DIAPHRAGMATIC-HERNIA IN AN 8-YEAR-OLD WITH EHLERS-DANLOS-SYNDROME

Ehlers-Danlos syndrome (EDS) includes a group of connective-tissue dis orders characterized by abnormal collagen metabolism. The diverse spec trum of this disease and its complications present a distinct challeng e to the surgeon. Patients with this hereditary disorder may have hype relastic or fragile skin, poor wound healing, hypermobile joints, clot ting abnormalities, spontaneous pneumothoraces, recurrent hernias, bow el 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.