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VACTERL association is a heterogeneous condition that includes at least three out of six core structural defects which occur together more commonly than would be expected by chance alone. We report a rare association of VACTERL with unilateral proximal focal femoral deficiency and trisomy 18 syndrome.

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BACKGROUND: Large wounds following surgery for neural tube defects are difficult to close; physical wound characteristics such as position and dimension would serve as a guide for their surgical closure. AIM: To study how wound dimension determines the choice between primary and rhomboid flap closure of skin defects following surgery for neural tube defects. MATERIALS AND METHODS: A retrospective study was carried out on cases of neural tube defects operated in the department of paediatric surgery at a tertiary center for 3 years from January 2015 to December 2017. Data regarding clinical features, location, wound dimensions following surgery, any bony deformity, method of closure used, distance of wound from anus, and postoperative complications were collected and analyzed. RESULTS: A total of 114 cases were operated during this period; 86/114 had primary closure, whereas 28/114 needed rhomboid flap for tension-free cover. Primarily closed wounds had a biphasic distribution of (long axis)/(short axis) ratio (with values either >1.65 or <0.63), whereas those covered by rhomboid flaps had a mean ratio of 1.25 (range, 0.71-1.45). All six cases with bony deformity needed rhomboid flaps. Although all lipomeningomyelocele defects could be primarily closed, all rachischisis needed flap cover. Infected lesions had a mean wound distance of 5.3cm from posterior anal margin. CONCLUSION: Defect's position, its size and shape, and any bony deformity determine the choice of closure of postoperative wound. The versatile, safe, and universal rhomboid flap is an aesthetic solution to the large skin defects in patients of neural tube defects.

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INTRODUCTION: Foreign bodies in the urogenital tract are not uncommon. Hairpins, glass rods, umbilical tapes, ball point pen are described in lower urogenital tract. Retained gauze piece (gossypiboma) in posterior urethra may cause diagnostic dilemma. Symptoms and investigations may mimic stricture of posterior urethra. PRESENTATION OF CASE: Two cases of retained gauze pieces in the urethra are described here. The micturating cystourethrogram was suggestive of posterior urethral stricture. DISCUSSION: Two cases described here had retained gauze piece as a cause of filling defect and abnormal appearance in the micturating cystourethrogram. Gossypiboma may be a possibility where posterior urethral stricture are seen after previous surgery in paediatric age group. CONCLUSION: In the setting of previous urogenital surgery gossypiboma should be kept in the differential diagnosis where posterior urethral stricture are seen in the paediatric age group.

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A crossed-fused ectopic kidney is a rare congenital anomaly, and uncommonly we see an aberrant vessel as a cause of hydronephrosis in a kidney. We report a case of 4-year-old boy with crossed-fused right renal ectopia with hydronephrosis in the both kidneys. The aberrant renal vessel was found pressing the left pelvi-ureteric junction causing hydronephrosis. Dismembered pyeloplasty of the left kidney was performed anterior to the aberrant vessel. The patient is doing well in the follow-up visits and his renal function is stabilized. Aberrant blood supply may be a cause of hydronephrosis in the crossed-fused ectopic ipsilateral kidney. Keeping this entity in differential diagnosis will help in successful management of the crossed-fused ectopic kidney.

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INTRODUCTION: The coexistent biliary atresia with aneurysmal dilatation of hepatic artery is a rare association. To know these anomalies will avoid many per operative complications. It is also important to mention that these children require liver transplantation in the long run then these vascular anomalies become more relevant. PRESENTATION OF CASE: A four month old male child presented with features of biliary atresia. On exploration a cystic expansile mass was detected beneath thread like common bile duct. Subsequent aspiration and studies proved it to be aneurysmal dilatation of hepatic artery. DISCUSSION: With biliary atresia many vascular and cardiac malformations have been described but aneurysmal dilatation of hepatic artery is a rare association. These anomalies may have impact on aetiopathogenesis of biliary atresia and also future liver transplantation. CONCLUSION: Awareness of rare association of hepatic artery aneurysm with biliary atresia will help in understanding aetiopathogenesis of biliary atresia and planning liver transplantation in such cases.

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Esophageal atresia with tracheoesophageal fistula (TEF with EA) is a common disease which is associated with many other congenital abnormalities. The most common association is with VACTERL syndrome.. Various types of ear malformations have been reported in CHARGE syndrome associated with EA and TEF. However, absence of external ear has not been described which is being reported in this article. We could not found this association even on extensive search of literature.

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Importance of day-care surgery in pediatric age group is ever increasing and nowadays bulk of surgery is performed as a day-care procedure. Day-care procedure requires short intra-operative period and less postoperative complications. The introduction of tissue adhesive has been received enthusiastically since they may result in equivalent tensile strength-improved cosmetic appearance of the scar and lower infection rate when compared to sutures, staples and adhesive tape. We have used isoamyl 2-cyanoacrylate as a tissue adhesive for skin closure in day-care surgery. Novocryl is ideal for such procedure as it is a sterile, inert, non-toxic, biocompatible and bacteriostatic liquid topical skin adhesive containing isoamyl 2-cyanoacrylate formulation. We applied isoamyl 2-cyanoacrylate glue in 100 patients over skin cut margin after stitching subcutaneous tissue. Skin margin were held together by means of skin hook for 1-2 min. The cosmesis was better as there were no suture marks or lumpiness. There were no major complications in our study. Thus, isoamyl 2-cyanoacrylate can be considered as an alternative to skin suturing in the pediatric day-care surgery.

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Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature-3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature.

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AIM: To evaluate the role of end-to-end oblique bowel anastomosis in bowel atresia. METHODS: End-to-end oblique anastomosis was done in 25 neonates of bowel atresia and the results were compared with traditional method of end to back anastomosis in 25 cases. RESULTS: We found less mortality and morbidity (5%) in our technique as compared to end to back technique (20%). CONCLUSIONS: We recommend this technique, as anastomosis is wide with less angulations, flow of effluent is linear, and there is less force exerted over post-anastomotic side wall.