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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
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January-February 2020
Volume 25 | Issue 1
Page Nos. 1-64

Online since Wednesday, November 27, 2019

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EDITORIAL  

Pediatric surgery in India: From inception to the travails of today and the way forward p. 1
Kuntal Bhaumik
DOI:10.4103/jiaps.JIAPS_198_19  
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ORIGINAL ARTICLES Top

Androgen receptor expression in hypospadias Highly accessed article p. 6
Dhanvanth Rajesh Balaji, Goutham Reddy, Ramesh Babu, Balamourougane Paramaswamy, Madhu Ramasundaram, Prakash Agarwal, Leena Dennis Joseph, Lawrence D'Cruze, Sandhya Sundaram
DOI:10.4103/jiaps.JIAPS_166_18  
Introduction: The exact mechanism behind the development of hypospadias is unclear. Research studies on androgen receptor (AR) expression are controversial with results stating all possible outcomes – AR elevated, similar, or reduced when compared to normal. Aims: The aim is to study the AR expression and hormone levels in hypospadias patients and compare them with children having normal genitalia. Methods: Group 1 (controls) involved patients who underwent circumcision for phimosis while Group 2 involved hypospadias patients who did not receive any preoperative testosterone. Preoperative hormonal assay included luteinizing hormone, follicle-stimulating hormone, and free testosterone levels in all the patients. The foreskin specimen was analyzed for AR expression using immunohistochemistry (anti-AR antibody PathnSitu, clone R441, 1/100 dilution). AR staining was expressed as H score. The H score was calculated by multiplying the intensity of staining and the percentage of stained cells showing cytoplasmic positivity at high power (×40). Results: There were 27 patients in Group 1 while 16 in Group 2 (distal 10; proximal 6).There was no significant difference in the age distribution. The mean H score was significantly higher (189.5) in hypospadias patients compared to controls (97.5) and was significantly higher in proximal (220) compared to distal (159) hypospadias. There was no significant difference in hormone levels between groups. Conclusion: AR expression was significantly elevated in hypospadias patients. It was higher in proximal compared to distal hypospadias, probably due to end-organ overexpression. Further larger trials are likely to through light into this controversial subject.
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Type IV congenital pouch colon in male children: Anatomical variations and a proposed new subclassification p. 10
Shailesh Solanki, Prema Menon, Shubhalakshmi Nayak, Ram Samujh, KL N. Rao
DOI:10.4103/jiaps.JIAPS_189_18  
Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review. Materials and Methods: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score. Results: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had “good” continence score compared to four children (three having anal canal) in Group 2. Conclusion: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence.
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Sacrococcygeal teratoma: Clinical characteristics, management, and long-term outcomes in a prospective study from a Tertiary Care Center p. 15
Devendra Kumar Yadav, Samir Kant Acharya, Deepak Bagga, Vishesh Jain, Anjan Dhua, Prabudh Goel
DOI:10.4103/jiaps.JIAPS_219_18  
Introduction: The study focuses on the clinical presentation, management, and outcomes (both short term and long term) in patients with sacrococcygeal teratoma managed over a decade in a tertiary care center. Materials and Methods: This is a prospective study on children with sacrococcygeal teratoma over 12 years data collected included antenatal diagnosis, mode of delivery, age at diagnosis, clinical presentation, physical extent of mass (including Altman classification), levels of alpha-fetoprotein, surgical approach, histopathology, clinical outcome, recurrence and long-term results including bladder-bowel dysfunction and neurological impairment. Functional results were evaluated clinically and radiologically. Results: During the study, 41 patients (male to female ratio of 1:3.1) with a median age of 36 days (1 day–11.6 years) with sacrococcygeal teratoma were managed at our center. The mean follow-up duration was 54 months (range 19–110 months). Nearly, two-thirds of the tumors were either Altman Type 1 or 2. Yolk sac tumor was present in 8 (19.5%) patients, while the rest has either mature or immature teratoma. Tumors were removed through a posterior sagittal or a chevron incision. In seven patients, abdominosacral approach was necessary. Eight patients with malignant disease received chemotherapy (neoadjuvant in 5). Overall survival was 95% at a mean follow-up of 54 months. Among the late complications, three patients had a local recurrence of tumor, and urinary dribbling was present in three patients. Conclusions: Teratomas are the most common germ cell tumors of the sacrococcygeal region. Most of the tumors are benign, and the incidence of malignancy increases with age. The evaluation of malignancy is, therefore, necessary in these children. Excellent survival of 95% was achieved in this series. Morbidity due to associated malformation, disease recurrence, and treatment may persist in these patients; hence, proper follow-up is needed.
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Pediatric pancreatitis: Outcomes and current understanding p. 22
Deepti Vepakomma
DOI:10.4103/jiaps.JIAPS_223_18  
