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Background : Recent SIOPEL studies have shown cisplatin monotherapy to be equally effective in management of Standard risk Hepatoblastoma (SRHB)as compared to PLADO. Aims and Objectives: To study the chemotherapy, response and outcomes in children with SRHB. Material and Methods: A retrospective study was conducted and all children with SRHB who presented to us from June 2007 to December 2017 were included. All patients with standard risk hepatoblastoma who had received at least 2 cycles 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. The disease was PRETEXT I in 5 (15.6%), II in 9 (28.1%) and 18 (56.2%). Nineteen children (59.4%) received Cisplatin monotherapy and of these 6 patients (all PREXT III) had poor response and the chemotherapy was upgraded to PLADO. The remaining 13 (40.6%) received upfront PLADO chemotherapy. Only 31 patients could be operated. Tumor recurred in 5 patients, 2 who had upfront PLADO and 3 patients had been upgraded to 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 with PRETEXT III disease in whom chemotherapy was upgraded to PLADO had significantly lower survival (p=0.036) compared to those who received upfront PLADO chemotherapy. Conclusion: Two thirds of 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: There is limited information on the impact of cytomegalovirus (CMV) infection on clinical outcomes and operative histopathology in children with biliary atresia (BA). We hypothesized that CMV infection is associated with greater histopathological damage and unfavorable short-term clinical outcomes. Materials and Methods: A prospective single-center study was conducted with effect from January 2011–July 2012 including all infants with BA who underwent surgery. Diagnosis of CMV infection was confirmed by serum immunoglobulin M (IgM) positivity or the presence of CMV-deoxyribonucleic acid (DNA) in the liver tissue. Four short-term outcome variables were observed. The cohort was divided into subgroups on the basis of seropositivity (IgM + or IgM−); the presence of CMV-DNA in the liver (polymerase chain reaction [PCR]+ or PCR−); and composite CMV groups (Group 1 – IgM+, PCR+; Group 2 – IgM+, PCR−; Group 3 –- IgM−, PCR+; and Group 4 – IgM−, PCR−). Outcomes and histopathology were compared in these subgroups. Results: A total of 32 infants with BA were operated at a mean age of 3.5 (range: 1–6) months. Serum IgM+ and PCR+ were observed in 50% and 37.5% of the patients. Unfavorable outcomes showed a significant association with IgM+ and not PCR+. Similarly, outcomes were poor for CMV Groups 1 and 2 at 1-month follow-up. Infants with IgM+ and PCR+ showed a greater degree of histopathological damage in terms of bile duct proliferation and severe bile duct fibrosis, respectively. Conclusion: In the present study, there was a high incidence of serum IgM+ (50%) and PCR+ of biopsy specimens (37.5%) in infants with BA. This CMV-infected subgroup was associated with greater histopathological damage and unfavorable short-term outcomes after surgery.

Acinar cell carcinoma (ACC) is a rare malignant tumor of the pancreas. A 10-year-old girl presented with a large tumor arising from the pancreatic head. Excision sans Whipple's procedure was performed. Histopathology revealed it as ACC. In the context of this case, this rare tumor is being reported to highlight that such tumors arising from the head of the pancreas can be managed successfully without always resorting to a Whipple's procedure.

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 (

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.

Abstract 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.

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.

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.

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.

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.

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.

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.

Purpose: Whipple's pancreaticoduodenectomy (WPD) is rarely required in children. However, WPD is the only option with pathologies involving the head of the pancreas requiring surgical excision. The objective of our study was to review our experience with WPD performed on children. Materials and Methods: A retrospective analysis of case records was conducted on all patients

A teratoma is not an uncommon tumor in the pediatric age group. It has a predilection for specific sites, but a teratoma arising from the hepatoduodenal ligament (HDL) is very rare. Only 15 cases of HDL teratoma have been reported so far. Due to the proximity to important structures, it is imperative that the correct anatomical localization is done preoperatively to avoid intraoperative surprises. Herein, we report a case of HDL teratoma in a toddler who underwent a successful excision. The relevant literature is also discussed briefly from a pediatric surgeon's perspective.

