Unilateral Carotid Cavernous Fistula masquerades bilateral thyroid eye disease
Author: Luke Tanner
Base Hospital / Institution: East Suffolk and North Essex FT NHS
ePoster presentation
Abstract ID: 24-524
Purpose
Carotid Cavernous Fistulas (CCF) can be associated with serious neuro-ophthalmologic morbidity and mortality. The clinical presentation can have significant variability and overlap with other causes of orbital inflammation. This case aims to present an unusual case of a patient with unilateral Carotid cavernous fistula (CCF) masquerading bilateral Thyroid Eye Disease (TED).
Methods
Clinical and radiological findings of a case report supported by photos, MRI scans and digital subtraction angiogram images.
Results
A 62-year-old female presented with painless bilateral conjunctival injection, eyelid oedema and proptosis with intermittent diplopia and Clinical Activity Score (CAS) 4. MRI of orbits showed bilateral severe radiological proptosis, bilateral orbital inflammation with enlargement of extraocular muscles and expansion of the retrobulbar fat with no enlargement of the superior ophthalmic vein. The patient was euthyroid with negative TRAb and treated as bilateral euthyroid severe TED with intravenous steroids and mycophenolate mofetil with improvement to CAS 2. In the next 8-months, unilateral relapse occurred in the right eye only, with episcleral vessel congestion, conjunctival chemosis and raised intraocular pressure with symptoms deterioration in the morning, which lead to further imaging investigation, including MRA sequences. The latter revealed right dural indirect CCF, confirmed also with digital subtraction angiogram.
Conclusion
There is considerable overlap in the clinical presentation and radiological features of CCF and TED and unilateral CCF could be the underlying pathology, even in bilateral ocular presentation. It also important the regular follow up of patients with orbital inflammation and further imaging, including MRA sequences, could be critical in atypical features or unusual relapses, especially for patients with worsening symptoms in the morning. Finally, normal superior ophthalmic veins finding on a standard orbital MRI head/orbits protocol does not exclude cortical draining variants of CCF.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Ourania | Fydanaki | East Suffolk and North Essex FT NHS |
| Gavin | Fatania | Imperial College Healthcare NHS Trust |
| Kunwar | Bhatia | Imperial College Healthcare NHS Trust |
| Vickie | Lee | Imperial College Healthcare NHS Trust |
