was recorded in 31 patients; mean and median ICCP were 15.64 and 14 mmHg, respectively (range = 6-30 mmHg). Cases with median ICCP < 14 had median cyst volume of 48 cc (range = 36-115) and amylase 2052 IU/L (range = 190-5052) whereas those with ICCP ≥ 15 had volume of 20 cc (range = 10-100) (P = 0.004) and amylase 36 IU/L (range = 0-2806) (P = 0.0004) suggesting inverse correlation. No significant correlation was found with bilirubin and electrolytes. ICCP directly correlated with parenchymal changes like hepatocellular damage (P = 0.002) and cholestasis (P = 0.001). It also correlated with bile duct changes. ICCP inversely correlated with cyst wall changes (P = 0.003, 0.0001, 0.023, 0.0013, respectively). High pressure cysts had normal pancreaticobiliary junction. Conclusion: High-pressure cysts tend to be smaller but have more severe backpressure changes in liver parenchyma. Low-pressure cysts have high volume and higher levels of amylase and lipase and therefore have more severe cyst wall changes.

Purpose: To evaluate the outcome of children with bilateral Wilms' tumor (BWT) treated on All India Institute of Medical Sciences-Wilms Tumor-99 (AIIMS-WT-99) protocol. Materials and Methods : All children with BWT, registered in our solid tumor clinic from August 1999 through December 2010 were included. Results: Of the 178 fresh cases of Wilms Tumor (WT) treated during this period, 11 (6.2%) had bilateral involvement. All patients except one (12 and 3 cm), had massive bilateral tumors of more than 10 cm on each side. There were eight boys and three girls in the age range 6-30 months. One patient had Denys-Drash syndrome. Twenty renal units were operated upon (12 tumorectomy, five partial nephrectomy, and three nephrectomies), while one patient with inferior vena cava (IVC) thrombus died of renal failure. Tumor spill occurred in three units, lymphnode was positive in two patients. Local recurrence occurred in four patients (six of 18 renal units (33%)-two bilateral and two unilateral). There was one recurrence in the liver that was treated with radio-frequency ablation. The 5-year overall survival (OS) was 90% (95% confidence interval (CI) = 50.8-98.6) and the relapse free survival (RFS) was 38% (95% CI = 6.1-71.6). Conclusion: Massive BWT respond poorly to preoperative chemotherapy, are often not amenable to partial nephrectomy/tumorectomy and have a higher local recurrence rate, giving a poor RFS.

To evaluate the management and outcome of children with pheochromocytoma and determine the role of cortex preservation in cases of bilateral disease.Retrospective review of children, below 12 y of age, with pheochromocytoma managed between November 2003 and December 2012 was done.Twelve patients, nine boys and three girls with median age 9 y were enrolled. Eleven (92 %) had adrenal tumors and in one it was extra-adrenal. Five (42 %) had bilateral disease. Ten presented with hypertension, one with headache and one with abdominal pain and fever. All were stabilized pre-operatively with alpha and beta blockers and volume expansion. Six children with unilateral disease underwent total adrenalectomy. Out of five with bilateral disease, one child underwent bilateral total adrenalectomy and was later started on hormone replacement. Remaining four underwent total adrenalectomy on one side and partial on the other side. Post-operatively all became symptom free and normotensive and were off medications within 1 mo. Two children developed recurrence 1 mo post-operatively, one with an initial unilateral pheochromocytoma and one with paraganglionoma. At the last follow up, 10 out of 12(83 %) were disease free while two with recurrence are still awaiting surgery.Surgical resection of pheochromocytoma is effective treatment to achieve cure and prolong survival. Cortex preservation should be done in bilateral disease as risk of recurrence in such cases seems to be of lesser significance as compared to the morbidity and mortality of adrenal insufficiency and consequent lifelong hormone replacement.

To assess the utility of fine needle aspiration cytology (FNAC) samples for molecular genetic analysis of neuroblastoma. The case files from the pediatric solid tumor clinic were reviewed to identify 20 neuroblastoma patients whose pre-treatment FNAC slides were preserved in the cytology laboratory. The FNAC slides were destained, air dried and hybridisation with fluorescence in situ hybridisation (FISH) probes was performed as per protocol. All slides were screened and analyzed carefully under the fluorescent microscope. Over four-fold increase of the N-myc signal numbers was defined as N-myc amplification. Focal occurrence of cells (at least 50) showing N-myc amplification surrounded by non-amplified tumor cells was defined as focal N-myc amplification. Presence of three or more signals for the long arm of chromosome 17 was defined as 17q gain. FISH analysis gave informative results for all the FNAC smears in our study. FISH analysis of FNAC smears showed N-myc amplification in 5 (25 %) out of 20 patients and 15 (75 %) showed normal N-myc copy number. Three out of these five patients had homogenous amplification and two patients had focal N-myc amplification, indicating tumor heterogeneity. On investigation of chromosome 17q status, 5 (25 %) out of 20 patients demonstrated gain of 17q and 15 (75 %) patients showed normal 17q status. Four out of the five patients with 17q gain also showed N-myc amplification. The current study indicates that FNAC is a rapid and atraumatic diagnostic method for neuroblastoma which provides sufficient material for molecular genetic analyses by means of FISH.

