Colonic atresia and hirschsprung disease: a case report and review of the literature.


Journal

Journal of medical case reports
ISSN: 1752-1947
Titre abrégé: J Med Case Rep
Pays: England
ID NLM: 101293382

Informations de publication

Date de publication:
07 Jun 2023
Historique:
received: 28 08 2022
accepted: 03 05 2023
medline: 8 6 2023
pubmed: 7 6 2023
entrez: 6 6 2023
Statut: epublish

Résumé

Colon atresia is one of the rarest congenital anomalies of the gastrointestinal tract, with an incident range of between 1 in 10,000 and 66,000 live births. Type I colonic atresia affects only the mucosal layer of the intestine and spares the intestinal wall and mesentery. Hirschsprung Disease is a rare association of Colon atresia and is usually diagnosed as a complication of atresia treatment. This study reports a 14-h term white middle-eastern female infant with type I transverse colonic atresia complicated by the association of Hirschsprung disease and provides a brief literature review of the topic. She presented with poor feeding, weakness, and failure to pass meconium, and her abdominal X-ray showed complete distal bowel obstruction. The presence of Hirschsprung disease was realized after complications of atresia surgery. The infant underwent a total of three surgeries involving an end-to-end anastomosis of the atresia, colostomy formation following anastomosis leakage, and Hirschsprung surgery. The patient ultimately expired. The association between colonic atresia and Hirschsprung disease poses a diagnostic and therapeutic challenge. Considering Hirschsprung disease as a possible association in colon atresia patients can facilitate proper decision-making in the course of treating colon atresia cases and achieving better outcomes.

Sections du résumé

BACKGROUND BACKGROUND
Colon atresia is one of the rarest congenital anomalies of the gastrointestinal tract, with an incident range of between 1 in 10,000 and 66,000 live births. Type I colonic atresia affects only the mucosal layer of the intestine and spares the intestinal wall and mesentery. Hirschsprung Disease is a rare association of Colon atresia and is usually diagnosed as a complication of atresia treatment.
CASE PRESENTATION METHODS
This study reports a 14-h term white middle-eastern female infant with type I transverse colonic atresia complicated by the association of Hirschsprung disease and provides a brief literature review of the topic. She presented with poor feeding, weakness, and failure to pass meconium, and her abdominal X-ray showed complete distal bowel obstruction. The presence of Hirschsprung disease was realized after complications of atresia surgery. The infant underwent a total of three surgeries involving an end-to-end anastomosis of the atresia, colostomy formation following anastomosis leakage, and Hirschsprung surgery. The patient ultimately expired.
CONCLUSIONS CONCLUSIONS
The association between colonic atresia and Hirschsprung disease poses a diagnostic and therapeutic challenge. Considering Hirschsprung disease as a possible association in colon atresia patients can facilitate proper decision-making in the course of treating colon atresia cases and achieving better outcomes.

Identifiants

pubmed: 37280703
doi: 10.1186/s13256-023-03969-z
pii: 10.1186/s13256-023-03969-z
pmc: PMC10246402
doi:

Types de publication

Review Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

233

Informations de copyright

© 2023. The Author(s).

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Auteurs

Amirhossein Ladan (A)

Department of Surgery, Ayatollah Mousavi Hospital, Zanjan University of Medical Sciences, Zanjan, Iran.

Reza Mahdian Jouybari (R)

Department of Pediatrics, Ayatollah Mousavi Hospital, Zanjan University of Medical Sciences, Zanjan, Iran.

Mahnaz Zareh Akbari (M)

Department of Pediatrics, Ayatollah Mousavi Hospital, Zanjan University of Medical Sciences, Zanjan, Iran. 507akbari@gmail.com.

Pegah Moharrami Yeganeh (P)

School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran. P.yeganeh@zums.ac.ir.

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