Atlas of Ophthalmology

Ptosis and Proptosis at OS in Carotid-cavernous Fistula (CCF) of Type D (Colour Image)

Ptosis and Proptosis at OS in Carotid-cavernous Fistula (CCF) of Type D (Colour Image)
Patient: 75 years of age, male, BCVA 1.0 at OD, 0.7 at OS, IOP 10/13 mmHg. Ocular Medical History: Since 3 weeks rapidly progressive exophthalmos, orbital pain, left-sided ophthalmoplegia, chemosis, and visual disturbance. General Medical History: headache, no trauma. Main Complaint: Visual loss. Purpose: to show proptosis in a spontaneous carotid-cavernous fistula. Methods: Colour Image, 3-Tesla MRA (Siemens). Findings: Colour Image: ptosis, exophthalmos, chemosis. Conventional MRI: orbital edema. Contrast-enhanced MRI in Coronal Orientation: enlargement of the superior ophthalmic vein, exophthalmos, enlargement of the orbital muscles. Time-resolved 3D-angiography (TWIST): retrograde drainage of the ophthalmic vein,, and enlargement of the superior ophthalmic vein. Discussion: The normal venous drainage of the superior and inferior ophthalmic veins as well as the sphenoparietal sinus and superficial middle cerebral veins is directed toward the cavernous sinus. A carotid-cavernous sinus fistula is an anomalous communication of dural branches from the intracerebral artery or external carotid artery to the cavernous sinus. Spontaneous carotid-cavernous sinus fistulas have been associated with the presence of ruptured carotid aneurysms, atherosclerosis, diabetes mellitus, and genetic disorders such as Ehlers-Danlos syndrome or fibromuscular dysplasia. Patients suffering from CCFs often present with dramatic ocular complications such as secondary glaucoma, visual loss, extraocular muscle palsy, progressive proptosis, conjunctival chemosis, and retinal vein occlusion. Barrow et al. (1) provided a detailed anatomical classification into four distinct types based on their arterial supply. Type A fistulas show a direct communication between the internal carotid artery and the cavernous sinus associated with high flow rates. Indirect fistulas are dural arterio-venous fistulas by the meningeal branches of the internal carotid artery (type B), the external carotid artery (type C), or both (type D). Traumatic fistulas are in most cases direct type A fistulas and show a single direct communication between the internal carotid artery and the cavernous sinus. About 20 % of type A fistulas are regarded as spontaneous, either due to rupture of a cavernous segment aneurysm or weakened atherosclerotic artery. Seeger et al. (2) reported, that the evaluation of the intracranial vasculature for CCF diagnosis has been carried out using conventional digital subtraction angiography (DSA) as gold standard, and magnetic resonance imaging (MRI) to rule out differential diagnoses. Literature: (1) Barrow DL, Spector RH, Braun IF, Landman JA, Tindall SC, Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas. J Neurosurg. 1985 Feb;62(2):248-56 (2) Seeger A, Kramer U, Bischof F, Schuettauf F, Ebner F, Danz S, Ernemann U, Hauser TK Feasibility of Noninvasive Diagnosis and Treatment Planning in a Case Series with Carotid-Cavernous Fistula using High-Resolution Time-Resolved MR-Angiography with Stochastic Trajectories (TWIST) and Extended Parallel Acquisition Technique (ePAT 6) at 3 T. Clin Neuroradiol. 2014 Mar 6.
Korbmacher, Judit,, Augenklinik, Universitätsklinikum Erlangen, Erlangen, Deutschland, Erlangen, Deutschland; Engelhorn, Tobias, Prof. Dr. med., Neuroradiologie, Universität Erlangen-Nürnberg, Erlangen, Erlangen, Deutschland; Michelson, Georg, Prof. Dr. med., Interdisziplinäres Zentrum für augenheilkundliche Präventivmedizin und Imaging, Augenklinik, Universität Erlangen-Nürnberg, Erlangen, Deutschland
H02.4 + I67.11
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