Aim of the Study: The aim of this study is to establish ventral glans length (VGL), meatus (M) size, and their ratio VGL/M (R) in normal Indian boys and to compare these with the respective equivalent dimensions in boys with distal and mid-penile hypospadias using anatomic landmarks. Methods: Normal boys were designated as Group A (n = 108), and the boys with hypospadias were designated as Group B (n = 81). The anatomical landmarks marked on the glans were measured using a digital camera and appropriate software. Results: The mean age of the boys in Groups A and B was 4.26 ± 3.59 (range 0.5–12) and 3.82 ± 2.85 (0.7–11) years, respectively. The observed mean values in Group A for VGL-A, M-A, and R-A were 3.05 ± 1.27 (range 1.19–8.09), 4.3 ± 1.23 (range 1.61–7.04), and 0.8 ± 0.45 (range 0.27–2.1) mm, respectively. The observed mean values in Group B for VGL-B, M-B, and R-B were 3.77 ± 0.81 (range 12.41–5.2), 3.27 ± 0.71 (range 2.25–5.34), and 1.17 ± 0.26 (range 0.84–1.86) mm, respectively. Comparison of R-A and R-B showed a statistically significant difference (P < 0.0001). Conclusions: The dimensions and the studied ratio between a hypospadiac glans and a normal glans differed significantly. The ratio between VGL and M was 0.8 in normal boys. In comparison, the ratio between the equivalent VGL and M in the hypospadiac boys was 1.17. During glansplasty in surgery for hypospadias, this factor should be taken into consideration to avoid the creation of long and tight ventral glans closure.

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

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

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.

Desmoid tumors (fibromatosis) are intermediate grade, locally aggressive soft-tissue tumors. A rare case of chest wall fibromatosis in a girl with congenital muscular torticollis is being reported. This report also highlights the need for long-term surveillance in such cases, despite being a benign pathology.

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.

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.

Renal Angiomyolipoma (AML) is not commonly seen in the pediatric age group other than patients of tuberous sclerosis where in they can have renal AMLs within the first decade with bilateral in involvement. Diagnosis of renal AML can generally be made by the current radiological modalities in the appropriate clinical setting, but it can be mistaken for other tumors when it is large and has low-fat content. Herein we report a case of giant renal AML that was initially misdiagnosed as a Wilms tumor in a 12-year-old girl.

Background and Aims: Extrahepatic biliary atresia is one of the most challenging conditions in pediatric surgery. The definition of prognostic factors is controversial. Surgical outcomes after bilioenteric drainage procedures are variable. This study attempts to correlate the pre- and post-operative liver histology with clinical factors in order to define early predictors of success. Materials and Methods: Twenty consecutive patients, treated by Kasai′s portoenterostomy (KP) over a 3 years period were included in this study. Tissue obtained from the porta hepatis was analyzed for duct size using an optical micrometer and was categorized into three types: I-No demonstrable ducts; II - 50 μ. Pre- and post-operative liver biopsy was analyzed for architectural changes and fibrosis; hepatic fibrosis was quantified using existing criteria. Pre- and post-operative liver function tests (LFTs) were also done. Surgical outcomes were defined as: (A) Disappearance of jaundice within 3 months; (B) initial disappearance of jaundice with recurrence by 6 months and (C) persistence of jaundice. Duct diameters, fibrosis score, and LFT were correlated with age and clinical outcomes. Results: The surgical outcomes were: A-6 patients (30%), B-6 patients (30%), C-8 patients (40%). The duct size at the porta was I-3 patients, II-11 patients, and III-4 patients (tissue was not available in 2 cases). The change in total serum bilirubin (mg%) from pre- to post-operative period was 13.6 ± 3.9 (Group A), 4.6 ± 2.8 (Group B), and 3.4 ± 3.9 (group C) (P < 0.001) and direct and indirect fractions followed a similar trend; the changes in liver enzymes were not significant. The changes in hepatic histopathological changes (ballooning of hepatocytes, giant cells, cholestasis, portal tract infiltration, ductular proliferation, lobular necrosis, and fibrosis) were also not significant but there was a definite trend in the change in fibrosis -1.500 ± 1.643 (Group A), 0.667 ± 2.582 (Group B), and 1.500 ± 1.852 (Group C) - reduction of fibrosis with good results and progression of fibrosis with poor results. Conclusions: Following KP, jaundice persisted in 40% patients; it disappeared in 60% patients but reappeared in half of these patients 6 months postoperatively. The duct size at the porta hepatis did not correlate with age or surgical outcome. Serum bilirubin showed the best correlation with surgical outcome. Postoperative changes in hepatic fibrosis seem to have some bearing on surgical outcomes-progressive fibrosis is a poor prognostic factor.