Aim: The aim of this study was to analyze the clinical spectrum of pediatric pancreatitis and review current literature with regard to concepts of disease and management approaches. Materials and Methods: This is a retrospective analysis of pancreatitis patients admitted to a tertiary referral pediatric surgical unit from March 2013 to September 2018. Results: There were 106 patients from 1 year to 18 years with median age group between 11 and 15 years and equal gender distribution. Sixty-two children had acute pancreatitis (AP), with 13 of them having recurrent episodes. Forty-four patients had chronic pancreatitis (CP). Definite etiology was found in 11 AP patients. All patients had radiological changes. All AP patients were treated symptomatically except those with correctable underlying etiology. Sixteen with CP required intervention in the form of endoscopic retrograde cholangiopancreatography sphincterotomy/stenting or surgery. There were two mortalities. One with acute necrotizing pancreatitis and one CP girl due to the complication of diabetes mellitus, unrelated to episode of pancreatitis. All others are well at the last follow-up. Conclusion: Pediatric pancreatitis is a disease with a wide spectrum, but management can be standardized. Newer studies reveal that certain genetic mutations make children more susceptible to pancreatitis. Increasing incidence must prompt us to evaluate further so as to better equip ourselves to managing this disease entity in all its forms and evolve preventive strategies.
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Our Laparoscopic Surgical technique and experience in treating pediatric inguinal hernia over the past decade p. 28
Dheidan Alshammari, Marina Sica, Isabelle Talon, Isabelle Kauffmann, Raphael Moog, Francois Becmeur, Anne Schneider
DOI:10.4103/jiaps.JIAPS_233_18  
Background: Over the past decade, laparoscopic hernia repair was the most performed operation in our department. Equally, it compromises 15% of all pediatric operations performed. We aim, in this study, to review all the cases performed and extrapolate important information like reoccurrences, the incidence of metachronous inguinal hernia, complications amongst other information. Material and Methods: All patients under the age of 18 whom underwent elective laparoscopic hernia repair between 03/01/2007 till the 18/05/2016 were included in our study. We recorded important clinical features and studied their post-operative follow up. Equally reoccurrences, the incidence of metachronous inguinal hernia, complications and other parameters were recorded and studied. Results: A total of 916 patients were operated on during the defined study period. There was a 0.17% reoccurrence rate and a 0.46% incidence of metachronous inguinal hernia. Equally a contralateral patent processus vaginalis was diagnosed and closed in 17.10%. There were no postoperative complications and we had a 0% postoperative hydrocele rate. Conclusion: Laparoscopic hernia repair is safe and carries all the benefits of minimally invasive surgery. We recommend that it is offered to patients and would like to refute previously claimed reports that it carries a higher reoccurrence rate or takes a long time to perform. Our reoccurrence rate of 0.17% is actually lower than many published reoccurrence rates after open repair.
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A novel technique for prevention of gastroesophageal reflux in staged repair of long gap esophageal atresia with tracheoesophageal fistula p. 34
Vipul Prakash Bothara, Gyan Prakash Singh, Shiv Narain Kureel
DOI:10.4103/jiaps.JIAPS_239_18  
Aim: The objective of the study is to report a novel technique of preventing gastroesophageal reflux and air leak from fistula to stomach in patients of tracheoesophageal fistula with long gap atresia, to buy time for the staged procedure. Methods: Seven patients of tracheoesophageal fistula with upper pouch of esophagus at 2nd thoracic vertebra were selected for the staged procedure. Weight ranged from 1.7 to 1.8 kg. During the 1st stage surgery for gastrostomy, midline strip of linea alba attached to xiphoid process was harvested and slinged around the gastroesophageal junction, along with right cervical esophagostomy. After radio-nuclear scan, the demonstration of abolition of gastroesophageal reflux, gastrostomy feed was started. The 2nd stage surgery performed after 6 weeks, included mobilization of esophagostomy, release of sling, thoracotomy, and tension-free esophageal anastomosis. Outcome measurement includes (1) prevention of air leak from esophagus into the stomach, (2) abolition of gastroesophageal reflux, (3) ability to start gastrostomy feeds, and (4) reversal of occlusion after release of the sling. Results: The placement of linea alba sling and elevation of gastroesophageal junction, abolished air leak from fistula to stomach in all. Radio nuclear scan demonstrated abolition of gastroesophageal reflux in 6 with weight gain after gastrostomy feeding. One patient expired due to sepsis. One patient underwent final repair with reversal of occlusion with release of the sling. Conclusion: Using a sling of the linea alba around the cardioesophageal junction, prevents gastroesophageal reflux and escape of air from esophagus into the stomach, gives time to improve the respiratory and nutritional status of the patient, for a subsequent safer delayed primary anastomosis.
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Correlation of hepatic artery resistive index with portal pressure and serum nitric oxide levels in patients with extrahepatic portal vein obstruction p. 38
Abhishek Gupta, Veereshwar Bhatnagar, Anjan Kumar Dhua, Manisha Jana, Archana Singh, Rohan Malik
DOI:10.4103/jiaps.JIAPS_244_18  
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.
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CASE REPORTS Top

