Diaphragmatic hernia in an 8-year-old with Ehlers-Danlos syndrome

Abstract

Ehlers-Danlos syndrome (EDS) includes a group of connective-tissue disorders characterized by abnormal collagen metabolism. The diverse spectrum of this disease and its complications present a distinct challenge to the surgeon. Patients with this hereditary disorder may have hyperelastic or fragile skin, poor wound healing, hypermobile joints, clotting abnormalities, spontaneous pneumothoraces, recurrent hernias, bowel perforation, and vascular complications. An 8-year-old female with EDS type VII and a newly diagnosed diaphragmatic hernia is presented. Surgical considerations of this syndrome are reviewed.