Literature DB >> 35420538

Successful Conservative Management of a Rare Surgical Complication of Vascular Ehlers-Danlos Syndrome: A Case Report.

Giuseppe Mangiameli1, Charles Al Zreibi1, Abderrahmen Ammar1, Alex Arame1, Françoise Le Pimpec-Barthes1.   

Abstract

Vascular Ehlers-Danlos syndrome is a hereditary disorder of the connective tissue characterized by a reduction in the production of type III collagen. Clinical evolution of the disease is unpredictable because vascular lesions can occur everywhere in the body. Bruising, arterial and bowel fragility, and uterine fragility during pregnancy are the most common symptoms. Thoracic surgeons should take with these patients because, in rare cases, thoracic complications may occur, such as recurrent pneumothorax or spontaneous hemothorax. We report an extra-pleural hematoma in a patient with vascular Ehlers-Danlos syndrome, an unusual complication treated successfully with conservative management.

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Year:  2021        PMID: 35420538      PMCID: PMC9007316          DOI: 10.7812/TPP/21.021

Source DB:  PubMed          Journal:  Perm J        ISSN: 1552-5767


  4 in total

Review 1.  Vascular aspects of the Ehlers-Danlos Syndromes.

Authors:  Fransiska Malfait
Journal:  Matrix Biol       Date:  2018-04-27       Impact factor: 11.583

2.  Management of Spontaneous Liver Hematoma in Ehlers-Danlos Syndrome Type IV: A Case Report.

Authors:  Brandon Imp; Samuel Mannarino; Anand Narayanan
Journal:  Perm J       Date:  2020-11

3.  A multi-institutional experience in vascular Ehlers-Danlos syndrome diagnosis.

Authors:  Sherene Shalhub; Peter H Byers; Kelli L Hicks; Dawn M Coleman; Frank M Davis; Giovanni De Caridi; K Nicole Weaver; Erin M Miller; Marc L Schermerhorn; Katie Shean; Gustavo Oderich; Mauricio Ribeiro; Cole Nishikawa; Kristofer Charlton-Ouw; Christian-Alexander Behrendt; E Sebastian Debus; Yskert von Kodolitsch; Devin Zarkowsky; Richard J Powell; Melanie Pepin; Dianna M Milewicz; Ellen S Regalado; Peter F Lawrence; Karen Woo
Journal:  J Vasc Surg       Date:  2019-07-26       Impact factor: 4.268

4.  Vascular Ehlers-Danlos Syndrome with a Novel Missense Mutation in COL3A1: A Man in His 50s with Aortic Dissection after Interventional Treatment for Hemothorax as the First Manifestation.

Authors:  Kosuke Sakai; Maiko Toda; Hiroyuki Kyoyama; Hiroaki Nishimura; Akitoshi Kojima; Yoshiki Kuwabara; Yumiko Kobayashi; Satoshi Kikuchi; Yusuke Hirata; Gaku Moriyama; Wataru Watanabe; Koichi Akutsu; Maki Nakai; Takeshi Yamada; Akihiko Gemma; Kazutsugu Uematsu
Journal:  Intern Med       Date:  2019-08-06       Impact factor: 1.271

  4 in total

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