Transvenous Embolization for Carotid-Cavernous Fistula in a Patient with Vascular Type of Ehlers-Danlos Syndrome—Direct Superior Ophthalmic Vein Approach: Case Report

Tanaka, Teppei; Hayakawa, Masayuki; Sadato, Akiyo; Adachi, Kazuhide; Watabe, Takeya; Maeda, Shingo; Ohmura, Masahiro; Hirose, Yuichi

doi:10.2176/nmc.cr.2013-0007

Cited by 12 publications

(3 citation statements)

References 19 publications

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“…Because of the high risk associated with endovascular treatment of CCF in EDS IV patients, it is recommended that all spontaneous CCF cases be approached with the possibility of EDS IV in mind. 150 Given the well-known vascular fragility of these patients, even a simple catheter angiography can lead to disastrous consequences in EDS IV 151 and therefore should be considered only if endovascular therapy is considered. As an alternative to transfemoral endovascular treatment, the ipsilateral carotid artery and internal jugular vein can be surgically exposed for direct insertion of endovascular sheaths.…”

Section: Eds and Marfan Syndrome And Ccfmentioning

confidence: 99%

Neurovascular manifestations of connective-tissue diseases: A review

et al. 2016

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Patients with connective tissue diseases are thought to be at a higher risk for a number of cerebrovascular diseases such as intracranial aneurysms, dissections, and acute ischemic strokes. In this report, we aim to understand the prevalence and occurrences of such neurovascular manifestations in four heritable connective tissue disorders: Marfan syndrome, Ehlers-Danlos syndrome, Neurofibromatosis Type 1, and Loeys-Dietz syndrome. We discuss the fact that although there are various case studies reporting neurovascular findings in these connective tissue diseases, there is a general lack of case-control and prospective studies investigating the true prevalence of these findings in these patient populations. Furthermore, the differences observed in the manifestations and histology of such disease pathologies encourages future multi-center registries and studies in better characterizing the pathophysiology, prevalence, and ideal treatment options of neurovascular lesions in patents with connective tissue diseases.

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Section: Eds and Marfan Syndrome And Ccfmentioning

confidence: 99%

Neurovascular manifestations of connective-tissue diseases: A review

et al. 2016

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“…In vEDS, ipsilateral headache may occur together with additional neurologic features, such as ophthalmoplegia and tinnitus, due to vascular accidents, including spontaneous direct cavernous‐carotid fistula [Chuman et al, ; Tanaka et al, ]. Similar presentations may equally be observed in other EDS subtypes with vascular fragility.…”

Section: Headache In Ehlers–danlos Syndromes: An Overviewmentioning

confidence: 99%

Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain

Castori¹,

Morlino²,

Ghibellini³

et al. 2015

American J of Med Genetics Pt C

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Ehlers-Danlos syndrome (EDS) is an umbrella term for a growing group of hereditary disorders of the connective tissue mainly manifesting with generalized joint hypermobility, skin hyperextensibility, and vascular and internal organ fragility. In contrast with other well known heritable connective tissue disorders with severe cardiovascular involvement (e.g., Marfan syndrome), most EDS patients share a nearly normal life span, but are severely limited by disabling features, such as pain, fatigue and headache. In this work, pertinent literature is reviewed with focus on prevalence, features and possible pathogenic mechanisms of headache in EDSs. Gathered data are fragmented and generally have a low level of evidence. Headache is reported in no less than 1/3 of the patients. Migraine results the most common type in the hypermobility type of EDS. Other possibly related headache disorders include tension-type headache, new daily persistent headache, headache attributed to spontaneous cerebrospinal fluid leakage, headache secondary to Chiari malformation, cervicogenic headache and neck-tongue syndrome, whose association still lacks of reliable prevalence studies. The underlying pathogenesis seems complex and variably associated with cardiovascular dysautonomia, cervical spine and temporomandibular joint instability/dysfunction, meningeal fragility, poor sleep quality, pain-killer drugs overuse and central sensitization. Particular attention is posed on a presumed subclinical cervical spine dysfunction. Standard treatment is always symptomatic and usually unsuccessful. Assessment and management procedures are discussed in order to put some basis for ameliorating the actual patients' needs and nurturing future research.

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“…One case of vEDS developed vision loss, proptosis, orbital pain, and orbital bruits [10]. Another case presented with tinnitus [11] but some cases have been detected on routine imaging [12].…”

Section: Neuro-ophthalmic Manifestationsmentioning

confidence: 99%

Neuro-ophthalmic manifestations of Ehlers–Danlos syndrome

Rachapudi,

Laylani,

Davila-Siliezar

et al. 2023

Current Opinion in Ophthalmology

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Purpose of review To review the neuro-ophthalmic manifestations of Ehlers–Danlos syndrome (EDS). Recent findings Ehlers–Danlos syndrome (EDS) is a rare genetic disorder with an estimated prevalence of 1 in 5000 individuals, but its true prevalence may be underestimated because of variable clinical presentations and limited awareness among healthcare professionals. The neuro-ophthalmic features of EDS may be difficult to recognize in context but new molecular genetic testing is now available for identification of specific subtypes of EDS. Summary Ophthalmic manifestations of EDS include loss of vision and double vision (strabismus), high myopia, retinal detachment, and blue sclera. The vascular subtype of EDS can present as a carotid-cavernous fistula, intracranial aneurysm, or arterial dissection.

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Transvenous Embolization for Carotid-Cavernous Fistula in a Patient with Vascular Type of Ehlers-Danlos Syndrome—Direct Superior Ophthalmic Vein Approach: Case Report

Cited by 12 publications

References 19 publications

Neurovascular manifestations of connective-tissue diseases: A review

Neurovascular manifestations of connective-tissue diseases: A review

Connective tissue, Ehlers–Danlos syndrome(s), and head and cervical pain

Neuro-ophthalmic manifestations of Ehlers–Danlos syndrome

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