Sigmoid Volvulus as a Clinical Manifestation of Acquired Colonic Hypoganglionosis: A Case Report

A reduced and limited number of myenteric ganglia and low acetylcholinesterase activity in the lamina propria characterizes an unusual form of intestinal innervation disorder known as acquired or adult-onset hypoganglionosis. Only a few cases have been observed in adults, while the majority are diag...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2023-01, Vol.15 (1), p.e33950
Main Authors: Alsulimani, Siraj, Haoues, Noomen, Aljuhani, Arwa M, Fayoumi, Noor, Al-Sawat, Abdullah
Format: Article
Language:English
Subjects:
Abdomen
Biopsy
Case reports
Colon
Congenital diseases
Constipation
Disease
Females
Gastroenterology
General Surgery
Histopathology
Intestinal obstruction
Laparoscopy
Ostomy
Pathology
Patients
Surgery
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Summary:A reduced and limited number of myenteric ganglia and low acetylcholinesterase activity in the lamina propria characterizes an unusual form of intestinal innervation disorder known as acquired or adult-onset hypoganglionosis. Only a few cases have been observed in adults, while the majority are diagnosed in infancy or youth. We report a rare case of colonic hypoganglionosis that presented as sigmoid volvulus in a 25-year-old female brought in to the ER. She underwent endoscopic decompression but developed a massive sigmoid volvulus with proximal colon dilatation. It was decided to do a total colectomy with an end ileostomy. The diagnosis was confirmed by histopathology, which revealed acquired hypoganglionosis. In order to prevent delayed or emergency presentation and the development of a stoma, the diagnosis of hypoganglionosis must be confirmed with full-thickness biopsies from all affected parts of the large bowel. Hypoganglionosis is rarely present, so young patients with a history of chronic constipation must be treated with a high index of suspicion.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.33950