Spontaneous rupture of an aneurysm of the right subclavian artery as a first presentation of Ehlers Danlos syndrome in a 15-year old boy

We report a case of a spontaneous rupture of a right subclavian aneurysm in a 15 year-old patient. This ruptured aneurysm was successfully treated in an endovascular manner by placing a covered stent-graft in the right subclavian artery via right brachial access. Subsequent work-up by skin biopsy an...

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Bibliographic Details
Published in:Acta chirurgica belgica 2013-09, Vol.113 (5), p.367-372
Main Authors: Verbert, A, Verbist, J, Peeters, P, Deferm, H, Haenen, L
Format: Article
Language:English
Subjects:
Adolescent
Aneurysm, Ruptured - diagnostic imaging
Aneurysm, Ruptured - etiology
Ehlers-Danlos Syndrome - complications
Ehlers-Danlos Syndrome - diagnosis
Ehlers-Danlos Syndrome - genetics
Humans
Male
Pneumothorax - complications
Rupture, Spontaneous
Subclavian Artery - diagnostic imaging
Tomography, X-Ray Computed
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Summary:We report a case of a spontaneous rupture of a right subclavian aneurysm in a 15 year-old patient. This ruptured aneurysm was successfully treated in an endovascular manner by placing a covered stent-graft in the right subclavian artery via right brachial access. Subsequent work-up by skin biopsy and fibroblast culture and by DNA-screening revealed the diagnosis of Ehlers Danlos type IV. Meanwhile, the patient developed twice a spontaneous pneumothorax, treated with thoracoscopic pleurodesis. This article provides a clear overview of the clinical and genetic characteristics of a case of Ehlers Danlos type IV and illustrates the importance of avoiding surgery in patients with connective tissue disease because of the high risk of perioperative complications.
ISSN:0001-5458
DOI:10.1080/00015458.2013.11680947