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Neuro-Ophthalmology in Dural Cavernous Sinus Fistulas (DCSFs) |
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Fig. 6.1 a–d. Classic clinical presentation of a traumatic direct CCF that is often acute and fulminant, but can be delayed for several days or weeks, in some cases for several months. A 24-year-old man after a car accident seen in July 2002. a–b
Severe exophthalmos and chemosis of the right eye, associated with audible bruit. c The arteriogram shows a massively enlarged CS (asterisks) with posterior bulging into the cranial cavity and dominant anterior drainage into a significantly enlarged SOV. No intracranial steal. Note that such severe chemosis is rare in DCSFs (see Fig. 6.4) d Fistula occlusion with one detachable balloon
signs pointing towards etiology, hemodynamics and prognosis. Therefore, knowledge and careful analysis of the neuroophthalmological symptoms are required for differential diagnosis, effective, individual, riskand prognosis-oriented use of diagnostic and therapeutic measures in patients with DCSFs.
6.1
Extraorbital Ocular Symptoms
6.1.1 Orbital Pain
Persistent frontal or periorbital cephalgia of varying intensity, often starting acutely, is a frequent initial symptom in patients with DCSF. These headaches are usually caused by local thrombosis within the CS or the SOV.
Furthermore, hemodynamic turbulences in the fistula leading to painful irritation of the meninges can increase during physical exercise or elevated blood pressure. Extraocular or extraorbital symptoms beside headaches are rare. Anecdotal reports of life-threatening epistaxis or intracerebral hemorrhages in case of leptomeningeal venous drainage (Keltner et al. 1987a) or ischemic brain stem infarcts after sinus venous thrombosis (Uchino et al. 1997) and atypical trigeminal neuralgia (Ott et al. 1993) exist. Fistulous connections in the posterior CS may cause mechanical compression of the Gasserian ganglion or vascular steal phenomena affecting in particular the first or second division, resulting into neuralgia and dysesthesia (Madsen 1970; Palestine et al. 1981). They can initially occur isolated and thus may easily be mistaken for idiopathic trigeminal neuralgia (Ott et al. 1993; Rizzo et al. 1982). Patients with periorbital cephalgia and accompany-