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 <60 days, 61-90 days, and >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.

Context: Care of a child having colostomy has not been institutionalized for a long time for economic and administrative reasons. After stoma formation, stoma care has to be provided to the child by caregivers at home. Hence, caregivers need to be provided with ongoing education and support, commencing from preoperative teaching to discharge from the hospital and home care. Aims: The aims of this study were to develop video-based learning resource material and to evaluate its effectiveness in terms of knowledge and skill attainment by caregivers. Settings and Design: The study design was time series, one group pretest and post test. This was conducted among 30 caregivers attending pediatric surgery outpatients and indoor departments of a tertiary level care center. Materials and Methods: A video teaching program (VTP) related to pediatric colostomy was developed and used to teach the caregivers about colostomy care. Pretested and validated knowledge questionnaire, observational checklist, and stoma assessment scale (SAS) were used to assess the knowledge and skills of caregivers before and after the administration of VTP immediately (post test 1) after and 2 weeks (post test 2) after the intervention. Statistical Analysis Used: Repeated measures analysis of variance (ANOVA), Bonferroni correction, Mann-Whitney U test, and Kruskal-Wallis test were used. Results: There were significant increases in knowledge (from 10.9 ± 2.5 to 16.4 ± 1.67 and 15.9 ± 4.02, P = 0.001, maximum score 20) and skill scores as assessed by the observation checklist (from 5.6 ± 2.0 to 9.8 ± 1.6 and 8.6 ± 2.1, P = 0.001, maximum score 12) immediately after and 2 weeks after the VTP. However, a decline in skills was observed at 2 weeks when compared with immediate scores, as measured by the observation checklist. There was no significant increase in the skill scores of caregivers as measured by SAS at 2 weeks compared to the immediate scores. Conclusion: The VTP was effective in bringing about an increase in the knowledge and skill of caregivers of children having colostomy. Therefore, video can be utilized for the counselling of caregivers of children with colostomy.

Aims and Objectives: To study wheather the facial features return to normal after surgery for muscular torticollis done in early childhood. Materials and Methods: This is a long-term study of the fate of facial asymmetry in four children who have undergone operation for muscular torticollis in early childhood. All the patients presented late, i.e., after the age of 4 years with a scarred sternomastoid and plagiocephaly, so conservative management with physiotherapy was not considered. All the patients had an x-ray of cervical spine and eye and dental checkup before making a diagnosis of muscular torticollis. Preoperative photograph of the patient's face was taken to counsel the parents about the secondary effect of short sternomastoid on facial features and the need for surgery. After division of sternomastoid muscle and release of cervical fascia when indicated, the head was maintained in a hyperextended position supported by sand bags for three days. Gradual physiotherapy was then started followed by wearing of a Minerva collar that the child wore for a maximum period of time in 24 h. Physiotherapy was continued three times a day till the range of movements of the head returned to normal. During the follow-up, serial photographs were taken to note the changes in the facial features. Results: In all four patients, the asymmetry of the face got corrected and the facial features returned to normal. Conclusion: Most of the deformity of facial asymmetry gets corrected in the first two years after surgery. By adolescence, the face returns to normal.

Aims: Scrutiny over the clinical behaviors, management, and the final outcome of some rare renal neoplasm in order to find out some hidden facts about these tumors which are playing an important role in the disease course and its management. Materials and Methods: Retrospective evaluation of uncommon (non-Wilms') renal neoplasm in the pediatric population in a tertiary care center. Fifteen cases of uncommon renal tumors were treated in our institution over the last 5 years (January 2008 to December 2012). The cases were tabulated in the form of age, sex, mode of presentation, preoperative investigations, intraoperative grading, pathological type, postoperative management and the final outcome. The patients were followed up for 2 years (clinically every 3 months and ultrasonography abdomen in every 6 months for first 2 years) in order to see any evidence of recurrence and complications related to postoperative chemotherapy. Results: Out of 15 cases, four cases were clear cell sarcoma (CCS) (26.6%), three cases were rhabdoid tumor (20%), three cases were congenital mesoblastic nephroma (20%), two cases were multilocular cystic nephroma (13.3%), two cases were renal teratoma (13.3%), and one case of teratoid Wilms' tumor (6.6%). There were two deaths (one CCS and one rhabdoid tumor) due to chemotherapy-related toxicity but no recurrence. Three patients were lost during postoperative follow-up; ten patients are doing well and getting a regular visit in the follow-up clinic. Conclusion: The clinical presentations of these uncommon renal tumors are similar to that of Wilms' tumor. Thus, preoperative diagnosis is difficult even with modern imaging techniques. Some of these tumors (CCS, rhabdoid tumor) are rapidly progressing and have a poor outcome. Hence, early intervention in the form of complete surgical resection of the tumor (whenever possible) and postoperative chemo/radiotherapy are imperative for fruitful outcome.

