Ruptured dissecting aneurysm in bilateral iliac arteries caused by Ehlers-Danlos syndrome type IV: report of a case

Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characte...

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Bibliographic Details
Published in:Surgery today (Tokyo, Japan) Japan), 2001-01, Vol.31 (1), p.85-89
Main Authors: Imamura, A, Nakamoto, H, Inoue, T, Yamada, H, Okuno, M, Takai, S, Komada, H, Kwon, A H, Kamiyama, Y
Format: Article
Language:English
Subjects:
Adult
Aneurysm, Dissecting - etiology
Aneurysm, Dissecting - surgery
Aneurysm, Ruptured - etiology
Aneurysm, Ruptured - surgery
Biopsy
Blood Vessel Prosthesis Implantation
Diagnosis, Differential
Ehlers-Danlos Syndrome - complications
Ehlers-Danlos Syndrome - diagnosis
Humans
Iliac Aneurysm - etiology
Iliac Aneurysm - surgery
Male
Skin - pathology
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Summary:Ehlers-Danlos syndrome (EDS) is an inherited disorder of connective tissue characterized by hyperextensible skin, hypermobile joints, and abnormalities of the cardiovascular system. Ten types and several subtypes of EDS have so far been recognized based on genetic, clinical, and biochemical characteristics. The spectrum of the disorder varies from mild to life-threatening vascular complications. EDS type IV is a particularly dangerous form with a lethal spontaneous rupture of the major arteries and aneurysmal formation. We present herein a case of a ruptured dissecting aneurysm in the bilateral iliac arteries caused by EDS type IV. A previously healthy 33-year-old man without any physical features of this connective tissue disorder experienced a metachronous vascular rupture two times. Successful synthetic bypass grafting was performed with great difficulty. The diagnosis of EDS type IV was made afterwards based on an electrophoresis analysis of a skin biopsy specimen which revealed a lack of type III collagen. Surgical intervention in cases of arterial complications in EDS type IV patients have been reported to be both difficult and frequently unsuccessful. The early clinical recognition of this syndrome is therefore of great importance due to the hazards of such surgical therapies.
ISSN:0941-1291
1436-2813
DOI:10.1007/s005950170228