Isolated adult hypoganglionosis resulting in toxic megacolon: A Case Report

Kahramanca, Şahin; Özgehan, Gülay; Celep, Bahadır; Şeker, Gaye; Arıkök, Ata Türker; Küçükpınar, Tevfik

doi:10.5505/kjms.2014.28291

Cited by 1 publication

(2 citation statements)

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“…The other disorders are exceptionally rare, therefore when they are observed in a clinical setting, evidence should be taken into account and expert views should be sought [ 11 ]. Hypoganglionosis can be differentiated by the reduced number and size of myenteric ganglion cells in the “intramuscular layers” (Auerbach plexus), low or absent AChE activity in the lamina propria, and hypertrophy of the muscularis mucosa of the intestinal wall [ 3 , 9 ]. The number of myenteric ganglia should be less than one and a half per 10 mm or fewer than two neurons per ganglion of colon tissue [ 9 , 10 ].…”

Section: Discussionmentioning

confidence: 99%

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

AlSulimani¹,

Haoues²,

Aljuhani³

et al. 2023

Cureus

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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.

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Section: Discussionmentioning

confidence: 99%

“…Differentiating between the types is difficult as they have the same clinical features: constipation or pseudo-obstruction [ 1 , 2 ]. For this reason, the differentiation is based on immunohistochemical examination and histological and electron microscopy findings through full-thickness biopsies for bowel segments [ 3 ].…”

Section: Introductionmentioning

confidence: 99%