not clear . Spontaneous bowel perforation is a well-known complication of patients with Type IV Ehlers-Danlos syndrome but is not a known complication of OI . 4 There is one additional documented case in which an adult with OI was found to have an absence of muscularis propria at the site of spontaneous colonic perforation . 5 A mechanism by which OI would cause a SAIM is unknown . 6 , 7
CONCLUSION
Our case highlights an exceedingly rare pathology , and to our knowledge , there are no other reported cases of SAIM resulting in spontaneous bowel perforation twice in the same adult patient . Furthermore , the familial presence of an underlying connective tissue disorder provides a unique opportunity to gain insight into the etiology of this poorly understood phenomenon .
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