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Lipomas are slow growing tumors rarely found in children. We report a very rare case of an intermuscular fibrolipoma found deep within the posterior triangle of the neck. A 3 year old child was brought with the complaints of right sided neck mass and an inability to fully turn his head for two months. Ultrasound and computed tomography of the neck suggested a diagnosis of intermuscular lipoma. The patient was successfully managed by complete surgical resection of the mass. Histopathological examination confirmed the diagnosis as intermuscular fibrolipoma. Due to the rarity of the case, we are prompted to report it.

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We describe a case of a 2-year-old female child who presented as emergency with acute gastroenteritis and severe dehydration. In this patient, there was a history of severe birth asphyxia, and the developmental milestones were delayed. The child was managed as hypotonic cerebral palsy elsewhere with antiepileptic drug and nutritional supplements. However, persistent abnormal pattern of breathing after adequate hydration and noncontributory metabolic profile raised the suspicion of alternate etiology. Later, the diagnosis of Joubert syndrome was established on contrast-enhanced magnetic resonance imaging of brain with findings of "molar tooth sign" appearance along with vermian hypoplasia. We present this case to alert the clinicians to explore all the differential diagnoses carefully whenever a child presents with the developmental delay associated with multisystem involvement.

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Advances in diagnostic and therapeutic interventions have improved the survival of neonates with sacrococcygeal teratoma in richer countries. These changes have not yet taken place in resource-limited settings. A retrospective analysis of neonates with sacrococcygeal teratoma operated at our centre during 1997-2016 was performed. Among 44 neonates enrolled, prenatal diagnosis of sacrococcygeal teratoma was available in 25% of pregnancies. All babies except one were born vaginally. Associated congenital malformations were seen in 20% (9/44). Types of sacrococcygeal teratoma were classified as Altman type I in 12 (27.3%), 24 (54.5%) as type II, 6 (13.6%) as type III and 2 (4.5%) as type IV. Morphologically, 77% (34/44) neonates had cystic lesions. All patients were managed successfully by total excision. Histopathology confirmed mature teratoma in 43 (97.7%). After a 12-month follow-up, no recurrence was observed. An uneventful pregnancy, absence of associated congenital anomalies, cystic morphology and a mature histology are good prognostic factors.

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With increasing use of ultrasound screening, the prenatal diagnosis of congenital diaphragmatic hernia (CDH) in better resourced areas has become the norm. However, early diagnosis is still not universal in resource-poor settings and late presentations of CDH continue. We retrospectively analysed the medical records of children operated for late-presenting CDH from 2001 to 2016 at our tertiary care centre in North India. A total of 32 patients were operated during the period with a male-to-female ratio of 3:1. Of these, 78% presented with respiratory symptoms, 37% with recurrent vomiting and 18% with an acute abdomen. Nine (28%) had been treated erroneously for gastroenteritis and another six (18%) had received anti-tubercular therapy for variable periods. A plain chest radiograph with a Ryle's tube in situ was confirmatory in 75% (24/32). In conclusion, initial misdiagnosis and subsequent unnecessary therapeutic interventions were the leading cause of morbidity .

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A series of five children with delayed presentation of congenital pulmonary airway malformation (CPAM), all initially misdiagnosed as pulmonary tuberculosis, is described. All five children presented with recurrent respiratory infections and failure to thrive, having shown poor response to multiple antibiotics course, including anti-tubercular therapy for variable periods. Successful surgical intervention was belatedly instigated.

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OBJECTIVE: The surgical repair of hypospadias is done in two stages in a select group of patients with severe anomaly. The first stage (I) procedure consists of correction of penile shaft curvature and second stage (II) repair involves the creation of a neourethra. This neourethra needs a cover of an intermediate layer in order to have good functional and cosmetic results. Among the various local flaps, tunica vaginalis flap is a good option for the use as an intermediate layer. METHODS: We have managed 22 patients of chordee with hypospadias by staged repair. In Stage I, chordee correction was done by dividing the urethral plate and covering the penile shaft with dorsal prepucial flaps. In Stage II, a neourethra was created and covered with tunica vaginalis flap either through the same incision (14/22) or via a subcutaneous tunnel (8/22). An indwelling catheter was kept for 10 to 12 days. RESULTS: Eighteen (81.8%) patients had successful functional and cosmetic repair. Two patients (9.1%) had urethrocutaneous fistula of which one healed on subsequent dilatation while the other one (4.5%) needed repair. Overall fistula formation rate was 4.5%. In two patients, the external urinary meatus could be made upto subglanular or coronal level. CONCLUSION: Staged repair of chordee with hypospadias is valuable in selected group of patients and tunica vaginalis flap is an excellent intermediate layer to cover the neourethra. However preoperative counseling is particularly essential in patients where the external urinary meatus can be created at coronal or subglanular level.

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Ninety-four patients with second branchial anomalies were retrospectively analysed at a tertiary care centre from January 2006 to September 2016 to determine the demographical data and management. Branchial sinus and fistula presented earlier as compared to branchial cyst. The mean age at presentation in case of branchial sinuses, fistulae and cysts was 5.07, 5.79 and 7.31 years respectively. There was preponderance in males as compared to females, more so in bilateral cases. Male to female sex ratio was 2.91:1. The branchial fistulae were the most common type of lesions, followed by the branchial sinuses. The branchial anomalies were more on the right side (65.96%) probably due to right handedness of the population. Only eight patients (8.51%) had bilateral anomalies. Four patients had familial association, it was seen in bilateral cases and they presented earlier than unilateral cases. Early and complete surgical excision is the treatment of choice. Preoperative sinogram/fistulogram and intraoperative methylene blue dye injection is not mandatory for excision of a branchial sinus/fistula. Post-operative wound infection was the most common complication (4.25%).

