Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

Background and Objectives: More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. Materials and Methods: During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Results: Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Conclusions: Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Study of Intrahepatic Biliary Architecture in Patients Operated for Extrahepatic Biliary Atresia using Magnetic Resonance Cholangiopancreatography.

Aim: The aim is to study intrahepatic biliary architecture in patients following Kasai's portoenterostomy for extrahepatic biliary atresia using magnetic resonance cholangiopancreatography (MRCP). Materials and Methods: It is a prospective observational study in a cohort of patients who have survived with a complete jaundice-free period for at least 1 year. MRCP was done to look for various intrahepatic architectural changes during their last visit. Findings were correlated with liver functions and growth and development. Results: Twenty-one patients were included in the study. The male-to-female sex ratio was 1.6:1, the median age at surgery was 75 days (18-140 days), and the median age at magnetic resonance imaging (MRI) was 4 years (18 months-18 years). More than 2 years of follow-up was in seven patients. Left lobe hypertrophy was observed in six patients, right lobe hypertrophy was in three patients, intrahepatic biliary tract dilatation was in five patients, and altered signal intensity between intrahepatic ducts was seen in five patients. In addition, hypointense foci in the spleen were seen in one patient. Twelve children had normal weight for their age, ten children had the normal height for their age, and gamma-glutamyl transferase was elevated in all children. Only four children had a history of fever and jaundice. Conclusions: The patients who survive after successful surgical intervention have near-normal liver gross architecture as on MRI/MRCP. Occasional findings of dilatation and lobar atrophy/hypertrophy are possible, especially in those with a history of cholangitis. The survivors also have acceptable growth parameters and normal liver functions.

Preoperative Triple-Phase Three-Dimensional-Multi-Detector Computed Tomography Imaging of the Hepatic Vascular Tree: An Accurate Road Map Prior to Tumor Resection in Hepatoblastoma.

Objective: To evaluate the hepatic vasculature/tumor relations in hepatoblastoma patients with three-dimensional (3D) reformatted images after triple-phase multi-detector computed tomography (MDCT) and to compare these with the surgical findings to judge the accuracy of this investigation. Materials and Methods: The study was carried out in hepatoblastoma patients after appropriate neo-adjuvant chemotherapy, prior to resection. Images were postprocessed at a dedicated workstation for multi-planar reformations, maximum intensity projection, curved planar reformations, and volume-rendered technique reconstructions. The reporting was done as per a specific protocol by both the radiologist and surgeon (per-operative findings) and the accuracy of MDCT ascertained as per concordance between the surgical and imaging findings. Results: Fourteen children (13 boys, 1 girl) underwent surgery. Clinically, relevant information regarding vascular, tumor involvement, and interface with vessels was provided by the study in all cases. Although all tumors were deemed resectable on preoperative imaging, one procedure was abandoned due to an unanticipated portal cavernoma. While a few anatomical variations were unexpectedly encountered during surgery, there was good concordance overall between findings on imaging and surgical exploration. Conclusions: MDCT with 3D reformatting provides accurate virtual representations of the hepatic tumor. This allows simulation of surgical resection with decreased risk of vascular injury and postoperative liver failure.

Impact of Cytomegalovirus Infection on Short-Term Clinical Outcomes and Operative Histopathology in Infants with Biliary Atresia: A Single-Center Prospective Cohort Study.

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 of the Uncinate Process of the Pancreas in a Child Managed Sans Whipple's Procedure-a Rare Case Scenario.

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.

Comparison of Cisplatin Monotherapy and PLADO in the Management in Children with Standard-Risk Hepatoblastoma in a Resource-Challenged Nation.

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.

Role of Diffusion Weighted MRI (DW-MR) in Detection of Satellite Lesions Not Detected with Multiphase CT Scans in Hepatoblastoma and Its Implications for Management.

OBJECTIVE: To evaluate the role of diffusion-weighted magnetic resonance imaging (DW-MR) in hepatoblastoma as compared to multiphase contrast enhanced computed tomography (CECT) scans for detection of satellite lesions. METHODS: In this prospective study on new cases of hepatoblastoma, multiphase CECT scans and DW-MR were performed before initiation of chemotherapy. Results of interpretation were compared for detection of satellite lesions. PRETEXT grouping and risk categorization was done according to SIOPEL based on CECT scan. RESULTS: Nine boys and 1 girl with hepatoblastoma, with median age of 11.5 mo were included. All patients were stratified as high-risk group with 2 (20%) in PRETEXT II and 8 (80%) in PRETEXT III. In 2 of 10 (20%) patients, additional satellite lesions were detected on DW-MR, which upgraded their stage. One of the two patients had one satellite lesion identified on CECT, while additional seven satellite lesions were identified on DW-MR imaging. For the other patient, CECT showed no satellite lesion while DW-MR detected six satellite lesions. CONCLUSION: MRI has become the gold standard investigation for evaluation of hepatoblastoma. DW-MR, which is a contrast free technique, is a better tool for assessment of satellite lesions which are usually missed on CECT. This would help in proper staging, planning of management and prognostication.

An Audit of the Provisional Date of Elective Surgery Allotted to Pediatric Surgery Outpatients of a Tertiary care, Public-funded Teaching Hospital of North India.

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 (<1%). The difference in the proportion of defaulters from both the reconstructive groups was statistically significant (P = 0.0001). On a further system-wise subcategorization of children in the day-care group, the genitourinary subgroup constituted the majority (80%). Children who had to undergo staged procedures showed a significantly better compliance (P < 0.0001) to provisional DAES as compared to those belonging to the single-stage group. CONCLUSIONS: Compliance to provisional DAES is poor among the children scheduled for day-care surgeries and those undergoing simple reconstructive surgeries in a single stage.

Management and outcomes of ureteroceles in children: An experience of 25 years.

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.

Neuroblastoma With Opsoclonus-Myoclonus-Ataxia Syndrome: Role of Chemotherapy in the Management: Experience From a Tertiary Care Center in a Resource-limited Setting.

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.

First-Year follow-up of Newborns Operated for Esophageal Atresia in a Developing Country: Just Operating is not Enough!

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.

Giant Gastric Polyp in Peutz-Jeghers Syndrome: Report of a Case.

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.

Gastric Adenomyosis: A Rare Cause of Pyloric Mass in Children.

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.

Correlation of Hepatic Artery Resistive Index with Portal Pressure and Serum Nitric Oxide Levels in Patients with Extrahepatic Portal Vein 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.

Evaluation of Serum Levels of Trace Elements in Children with Biliary Atresia and their Correlation with Liver Histopathology.

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.

Outcome of Children with Stage IV Wilms Tumor - Our Experience of 15 Years.

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.

Surgical Correction of Pectus Excavatum Using a Rib Graft Strut Following Excision of Costal Cartilages.

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.

Developmental Status of Children Operated for Esophageal Atresia with or without Tracheoesophageal Fistula Along with Maternal Stress, Their Quality of life, and Coping Abilities at AIIMS, New Delhi.

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.

Need for urodynamic evaluation as a regular follow-up tool in assessment of long-term urological outcomes in patients with sacrococcygeal teratoma.

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).

Abernethy malformation type 2: varied presentation, management and outcome.

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.