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.

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.

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.

Aim: Our study aims to find various enzymatic and biochemical components of bile and their clinical or prognostic correlation with regard to progression and severity of hepatobiliary diseases. Materials and Methods: It was a cross-sectional study where all the patients suffering from choledochal cyst (CDC), extrahepatic portal venous obstruction (EHPVO), and infantile obstructive cholangiopathy undergoing diagnostic preoperative cholangiogram; and patients with history of total parenteral nutrition (TPN) undergoing surgery for some other condition were included in the study. Intraoperatively, bile was collected from the gallbladder and sent for estimation of amylase, lipase, sodium, potassium, calcium, chloride, bicarbonate, total bilirubin, pH, cholesterol, triglycerides, and total bile acid. Results: A total of 80 patients were included in the study (20 in each of the four disease-based groups). Amylase, lipase, and pH were significantly different among the patients of CDC when compared with the presence or absence of dilated intrahepatic biliary radicals. Similarly, amylase, lipase, and pH were also significantly different among the patients of EHPVO when compared with presence or absence of biliopathy. Levels of cholesterol and bile acid were significantly higher in patients who were evaluated after 1 year following TPN than those who were evaluated before 1 year. The patients of infantile cholangiopathy, who had history of fever, had significantly higher level of calcium. Conclusion: The components of bile show close correlation with various clinical and prognostic markers, there is a very close correlation between these parameters and the clinical severity, disease progression, and final outcome.

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.

Aim: Crossed fused renal ectopia is a rare congenital malformation, which is reported to be usually asymptomatic but may have varied presentations. This survey was conducted to study the clinical profile and the challenges posed in the management of this entity. Materials and Methods: Retrospective analysis of 6 patients diagnosed to have crossed fused renal ectopia during 1997-2010. The diagnosis was confirmed during surgical exploration in one patient. In one patient it was detected on antenatal ultrasonography and in the other 4 patients it was detected during investigations for abdominal pain, abdominal mass, anorectal malformation and urinary tract infection. Results: The left moiety was crossed and fused with the right moiety in 4 cases. Ultrasonography was found to be a good screening investigation with useful diagnostic contributions from CT scans, radionuclide scintigraphy and magnetic resonance urography. Micturating cystourethrography revealed presence of VUR in 4 cases, 3 of whom have undergone ureteric reimplantation. Two patients required pyeloplasty for pelviureteric junction obstruction; in one of these patients the upper ureter was entrapped in the isthmus. In one patient, a non-functioning moiety resulted in nephrectomy. All children were asymptomatic at last follow-up with stable renal functions. Conclusions: Crossed fused renal ectopia was detected in most patients during investigation for other problems. It was found more commonly in boys. The left moiety was crossed to the right in the majority of cases. Associated urological problems were found in most cases and required the appropriate surgical management.

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.

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.

