A case of acute thrombosis of an ulnar artery aneurysm in a patient with osteogenesis imperfecta tarda is described. The pathophysiology of osteogenesis imperfecta is reviewed and the possible association of defective collagen maturation and aneurysm formation is postulated. Most ulnar artery aneurysms or thromboses should be surgically resected and the artery repaired by microsurgical technique with primary anastomosis if possible or a venous interpositional graft if direct repair is not possible without tension.
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