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Updated: Sep 20, 2025
Murine Ileocolic Bowel Resection with Primary Anastomosis
Published on: October 29, 2014
Case Report: Jejunoileal Atresia With Persistent Poor Bowel Function Can Occur After Surgical Correction for
Bassam N AlBassam1,2, Ahmad A Al-Shammari1,2, Saleh A AlQahtani1,2
1Department of Pediatrics, College of Medicine, Imam Abdulrahman bin Faisal University, Dammam, Saudi Arabia.
Insights
Jejunoileal atresia (JIA) and Hirschsprung disease (HD) rarely coexist. This case highlights a neonate with both conditions, experiencing severe complications including sepsis and organ failure, underscoring the need for vigilance in managing persistent poor bowel function post-surgery.
Area of Science:
- Pediatric Surgery
- Neonatal Gastroenterology
- Clinical Case Reports
Background:
- Jejunoileal atresia (JIA) is a frequent cause of neonatal intestinal obstruction.
- Concomitant occurrence of JIA and Hirschsprung disease (HD) is exceptionally rare.
- HD involves colonic aganglionosis, leading to functional obstruction.
Observation:
- A male infant presented with JIA and subsequently diagnosed with HD.
- The patient underwent multiple laparotomies for JIA and feeding intolerance.
- He developed recurrent bacteremia, fungemia, hypoglycemia, central hypothyroidism, and multi-organ failure.
Findings:
- The rare co-occurrence of JIA and HD was observed.
- A proposed etiology involves an intrauterine vascular accident affecting bowel and neural crest cell migration.
- Unique associations included ileal atresia with colonic aganglionosis, central hypothyroidism, and persistent bacteremia.
Implications:
- Persistent poor bowel function after surgical correction of concomitant HD in JIA cases warrants careful consideration.
- This case expands the understanding of rare congenital gastrointestinal anomalies and their complex management.
- Highlights the potential for severe systemic complications in neonates with combined JIA and HD.
Abstract:
Jejunoileal atresia (JIA) is one of the common etiologies of intestinal obtrusion in neonates. However, cases of concomitant ileal atresia and Hirschsprung disease (HD) rarely occur. We report the case of a male infant who had JIA concomitantly with HD that was re-anastomosed. The patient underwent an exploratory laparotomy to resect the dilated terminal ileum. Subsequently, owing to a significantly dilated proximal bowel, he underwent a second exploratory laparotomy. However, he continued to have feeding intolerance postoperatively. He had colonic aganglionosis and was diagnosed with HD. A third laparotomy was then performed. Additionally, he had recurrent episodes of gram-negative bacteremia, especially candida parapsilosis fungemia, despite receiving antibiotics and antifungal, and there were no identifiable underlying genetic or immunological causes. Finally, the patient had recurrent episodes of hypoglycemia, central hypothyroidism, and multiple organ failure and died at the age of 7 months. The concomitant ileal atresia and HD was thought to be due to a common intrauterine vascular accident, together with loss of bowel, thereby acting as a barrier for the caudal migration of neuromeric cells and leading to colonic aganglionosis. In this case, ileal atresia was associated with colonic aganglionosis, central hypothyroidism, and persistent bacteremia, which is a unique finding. In cases of JIA, persistent poor bowel function after surgical correction of concomitant HD should be considered.