Background: A majority of cystic lesions in the western world are detected antenatally, whereas, the diagnosis in our setup occurs once the child becomes symptomatic. Surgical management is primarily dictated by the presence of symptoms, recurrent infection, and rarely by the potential risk of malignant transformation. Materials and Methods: A retrospective analysis was carried out on all consecutive patients with cystic lung lesions managed at our center from January 2000 through June 2011 for antenatal diagnosis, presentation, diagnostic modalities, treatment, and complications. Results: Forty cystic lung lesions were identified. Only 8% were antenatally detected. Out of 40, the final diagnosis was congenital cystic adenomatoid malformation in 19, congenital lobar emphysema in 11, and bronchogenic cysts and pulmonary sequestration in five each. Of these, 20% had received a course of prior antitubercular therapy and 30% had an intercostal drain inserted prior to referral to our center. Postoperative morbidity in the form of bronchopleural fistula, pneumothorax, and non-expansion of the residual lung was noted in 10% of the patients. Conclusion: Antenatal diagnosis of these lesions is still uncommon in third world countries. Prior to referral to a pediatric surgical center a large number of patients received antitubercular drugs and an intercostal drain insertion, due to incorrect diagnosis.

Background: The surgical management of urinary bladder exstrophy is challenging. This paper describes the personal experience in a tertiary care hospital over a period exceeding a quarter of a century. Methods: During the period 1984-2010, 248 patients of the epispadias-exstrophy complex have been treated. The cases of classical bladder exstrophy (n = 210) form the basis of this paper. The stages/procedures used in the surgical reconstruction of bladder exstrophy included bladder closure with anterior abdominal wall reconstruction, bladder neck repair, ureteric reimplantation, epispadias repair and augmentation colocystoplasty in various combinations. Some of these patients had their initial operations done prior to 1984 or in other hospitals. Evaluation methods included, amongst others, clinical evaluation and urodynamic assessment. Eight patients opted out of treatment; 15 patients underwent permanent urinary diversion by either ureterosigmoidostomy or colon conduit. The remaining 187 patients were treated with bladder reconstruction, and of these, 132 patients have had at least one attempt at bladder neck reconstruction with 56 of these patients having undergone an augmentation colocystoplasty. Results: A total of 105 patients had socially acceptable continence: 57 from the bladder neck reconstruction group and 48 from the bladder augmentation group. Further attempts at continence surgery have been offered to the inadequately continent patients. Conclusions: Surgical management of bladder exstrophy demands patience and perseverance. It is possible to provide all patients with socially acceptable continence with bladder neck division and catheterizable continent stoma as the last resort. Urodynamic assessment has emerged as an essential tool in the follow-up evaluation of these patients. Anticholinergic medication with imipramine or oxybutinin is a useful adjunct in the overall management.

Malignant rhabdoid tumor (MRT) is a rare, but aggressive tumor commonly arising from the kidney in young children. Extrarenal MRT has been reported in the literature in various other sites including the liver, pelvis, CNS, abdomen, heart and other soft-tissues. Reported herein are the presentation, radiology, histopathology, immunohistochemistry, treatment and outcome of a 6 month infant with primary MRT of liver.

Two uncommon cases of locally invasive pulmonary inflammatory myofibroblastic tumor are reported. Diagnosis was established by a prior thoracotomy and incisional biopsy. Complete excision was curative and both children remain asymptomatic at last follow up.

Antireflux surgery (ARS) was performed on 45 renal units during 1991-96. Pre-operative renal length meas-urements were available in 20 renal units and 23 renal units were evaluated for renal length postoperatively. Pre-and post-ARS renal length measurements were available in 15 renal units. Renal length measurements were made by real time B mode ultrasonography. The post-ARS renal growth was accelerated in 69.5% units, normal in 13% units and impaired in 17.3% units. Accelerated renal growth occurred mainly in the first year after ARS but continued up to 2 years. Pre-existing renal scars, hyper-tension, poor renal function and post-operative urinary tract infection did not affect post-ARS renal growth. Fail-ure of ARS resulted in renal growth retardation.