Neuroendovascular Procedures in Patients with Ehlers-Danlos Type IV: Multicenter Case Series and Systematic Review.
World Neurosurg
; 170: e529-e541, 2023 Feb.
Article
em En
| MEDLINE
| ID: mdl-36402305
ABSTRACT
BACKGROUND:
Ehlers-Danlos type IV or vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited disorder characterized by profound vascular fragility resulting from defective production of type III procollagen. Cerebrovascular diseases including spontaneous dissections, cerebral aneurysms, and cavernous carotid fistulae are common. Endovascular therapies in this patient population are known to be higher risk, although many studies (before 2000) involved older techniques and equipment. The purpose of this study is to investigate the safety and efficacy of modern neuroendovascular techniques in the treatment of cerebrovascular diseases in patients with vEDS.METHODS:
We combined a multi-institutional retrospective case series at 3 quaternary-care centers with a systematic literature review of individual case reports and case series spanning 2000-2021 to evaluate the safety and efficacy of neuroendovascular procedure in patients with vEDS with cerebrovascular diseases.RESULTS:
Fifty-nine patients who underwent 66 neuroendovascular procedures were evaluated. Most of the patients had direct cavernous carotid fistulas (DCCF). Neuroendovascular procedures had a 94% success rate, with a complication rate of 30% and a mortality of 7.5%.CONCLUSIONS:
Neuroendovascular procedures can be performed with a high rate of success in the treatment of cerebrovascular diseases in patients with vEDS, although special care is required because complication rates and mortality are high. Access site and procedure-related vascular injuries remain a significant hurdle in treating vEDS with cerebrovascular diseases, even with modern techniques.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Seio Cavernoso
/
Aneurisma Intracraniano
/
Síndrome de Ehlers-Danlos
/
Síndrome de Ehlers-Danlos Tipo IV
Tipo de estudo:
Systematic_reviews
Limite:
Humans
Idioma:
En
Revista:
World Neurosurg
Assunto da revista:
NEUROCIRURGIA
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Estados Unidos