Cloacal Exstrophy with Normal Penis and Urethra - A Rare Variant.

Classically, cloacal exstrophy presents with omphalocele, bladder exstrophy, imperforate anus, and spinal defects. We report a rare variant of cloacal exstrophy in a 6-day-old male with an intact lower abdominal wall, normal penis, and urethra. Only two such cases have been reported in the literature.

Distal duodenal web with malrotation: An unusual finding.

The most common site of the congenital duodenal web is the second part. Web distal to the second part of the duodenum is rare. It mimics the windsock deformity. Diagnosis may be missed if accompanying malrotation is present. We hereby report two cases of distal duodenal webs associated with malrotation and challenges in their diagnosis and management.

Fetus-in-Fetu: A Tale of the Trapped Triplets.

A 7-day-old male child presented with abdominal distention and jaundice. Radiological investigations revealed an encapsulated sac encasing three fetus-in-fetu (FIF) in the retroperitoneum. Laparotomy revealed a sac occupying almost the whole of the abdomen. The sac was stretching the duodenum and barely visible common bile duct, which were carefully separated. The rest of the bowel was displaced to the left. The sac containing three FIFs was excised intact. One of the fetuses was highly differentiated and had thoracic meningomyelocele, which has never been reported in FIF.

Rapunzel Syndrome: A Concealed Tale of the Misleading Tail.

The diagnosis of Rapunzel syndrome can sometimes be challenging. The silent features such as poor appetite, constipation, anemia, hypoalbuminemia, and hypoproteinemia were all overlooked in our patient initially. The Rapunzel tail leads to multiple intussusceptions and an unexpected perforation sealed by intussusceptions. Perforation sealed by intussusceptions in a clinically stable patient of Rapunzel syndrome has never been reported.

Pediatric Appendicitis Score or Ultrasonography? In Search of a Better Diagnostic Tool in Indian Children with Lower Abdominal Pain.

OBJECTIVES: To prospectively assess the performance of pediatric appendicitis score (PAS) in diagnosing acute appendicitis in the children with lower abdominal pain and correlated with ultrasound findings; and to assess the impact of the PAS on clinical outcome and its efficacy in differentiating between complicated and uncomplicated appendicitis. METHODS: A prospective study was done which included cases of lower abdominal pain. Appendectomy was done for PAS ≥ 6, and diagnosis was confirmed on histopathology. A receiver operator characteristic (ROC) curve was created to assess the PAS performance. The sensitivity, specificity, and accuracy of ultrasonography in diagnosing appendicitis were assessed, and analysis of agreement between ultrasonography and PAS score was done by kappa statistics. RESULTS: Of 260 cases with lower abdominal pain, 205 were suspected of having appendicitis. One hundred fifty-nine had PAS ≥ 6. There were 2/159 (1.26%) cases of negative appendectomies and 2/46 (4.34%) cases of missed appendicitis. The mean PAS was significantly higher in patients with appendicitis than in those without appendicitis. The area under the ROC curve was 0.9925. Sensitivity, specificity, and positive and negative predictive value of PAS were 98.74%, 95.65%, 95.7% and 95.65%, respectively. Complicated appendicitis had significantly more PAS, fever, and cough tenderness than uncomplicated appendicitis. The sensitivity and specificity of ultrasonography were 86.79% and 17.39%, respectively. Agreement between ultrasonography-proven appendicitis and PAS-dependent appendicitis was weak. CONCLUSION: PAS has high efficacy in diagnosing acute appendicitis. Clinical outcome was more favorable with the use of PAS. Ultrasonography should be used judiciously and in combination with clinical judgment.

Congenital Segmental Dilatation of Ascending Colon with Distal Microcolon: A Diagnostic Dilemma.

A 5-day-old male presented with bilious vomiting, a grossly distended abdomen, and passage of a small amount of stool. The anal opening was at a normal position. X-ray abdomen showed a large bowel loop with a single air-fluid level occupying more than half of the abdominal width. On laparotomy, the ascending colon was dilated to form a pouch-like structure, and the ileum and appendix were opening into it. Colon distal to pouch was present as microcolon. Histopathology of the dilated segment was suggestive of congenital segmental dilatation (CSD). In CSD, the distal bowel is of normal caliber. This is a rare case of CSD of ascending colon with distal microcolon.

Evaluation of Interstitial Cells of Cajal-Like Cells at the Pelviureteric Junction in Children with Pelviureteric Junction Obstruction: A Prospective Study and Review of Literature.

Introduction: The etiopathogenesis of pelviureteric junction obstruction (PUJO) has been debated. Recently, the role of interstitial cells of Cajal-like cells (ICC-LC)has been studied and reported to be the cause of this functional obstruction. We studied the histopathology and ICC-LC density at PUJ and compared it with that of PUJ of the control group and distal ureteric margin of the study group. Methods: A prospective study was conducted which included PUJO patients in the study group and the renal tumor patients in the control group. Histopathological examination (muscle hypertrophy and fibrosis) and immunohistochemistry (ICC-LC density) were done. The muscle hypertrophy, fibrosis, and ICC-LC density at the PUJ in both the groups were compared. A similar comparison was performed between the findings at the PUJ and the distal margin in the study group. Results: The study and control groups included 37 PUJO patients and 13 Wilms tumor patients. The ICC-LC density at PUJ in the study group was significantly lower than that in the control group (P < 0.001) and that at the distal resected margin of the study group (P < 0.001). Significantly increased muscle hypertrophy (P < 0.001) and fibrosis (P = 0.002) were seen at PUJ in the study group compared to the control group. No significant association was noted between the ICC-LC density and muscle hypertrophy at PUJ and the distal resected margin in the study group. Conclusion: A significant decrease in the density of ICC-LC and increased fibrosis and muscle hypertrophy at PUJ in children with PUJO play a role in the etiopathogenesis of the disease.

