Chronic Intestinal Pseudo-obstruction Due to Adult-onset Acquired Isolated Hypoganglionosis with Muscular Atrophy in the Small Intestine: a Case Report and Review of Literature

Overview

Journal Clin J Gastroenterol

Specialty Gastroenterology

Date 2024 Jan 9

PMID 38193986

Authors

Sayoko Tayama

Yoki Furuta

Miyuki Morito

Hideaki Naoe

Daiki Yoshii

Yasuyuki Uchida

Koichiro Yoshimaru

Yuji Miyamoto

Tomoaki Taguchi

Yasuhito Tanaka

Affiliations

Soon will be listed here.

Abstract

Background: Chronic intestinal pseudo-obstruction (CIPO) is a rare intestinal disorder characterized by impaired propulsion of the digestive tract and associated with symptoms of intestinal obstruction, despite the absence of obstructive lesions. CIPO includes several diseases. However, definitive diagnosis of its etiology is difficult only with symptoms or imaging findings.

Case Presentation: A 56-year-old man was referred to our hospital due to a 3-year history of continuous abdominal distention. Imaging, including computed tomography of the abdomen, and endoscopy revealed marked dilatation of the entire small intestine without any obstruction point. Therefore, he was diagnosed with CIPO. Since medical therapy didn't improve his symptoms, enterostomy and percutaneous endoscopic gastro-jejunostomy were performed. These procedures improved abdominal symptoms. However, he required home central venous nutrition due to dehydration. The pathological findings of full-thickness biopsies of the small intestine taken during surgery revealed decreased number and degeneration of ganglion cells in the normal plexus. These findings led to a final diagnosis of CIPO due to acquired isolated hypoganglionosis (AIHG).

Conclusions: Here, we report the case of a patient with CIPO secondary to adult-onset AIHG of the small intestine. Since AIHG cannot be solely diagnosed using clinical findings, biopsy is important for its diagnosis.

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