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Newborn babies presenting with difficulties related to the aerodigestive tract (ADT) are often provisionally diagnosed and managed as having oesophageal atresia +/- tracheo-oesophageal fistula. Continuing difficulties with management and abnormal findings on investigations should lead to the consideration of other congenital anomalies of the ADT, including complete larnygo-tracheo-oesophageal cleft (LTOC). We present two patients who were eventually diagnosed with complete LTOC and care was withdrawn. We discuss the inherent difficulties in reaching this diagnosis and present an algorithm to help manage these rare and challenging situations., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Indian Association of Pediatric Surgeons.)

Aim: To evaluate the range and scientific nature of oral presentations in the Scientific Program of IAPSCON from 2014-18., Methods: The oral presentations in Scientific Program of IAPSCON from 2014 - 18 were tabulated and categorised as per: type of presentation, Paediatric Surgery related clinical subject heading, nature of the content and scientific quality as per Oxford levels of clinical evidence., Results: Most oral presentations were in the form of Short papers. The most common clinical section and clinical condition presented were Urology and Hypospadias respectively. Clinical management followed by operative management represent the majority of the presentations when categorized by the type of content. Certain sections of clinical practice were not represented at all, notably anterior abdominal wall defects and gastro-oesophageal reflux. Most presentations (82.5%) are in Level 4 and 5 as per Oxford levels of clinical evidence, with Levels 1-3 accounting for 5% of presentations. Minimally Invasive Surgery (including robotic) accounted for 8.4% presentations., Conclusions: This qualitative evaluation shows the variety of oral presentations and areas of greater and lesser interest in the conference meetings. It shows that the scientific quality of the presentations can be improved and highlights certain areas of clinical and scientific research for future consideration., Competing Interests: There are no conflicts of interest., (Copyright: © 2020 Journal of Indian Association of Pediatric Surgeons.)

Aims: The purpose of this study was to assess bowel function and quality of life (QoL) in patients with Hirschsprung's disease (HD) and identify patients who have 'failed' treatment., Methods: A review of a single-center HD cohort treated with pull-through surgery from 2004 to 2017 was completed. Bowel function of patients five years and above and QoL of all patients were assessed using validated questionnaires. Patients who 'failed' treatment were defined as above five years with one or more of: a) long-term stoma, b) needing an antegrade continence enema or transanal irrigation, c) severe soiling, or d) severe constipation. Statistical analysis was performed with P < 0.05 deemed significant. Data are given as mean [range]., Results: Seventy-one patients presented with HD within the study period. Mean follow-up was 5.4 years [0.7-13.3]. Of 38 eligible patients, bowel function was assessed in 24 patients (nine had a stoma, five lost to follow-up). The mean incontinence score was 17 [0-28)], and the mean constipation score was 17 [5-25]. Incontinence and constipation scores were worse than healthy controls (P < 0.001 and P = 0.001, respectively) and did not improve with age. Fifty-six patients had QoL assessed with no difference between our cohort (81 [25-100]) and healthy controls (81 [unknown]); (P = 0.85). Thirty-three patients were assessed for 'failure' (bowel function score n = 24; stoma n = 9). Thirty of 33 (91%) children older than five years can be considered to have 'failed' treatment., Conclusions: Patients have worse bowel function than healthy children, which does not improve with age. QoL is comparable to healthy controls. A significant proportion of patients have poor outcomes and have 'failed' treatment., Level of Evidence: Level III., (Copyright © 2019. Published by Elsevier Inc.)

Purpose: Screening investigations for the vertebral, anorectal, cardiac, tracheo-oesophageal, renal and limb (VACTERL) association form an important part of the management of neonates with anorectal malformations (ARMs). We developed a proforma to define investigations and indications for referral. The aim of the current study was to determine if the proforma could improve screening rigour., Methods: Four centres performed a 3-year retrospective audit of neonates with ARM. Following introduction of a proforma, the same data were collected prospectively for consecutive neonates over a further 2 years. The appropriate investigation of each component of the VACTERL association and the corresponding referral required for each abnormal result were defined. The proportion of patients undergoing appropriate investigation and referral was compared against these standards. An audit standard of 90% was set for each criteria., Results: Prior to implementation of the proforma, 86 patients were audited, with a further 69 patients after. The audit standard was met in 7 criteria before introduction of the proforma in comparison to 10 criteria afterwards., Conclusion: The completeness of VACTERL screening and its documentation improved following introduction of the proforma. Performance remains imperfect. Review of specific criteria (such as definition of vertebral body screening) will help address this.