Aims: In 1955, Professor Morio Kasai first performed a hepatic portoenterostomy. Since then, the procedure has changed the lives of children with biliary atresia (BA). We report our initial experience in performing "extended" Kasai portoenterostomy (KPE), a modification of the original procedure. Materials and Methods: Since 2013, we have used the technique of "extended KPE" and prospectively recorded data on all children undergoing this operation. Data on demographics, clinical features, liver function tests, and perioperative cholangiogram findings were collected. Outcome of KPE was measured by Jaundice Disappearance Rate (JDR) and Native Liver Survival Rate (NLSR). We present our preliminary results from a 30-month period (February 2013 to May 2015). Results: Thirty-one children underwent KPE during this period (19 males) and only 1 child had biliary atresia splenic malformation (BASM). The mean age at KPE was 73 ΁ 24 days. Five (16.1%) children were more than 90 days old at the time of KPE. Fourteen children cleared jaundice (JDR 45.2%). Eleven (35.5%) children developed episodes of cholangitis, of whom 8 had early cholangitis (within 3 months of the operation). The proportion of children who survived with their own liver 6 months after KPE (NLSR) was 84.2%. Of those children older than 90 days, 2 cleared jaundice and have survived with their native livers for more than 16 months. Conclusion: In our preliminary report of 31 children, we conclude that the extended KPE leads to increased jaundice clearance and improved NLSR in children with BA.

A 3-day-old male neonate delivered at 34 weeks of gestational age was brought with breathing difficulty since birth. The abdomen was massively distended. A soft cystic mass was occupying almost the entire abdomen and causing obvious respiratory distress. On exploration, a huge, solitary, unilocular cyst was found between the two lobes of the liver. Growing extrahepatically between the two lobes, it had displaced them laterally on either side. Enucleation of the cyst and marsupialization of its base was done. Histopathology showed evidence of congenital solitary nonparasitic cyst of the liver. Symptomatic presentation of CSNCL in children, especially in a neonate is extremely rare and not considered as a differential diagnosis of an abdominal mass. Hence, the case report.

There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms' tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms' tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction.

Complete midline sternal cleft is a rare congenital anomaly resulting from failed midline ventral fusion of the sternal bars. Very few cases of complete sternal cleft have been described in literature. We present a case of complete sternal cleft in a 3-month-old child. The patient underwent primary closure of the defect using stainless steel wires.

Rhabdomyosarcoma (RMS) is a common soft-tissue neoplasm in the pediatric age group. Common locations are head and neck, genitourinary areas, trunk, and extremities. Two pathologic variants of this malignancy are embryonal and alveolar. The involvement of breast is rare. Herein, we report two cases of alveolar RMS of the breast of which one is an isolated breast metastasis from an orbital primary, whereas the other is a primary RMS of the breast. Both the patients were treated with surgery followed by adjuvant chemotherapy and radiotherapy and are currently disease free at six and three year respectively, following completion of treatment.

Clinicoradiological and histopathological differentiation of pancreatoblastoma from hepatoblastoma can often be a challenge in clinical practice owing to their peculiar resemblance. We report a case of a 4-year-old boy with a right hypochondriac region mass, which was diagnosed as hepatoblastoma on the basis of imaging, raised tumor marker, and biopsy; however, pancreatic origin of the mass was ascertained on exploration and pancreatoblastoma was confirmed on histopathology.

A full-term male neonate was initially managed for respiratory distress which developed few hours after birth. His initial chest radiograph was normal, and blood culture revealed Group B streptococcal (GBS) sepsis. He subsequently developed progressive right chest opacification that did not improve with medical management. Imaging done few days later revealed right-sided diaphragmatic hernia. The 12-day-old neonate underwent primary repair of the diaphragmatic defect and had an uneventful recovery. This case report intends to highlight this unique association between early onset GBS sepsis and delayed onset of the right congenital diaphragmatic hernia.

A number of techniques have been described for intestinal anastomosis. We describe a different, simple, and safe technique that can be used in patients with intestinal diseases, such as jejunoileal atresia and perforation that has proximal dilated segments. In this technique, an atraumatic bowel clamp was applied on the proximal dilated bowel at a 90° angle. In the narrow distal segment, we resected the bowel at a 0° angle and continued at a 30° angle from the antimesenteric side. Finally, a two-layer interrupted anastomosis was performed. We applied this technique to a 31-day-old patient who had a divided jejunostomy due to malrotation and perforation with a proximal dilated bowel. Neither anastomotic complications nor feeding and passage problems were seen postoperatively.