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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
October-December 2018
Volume 23 | Issue 4
Page Nos. 179-242

Online since Thursday, October 4, 2018

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OBITUARY  

Obituary Highly accessed article p. 179
Subir K Chatterjee
DOI:10.4103/0971-9261.242724  
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EDITORIAL Top

Pediatric surgical diseases and legacy of pediatric surgery in adults - Responsibility of pediatric surgeons p. 180
Sachchidananda Das
DOI:10.4103/jiaps.JIAPS_131_18  
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ORIGINAL ARTICLES Top

Vestibulo-rectal pull through in H-fistula in girls p. 182
Kuntal Bhaumik, Sachchidananda Das, Subir K Chatterjee
DOI:10.4103/jiaps.JIAPS_59_18  
Aims and Objectives: Vestibulo-rectal pull-through (VRPT) in H-fistula in girls was first described by Chatterjee et al. We are presenting our experience with this approach in 47 cases. Materials and Methods: We have total 47 cases of which one is a recurrent fistula operated outside. A circumferential incision is made around the fistula in the vestibule; fistula tract is dissected liberally and delivered by invagination into the bowel. Then, the fistula tract is excised adequately and closed from within the bowel lumen so that no anterior outpouching of the rectum remains. The perineal body is repaired through the vestibular incision. In no cases, protective colostomy was performed. Only the recurrent fistula case had colostomy done in another institution. Results: Complete cure was obtained in 45 out of 47 cases. Two of our earlier cases had recurrences perhaps due to inadequate mobilization, but in later cases, we had no recurrence. Conclusion: VRPT yields good result without the need for colostomy. Incisions on the perineal skin or the anal verge are avoided, thus improving the cosmetic outcome.
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A randomized control trial comparing outcome after stented and nonstented Anderson-Hynes dismembered pyeloplasty p. 186
Nilesh G Nagdeve, Pravin D Bhingare, Prashant Sarawade
DOI:10.4103/jiaps.JIAPS_58_17  
Aim: The aim of the study was to compare the efficacy and postoperative complications of stented and nonstented open pediatric dismembered pyeloplasty for ureteropelvic junction (UPJ) obstruction. Settings and Design: A balanced, parallel group, prospective randomized controlled trial comparing stented and nonstented Anderson-Hynes Dismembered Pyeloplasty. Subjects and Methods: It included 42 children who required Anderson-Hynes dismembered pyeloplasty for UPJ obstruction (UPJO). Patients were randomized into stented (double “J” [DJ] stent) and nonstented pyeloplasty groups. The intraoperative and postoperative course was compared. Both groups were analyzed for problems such as dysuria, frequency, pain, hematuria and urinary tract infection, and postoperative renal status. Statistical Analysis: Mann–Whitney U-test, Fisher's exact test, Student's t-tests, and Chi-squared test were used. Results: Surgical duration was significantly shorter for the nonstented group as compared to the stented group (60.4 ± 6.49 min vs. 78.9 ± 8.17 min). The intraoperative negotiation of DJ stent was troublesome in 21.7% patients belonging to the stented group. The hospital stay was comparable in both groups (4.67 ± 1.9 vs. 4.28 ± 0.67 days). Patients in stented group had experienced dysuria, loin pain, lower abdominal spasmodic pain, and frequency significantly higher than nonstented group. However, the other problems such as fever, hematuria, and urinary tract infections were more common in stented group, but the difference was not statistically significant. There was no difference in resolution of hydronephrosis in both groups. Conclusions: There is no statistically significant difference in resolution of hydronephrosis following nonstented or stented dismembered pyeloplasty in children with UPJO. However, the patient is more symptomatic due to stent in the postoperative period.
