IMAGES

Indirect Carotid-Cavernous Fistula Mimicking Scleritis

Andrés Alberto Hormaza, MD,*† Gabriel J. Tobón, MD, PhD,†‡ Carlos A. Cañas, MD,†
Juan Pablo Suso, MD,†§ and Fabio Bonilla-Abadía, MD†

C arotid-cavernous sinus fistulas (CCF) are abnormal commu-

nications between the carotid system and the cavernous sinus
and often present with ocular signs and symptoms.1 Direct fistulas
usually account for 70 to 90% of all, being indirect fistulas
very rare.
A 51-year-old man presented with 6 months of red eye and
eyelid edema, without changes in visual acuity. Physical examina-
tion of the left eye revealed edema, mild proptosis, and conjunc-
tiva injection (Fig. 1A, B). This patient had been diagnosed and
treated as having scleritis for several months. No clinical signs
or positive laboratory of autoimmunity were found. Magnetic res-
onance angiography reported indirect signs that suggest left indi-
rect carotid-cavernous fistula without thrombosis (Fig. 2). FIGURE 2. Magnetic resonance angiography. Indirect radiologic
Because of the persistent symptoms, risk of vision loss, and intra- signs of indirect cavernous-carotid fistula in the patient. (A) Axial
FLAIR sequence with tortuous and prominent left superior
cranial bleeding, a vascular embolization was done, diminishing
ophthalmic vein (white arrow) with hypertension signs.
edema and conjunctival injection. (B) Coronal T2, proptosis, thickening of extraocular muscles (thick
This rare variety of CCF may be mistaken for chronic arrow: superior oblique; thin arrow: medial rectus; unfilled arrow:
conjunctivitis, orbital pseudotumor, orbital cellulitis, or thyroid inferior rectus) comparative with contralateral eye.
disease delaying diagnosis and early treatment. The visual impair-
ment often is caused by glaucoma associated to venous hyperten-
sion, but also by diplopia, proptosis with corneal exposure,
index of suspicion is the key factor in diagnosing a case of CCF
macular edema, and retinopathy or optic nerve ischemia. A high
without any history of trauma and collagen vascular disease.2
Considering the differential diagnosis, scleritis has been associated
as a manifestation of systemic rheumatic diseases (rheumatoid
arthritis, vasculitis, among others),3 contrary to CCF that is more
related to localized vascular abnormalities. The CCF vascular
findings are dilatated and tortuous conjunctival and epibulbar
veins, with a thickened wall making an angle near to the limbus,
leading to conjunctival injection, in contrast to scleritis that is
characterized by scleral edema and dilatation or closure of deep
episcleral vascular plexus.4,5

REFERENCE
1. Tan AC, Farooqui S, Li X, et al. Ocular manifestations and the clinical course
of carotid cavernous sinus fistulas in Asian patients. Orbit. 2014;33:45–51.
2. Das JK, Medhi J, Bhattacharya P, et al. Clinical spectrum of spontaneous
carotid-cavernous fistula. Indian J Ophthalmol. 2007;55:310–312.
3. Jabs DA, Mudun A, Dunn JP, et al. Episcleritis and scleritis: clinical features
and treatment results. Am J Ophthalmol. 2000;130:469–476.
4. De Keizer RJ. Spontaneous carotico-cavernous fistulas. The importance of
FIGURE 1. Left eye shows (A) conjunctival injection, eyelid edema, the typical limbal vascular loops for the diagnosis, the recognition of
and proptosis. (B) Conjunctival veins dilatated and tortuous. glaucoma and the uses of conservative therapy in this condition.
Color figures are available online at http://www.jclinrheum.com. Doc Ophthalmol. 1979;46:403–412.
5. Okhravi N, Odufuwa B, McCluskey P, et al. Scleritis. Surv Ophthalmol.
2005;50:351–363.

From the *ICESI University, Cali; †Rheumatology Unit, Fundación Valle del
Lili, Cali; ‡Laboratory of Immunology, Fundación Valle del Lili, Cali;
and §Instituto de Investigaciones Clínicas, Fundación Valle del Lili, Cali,
Colombia.
The authors declare no conflict of interest.
Correspondence: Fabio Bonilla, MD, Unit of Rheumatology, Fundación Valle
del Lili, Cra. 98 18-49, Cali, Colombia. E-mail: fbac1982@hotmail.com.
Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.
ISSN: 1076-1608
DOI: 10.1097/RHU.0000000000000454

JCR: Journal of Clinical Rheumatology • Volume 23, Number 2, March 2017 www.jclinrheum.com 117

Copyright © 2017 Wolters Kluwer Health, Inc. All rights reserved.