Spontaneous carotid cavernous fistula in a case with protein S deficiency that newly developed ophthalmoplegia after embolization

Chi Kyung Kim, Je Young Shin, Jun Young Chang, Seung Hoon Lee

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Background Carotid cavernous fistula (CCF) is an abnormal communication between the carotid artery and the cavernous sinus. The pathogenesis of spontaneous CCF remains unclear, although sinus thrombosis is known to be a predisposing factor for dural arteriovenous fistula. Because spontaneous CCFs are mainly of the dural type, we considered that thrombogenic conditions, such as, protein S deficiency might be associated with CCF. Case Report A 42-year-old woman complained of conjunctival injection and retro-orbital pain that first appeared 1-month before visiting our hospital. She had no history of head trauma or intracranial surgery. Exophthalmos and chemosis were observed in her left eye, which also had lower visual acuity and higher intraocular pressure than the right eye. Magnetic resonance images and cerebral angiography revealed a left dural CCF. Her protein S was low, at 41% (normal range: 70-140%), but other hematologic values related to coagulation were normal. Her symptoms were relieved after initial transvenous coil embolization. However, a newly developed sixth-nerve palsy was detected 4 days after initial embolization. Follow-up angiography revealed a minimal shunt, and thus transvenous coil embolization was repeated. Two days later, the ophthalmoplegia started reducing, and 1-month later it had almost disappeared. Conclusions To the best of our knowledge, this is the first report of spontaneous dural CCF in a Korean patient with concurrent protein S deficiency. Interestingly, transient sixth-nerve palsy developed after transvenous coil embolization in this patient. This additional symptom caused by the residual fistula was relieved after additional transarterial embolization.

Original languageEnglish
Pages (from-to)164-167
Number of pages4
JournalJournal of Clinical Neurology (Korea)
Volume7
Issue number3
DOIs
Publication statusPublished - 2011 Jan 1
Externally publishedYes

Fingerprint

Protein S Deficiency
Ophthalmoplegia
Fistula
Abducens Nerve Diseases
Central Nervous System Vascular Malformations
Intracranial Sinus Thrombosis
Cavernous Sinus
Cerebral Angiography
Exophthalmos
Magnetic Resonance Angiography
Protein S
Intraocular Pressure
Craniocerebral Trauma
Carotid Arteries
Causality
Visual Acuity
Angiography
Reference Values
Communication
Pain

Keywords

  • Carotid cavernous fistula
  • Complication
  • Protein s deficiency
  • Sixth-nerve palsy
  • Transvenous embolization

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Spontaneous carotid cavernous fistula in a case with protein S deficiency that newly developed ophthalmoplegia after embolization. / Kim, Chi Kyung; Shin, Je Young; Chang, Jun Young; Lee, Seung Hoon.

In: Journal of Clinical Neurology (Korea), Vol. 7, No. 3, 01.01.2011, p. 164-167.

Research output: Contribution to journalArticle

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