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Stricture urethra in children: An Indian perspective p. 192
Koushik Herle, Susan Jehangir, Reju J Thomas
DOI:10.4103/jiaps.JIAPS_146_17  
Background: Pediatric urethral stricture and its treatment have functional implications in the growing child. Subjects and Methods: A retrospective study of records on urethral strictures encountered in our institution between January 2005 and May 2016 yielded 23 boys against a backdrop of 19,250 admissions during the same period; stenosis and strictures after hypospadias repair were not included in this study. Demographic data were collected from the charts, and the success of repair was assessed clinically by success of repair was assessed clinically by observing for presence or absence of symptoms such as dribbling, straining at voiding, adequacy of urinary stream and radiologicaly by assessing the micturition phase of voiding cystourethrogram. Success was defined as successful initiation, flow, and completion of voiding with radiological evidence of reestablishment of urethral continuity. Results: The most common cause of urethral stricture was perineal or pelvic trauma (56.5%). Three after surgery for anorectal malformation (13.04%) and 2 (8.6%) followed otherwise unspecified urethritis. Transperineal and transpubic anastomotic routes were used for surgery. Redo surgery was required in 47.8%. The overall success rate was 82%. A self-catheterizable mitrofanoff channel was created as part of the primary procedure in 63.6% (7/11) or after the failure of the first procedure in 36.3% (4/11). Conclusion: The majority of urethral strictures are long-segment strictures or those with complete disruption not amenable to endoscopic techniques. The aim of the surgery is to obtain end-to-end opposition of healthy proximal and distal urethra. The route – transperineal or transpubic – which will give the best access to the ends of the urethra is determined by the location and extent of the stricture and the alteration in anatomy as a consequence of the pelvic fracture. Even after the introduction of laser and endoscopic techniques, surgical repair is required to tackle the majority of urethral strictures in children.
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Trends in referral pattern of antenatally diagnosed surgical abnormalities in a tertiary care center in North India p. 198
Prema Menon, Vineet Binu, Katragadda Lakshmi Narasimha Rao, Vanita Suri
DOI:10.4103/jiaps.JIAPS_232_17  
Aim: Pregnant women with antenatally diagnosed surgical abnormalities of the fetus are often referred for pediatric surgical consultation for likely outcome and prognosis. In this study, we analyzed the gestational age at referral to see the benefits of the same in decision-making and also looked for any change in trends of referral pattern over a period with the increasing availability of ultrasonography (USG). Materials and Methods: Records of pregnant women referred for antenatal counselling over an eight year period were analyzed. This was an observational study conducted in the Pediatric surgery outpatient department of a tertiary care centre from 2008-2015. All pregnant women referred for antenatal counseling were included, with the diagnosis of fetal abnormalities being based on USG. Abnormalities were classified by system involved. Comparison of data before and after the year 2012 was done to look for change in trends. Patients with poor prognostic outcome were further analyzed. Results: A total of 1211 women were analyzed, with 490 (40.7%) presenting between 2008 and 2011 and 719 (59.3%) between 2012 and 2015. Overall, in those with likely poor prognostic outcome (n = 482), 189 (39.2%) patients presented at <20 weeks gestation, 106 (22%) between 21 and 24 weeks gestation, and 187 (38.8%) after 24 weeks of gestation. Genitourinary abnormalities (n = 220; 46%) accounted for the most common poor prognostic outcome group in all gestational ages. Conclusion: Nearly two-thirds (61%) of the patients with poor outcome were referred after 20 weeks of gestation, i.e., beyond the permissible time for termination. Fetal sonography expertise between 16 and 20 weeks should be improved as a priority by the government in peripheral centers.
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Persistent mullerian duct syndrome: A single-center experience p. 203
Saravanan Natarajan, Manikandhan Periasamy, Saminathan Rangasamy, Shankar Mohan, Prabakaran Sundararajan
DOI:10.4103/jiaps.JIAPS_61_17  
Context: Persistent Mullerian duct syndrome (PMDS) is a rare disorder. It is a type of male pseudohermaphroditism, usually presenting as “Hernia Uteri Inguinalis”. Aims: This study aims to present our experience of PMDS, over a 7-year period. Settings and Design: Our center is a tertiary care facility, situated in Tamil Nadu, a southern state of India. Subjects and Methods: This is a retrospective study. The study period was from 2007 to 2015. Seven cases presented during that period. The difficulties in diagnosis, treatment options discussed, along with a review of literature are presented. Results: Seven cases of PMDS presented over 8 years. Only four were diagnosed preoperatively. Mullerian remnants were excised in five cases. Conclusions: PMDS is rare. Orchiopexy should be the goal of treatment.
