Ileal atresia and total colonic hirschsprung disease in a 36‐week neonate: A case report

Atqiaee, Khashayar; Hiradfar, Mehran; Mashhadi, Mahdi Parvizi; Khanghah, Ali Samady

doi:10.1002/ccr3.7079

Cited by 1 publication

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References 13 publications

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“…Ileal atresia and Hirschsprung disease are relatively common causes of neonatal obstruction however concomitant occurrence of both is rare with few reported cases. 4,[9][10][11][12][13] While the exact etiology is unknown, it is believed that the vascular insult within the 6 th to 8 th week of life that is presumed to cause ileal atresia could also interrupt the caudal migration of neural crest cells/ganglion cells resulting in both total colonic and small intestinal aganglionosis distal to the site of atresia. 13 It's critical to recognize these cases because an unrecognized distal obstruction threatens any anastomosis made during the treatment of jejunal atresia.…”

Section: Discussionmentioning

confidence: 99%

Achieving Digestive Autonomy and Gastrointestinal Continuity in a Patient with Short Bowel Syndrome Secondary to Concomitant Jejunal Atresia and Small Intestinal Hirschsprung's Disease

Velasquez,

Xu,

Liu

et al. 2024

European J Pediatr Surg Rep.

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Introduction: Concomitant presentation of jejunal atresia and Hirschsprung disease is rare and places children at high risk for developing short bowel syndrome and parenteral nutrition dependence which can affect the feasibility/timing of pull-through. Case description: A patient was born with jejunal atresia with a delayed diagnosis of Hirschsprung disease. After several procedures and bowel resections, the patient was ultimately left with an end jejunostomy and long Hartman’s pouch with short bowel syndrome, dependent on parenteral nutrition. The patient initially presented to our institution at age 2 with failure to thrive secondary to an obstructed/dilated jejunostomy and mild enterocolitis of their defunctionalized segment. The patient subsequently underwent completion of subtotal colectomy and revision of jejunostomy utilizing a serial transverse enteroplasty to manage the dilated bowel and gain length. The patient was able to wean off parenteral nutrition and achieve nutritional autonomy and by age 5. Following which, the patient was able to undergo ileoanal pull-through. After pull-through, the patient was able to stool independently and has suffered no major complications to date. Conclusion: Serial transverse enteroplasty can be successfully utilized in patients with a history of Hirschsprung disease and jejunal atresia to achieve nutritional autonomy and ultimately, re-establish gastrointestinal continuity with pull-through.

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