Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome: a Case Report Managed by Coil Embolization of the Splenic Artery

Authors

  • Marta Ferreira Martins Unidade Local de Saúde do Tâmega e Sousa – Department of General Surgery, Portugal https://orcid.org/0000-0003-0017-9533
  • Miguel Maia Unidade Local de Saúde do Tâmega e Sousa – Department of Vascular Surgery, Portugal
  • Bernardo Moreira Unidade Local de Saúde do Tâmega e Sousa – Department of General Surgery, Portugal
  • Paula Ferreira Pinto Instituto Português de Oncologia do Porto – Department of General Surgery
  • João Almeida Pinto Unidade Local de Saúde do Tâmega e Sousa – Department of Vascular Surgery, Portugal
  • Carla Freitas Unidade Local de Saúde do Tâmega e Sousa – Department of General Surgery, Portugal

DOI:

https://doi.org/10.48729/pjctvs.567

Keywords:

ascular Ehlers-Danlos Syndrome, spontaneous splenic rupture, endovascular embolization, arterial fragility, hemorrhagic shock

Abstract

Vascular Ehlers-Danlos Syndrome (VEDS) is a rare genetic disorder characterized by arterial fragility and a high risk of spontaneous vascular rupture. Splenic artery rupture is an uncommon but life-threatening manifestation of this condition.

We report the case of a 34-year-old male with VEDS who presented with sudden abdominal pain and rapid progression to hemorrhagic shock. Imaging confirmed active bleeding from the distal splenic artery. After multidisciplinary evaluation, endovascular embolization via a left humeral approach was performed using multiple coils. The procedure achieved hemostasis, and the patient showed immediate clinical improvement. He was discharged after 27 days following immunization against encapsulated organisms due to presumed hyposplenism.

This case highlights the critical role of endovascular techniques in managing vascular emergencies in VEDS patients, offering a minimally invasive and effective alternative to open surgery, minimizing vascular manipulation in fragile tissues. 

Splenic artery embolization may be life-saving, but long-term surveillance and preventive strategies are crucial, given the risk of recurrence and complications.

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References

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Published

10-05-2026

How to Cite

1.
Ferreira Martins M, Maia M, Moreira B, Ferreira Pinto P, Almeida Pinto J, Freitas C. Spontaneous Splenic Rupture in Vascular Ehlers-Danlos Syndrome: a Case Report Managed by Coil Embolization of the Splenic Artery. Rev Port Cir Cardiotorac Vasc [Internet]. 2026 May 10 [cited 2026 May 11];33(1):45-9. Available from: https://pjctvs.com/index.php/journal/article/view/567

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