Background and Aims: At routine groin surgery in male paediatric patients occasionally the vas deferens may be absent. This finding usually leads to investigations to establish the status of the contralateral vas deferens and the status of the kidneys. It is not uncommon to find either an ipsilateral renal agenesis or congenital bilateral absence of the vas deferens. The latter finding prompts a test for cystic fibrosis. We report three patients who upon investigation were found to have the rare combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, and discuss the possible embryological basis, the clinical management and the long-term implications of these findings., Patients and Methods: We present three patients who were incidentally found to have absence of the vas deferens whilst undergoing elective groin surgery and following further tests were diagnosed with congenital bilateral absence of the vas deferens and unilateral renal agenesis. The case notes were reviewed, together with the results of radiological investigations, cystic fibrosis screening and the status of the contralateral vas deferens., Results: All three patients were found to have congenital bilateral absence of the vas deferens, unilateral renal agenesis and were not found to have cystic fibrosis., Conclusions: The combination of congenital bilateral absence of the vas deferens and unilateral renal agenesis, without cystic fibrosis, is rare and not reported previously in the paediatric literature. These findings require appropriate counselling of the parents and child, with regards to the long-term implications of infertility and renal function.

Magnetic resonance imaging (MRI) of a 27 week gestation female fetus suggested an abnormal rectum. Subsequent examination of the term newborn confirmed an imperforate anus present in the normal position. No meconium was seen on the perineum by 18 hours. A prone cross-table lateral radiograph confirmed that rectal gas was present 3 mm beneath the membrane leading to the diagnosis of complete anal membrane, and an anoplasty rather than a colostomy was performed., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Purpose: Proximal colonic atresia often results in a marked discrepancy of the atretic ends presenting a technical challenge for restoration of bowel continuity. We review our series of colonic atresia patients with particular reference to the techniques for restoring bowel continuity., Methods: Case notes of all patients diagnosed with colonic atresia and admitted to our unit between 1997 and 2011 were reviewed., Results: There were 13 patients with a median gestational age of 39 weeks. Location of the atresia was ascending colon (n = 1), hepatic flexure (n = 9), proximal transverse colon (n = 2) and sigmoid colon (n = 1). The primary procedure was: primary anastomosis (n = 1), right hemicolectomy followed by ileo-colic anastomosis (n = 2), right hemicolectomy and stoma formation (n = 3). Six patients underwent stoma formation, of which five subsequently had a right hemicolectomy at a later procedure. In total, 10/12 patients required right hemicolectomy to facilitate restoration of bowel continuity. Median time to full feeds was 11 days (4-46). Median follow up time was 11 months (22 days-5.6 years)., Conclusion: A temporising stoma does not reduce the discrepancy in the calibre of the atretic ends in proximal colonic atresia. Right hemicolectomy and ileo-colic anastomosis should therefore be considered at the initial surgery.

Splenic trauma in children following blunt abdominal injury is usually treated by nonoperative management (NOM). Splenectomy following abdominal trauma is rare in children. NOM is successful as in the majority of instances the injury to the spleen is contained within its capsule or a localised haematoma. Rarely, the spleen may suffer from an avulsion injury that causes severe uncontrollable bleeding and necessitates an emergency laparotomy and splenectomy. We report two cases of children requiring splenectomy following severe blunt abdominal injury. In both instances emergency laparotomy was undertaken for uncontrollable bleeding despite resuscitation. The operating team was unaware of the precise source of bleeding preoperatively. Retrospective review of the computed tomography (CT) scans revealed subtle radiological features that indicate splenic avulsion. We wish to highlight these radiological features of splenic avulsion as they can help to focus management decisions regarding the need/timing for a laparotomy following blunt abdominal trauma in children.

Ano-rectal malformations (ARM) in the male patient may be associated with a fistulous communication between the rectum and urethra. Pre-operative radiological assessment is important to delineate (a) the presence and level of the fistula, (b) the anatomy of the posterior urethra and (c) any anomalies in adjacent structures. Bladder catheterisation can be technically difficult when performing an MCUG and distal loopogram in such patients. This can be due to urethral stricture, tortuous or kinked urethra or preferential passage of catheter into a large fistula and leads to an inadequate study. We describe a "double urethral catheter technique" to enable urethral catheterisation when the fistula is large.

Primary intrarenal neuroblastoma is an extremely rare and aggressive tumour of the kidney. We report on a case that mimicked Wilms' tumour. Differentiating primary intrarenal neuroblastoma from Wilms' tumour is important both for management and prognosis.

Aim: To review the surgical management of Wilms' tumour with persistent intravascular (vena caval +/- atrial) tumour extension., Patients and Methods: Data were collected regarding operative details, tumour and 'thrombus' histology, and long-term outcome for patients with Wilms' tumour with cavo-artial extension., Result: From 1988 to 2004, 13 patients underwent treatment for Wilms' tumour with persistent intravascular extension. Preoperative chemotherapy was administered in 11/13 patients and postoperative radiotherapy in eight patients. Intravascular involvement was upto IVC (5), and right atrium (8) patients. Techniques employed for excision of intra-vascular component were: local cavotomy (3), extensive infra-diaphragmatic cavotomy without cardiopulmonary bypass (CPB) (1), and excision of cavo-atrial tumour with CPB (+/- hypothermia and cardiac arrest) (9). Mean time on CPB was 90 min. Caval repair was accomplished by primary repair (6) and pericardial graft in (7) patients. There were no intraoperative deaths and few major complications. Tumour thrombus contained malignant cells in 10/13 cases. Mean follow up has been for 55.4 months. To date, seven patients remain disease-free (one lost to follow up), disease recurred in five patients, three of whom have died. There were no symptoms related to the graft., Conclusions: Surgery for Wilms' tumour with persistent intravascular extension despite chemotherapy is technically challenging. CPB +/- hypothermia and cardiac arrest and extensive caval repair with a graft is safe and reliable in the long term.

