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Surgical management of vascular Ehlers-Danlos syndrome and its challenges: a case report

Francisco da Rocha de Sousa1, Nicola Colucci2, Arnaud Dupuis1

  • 1Department of Surgery, Division of Visceral Surgery, Geneva University Hospitals, Geneva, Switzerland.

Swiss Medical Weekly
|December 5, 2020
PubMed

Insights

Vascular Ehlers-Danlos syndrome (vEDS) management is complex. This case highlights challenges in surgical intervention for vEDS patients with arterial rupture and colonic complications, emphasizing individualized care.

Area of Science:

  • Genetics
  • Vascular Surgery
  • Gastroenterology

Background:

  • Ehlers-Danlos syndrome (EDS) comprises rare inherited connective tissue disorders.
  • Vascular EDS (vEDS) involves type III collagen defects, leading to life-threatening arterial and visceral complications.
  • Surgical intervention in vEDS patients requires careful consideration due to high risks.

Observation:

  • A 42-year-old male with suspected vEDS presented with hemorrhagic shock from spontaneous left colic artery rupture.
  • Abdominal vascular abnormalities supported the vEDS diagnosis, confirmed by a novel pathogenic COL3A1 gene mutation.
  • Postoperatively, the patient developed mechanical ileus due to descending colon ischemic stenosis.

Findings:

  • Conservative management failed for the ischemic stenosis, posing a risk of colonic perforation.
  • A diverting ileostomy was performed due to the high risk of perforation in this vEDS patient.
  • This case underscores the diagnostic and therapeutic challenges in managing vEDS complications.

Implications:

  • Management of vEDS patients necessitates meticulous case-by-case risk-benefit analysis.
  • Minimally invasive approaches should be prioritized when feasible in vEDS management.
  • Further research into optimal surgical strategies for vEDS-related complications is warranted.
Abstract

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