Progressive Systemic Sclerosis Resulting in Megacolon

Shamberger, Robert C.

doi:10.1001/jama.1983.03340080041025

Cited by 12 publications

(2 citation statements)

References 4 publications

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“…Thus, it has been postulated that disruption of the connective tissue network in the muscularis propria results in impairment of the coordinated movements of the longitudinal and circular muscle layers [26,27]. Indeed, megacolon has been observed in patients with certain systemic connective tissue disorders, including Ehlers-Danlos Syndrome [EDS [28-31]], amyloidosis [32] and scleroderma [33,34]. …”

Section: Discussionmentioning

confidence: 99%

Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults

et al. 2015

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Background Chronic megacolon is a rare disease of the colonic motor function characterized by a permanent increase in colonic diameter. Methods We reviewed electronic medical records of all patients diagnosed with chronic megacolon from 1999 to 2014 at Mayo Clinic. Our aim was to summarize clinical and motility features, including colonic compliance and tone measured by colonic barostat-controlled 10cm long infinitely compliant balloon. Colonic compliance curves were compared to healthy (40) and disease (47) control groups. Results Among 24 identified patients, the mean maximal colonic diameter on abdominal radiograph was 12.7±0.8cm. The cause of megacolon was idiopathic in 16/24, and secondary in 8/24. A relatively high prevalence (10/24) of comorbid pelvic floor dyssynergia was identified. At the time of this report, 16 patients had undergone colectomy. In general, megacolon presented high fasting colonic volume at relatively low pressures (16-20mmHg), suggesting high colonic compliance; similarly, volumes at operating pressures that ensured apposition of the balloon to the colonic wall suggested low colonic tone. Median balloon volume at 44mmHg distension was 584mL (IQR 556.5-600) in patients with megacolon compared to 251mL (212-281) in healthy, 240mL (207-286) in functional constipation and 241mL (210.8-277.5) in diarrhea-predominant irritable bowel syndrome controls. Colon's tonic response to feeding was generally intact, and there was frequently maintained phasic contractile response to feeding. Conclusions Chronic megacolon is a severe colonic dysmotility, manifesting radiologically with increased colonic diameter; it can be proven by measuring colonic compliance, and typically requires colectomy because of failed medical therapy.

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Section: Discussionmentioning

confidence: 99%

Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults

et al. 2015

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“…If enemas or manual disimpaction are ineffective, then neostigmine, a cholinesterase inhibitor that promotes colonic motility, can be safe and effective for treatment (65). Rarely, colonic pseudo-obstruction can progress to megacolon requiring urgent surgical resection to prevent perforation (66). Elective segmental surgical resection may be a good option for colon inertia related to segmental involvement, particularly when small intestinal motility and anorectal function are relatively normal (67).…”

Section: Colon and Anorectummentioning

confidence: 99%

Gastrointestinal manifestations of systemic sclerosis

Shreiner

Murray

Denton

et al. 2016

Journal of Scleroderma and Related Disorders

131

106

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In patients with systemic sclerosis (SSc), gastrointestinal (GI) tract involvement is almost universal. Any segment of the GI tract from mouth to anus can be involved, and GI symptoms are a frequent cause of morbidity. In severe cases, GI tract involvement can progress to the point of malnutrition requiring parenteral nutrition. GI tract involvement in SSc contributes to disease-related mortality although mostly as a co-morbidity rather than direct cause of death. The review is intended to help address challenges in the assessment and treatment of GI tract involvement in SSc.

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