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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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   Table of Contents - Current issue
Coverpage
January-March 2019
Volume 24 | Issue 1
Page Nos. 1-80

Online since Wednesday, December 19, 2018

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EDITORIAL  

Progress in fetal surgery: A buoyant start or watchful reluctance? Perspectives for India p. 1
Shilpa Sharma
DOI:10.4103/jiaps.JIAPS_84_18  
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REVIEW ARTICLES Top

Consensus on the management of posterior urethral valves from antenatal period to puberty Highly accessed article p. 4
Shilpa Sharma, Manoj Joshi, Devendra K Gupta, Mohan Abraham, Praveen Mathur, JK Mahajan, AN Gangopadhyay, Simmi K Rattan, Ravindra Vora, G Raghavendra Prasad, NC Bhattacharya, Ram Samuj, KL N. Rao, AK Basu
DOI:10.4103/jiaps.JIAPS_148_18  
The need for successful management of posterior urethral valves always captivates the minds of pediatric surgeons. Its success, however, depends on several factors ranging from prenatal preservation of upper tracts to postoperative pharmacological compliance. Regardless of measures available, some cases do not respond and progress to end stage. The management depends on several issues ranging from age and severity at presentation to long-term follow-up and prevention of secondary renal damage and managing valve bladder syndrome. This article is based on a consensus to the set of questionnaires, prepared by research section of Indian Association of Paediatric Surgeons and discussed by experienced pediatric surgeons based in different institutions in the country. Standard operating procedures for conducting a voiding cystourethrogram and cystoscopy were formulated. Age-wise contrast dosage was calculated for ready reference. Current evidence from literature was also reviewed and included to complete the topic.
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Formulation of research question – Stepwise approach p. 15
Simmi K Ratan, Tanu Anand, John Ratan
DOI:10.4103/jiaps.JIAPS_76_18  
Formulation of research question (RQ) is an essentiality before starting any research. It aims to explore an existing uncertainty in an area of concern and points to a need for deliberate investigation. It is, therefore, pertinent to formulate a good RQ. The present paper aims to discuss the process of formulation of RQ with stepwise approach. The characteristics of good RQ are expressed by acronym “FINERMAPS” expanded as feasible, interesting, novel, ethical, relevant, manageable, appropriate, potential value, publishability, and systematic. A RQ can address different formats depending on the aspect to be evaluated. Based on this, there can be different types of RQ such as based on the existence of the phenomenon, description and classification, composition, relationship, comparative, and causality. To develop a RQ, one needs to begin by identifying the subject of interest and then do preliminary research on that subject. The researcher then defines what still needs to be known in that particular subject and assesses the implied questions. After narrowing the focus and scope of the research subject, researcher frames a RQ and then evaluates it. Thus, conception to formulation of RQ is very systematic process and has to be performed meticulously as research guided by such question can have wider impact in the field of social and health research by leading to formulation of policies for the benefit of larger population.
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ORIGINAL ARTICLES Top

