The journal of the Japanese Practical Surgeon Society
Online ISSN : 2189-2075
Print ISSN : 0386-9776
ISSN-L : 0386-9776
A CASE OF COLONIC RUPTURE WITH EHLERS-DANLOS SYSNDROME
Hideki KITOZensuke YANAGIKenzo ASADAKanji TOKURAJun TAKEBAYASHIIsao TANAKA
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1990 Volume 51 Issue 7 Pages 1519-1526

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Abstract

A case of spontaneous rupture of the sigmoid colon with Ehlers-Danlos syndrome in a 60-year-old woman is reported, whose life could be successfully saved. The patient was admitted with sever lower abdominal pain. On laparotomy a 12cm perforation was found on the antimesenteric side of the sigmoid colon, and Hartmann's operation was performed. She was diagnosed as Ehlers-Danlos syndrome by clinical signs and histopathological findings of the skin, but immunofluorescence with anti-collagen antibody showed that the distribution pattern of type I, III, IV and V collagen in the skin was normal. So, it seems that further examination of cultured skin fibroblasts from the patient is necessary.
In the domestic literature, this is the first case of colonic perforation With Ehlers-Danlos syndrome, in which patient's life could be saved. For better management of this syndrome, it is important to avoid an increase in intraintestinal pressure. Moreover, primary anastomosis and subsequent closure of the colostomy should be avoided for the colonic perforation associated with this syndrome.

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© Japan Surgical Association
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