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Type IV Ehlers-Danlos syndrome with isolated arterial involvement

  • Papers From The French Vascular Surgery Society 1988 Meeting
  • Published:
Annals of Vascular Surgery

Abstract

Ehlers-Danlos syndrome is a connective tissue disorder characterized by skin hyperelasticity and friability, hyperextensible small articulations, vascular fragility, and ocular and visceral symptoms. Ten types of Ehlers-Danlos syndrome have been described to date. Type IV is characterized by isolated arterial involvement with the risk of rupture, making diagnosis difficult. A 25-year-old man was operated on for a ruptured left and then right iliac artery and once again for a hepatic artery aneurysm within a nine month period. The diagnosis of Ehlers-Danlos syndrome was based on clinical and histopathologic findings. An asymptomatic iliac artery aneurysm was found in the patient's 23-year-old brother.

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Authors and Affiliations

  1. From the Service de Chirurgie Thoracique et Vasculaire and Service d'Anatomo-Pathologie, Centre Hospitalier et Universitaire de Clermont-Ferrand, France

    François Bellenot MD, Stephane Boisgard MD, Bernadette Kantelip MD, Philippe Maillard MD, Philippe Tissandier MD, Jean-Pierre Ribal MD & Gérard Glanddier MD

Authors
  1. François Bellenot MD
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  2. Stephane Boisgard MD
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  3. Bernadette Kantelip MD
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  4. Philippe Maillard MD
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  5. Philippe Tissandier MD
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  6. Jean-Pierre Ribal MD
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  7. Gérard Glanddier MD
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Cite this article

Bellenot, F., Boisgard, S., Kantelip, B. et al. Type IV Ehlers-Danlos syndrome with isolated arterial involvement. Annals of Vascular Surgery 4, 15–19 (1990). https://doi.org/10.1007/BF02042682

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  • DOI: https://doi.org/10.1007/BF02042682

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