Encouraging results of bowel and bladder management in spina bifida aperta in South India with quality of life scores in a tertiary care institution in South India p. 21
Jujju Jacob Kurian, Tarun John K. Jacob, John Mathai
DOI:10.4103/jiaps.JIAPS_195_17  
Context: It is often a challenge to counsel parents with children operated for spina bifida aperta in developing countries. Data regarding the efficacy of simple measures and preventive are scarce. Aims: The aim of this study is to study such children for the incidence, prevalence of bowel bladder dysfunction, and the quality of life (QOL) in children who are involved with a multidisciplinary team in India. Materials and Methods: All children with spina bifida occulta were followed – QOL questionnaires (PIN Q, modified Barthels activities of daily living , and the visual analog score [VAS]) were used. Interventions, such as clean intermittent catheterization (CIC), bowel enemas, and surgical procedures, were studied. Results: A total of 68 children were assessed. Twenty-nine of these children over five were evaluated with QOL scores. The prevalence of incontinence of bowel and bladder was studied. The primary outcomes included the QOL scores, and the various surgical options help bowel and bladder management. Hydronephrosis in 17.95% of children <5 years and 65.5% of children over 5 years was noted. Nineteen children were socially independent for their bowel management. The Barthel index and PIN-Q showed a poor QOL in 27.6% and the VAS in 34.5% had the same. This translated to an acceptable QOL for over two-thirds of the children. Conclusions: Simple procedures and training for bowel management translate to a significant number of children being able to independently manage bowel care. About 30% of children develop hydronephrosis by 5 years; the decision to teach CIC must be made by then. We believe that positive counseling is given to the parents of children with spina bifida aperta as the children are capable of a reasonable QOL.
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Role of chemoports in children with hematological/solid tumor malignancies - Technical implications and complications: An institutional experience p. 27
Rajeev Redkar, Anant Bangar, Janani Krishnan, Vinod Raj, C Swathi, Shirin Joshi
DOI:10.4103/jiaps.JIAPS_212_17  
Aim: The aim of this retrospective analysis was to identify the variously related complications and to study preventive and therapeutic measures for these complications. Materials and Methods: A total of 72 catheters were inserted in 69 patients (mean follow-up of 1140 days) from December 2002 to May 2017. Sixty-four children were diagnosed to have hematological malignancies, and five children had solid tumors. The youngest child was 2 months of age, and the oldest was 15 years, 5 months. Records were analyzed retrospectively for the age, indication, route of insertion, and postoperative complications. A protocol-based insertion and postinsertion handling by trained nursing staff/doctors were instituted, including a periodic training program for those concerned. Results: Chemoport-related complications were infection in 3 (4.16%), necessitating port removal in one patient. The rest were managed by antibiotic-lock therapy. The other problems were catheter tip occlusion in 1 (1.38%) and extravasation in two patients (2.77%) leading to a sterile collection around the port chamber. An unsightly scar in 4 (5.55%) and granuloma formation at scar site in 1 (1.38%) patient were noted. Conclusion: Totally implantable chemoports are preferred in children with solid and hematological malignancies because of decreased pain, the rate of infection, and ability to maintain patency for the long term. Despite significant advantages over other types of central venous access, chemoports have their own complications. It was also noted that the rate of complications could be minimized by periodic training of all the personnel concerned and following protocol-based handling of ports.
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Urodynamic changes after valve fulguration alone and valve fulguration with bladder neck incision p. 31
Sanjeet Kumar Singh, Vijay Sharma, Anjana Singh
DOI:10.4103/jiaps.JIAPS_141_17  
Aims: The aim of this study is to compare urodynamic changes after valve fulguration alone and valve fulguration with bladder neck incision (BNI). Settings and Design: A total of 81 patients with posterior urethral valve were treated at our center from July 2010 to July 2016. Patients were randomized into two groups using simple randomization. Forty patients underwent BNI in addition to valve fulguration (Group I), and the remaining 41 patients underwent conventional transurethral valve fulguration (Group II). Subjects and Methods: The exclusion criteria for both the groups were the presence of simultaneous urogenital anomalies, any neurological condition, history of any urethral manipulation, and urinary diversion. Urodynamic changes were compared in both groups postoperatively. All patients were evaluated throughout their follow-up, according to the following protocol: (a) Voiding cystourethrography at 6 weeks after surgery; (b) Renal function test and urine culture at 6 weeks and then 3 monthly; (c) Ultrasound kidney, ureter, and bladder region and urodynamics at 3 and 6 months after surgery and then yearly. Median follow-up period for Group I was 27.5 months (13–72 months) and 14 months (14.5–72 months) for Group II. Statistical Analysis Used: Statistical analysis was done using the Student's t-test for parametric data and Chi-square test for categorical variable. P ≤ 0.05 was considered as statistically significant. Results: The mean age was 7.26 years in Group I and 7.66 years in Group II at the end of follow-up. There was no statistically significant difference found regarding detrusor overactivity (P = 0.68), compliance (P = 0.052), end-filling pressure (P = 0.08), and max Pdet at Qmax (P = 0.08) in the both groups. However, there was a statistically significant difference regarding improvement of peak flow (P = 0.038) and postvoid residue (PVR) (P = 0.045) in Group I in comparison to Group II. Conclusions: Valve ablation with BNI gives statistically significant better urodynamics in voiding phase regarding flow and lesser PVR in comparison to valve ablation.
