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Doppler sonography in the diagnosis of dural carotidcavernous fistula
Insights
A young man with hemophilia presented with a rare cavernous sinus fistula. Directional Doppler ultrasonography identified the arteriovenous shunt, aiding diagnosis and confirming spontaneous symptom resolution.
Area of Science:
- Neurology
- Vascular Surgery
- Radiology
Background:
- Intracranial dural fistulas typically affect older women, often with subtle physical findings.
- Carotid-cavernous sinus fistulas (CCSF) can present atypically, especially in younger patients.
- Hemophilia presents unique challenges in managing vascular abnormalities.
Purpose of the Study:
- To report a case of a young male with hemophilia presenting with a CCSF.
- To highlight the diagnostic utility of directional Doppler ultrasonography in atypical CCSF.
- To document the spontaneous resolution of CCSF in this patient.
Main Methods:
- Clinical examination of a 19-year-old male with a red, proptosed eye.
- Directional Doppler ultrasonography to assess for arteriovenous shunting.
- Selective internal carotid angiography to delineate the fistula's origin.
Main Results:
- Dilated, tortuous episcleral vessels were noted, but typical CCSF signs were absent.
- Directional Doppler ultrasound revealed retrograde flow in the left frontal vein, indicating an arteriovenous shunt.
- Angiography confirmed a dural fistula originating from an internal carotid artery branch.
Conclusions:
- This case demonstrates an unusual presentation of CCSF in a young male with hemophilia.
- Directional Doppler ultrasonography is a valuable noninvasive tool for detecting low-flow retrobulbar arteriovenous shunts.
- Spontaneous remission of CCSF is possible, even in atypical presentations.
Abstract:
Intracranial dural fistulas in the region of the cavernous sinus usually occur in middle-aged or elderly women in whom physical findings of ocular pulsation, orbital bruit, and ocular injection are subtle or absent. We examined a 19-year-old man with hemophilia who had a red, mildly proptosed left eye. Typical features of a carotidcavernous sinus fistula were absent except for the presence of dilated, tortuous episcleral vessels. Directional Doppler ultrasound evaluation detected retrograde blood flow in the left frontal vein and thus confirmed the presence of an arteriovenous shunt. After a selective internal carotid angiogram demonstrated a shunt from a dural branch of the internal carotid artery, the patient's symptoms remitted spontaneously. Directional Doppler ultrasonography is a noninvasive test that can detect the presence of a low flow, retrobulbar arteriovenous shunt in the region of the cavernous sinus.