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Demographic study and management of colonic atresia: Single-center experience with review of literature p. 206
Hinglaj Saha, Dipak Ghosh, Tapanjyoti Ghosh, Shibshankar Burman, Kaushik Saha
DOI:10.4103/jiaps.JIAPS_219_17  
Context and Aim: The aim of this study is to highlight the rarity of this disorder and its associated anomalies and our objective was to review our experience in the management of colonic atresia (CA) with respect to staged surgery versus one-step procedure for a better outcome of the disease. Settings and Design: A randomized, controlled, single-center study conducted over a period of 4 years from October 2013 to 2017. Subjects and Methods: Nine cases were operated for CA of which three underwent primary anastomosis and six underwent initial colostomy followed by definitive surgery. Age, sex, body weight, clinical presentation, type of atresia, site, time interval of operation, associated anomalies, initial procedure, postoperative complications, final procedure, biopsy, hospital stay, and outcome were noted. Statistical Analysis Used: Microsoft Excel was used for statistical analysis. Results: Out of 9 patients, 6 were males and 3 were females, 7 patients survived and 2 patients died (mortality 22.2%), of which one patient with primary anastomosis had leak and sepsis and one patient with primary anastomosis had associated Hirschsprung's disease (HD). Mean time gap for operation was 12.8 h and mean hospital stay was 3.5 days for initial colostomy and 21 days for primary anastomosis. Wound dehiscence occurred in 4 patients. Malrotation was found in 3 patients, HD in 2 patients, cardiac anomaly in 2, Meckel's diverticulum in 1, and cleft lip in 1 patient. Type IIIa atresia was found in in 6 patients, Type I in 2, and Type II in 1 patient. The most common site was transverse colon (n = 5). Conclusion: Diagnosis and management of CA is a challenge. Early presentation and diagnosis should be prompt. Staged procedure with initial colostomy followed by definitive procedure is the preferred choice. Associated anomaly HD must be ruled out.
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Whipple's pancreaticoduodenectomy in pediatric patients: An experience from a tertiary care center p. 212
Abhimanyu Varshney, Anjan Kumar Dhua, Vishesh Jain, Sandeep Agarwala, Veereshwar Bhatnagar
DOI:10.4103/jiaps.JIAPS_35_18  
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 <18 years of age, who underwent WPD at our center over the last 20 years. Data regarding demographics, signs, and symptoms at presentation, diagnostic imaging and procedures, pathologic reports, surgical and medical treatment, and follow-up were collected to study the indications and safety and outcomes of WPD in children. Results: Five patients had been planned for a WPD during the study (1995–2015); but in one patient, the procedure was abandoned, the rest four patients formed the study group. Male to female ratio was 3:1. Median age at the time of surgery was 9 years (11 months–12 years). The most common presentation was obstructive jaundice (50%, 2/4). Radiological imaging was able to accurately predict the surgical procedure required in all except one case. The mean operating time was 205 min (180–240 min). There were no intraoperative complications. The mean intraoperative blood loss was 85 mL (20–150 mL). The youngest patient requiring WPD was an 11-month-old child. Oral feeding was established by the 7th postoperative day (range 5–7 days) in all cases. There were no cases of anastomotic leak or pancreatic or jejunal fistulae. One patient developed features of subacute intestinal obstruction after discharge and required re-exploration. There was no intra- or post-operative mortality. Conclusion: WPD is safe and efficacious procedure in a selected group of children. The overall efficacy of surgical treatment combined with the relatively low severity of complications leads us to recommend WPD in children when indicated.
