title: Spontaneous Carotid Cavernous Fistula in a Case with Protein S Deficiency that Newly Developed Ophthalmoplegia after Embolization.
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.
  
  
  METHODS
  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.
authors.affiliation: Department of Neurology, Seoul National University Hospital, Seoul, Korea.
authors.forename: Chi Kyung
authors.initials: CK
authors.lastname: Kim
authors.forename: Je-Young
authors.initials: JY
authors.lastname: Shin
authors.forename: Jun-Young
authors.initials: JY
authors.lastname: Chang
authors.forename: Seung-Hoon
authors.initials: SH
authors.lastname: Lee
references.citation: Neurol Res. 2003 Oct;25(7):745-8
references.pmid: 14579793
references.citation: Neurosurgery. 2008 Oct;63(4):693-98; discussion 698-9
references.pmid: 18824942
references.citation: Neurosurgery. 2007 Aug;61(2):262-8; discussion 268-9
references.pmid: 17762738
references.citation: Surg Neurol. 1975 Nov;4(5):433-7
references.pmid: 1188578
references.citation: J Clin Neurol. 2005 Oct;1(2):134-41
references.pmid: 20396460
references.citation: Curr Opin Hematol. 2008 Sep;15(5):529-36
references.pmid: 18695379
references.citation: Stroke. 1996 Feb;27(2):243-6
references.pmid: 8571417
references.citation: Br J Haematol. 2001 Jun;113(3):636-41
references.pmid: 11380449
references.citation: N Engl J Med. 1984 Dec 13;311(24):1525-8
references.pmid: 6239102
references.citation: J Neurosurg. 1994 Jul;81(1):122-5
references.pmid: 8207513
references.citation: J Neurosurg. 2008 Aug;109(2):208-14
references.pmid: 18671631
references.citation: J Neurosurg. 1985 Feb;62(2):248-56
references.pmid: 3968564
references.citation: Surg Neurol. 2002 Jul;58(1):59-64; discussion 64
references.pmid: 12361657
references.citation: Neurosurgery. 2003 Oct;53(4):836-56; discussion 856-7
references.pmid: 14519216
references.citation: Stroke. 2005 Jun;36(6):1134-9
references.pmid: 15890994
references.citation: AJNR Am J Neuroradiol. 2001 Mar;22(3):537-40
references.pmid: 11237982
references.citation: AJNR Am J Neuroradiol. 2006 Nov-Dec;27(10):2078-82
references.pmid: 17110671
references.citation: Surg Neurol. 2007 Sep;68(3):277-84; discussion 284
references.pmid: 17719966
references.citation: Neurology. 1998 Mar;50(3):814-6
references.pmid: 9521287
references.citation: Brain. 1973 Sep;96(3):601-12
references.pmid: 4743934