Spontaneous small bowel perforation: a rare GI manifestation of type IV Ehlers-Danlos syndrome

Isolated small bowel perforation is low in the differential diagnosis of abdominal pain in the young, relatively healthy patient. It is, however, a rare manifestation of type IV (vascular) Ehlers-Danlos syndrome (EDS). In addition, there is no general consensus on the management of GI manifestations in patients with type IV EDS. We present the case of a 31-year-old male with history of type IV EDS, presenting with acute onset abdominal pain. Imaging was notable for intra-abdominal free air and thickened loops of small bowel in the pelvis. The patient underwent exploratory laparotomy with resection of the small bowel perforation with enteroenteric anastomosis. In our literature review, we evaluated gastrointestinal manifestations observed in patients with type IV EDS, management recommendations and potential complications to be mindful of in this population.