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CASE REPORTS Top

Gastric Deserosalization: What Lies Behind Closed Doors p. 216
Prabudh Goel, Minu Bajpai, Murli Krishna Nagendla, Akhil Singh
DOI:10.4103/jiaps.JIAPS_78_17  
Congenital intestinal malrotation has a wide spectrum of presentation varying from incidental detection to recurrent episodes of benign abdominal pain to frank gastrointestinal obstruction, mid-gut volvulus, and bowel gangrene. Herein, we report the first case of congenital intestinal malrotation leading to gastric deserosalization. Intraoperative findings were conspicuous by the presence of midgut volvulus and gastric perforation in the posterior wall. There are a few more dimensions uncovered by this case, a brief reference to each has been considered necessary.
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Concurrent congenital fibrolipomatous hamartoma and congenital nevus of infancy: A syndromic or chance association p. 219
Rajesh Kumar, Chandni Garg, Uma Nahar Saikia, KL N. Rao
DOI:10.4103/jiaps.JIAPS_163_17  
Congenital nevi and fibrolipomatous hamartoma are benign tumors of childhood, the latter being very uncommon. Fibrous hamartoma of infancy typically occurs in <2 years of life. The concurrence of these two lesions is extremely rare. We report a case of congenital fibrolipomatous hamartoma and congenital nevus of infancy in a 6-month-old male infant. Clinically, a suspicion of benign versus malignant lesion beneath the giant congenital nevus prompted its surgical removal. The histopathology confirmed it to be a compound lesion with benign melanocytic nevi and fascicles of spindle cells with eosinophilic cytoplasm representing hamartoma. Several types of melanocytic combined lesions have been noted with neuroectodermal and mesenchymal components. Sometimes, malignant soft-tissue neoplasm such as liposarcoma, rhabdomyosarcoma, and ganglioneuroblastoma do occur. This case report highlights the role of prompt surgical excision and histopathological examination.
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Primary iliopsoas abscess in an infant: A case report p. 222
Ajay Kumar Chintakrinda, Bikramjit Das, Shivani Dogra, Debasish Mitra
DOI:10.4103/jiaps.JIAPS_215_17  
Primary iliopsoas abscess (IPA) is rare in the infantile period. However, if treatment is not initiated soon, aggressive, and adequate, there is high risk for mortality or permanent damage to the hip joints. We present the case of a preterm neonate who presented at 2 months of age with swelling and restricted movements of the left leg for the past 1 week. A diagnosis of idiopathic left IPA was made due to Staphylococcus aureus which was drained surgically and treated with systemic antibiotics. Currently, the baby is 6 months old with no evidence of any sequelae.
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Report of Massive Bleed after Chest-drain Insertion in a Case of Job's (hyper-IgE) Syndrome p. 225
Prabudh Goel, Vishesh Jain, Sanjeev Kumar, Sanjiv Sharma, Minu Bajpai
DOI:10.4103/jiaps.JIAPS_72_17  
Chest-drain insertion is considered to be a benign procedure. A 6-year-old girl who is a known case of hyper-IgE/Job's syndrome presented to us following three episodes of fresh bleed following chest-drain insertion for the left lung abscess. The site of bleed was localized to be the ruptured pseudoaneurysm of the anterior segmental branch of the left pulmonary artery. For successful management, various pitfalls and gray areas have been highlighted: (a) the procedure of chest-drain insertion has a potential for many complications some of which may be life-threatening, (b) the importance of proper history taking and a complete evaluation of all patients before surgical intervention, (c) role of ultrasound-guided chest-drain insertion, and (d) the importance of lateral and pragmatic approach with multidisciplinary involvement in such unusual and challenging cases cannot be overemphasized.
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Teratoma in the hepatoduodenal ligament p. 228
Sreekar Gundapaneni, Anjan Kumar Dhua, Vishesh Jain, Manisha Jana, Sandeep Agarwala, Veereshwar Bhatnagar
DOI:10.4103/jiaps.JIAPS_36_18  
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.
