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Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome

Vascular Ehlers-Danlos Syndrome (vEDS) is a rare connective tissue disorder associated with COL3A1 gene mutation encoding type III collagen. Given the possible fatal prognosis if not treated timely, it is important to suspect and diagnose as soon as possible. Despite advances in endovascular techniq...

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Autores principales: Park, Suehyun, Hwang, Deokbi, Yun, Woo-Sung, Kim, Hyung-Kee, Huh, Seung
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627186/
https://www.ncbi.nlm.nih.gov/pubmed/37936948
http://dx.doi.org/10.3389/fsurg.2023.1268671
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author Park, Suehyun
Hwang, Deokbi
Yun, Woo-Sung
Kim, Hyung-Kee
Huh, Seung
author_facet Park, Suehyun
Hwang, Deokbi
Yun, Woo-Sung
Kim, Hyung-Kee
Huh, Seung
author_sort Park, Suehyun
collection PubMed
description Vascular Ehlers-Danlos Syndrome (vEDS) is a rare connective tissue disorder associated with COL3A1 gene mutation encoding type III collagen. Given the possible fatal prognosis if not treated timely, it is important to suspect and diagnose as soon as possible. Despite advances in endovascular technique, access point complications remain a serious challenge in patients with vEDS. Here, we describe a 30-year-old male patient who was diagnosed with vEDS after consecutive events of bilateral iliac vessels at an interval of 3 months: (1) spontaneous dissecting aneurysm of right iliac artery and (2) arteriovenous fistula between left internal iliac artery (IIA) and left common iliac vein. This patient was treated with iliac stent-grafts and overlapping femoral interposition graft (Dacron) in the 1st operation and access artery repair with surgical dissection after coil embolization of IIA and stent-graft insertion into left common to external iliac arteries in the 2nd operation. The patient has been treated with beta-blockers and anticoagulants for the management of vEDS and postoperative deep vein thrombosis, respectively. The stent-grafts in both iliac arteries and the access sites have been well-tolerated without any adverse effects for 14 months following the 2nd operation. In conclusion, given the vascular fragility and the potential for future events, additional vascular manipulation should be avoided unless it is in a life-threatening condition. In particular, meticulous hybrid interventions can be effective treatments.
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spelling pubmed-106271862023-11-07 Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome Park, Suehyun Hwang, Deokbi Yun, Woo-Sung Kim, Hyung-Kee Huh, Seung Front Surg Surgery Vascular Ehlers-Danlos Syndrome (vEDS) is a rare connective tissue disorder associated with COL3A1 gene mutation encoding type III collagen. Given the possible fatal prognosis if not treated timely, it is important to suspect and diagnose as soon as possible. Despite advances in endovascular technique, access point complications remain a serious challenge in patients with vEDS. Here, we describe a 30-year-old male patient who was diagnosed with vEDS after consecutive events of bilateral iliac vessels at an interval of 3 months: (1) spontaneous dissecting aneurysm of right iliac artery and (2) arteriovenous fistula between left internal iliac artery (IIA) and left common iliac vein. This patient was treated with iliac stent-grafts and overlapping femoral interposition graft (Dacron) in the 1st operation and access artery repair with surgical dissection after coil embolization of IIA and stent-graft insertion into left common to external iliac arteries in the 2nd operation. The patient has been treated with beta-blockers and anticoagulants for the management of vEDS and postoperative deep vein thrombosis, respectively. The stent-grafts in both iliac arteries and the access sites have been well-tolerated without any adverse effects for 14 months following the 2nd operation. In conclusion, given the vascular fragility and the potential for future events, additional vascular manipulation should be avoided unless it is in a life-threatening condition. In particular, meticulous hybrid interventions can be effective treatments. Frontiers Media S.A. 2023-10-23 /pmc/articles/PMC10627186/ /pubmed/37936948 http://dx.doi.org/10.3389/fsurg.2023.1268671 Text en © 2023 Park, Hwang, Yun, Kim and Huh. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY) (https://creativecommons.org/licenses/by/4.0/) . The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Surgery
Park, Suehyun
Hwang, Deokbi
Yun, Woo-Sung
Kim, Hyung-Kee
Huh, Seung
Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title_full Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title_fullStr Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title_full_unstemmed Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title_short Case Report: Hybrid approach as a Rescue Treatment in a patient with vascular Ehlers–Danlos Syndrome
title_sort case report: hybrid approach as a rescue treatment in a patient with vascular ehlers–danlos syndrome
topic Surgery
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627186/
https://www.ncbi.nlm.nih.gov/pubmed/37936948
http://dx.doi.org/10.3389/fsurg.2023.1268671
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