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Innominate artery involvement in type IV Ehlers-Danlos syndrome.

A Valverde1, J F Tricot, B de Crepy

  • 1Service de Chirurgie Vasculaire et Thoracique, Hôpital André Mignot, Le Chesnay, France.

Annals of Vascular Surgery
|January 1, 1991
PubMed
Summary

Ehlers-Danlos syndrome can cause rare but severe innominate artery issues, including dissection and rupture. Vascular surgeons face challenges managing these fragile arterial complications.

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Area of Science:

  • Vascular Surgery
  • Genetics
  • Cardiology

Background:

  • Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
  • Arterial complications are a known, albeit rare, manifestation of EDS.
  • These complications are often due to inherent arterial wall fragility.

Observation:

  • Two cases of innominate artery involvement in patients with Ehlers-Danlos syndrome are presented.
  • Case 1: Spontaneous dissection of the innominate artery successfully treated.
  • Case 2: Spontaneous rupture of the innominate artery leading to mortality postoperatively.

Findings:

  • Innominate artery dissection and rupture can occur in Ehlers-Danlos syndrome patients.
  • Arterial wall fragility in EDS significantly complicates surgical management.

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  • Outcomes can be variable, ranging from successful treatment to fatal complications.

    • Implications:

    • Highlights the critical need for heightened awareness of vascular risks in EDS patients.
    • Underscores the diagnostic and therapeutic challenges faced by vascular surgeons.
    • Emphasizes the importance of careful preoperative assessment and management strategies for EDS patients with arterial abnormalities.