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Yolk sac tumor of stomach: Case report and review of literature p. 232
Ankur Mandelia, Nijagal Mutt, Richa Lal, Raghunandan Prasad
DOI:10.4103/jiaps.JIAPS_17_18  
Gastric yolk sac tumors (YSTs) are very rare. Only seven cases of pure YST of the stomach, with a median age of 65 years, and five cases of YST combined with adenocarcinoma have been reported. We report a 3-year-old boy who presented to us with recurrent episodes of melena and anemia of 2 months' duration. Upper gastrointestinal endoscopy revealed a deeply excavated ulcer on the lesser curvature, 4 cm from the gastroesophageal junction. Computed tomography (CT) abdomen revealed large heterogeneously enhancing soft-tissue mass lesion in the lesser sac with indistinct fat planes with the lesser curvature of the stomach with multiple peripherally enhancing lesions in both lobes of liver and omentum, suggestive of metastasis. Serum alpha-fetoprotein (AFP) was 21,000 ng/ml. Tru-cut biopsy from the mass was suggestive of YST. Following three courses of chemotherapy based on Cisplatin, Etoposide and Bleomycin (PEB), sleeve gastrectomy with excision of the mass with omentectomy with biopsy of the liver nodules with a sampling of perigastric lymph nodes was done. Histopathology revealed only necrotic tissue with no evidence of residual malignancy. Three-month follow-up CT revealed no residual mass with AFP of 3 ng/ml.
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Anesthetic and surgical management of mucoid retention cyst in the vallecular region: An airway challenge p. 234
Christina George, Aditya Martin, Narjeet Meena Osahan, Dootika Liddle
DOI:10.4103/jiaps.JIAPS_170_17  
Growth of any kind in the oropharynx poses a threat to the airway especially during anaesthesia. Being aware of the challenges and adequately equipped to handle the situation is the duty of the anesthesiologist. Methods and Material: An eight year old girl diagnosed case of multiple mucous cysts presented to the outpatient department for excision of a lower lip mucous cyst. So an ultrasound was done which revealed a large intramural mucous cyst. After a preanaesthetic checkup she was posted for the excision of the cyst. Results: The girl was posted for Elective surgery and was induced without prior premedication as she had revealed a history of snoring in supine position. After preoxygenating with 100% oxygen, she was induced with Injection Ketamine and Sevoflurane. She started desaturated abruptly so nasal airway and finally # 2.5 LMA was inserted but SpO2 didn't improve. With backup of Ent and paediatric surgeons direct larngoscopy was attempted and #5 ETT was inserted successfully. Following which the huge vallecular cyst was removed.
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IMAGES Top

An enigmatic route to the contralateral pelvicalyceal system on antegrade pyelogram p. 236
Prabudh Goel, Minu Bajpai, Devasenathipathy Kandasamy
DOI:10.4103/jiaps.JIAPS_139_18  
Introduction: The authors present the rare yet enigmatic phenomenon of ‘pyelo-renal’ backflow. Case Presentation: An eight-month-old boy with multiple congenital anomalies underwent left Anderson-Hynes Pyeloplasty for pelvi-ureteric junction obstruction. Antegrade dye-study done through the nephrostomy revealed obstruction at the level of the pelvi-ureteric junction, yet the contrast was visualized in the pelvis of contralateral kidney and urinary bladder (pyelo-venous backflow) masquerading as vesicoureteric reflux. The phenomenon of ‘pyelo-renal’ backflow along with pyelo-tubular, pyelo-interstitial, pyelo-sinusal and pyelo-lymphatic backflow have been described and the respective mechanisms discussed. Conclusion: The phenomenon is known to happen in the presence of obstruction to outflow from renal pelvis thereby creating a closed compartment. Injection of contrast at a pressure above the critical limit may result in forniceal tears and back-flow of contrast into the renal tubules and beyond.
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LETTERS TO THE EDITOR Top

Penile dislocation with inversion p. 239
Yogesh Kumar Sarin
DOI:10.4103/jiaps.JIAPS_66_18  
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Pneumoperitoneum: Not always due to an intestinal perforation!! p. 240
Nidhi Sugandhi, Pratheep Samraj, Samir Kant Acharya, Amit Jadhav, Narinder Tekchandani, Deepak Bagga
DOI:10.4103/jiaps.JIAPS_248_17  
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Male cloaca – An additional rare variant of anorectal malformation p. 241
Shilpa Sharma, Devendra K Gupta
DOI:10.4103/jiaps.JIAPS_82_18  
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