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A pilot study on neonatal surgical mortality: A multivariable analysis of predictors of mortality in a resource-limited setting p. 36
Archana Puri, Brahmanand Lal, Sushma Nangia
DOI:10.4103/jiaps.JIAPS_30_18  
Purpose: The aim of this research is to study the predictors of neonatal surgical mortality (NSM)-defined as in-hospital death or death within 30 days of neonatal surgery. Materials and Methods: All neonates operated over the study period of 18 months were included to evaluate NSM. The evaluated preoperative and intraoperative variables were birth weight, gestation age, age at presentation, associated anomalies, site and duration of surgery, intraoperative blood loss, and temperature after surgery. Assessed postoperative variables included the need for vasopressors, postoperative ventilation, sepsis, reoperations, and time taken to achieve full enteral nutrition. Univariate and multivariate logistic regression was applied to find the predictors of mortality. Results: Based on patient's final outcome, patients were divided into two groups (Group 1-survival, n = 100 and Group 2-mortality, n = 50). Incidence of NSM in this series was 33.33%. Factors identified as predictors of NSM were duration of surgery >120 min (P = 0.007, odds ratio [OR]: 9.76), need for prolonged ventilation (P = 0.037, OR: 5.77), requirement of high dose of vasopressors (P = 0.003, OR: 25.65) and reoperations (P = 0.031, OR: 7.16 (1.20–42.81). Conclusion: NSM was largely dependent on intraoperative stress factors and postoperative care. Neonatal surgery has a negligible margin of error and warrants expertize to minimize the duration of surgery and complications requiring reoperations. Based on our observations, we suggest a risk stratification score for neonatal surgery.
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Experience with the redo pull-through for hirschsprung's disease p. 45
Devendra K Gupta, Kashish Khanna, Shilpa Sharma
DOI:10.4103/jiaps.JIAPS_52_18  
Aim: This study aims to evaluate the need of Redo pull-through (Re PT) procedures for Hirschsprung's disease (HD) and suggest preventive strategies. Materials and Methods: Patients who underwent redo procedures for HD from 1980 to 2016 by a single surgeon were retrospectively reviewed. Results: Of 167 patients operated for HD, 32 underwent Re PT; 7 were previously operated by the same surgeon, while 25 were referred from outside. Indication for Re PT included residual disease including the rectal pouch following-Duhamel (12), false-negative biopsy (3), retraction of bowel (5), anorectal stricture (2), bowel twist (1), cuff inversion (2), postmyectomy continued symptoms after primary PT (1), fecal fistula (1), Re PT after surgery for adhesive intestinal obstruction (3), bleeding (1), and combination of causes, including scarred perineum (1). Age at follow-up ranged from 2.5 to 26 years. Proximal diversion was performed in 19 and 14 underwent open Scott Boley's/Soave PT and 5 ileoanal anastomosis. Of the remaining, nondiverted 13 patients, 5 underwent transanal endorectal PT and 8 underwent PT of colostomy. Conclusions: Most patients of Re PT came after an initial Duhamel's procedure. Retraction of bowel, inversion of cuff, twist, distal bowel stricture, and perianal fibrosis were found after Scott Boley procedure. Proper planning with an initial diversion, nutritional buildup, barium study evaluation, frozen section facility, experienced pathologist, and an expert surgeon are prerequisites for a successful outcome after an initial as well as Re PT.
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Synchronous bilateral wilms tumor: Five-year single-center experience with assessment of quality of life p. 52
Hinglaj Saha, Dipak Ghosh, Somak Kumar Biswas, Prafulla Kumar Mishra, Kaushik Saha, Uttara Chatterjee
DOI:10.4103/jiaps.JIAPS_42_18  
Context: Synchronous Bilateral Wilms tumor (sBWT). Aims: This study aimed to assess the outcome of patients with sBWT treated on SIOP protocol. Settings and Design: Retrospective and prospective randomized study. Subjects and Methods: SIOP 93-01 protocol was used to study nine patients of sBWT in a single center and followed up over a period from 2 to 5 years. Statistical Analysis Used: Unpaired t-test and Mann–Whitney U-test were used for analysis. Results: Of nine patients, six were included in the study as three patients lost to follow-up. Among the six patients, there were four girls and two boys with a median age of 2 years. Mean regression in the size of tumor was 87% in four out of six patients. Tumor with unfavorable histology showed 32% response (ratio of favorable: unfavorable histology 2:1). Event-free survival rate was 81.3% and overall survival was 90% over 2–5 years. Recurrence was seen in two patients of whom one had Denys–Drash syndrome. Mean DTPA glomerular filtration rate was 91.4/ml/min/1.73 m2 preoperatively and that of 3 months after completion of treatment was 84/ml/min/1.73 m2. Health-related quality of life (HRQOL) using Pediatric Quality of Life Inventory and Lansky Play Performance Scale revealed significant improvement results of all functioning domains such as physical, social, emotional, and school subscales with P < 0.05 and performance scale (P < 0.04). Conclusions: We suggest SIOP protocol for sBWT and bilateral nephron-sparing surgery in two stages. However, long-term follow-up is required to assess the ultimate renal function outcome. HRQOL is an essential guide in improving the conditions of pediatric cancer survivors.
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CASE REPORTS Top

