High-Flow Carotid-Cavernous Fistula in an 12-Year-Old Presenting with Progressive Proptosis and Vision Loss: A Case Report
Year: 2025
Type: article
Study design: Case Report
Abstract: Objectives: To report a rare pediatric case of spontaneous high-flow direct carotid–cavernous fistula (CCF) presenting with rapid, vision-threatening orbital congestion, and to highlight the diagnostic pathway and management barriers in a low-resource setting. Case Presentation: An 12-year-old boy presented with 3–4 weeks of progressive left-eye proptosis, orbital pain, and visual decline, culminating in no light perception. Examination showed marked left-sided proptosis, afferent pupillary defect, and elevated intraocular pressure (24–25 mmHg; right eye 12 mmHg). Funduscopy revealed dilated, tortuous retinal veins with venous pulsations, without cherry-red spot or disc edema. Orbital B-scan demonstrated a well-defined hypoechoic retrobulbar structure suggestive of vascular dilation. Brain-orbit MRI identified multiple T2 flow voids, a markedly dilated superior ophthalmic vein draining into the cavernous sinus and appearing connected to the left internal carotid artery, associated enlarged extraocular muscles, and retrobulbar edema—findings consistent with a high-flow direct CCF. Urgent digital subtraction angiography and endovascular embolization were recommended to prevent intracranial venous hypertension and irreversible optic neuropathy. Due to the absence of interventional neuroradiology services and endovascular equipment locally, the patient was referred abroad for definitive management, with close ophthalmic monitoring advised pending transfer. Conclusion: CCF, uncommon in children, should be considered in pediatric proptosis with visual decline—even without trauma. Imaging enables early recognition; however, timely access to endovascular therapy determines visual and neurological outcomes. This case underscores gaps in neurovascular care within low-resource settings and supports investment in endovascular capacity, clinician training, and referral networks to mitigate preventable vision loss in presentations.
Institution: Herat University
Language: English
Subfield: Clinical Neurology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
OpenAlex ID: W4416289062