Stomal stenosis is a frequent complication after creation of a Mitrofanoff stoma for urinary diversion. We describe a new technique for achieving a skin-lined stoma opening that is easy to construct and decreases the risk of stenosis in the long term.

Pyloric stenosis is a common paediatric surgical condition with a well-recognised pattern of clinical features. Diagnosis is usually straightforward and a Ramstedt pyloromyotomy is curative. We present three patients who developed pyloric stenosis incidentally during the management of other primary surgical conditions. Issues of diagnostic difficulty following presentation of pyloric stenosis in the post-operative period (all three patients), its rare syndromic associations (one patient) and its management in this unusual situation are discussed. An awareness of the unusual presentations of pyloric stenosis during treatment for other primary surgical conditions can help in earlier diagnosis and treatment.

Mesenchymal hamartoma of the liver is generally considered to be benign in nature, and surgical excision has been the treatment of choice. There have been 3 reports on the association of cytogenetic abnormalities with this tumor, and the possibility of malignant potential has been raised. The authors report a fourth case with a previously unreported complex chromosomal translocation between chromosomes 11, 17, and 19 at bands q12, p11, and q13.3, respectively in all cells examined and discuss the significance of this finding.

With widespread use of antenatal ultrasound, an increasing number of urinary-tract lesions are being detected, and the clinician is called upon to make management decisions. Parenchymal lesions of the kidney pose a special challenge because of the associated risk of the occurrence of malignancy. A case of an antenatally-detected solid/cystic lesion of the kidney diagnosed as a congenital cystic mesoblastic nephroma following nephrectomy is presented and the issues involved in therapeutic decision-making are discussed.

Background/purpose: The purpose of the investigation was to apply a semiquantitative scoring system for bowel function to patients who had undergone endorectal pull-through (ERPT) for Hirschsprung's disease (HD) and to use this to analyse the clinical factors relating to functional outcome., Methods: The case note details of 63 patients undergoing ERPT for HD were reviewed. A questionnaire using a semiquantitative scoring system for 6 aspects of bowel function was sent to parents of 55 patients. The total functional score (TFS) for each patient was calculated, and a statistical analysis was performed to determine which clinical factors were significantly related to bowel functional outcome., Results: Fifty (91%) of the parents returned the questionnaire. TFS was "good" in 45%, "fair" in 33% and "poor" in 22%. There was a statistically significant difference in the scores of those who had preoperative Hirschsprung's associated enterocolitis (HAEC) TFS = 8.0 and those who did not, TFS 12.7 (P <.01) The sex of the patient, length of aganglionic segment, timing of ERPT (early/late), staging of ERPT (1 or 2), presence/absence of anastomotic stricture, and presence or absence of Down's syndrome did not statistically significantly affect TFS., Conclusions: Preoperative HAEC was the most important factor in relation to functional outcome after ERPT for HD. The reason remains unclear., (Copyright 2003, Elsevier Science (USA). All rights reserved.)

Perianal abscess (PA) is a common condition encountered in childhood, but its optimal primary treatment is uncertain. Treatment of PA by incision and drainage (I & D) alone is associated with an unacceptably high recurrence rate, either as fistula-in-ano (FIA) or as PA. To identify possible causes of recurrence and assess the value of concomitant laying open of a fistulous tract at the time of primary incision and drainage, the case notes of all children who presented to our institution with a PA between January 1992 and January 1997 were reviewed retrospectively. Thirty-three cases were identified (29 boys and 4 girls). A fistulous tract was identified and laid open at the time of primary drainage in 20 cases, whilst 13 were treated by I & D alone. Following primary drainage, there were 7 recurrences (21.2%) (FIA 5 and PA 2). All recurrences had been primarily treated by drainage alone, whilst there were no recurrences in patients who had also undergone fistulotomy at the time of primary drainage. Thus the primary treatment of PA in childhood should involve a careful search for a coexisting fistula and treatment of this by fistulotomy.

Superior mesenteric artery syndrome is an uncommon clinical condition characterised by features of acute or chronic upper gastrointestinal tract obstruction. It sometimes has particular clinical associations such as weight loss, spinal surgery cast application or adhesions. Associated multiple clinical problems can complicate the picture and delay diagnosis. Diagnosis is usually by barium meal showing compression of the 3rd part of duodenum. Treatment can be conservative or surgical. Surgical treatment consists of division of the ligament of Treitz and derotation of the gut. We review our experience of four children with this syndrome.

Simple renal cysts are uncommon in children and their presentation and management has changed with increasing use of ultrasound scans. The aim of this study was to review our experience and highlight some peculiarities in diagnosis and management of these cases. Eight cases were diagnosed and two symptomatic cases underwent aspiration under ultrasound guidance; one case recurred and required re-aspiration. Differentiation of simple renal cysts from other cystic lesions of the kidney, aspiration of symptomatic cysts and the importance of long-term follow-up are discussed.