Jl. Iglesias et T. Renard, DIAPHRAGMATIC-HERNIA IN AN 8-YEAR-OLD WITH EHLERS-DANLOS-SYNDROME, Pediatric surgery international, 13(8), 1998, pp. 553-555
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.