Objective: To study the incidence and type of musculoskeletal and aesthetic abnormalities after thoracotomy in children. Materials and Methods: Children below 12 years of age who had undergone thoracotomy for any condition and have at least 2 years follow up were included in the study. Detailed assessment of the patients included: history and general examination, clinical examination of chest and musculoskeletal system, X-ray chest including bilateral shoulders [antero-posterior (AP), lateral, oblique], X-ray whole spine (AP, lateral, right and left side bending AP view). Results: Fifty-two pateints were recruited.The incidences of various clinically and radiologically assessed musculoskeletal and aesthetic abnormalities observed were: winging of scapula (5.7%), ipsilateral elevation of shoulder (5.7%), fixation of skin cicatrix to bony thorax (7.7%), maldevelopment of pectoral muscles (11.5%), asymmetry of the level of nipples (1.9%), rib fusion (5.7%), crowding of ribs (9.6%), mediastinal shift (3.8%), decreased space available for lungs (3.8%), Scoliosis with Cobb′s angle >10 0 (1.9%). Conclusions: The incidences of musculoskeletal and aesthetic abnormalities after posterolateral thoracotomies in children were low. Longer follow up of patients after thoracotomy is needed to pick up these abnormalities as the children grow.

To correlate the functional outcomes in children operated for anorectal malformations (ARM) using the Krickenbeck consensus and their quality of life (QOL). Thirty-three children operated at a tertiary care Pediatric surgery centre were studied 2 y after completion of all the surgeries and if more than 3 y of age. The functional stooling outcomes, type of anomalies and surgical procedures were tabulated using the Krickenbeck classification. The QOL questionnaire consisted of five parameters (Social habit, school attendance, daily activity, relation to peers and feeling) and the scoring ranged between 0 and 12: Good (8–12), Fair (5–7) and Poor (0–4). Median age of the children at the time of study was 7 y (Range 4–14). Comparison of stooling outcomes and QOL showed no significant difference (p 0.48). QOL was not affected by the age at evaluation, type of anomaly and type of surgical procedure. Children with vertebral, anorectal, cardiac, tracheoesophageal, radial and limb abnormalities (VACTERL) association had poorer QOL as compared to children without VACTERL association (p 0.02). Poor stooling outcomes were seen in children who underwent abdominoperineal pull through procedure (p 0.02). QOL appears to be independent of the stooling outcomes in operated children of ARM with majority enjoying a good QOL (93.9%).

Aim: To evaluate the incidence of portal hypertension (PHT) in biliary atresia (BA) patients and to monitor its progress after Kasai portoenterostomy (KP) by measuring nitric oxide (NO) levels in peripheral blood. Materials and Methods: A prospective cross-sectional study conducted over a period of 2 years. Intraoperative portal pressure (PP) and blood NO levels at presentation, 1-month, 3-month, and 6-month follow-up, were correlated with clinical and biochemical parameters in BA patients. The mean NO level in age-matched control group was 4.64 ± 2.32 μmol/L. Results: Thirty-four BA patients underwent KP over a period of 2 years. The mean age of presentation was 2.7 months (range 1-4 months). The mean intraoperative PP was 21.3 ± 5.4 mmHg. The mean PP in patients aged 90 days was 18.53 ± 4.45 mmHg, 20.33 ± 3.07 mmHg, and 26.5 ± 5.01 mmHg, respectively. The mean PP in the patients who underwent successful KP was 16.75 ± 3.54 mmHg while for those who continued to have jaundice it was 23.94 ± 4.63 mmHg (P < 0.001). NO levels closely followed the PP as shown by the regression equation NO = 4.79 + 0.64 PP mmHg, R2 = 0.69. The mean NO level at presentation was 18.48 ± 4.17 μmol/L and at 1-month, 3-month, and 6-month follow-up was 11.94 ± 5.62 μmol/L, 10.79 ± 6.02 μmol/L, and 9.93 ± 6.53 μmol/L, respectively (P < 0.001). The difference in NO levels was also statistically significant between the patients who cleared jaundice and those with persisting jaundice. Conclusion: All BA patients had PHT at presentation. PHT worsens with age and has an adverse effect on outcome of KP. NO levels in blood closely follow PP and higher levels are associated with poor outcome.