Traumatic rupture of jejunal burkitt's lymphoma with intestinal transection p. 61
Anoop Singh, Girish Prabhakar
DOI:10.4103/jiaps.JIAPS_67_18  
Burkitt's lymphoma (BL) is an aggressive non-Hodgkin lymphoma.[1] BL of the small intestine, presenting as a surgical emergency needing emergency laparotomy, is an uncommon presentation of this tumor. We present a case of BL presenting as perforation peritonitis after blunt trauma abdomen.
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Double jejunoileal fistula after ingestion of magnets p. 63
Konstantinos Zachos, Antonios Panagidis, George Georgiou, Vasileios Alexopoulos, Xenophon Sinopidis
DOI:10.4103/jiaps.JIAPS_92_18  
A double jejunoileal fistula was diagnosed intraoperatively 10 months since a 4-year-old boy had swallowed an object consisted of multiple magnetic items. Magnet ingestion presents characteristics that are misdiagnosing, critical time-consuming, dangerous, and occasionally fatal. The particularities and pitfalls of ingested magnetic items are outlined.
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Split-appendix technique for simultaneous use in the mitrofanoff principle and posterior urethral substitution in a bladder exstrophy–epispadias complex patient p. 65
Yogesh Kumar Sarin, Parveen Kumar
DOI:10.4103/jiaps.JIAPS_71_18  
We describe the case of a bladder exstrophy–epispadias complex (BEEC), in which a “split” appendix was used simultaneously as a posterior urethral replacement (for strictured Mitchell's repair) as well as a Mitrofanoff channel. Split appendix had been used before for simultaneous Mitrofanoff and Malone's antegrade contrast enema channels to manage neurogenic bladder and bowel. To the best of our knowledge, this is the first reported case for the use of split appendix for urethral reconstruction in a child with BEEC.
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Hepatoblastoma with precocious puberty p. 68
Enono Yhoshu, Yasir A Lone, Jai K Mahajan, Umesh Bahadur Singh
DOI:10.4103/jiaps.JIAPS_102_18  
Hepatoblastoma (HB), a primary liver tumor of childhood, is often accompanied by raised levels of alpha-fetoprotein (α-FP). Rarely, the beta-human chorionic gonadotropin (β-hCG) levels may also be elevated, which can cause peripheral precocious puberty (PPP). We report a case of HB with precocious puberty wherein hormonal assays showed an increase in α-FP, β-hCG, and testosterone levels, and suppression of follicle-stimulating and luteinizing hormone levels. After chemotherapy and surgery, α-FP, β-hCG, and testosterone levels normalized, and the signs of virilization did not progress further. New therapeutic approaches have made a previously reported grim prognosis of virilizing HB, more favorable now. In the assessment of PPP, the possibility of a tumoral source for the hormones should also be considered.
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Enterogenous cyst presenting as mediastinal mass in a newborn p. 72
Nidhi Jain, Vijay Gupta, NB Mathur, Ajay Kumar, Nita Khurana, Yogesh Kumar Sarin
DOI:10.4103/jiaps.JIAPS_34_18  
Duplication cyst is a rare congenital malformation. Enterogenous cyst are foregut duplication cyst with or without a vertebral defect. We report a case of a 36hr old neonate with mediastinal enterogenous cyst associated with vertebral defects. The embryology, differentials, and management of enterogenous cyst in the newborn have been discussed.
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Two cases of progressive familial intrahepatic cholestasis type 2: Role of surgery with brief review of literature p. 75
Hinglaj Saha, Ghosh Tapanjyoti, Somak Biswas, Prafulla Kumar Mishra, Kalyani Saha Basu, Uttara Chatterjee
DOI:10.4103/jiaps.JIAPS_235_17  
Progressive familial intrahepatic cholestasis (PFIC) is a rare bile acid transporter defect and autosomal recessive disorder with type 2 being the most common type. Partial internal or external biliary diversion delays its progression to end-stage liver disease. Here, we discuss two cases of type 2 PFIC.
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LETTERS TO THE EDITOR Top

Cecal hematoma associated with suspected necrotizing enterocolitis in a neonate p. 78
M Bilal Mirza, Muhammad Salman Saleem, Abrar Kamran, Mahvish Hussain, Tariq Khawaja, Kashif Arsalan, Nabila Talat
DOI:10.4103/jiaps.JIAPS_211_17  
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Interstitial cells of cajal and their correlation to outcome in congenital urological abnormalities p. 79
Salvatore Arena, Carmelo Romeo
DOI:10.4103/jiaps.JIAPS_79_18  
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