br Case Report A day old male child weighing

Case Report A 5-day-old male child, weighing 2.5 kg, was admitted in the emergency department with complaints of an inability to pass meconium, severe abdominal distension, and bilious vomiting. The child had passed meconium once within 24 hours of life. On examination, the heart rate was 140 beats/min, respiratory rate was 48 breaths/min, and the capillary refill time was < 3 seconds with a severely distended abdomen and palpable bowel loops. Hemogram and blood chemistry were within normal limits, and an X-ray of the abdomen revealed multiple air fluid levels suggesting distal intestinal obstruction. After informed consent was obtained, the patient was prepared for exploratory laparotomy with a right supra-umbilical transverse incision, which revealed a 3-cm-long narrow segment of the rectum proximal to the peritoneal reflection, followed by a zone of transition and a proximal largely distended intestine. A provisional diagnosis of HD was made. Seromuscular biopsy was obtained from the collapsed rectum, and right transverse colostomy was performed. Postoperative recovery was uneventful and the patient was discharged on Postoperative Day 8, with advice to follow-up in the pediatric surgery outpatient department. The patient was lost to follow-up and again attended the outpatient department 6 years after the previous procedure. Barium enema study was performed, which was normal except for a slight narrowing at the sigmoid colon level (Figure 1). A review of a previous histopathological examination report showed the presence of ganglion csf-1r and absent hypertrophic nerve fibers in the provided specimen (Figure 2). A decision to close the stoma was made. In the postoperative period, the patient developed abdominal distension and did not pass stools but was passing flatus. We suspected impacted fecaloma in the rectosigmoid region; colonoscopy was performed, which revealed a diaphragm obstructing the lumen. The patient was again examined; the finding simulated a transition zone with a collapsed distal and dilated proximal segment (Figure 3). Colon was opened longitudinally at the transition zone and type I sigmoid atresia (windsock-like deformity) was found (Figure 4).
Discussion There are three anatomical types of CA; the least frequent is type I, which is characterized by a mucosal diaphragm completely occupying the lumen without seromuscular interruption. These webs can have a small opening, thus preventing complete obstruction and often resulting in delayed diagnosis. HD is recognized in at least 2% of patients with CA. A study hypothesized that when a vascular insult occurs before the retroperitoneal fixation of the colon at 11 weeks of gestation, caudal migration of the myenteric nerve is interrupted. Therefore, it is imperative to rule out HD in every patient with CA. Approximately 50–90% of children with HD present during the neonatal period with abdominal distension, bilious vomiting, and feeding intolerance. Plain abdominal radiographs generally show dilated bowel loops and the air fluid level throughout the abdomen. A differential diagnosis includes intestinal atresia, meconium ileus, meconium plug syndrome, and other less common conditions such as colonic atresia. A water-soluble contrast enema is generally administered as a first step in the diagnostic process. The salient findings on a contrast enema is the narrowing of a variable length of the distal bowel that has an irregular mucosal outline and involves the rectum and distal sigmoid colon with the reversal of the recto-sigmoid index and a funnel-like transition into a dilated proximal sigmoid and descending colon, and there is a retention of contrast in the colon on the 24-hour postevacuation film. A diagnosis of CA is straightforward when facilitated by a water-soluble contrast enema, and preoperative contrast is recommended in neonates with distal intestinal obstruction. Early operative management is undertaken because of the risk of perforation and volvulus. Because of the association with HD, suction rectal biopsy for evaluating ganglionic cells should be performed in the operating room if primary anastomosis is planned. Similarly, if colostomy is appropriate, the myenteric plexus at the colostomy site should be examined during operation, and suction rectal biopsy should be performed before repair.