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.

A rare case of spontaneous splenic rupture complicated by hemophilia A.

Splenic lymphangiomas are benign neoplasms resulting from congenital malformations of lymphatic channels manifesting as cystic lesions, occurring mostly in childhood. This process usually involves additional sites in a diffuse or multifocal fashion but although rare, can also present isolated to the spleen. The clinical picture varies from asymptomatic identified incidentally to nonspecific symptoms from compression of adjacent organs. Spontaneous rupture of these lesions can lead to hemoperitoneum, acute abdomen and hemorrhagic shock. We present the case of a 33-year-old male who required urgent exploration and splenectomy secondary to ruptured splenic lymphangioma, complicated by postoperative bleeding, re-exploration and blood transfusion from unknown Hemophilia A. Overall, it is important to be cognizant of this condition in the setting of left upper quadrant pain, even in an adult, as any delay in diagnosis or treatment can lead to life-threatening complication.