Congenital Hernia of the Umbilical Cord associated with type IIIa Ileal Atresia

A 2-day-old, term male neonate, weighing 2.1 kg, 1st in birth order was born by normal delivery. He presented to our department with a small fleshy swelling at the base of the umbilicus, bilious vomiting and epigastric fullness. Antenatal ultrasounds were not done. On examination, the baby was hemodynamically stable, pulse rate150/min and respiratory rate-50/min. anicteric and mildly dehydrated. Abdominal examination revealed hernia of umbilical cord. There was mild epigastric distension, soft on palpation and absent bowel sounds; nasogastric aspirate was bilious. Laboratory tests were normal except for hypocalcaemia (serum calcium-8 mg/dl) and raised C-reactive protein. Upright abdominal radiograph showed dilated small bowel loops with multiple airfluid levels with paucity of distal air; there were multiple defects involving the lumbar vertebra (Fig.1). Laparotomy revealed proximal ileal atresia, type IIIa. The proximal part of distal unused ileum was herniating into the umbilical cord, ending blindly and stuck inside the sac of hernia of umbilical cord (Fig.1). It was dissected and freed; end to back ileoileal anastomosis was performed after excision of 10-12cms of dilated loop and confirming distal bowel patency. Post-operative recovery was uneventful and the child is doing well at follow-up. Figure 1: Upright abdominal radiograph showing dilated small bowel loops, multiple air-fluid levels with absence of air distally (A); Intra-operative picture showing type IIIa ileal atresia with distal unused ileum is seen herniating into the umbilical cord (B); hernia of umbilical cord (blue arrow) is seen along with proximal part of distal ileum after its dissection from inside the sac (C).