Pediatric Neuroblastoma - Impact of Nutritional Status on Complications and Outcomes.

Aim: The aim is to study the impact of nutritional status on outcomes and treatment-related complications in patients of neuroblastoma. Materials and Methods: Anthropometry (height, weight, weight for height [in <5 years], mid-arm circumference [MAC] [in <5 years], and body mass index [BMI]), hemoglobin level, and serum albumin level were recorded. Results: Twenty-four neuroblastoma patients, 16 males and 8 females, with a mean age of 42.7 months were treated and followed up over a period of 0.03-170 months. Of 24 patients, 16 survived (complete response - 9, partial response - 2, no response - 2, progressive disease - 3). Height <2 standard deviation [SD] was seen in 12.5% (3/24). Mortality and complications were found in 66.7% (2/3) and 100% (3/3), respectively. Weight <2SD was seen in 37.5% (9/24). Mortality and complications were found in 33.3% (3/9) and 67% (6/9), respectively. Weight for height <2 SD was seen in 35% (7/20). Mortality and complications were found in 42.9% (3/7) and 71.4% (5/7), respectively. MAC <2 SD was seen in 15.8% (3/19). Mortality and complications were found in 66.7% (2/3) and 66.7% (2/3). BMI <2SD was seen in 29.2% (7/24). Mortality and complications were found in 42.8% (3/7) and 28.6% (2/7). Anemia was seen in 62.5% (15/24), of which nine survived. Complications were seen in 58.3% (14/24) (P = 0.001). Hypoproteinemia was seen in 20.8% (5/24). Mortality and complications were 40% (2/5) and 80% (4/5). Conclusion: Neuroblastoma has poor outcome in Indian children, and there does not appear to be a major role played by nutrition in determining the response to treatment and survival.

Delayed Presentation of Jejunal Atresia with Multiple Enteric Fistula: A Case Report and Review of Literature.

A 6-month-old boy presented with features of intestinal obstruction. Laparotomy revealed Type IIIa jejunal atresia. The proximal and distal bowel loops were in continuity through multiple fistulae between the adjoining bowel loops. To the best of our knowledge, it is the first report of congenital Type IIIa intestinal atresia surviving beyond the neonatal age without surgery.

Fogarty Catheter: An Indispensable Tool to Complement the Ingenuity of the Endoscopist for Extraction of Airway "Foreign Body with a Hole".

Introduction: The extraction of smooth spherical objects is challenging as they are difficult to grasp within the jaws of the forceps and tend to slip distally. Objective: The authors herein have shared their experience with the use of a Fogarty catheter (FC) for safe extraction of smooth and spherical "foreign body (FB) with a hole." Materials and Methods: Report on pediatric cases (n = 4) of airway "FB with a hole" wherein the FC was used for their extraction. Mean age was 27.5 months (range: 17 months-39 months). The male: female ratio was 3:1. The technique of FB extraction with a FC has been described, including the principle of the technique, indications, and contra-indications, technical problems and troubleshooting. Results: The FB spectrum included a necklace bead (n = 2), nonnecklace bead (n = 1) and a fragmented end-piece of the housing of a ball-pen (n = 1). The locations of the FBs were right main bronchus (n = 1), secondary bronchus on the right (n = 1), and in the left main bronchus (n = 2). Successful removal of FB with use of FC during rigid broncoscopy was possible. The bead had to be rotated in n = 2 patients to align the hole with the FC. Problems associated with threading the hole and disimpaction of the FB have been highlighted. No complications were observed. The advantages and limitation of the technique have been discussed. Conclusions: The use of FC with the described technique offers a safe, effective and reproducible method for removal of airway "FB with a hole" in a controlled environment while minimizing the possibility of iatrogenic injury to the wall of the surrounding airways.

Cystic biliary atresia with congenital absence of portal vein.

A 126-day female child presented with jaundice since day 7 of life. She was icteric and had hepatosplenomegaly. Radiological investigations revealed three extrahepatic and multiple intrahepatic biliary cysts, absent gall bladder and portal vein. These findings were confirmed on laparotomy. To the best of our knowledge, this is the first report of cystic biliary atresia associated with congenital absence of portal vein.

Congenital colonic stenosis with absent caecum and appendix: a rare association.

Congenital colonic stenosis is a rare condition with less than 20 cases reported in the literature since 1966. We report an interesting case of a 7-month-old baby girl who presented with features suggestive of acute intestinal obstruction. On exploration, it was a case of ascending colon stenosis with absence of caecum and appendix. A double barrel ileocolostomy was performed. The histopathology confirmed the diagnosis of colonic stenosis and ruled out the presence of Hirschsprung's disease in the distal colon. The child underwent second stage surgery (stoma closure) after 9 months. In complex cases of congenital colonic stenosis, an early decompressive surgery followed by a delayed second stage closure is recommended in patients with poor general condition.