Background/Purpose: This study aims to analyze the incidence and type of various associated anomalies among infants with extrahepatic biliary atresia (EHBA), compare their frequency with those quoted in the existing literature and assess their role in the overall management. Materials and Methods: A retrospective study was performed on 137 infants who underwent the Kasai procedure for EHBA during the past 12 years. The medical records were reviewed for the incidence and type of associated anomalies in addition to the details of the management of the EHBA. Results: Of the137 infants, 40 (29.2%) were diagnosed as having 58 anomalies. The majority of patients had presented in the 3 rd month of life; mean age was 81 ± 33 days (range = 20-150 days). There were 32 males and 8 females; boys with EHBA had a higher incidence of associated anomalies. Of these 40 patients, 22 (37.9%) had vascular anomalies, 13 patients (22.4%) had hernias (umbilical-10, inguinal-3), 7 patients (12.1%) had intestinal malrotation, 4 patients (6.8%) had choledochal cyst, 1 patient (1.7%) had Meckel′s diverticulum, 3 patients (5%) had undergone prior treatment for jejunoileal atresias (jejunal-2, ileal-1), 2 patients (3.4%) had undergone prior treatment for esophageal atresia and tracheoesophageal fistula, 2 patients (3.4%) had spleniculi, and 2 patients (3.4%) were diagnosed as having situs inversus. Conclusions: The most common associated anomalies in our study were related to the vascular variation at the porta hepatis and the digestive system. The existence of anomalies in distantly developing anatomic regions in patients with EHBA supports the possibility of a "generalized" insult during embryogenesis rather than a "localized" defect. In addition, male infants were observed to have significantly more associated anomalies as compared with the female infants in contrast to earlier reports.

A 6-year-old boy presented with repeated attacks of fever, abdominal pain, and obstructive jaundice. Clinical examination and preoperative imaging suggested the diagnosis of a type I choledochal cyst. During surgery, a hydatid cyst was found occupying the head of pancreas, causing obstruction of the common bile duct. The authors emphasize that in endemic areas, hydatid cyst should be included in the list of conditions in the differential diagnoses of obstructive jaundice and cystic lesions located around the bilio-pancreatic junction in children.

Aims: To correlate the age at surgery, liver function tests, and hepatic and portal tract histo-pathological changes with surgical outcome in the form of disappearance of jaundice in extrahepatic biliary atresia (EHBA). Materials and Methods : This is a retrospective study of 39 cases of EHBA. There were 19 males and 10 females. Kasai's portoenterostomy (KPE) along with liver biopsy was performed in these patients; for purpose of correlation this biopsy was considered to be the preoperative biopsy. These patients were divided into three groups based upon surgical outcome: (A) disappearance of jaundice; (B) initial disappearance of jaundice with recurrence after 3 months; and (C) persistence of jaundice. Postoperatively, liver function tests and liver biopsies were repeated at 3 months after the KPE. Results : There were 11 patients in group A (28%), 21 patients in group B (54%), and seven patients in group C (18%). The age at surgery was comparable in all the three groups. The postoperative levels of serum bilirubin, alkaline phosphatase (ALP), and gamma glutamyl transpeptidase (GGTP) showed statistically significant improvement as compared with the preoperative levels in group A and B patients. Patients belonging to group C showed no improvement in the liver functions following surgery. The preoperative hepatic histopathological changes (hepatocellular alteration, cholestasis, bile ductular proliferation, and bile duct inflammation) showed a significant difference among the three groups; patients with lesser degrees of pre-existing histopathological changes had better outcome following surgery. Fibrosis was seen in all the three groups preoperatively but the difference was not statistically significant. Group C had significant fibrosis in more than 50% patients. Additional findings, viz. ductal plate malformation (9 patients, 23%) and giant cell transformation (19 patients, 49%) did not show any correlation with surgical outcomes. Conclusions : The liver function tests and the histopathological features appeared to affect the final surgical outcome of these patients. Higher degree of cholestasis, hepatocellular alteration, bile ductule proliferation, bile duct inflammation showed definite correlation with poor surgical outcome. High grade hepatic fibrosis and portal edema showed a trend towards poor outcome but did not achieve statistical significance.