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OBJECTIVE: The patients with anorectal malformations (ARM) have been identified with specific and non-specific pathological changes. The present study was conducted with the aim to study histomorphological changes and various immunohistochemical (IHC) markers (calretinin, S-100, CD117) in intestinal wall specimens to assess neuronal dysfunction in ARM patients. MATERIAL AND METHODS: Thirty children having ARM were included in our study. In all the cases, a representative biopsy was received. The tissue sections were processed and wax blocks were prepared. Various histopathological changes were examined on routine H&E. Representative sections were further subjected to IHC staining for ganglion cells (calretinin), interstitial cells of Cajal (CD117) and nerve bundles (S-100 protein). Descriptive variables were analyzed to assess neuronal dysfunction in cases of ARM. Chi-square was used to compare the categorical values. P-value <0.05 was accepted as statistically significant. RESULTS: Biopsies were studied for histological changes using H&E stain. The most frequently observed histological finding in mucosa was inflammation and congestion in 87% and 67% of cases respectively. Disrupted muscularis mucosa was observed in 60%, eroded mucosa in 57%, and hemorrhage in 40% of cases. Submucosal inflammation and congestion were most common finding observed in submucosa in 87% and 80% cases respectively. CD117 was used to demonstrate altered density and distribution of interstitial cells of Cajal (ICC) in cases of ARM. Majority of them belong to grade 2+ category (n=17, 57%) followed by grade 1+ (n=8, 17%) for ICC cells. Altered density and distribution of ICC was observed in ARM which was statistically significant (p=0.02). CONCLUSION: The malformed segments in ARM show various specific and non-specific histomorphological changes. Examination of H&E sections along with IHC stains evaluation can minimize need for repeated biopsies and unnecessary radical treatment. CD117 immunohistochemistry is reliable adjunctive test in evaluation of ICC in motility disorders of bowel. Calretinin is good marker for identification of ganglion cells. In ARM, density and distribution of ICCs is significantly altered which can explain postoperative dysmotility.

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Trichobezoar is less common in boys. We are reporting a case of isolated ileal trichobezoars in a 4-year old boy causing intestinal obstruction and gut ischemia with perforation and peritonitis. The case was managed surgically with ileal resection and anastomosis. Postoperative period was uneventful.

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Transverse testicular ectopia is an extremely rare anomaly, in which both the testis migrate towards the same hemiscrotum through the same inguinal canal. It is usually associated with other abnormalities such as persistent Mullerian duct syndrome, true hermaphroditism, inguinal hernia, pseudohermaphroditism, and scrotal anomalies; the association with inguinal hernia being the commonest. We, here, report a case of transverse testicular ectopia in a 12 years old boy having the ectopic testis in contralateral hemiscrotum without any inguinal hernia but having scrotal hypospadias with severe chordee. The diagnosis was made on clinical examination and confirmed on ultrasonography. Trans-septal orchiopexy was done for ectopic testis and hypospadias and chordee were managed by staged repair.

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BACKGROUND: Gastrointestinal tract (GIT) duplications are one of the rare congenital anomalies and can occur in any portion of the gastrointestinal tract but are more commonly encountered in small intestine. The duplication cysts cause symptoms like abdominal mass and intestinal obstruction requiring surgery or may remain asymptomatic. We are reporting our 15 years' experience duplication cysts presenting in neonates. METHODS: It is a retrospective study undertaken in the department of pediatric surgery between 2001 and 2015 for GIT duplications in neonates. Patients were analyzed for their antenatal diagnosis, age, sex, clinical diagnosis, investigatory approach, operative management and surgical outcomes. RESULTS: Total number of neonates, diagnosed with gastrointestinal duplication in the last 15 years, was 17. Male to female ratio was 3.3:1. The most common location was found to be the ileum occurring in 71% of cases. Apart from ileum, 2 cases of duodenal and 1 case each of gastric, colonic and cecal duplication cyst were encountered. Majority cases presented with sub-acute intestinal obstruction and were managed successfully by resection and end to end anastomosis. Associated gut atresia was found in 4 cases while 1 case was found to be associated with perforation of gut. CONCLUSION: Gastrointestinal tract duplications often present with typical symptoms of gastrointestinal tract obstruction. Early diagnosis and management is required to prevent postoperative morbidity and mortality.

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Esophageal atresia with tracheoesophageal fistula (EA/TEF) associated with distal congenital esophageal stenosis (CES) is a well-known entity. We encountered three patients of EA/TEF associated with long and unusual CES.

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BACKGROUND: Type III and IV jejunoileal atresias are associated with loss of significant length of the gut and can lead to short gut syndrome if further resection of proximal dilated gut is done. We modified the anastomotic technique so that proximal dilated segment of the gut is not resected as to prevent short gut syndrome. MATERIAL AND METHODS: Medical Record of patients of Type III and IV jejuno-ileal atresias managed with modified anastomotic technique in our center during 5-years was reviewed. RESULTS: Fifteen patients were managed with our modified technique. There were no anastomotic leak observed and there was 6% mortality seen in our modified technique. CONCLUSION: We found less mortality and morbidity in our technique compared to recommended techniques described in literature.

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Lipoma rarely involves parotid gland especially in children. An 11-year-old boy presented with right parotid swelling. Preoperative workup including CT scan and FNAC gave suspicion of parotid gland lipoma. The diagnosis was confirmed on histopathology after complete excision of the mass.