Management of choledocholithiasis in a 5-month-old male postprimary repair of esophageal atresia with tracheoesophageal fistula p. 43
Meera Luthra, Chiranjiv Kumar, Sanjay Saran Baijal
DOI:10.4103/jiaps.JIAPS_53_18  
Choledocholithiasis (CDL) in neonates and infants is <1 in 5000. There is no gold standard for the management of CDL in the pediatric population. In English literature, this is the first case report of a baby born with esophageal atresia and tracheoesophageal fistula (Type C) with cholangitis, who underwent percutaneous transhepatic biliary drainage and peroral extraction of a large calculus in the common bile duct after primary repair of esophagus.
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Repair of ruptured omphalocele sac in the neonatal period and beyond p. 46
Nidhi Sugandhi, Manoj Saha, Veereshwar Bhatnagar, Anjan Kumar Dhua
DOI:10.4103/jiaps.JIAPS_195_18  
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.
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Generalized lymphangioma of the tongue: A rare cause of macroglossia p. 49
Amar A Shah, Khalid Mahmud, Anirudh V Shah
DOI:10.4103/jiaps.JIAPS_210_18  
Generalized lymphangioma of tongue is a rare cause of macroglossia in children. It causes mechanical discomfort and functional and psychological disturbances. We report a case of macroglossia due to generalized lymphangioma in a 10 year old where partial glossectomy was done to achieve symptomatic relief.
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Acute lymphoblastic leukemia in a child presenting primarily with priapism p. 52
Garima Gupta, Deepak Kumar, Maharishi Trivedi
DOI:10.4103/jiaps.JIAPS_214_18  
Priapism is an uncommon presentation in children and adults with a chronic myeloid type of leukemia. Its association is rarely found in an acute lymphoblastic type of leukemia (ALL). Timely management is important to prevent irreversible complications. We report a case of ALL with presenting complaint of priapism.
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Laparoscopic Puestow procedure for chronic pancreatitis in children p. 55
Apurva Arora, Prakash Agarwal, Rajkishore Bagdi, Madhu Ramasundaram, ML Sankar Narayanan
DOI:10.4103/jiaps.JIAPS_235_18  
Chronic pancreatitis (CP) is a sequelae characterized by recurrent attacks of pancreatitis leading on to fibrosis and calcification of both ductal and parenchymal elements of the pancreas. Most of these, if left untreated at the right time, will lead on to burnt-out pancreas with complete atrophy of the parenchyma, leading on to endocrine/exocrine insufficiency. The pain in such patients is most likely due to ductal hypertension, parenchymal hypertension, and head mass causing perineural inflammation. Puestow procedure (longitudinal pancreaticojejunostomy) is the surgery of choice in selected patients with ductal hypertension where decompression of the duct provides symptomatic relief. Performance of such procedures in children by minimal access laparoscopic method is still evolving. We report a series of three patients (aged 10, 12, and 16 years) diagnosed with CP and successfully managed by laparoscopic Puestow procedure.
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Mesonephric remnant (paroophoron) presenting as a congenital lumbar sinus in a child p. 58
Saurabh Garge, Geetika Paliwal, Sharmila Mittal, Neha Kakani
DOI:10.4103/jiaps.JIAPS_242_18  
Presence of symptomatic mesonephric duct remnants is rare in pediatric age group. These can be present in dormant physiological or symptomatic pathological states. Pathologies can vary from benign cysts to malignant tumors. We here report a rare presentation in a 2-year-old female, of a mesonephric remnant in the form of a sinus above the iliac crest.
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IMAGES Top

A rare case of neonatal extensive small intestinal diverticulosis associated with jejunal atresia p. 60
Ramesh B Hatti, Anita H Nyamagoudar, Timman Gouda R. Patil, Rajendra T Patil, Sunil J Patil
DOI:10.4103/jiaps.JIAPS_200_18  
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Large pediatric abdomino-groin lipoblastoma: A rare case p. 61
Enono Yhoshu, Intezar Ahmed, Gyanendra Chaudhary, Manish Kumar Gupta
DOI:10.4103/jiaps.JIAPS_42_19  
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LETTERS TO THE EDITOR Top

Formulation of research question p. 62
Beuy Joob, Viroj Wiwanitkit
DOI:10.4103/jiaps.JIAPS_247_18  
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Testicular mixed germ cell tumor in a newborn child: A rare case p. 62
Mahmood Dhahir Al-Mendalawi
DOI:10.4103/jiaps.JIAPS_46_19  
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Umbilical hernia and comorbidities in children p. 63
Gabriel Ngom, Salsabil Mohamed Sabounji, Mbaye Fall, Ndeye Fatou Seck, Ibrahima Bocar Welle, Oumar Ndour
DOI:10.4103/jiaps.JIAPS_49_19  
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  2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

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