Customize your JAMA Network experience by selecting one or more topics from the list below.
Copyright 2006 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2006
A dural cavernous sinus fistula can cause a number of different ocular findings. We report a case of dural cavernous sinus fistula causing an isolated macular exudative detachment.
An 84-year-old woman was initially seen because of a 1-month history of diplopia and left periorbital pain. She had well-controlled hypertension and a history of a rhegmatogenous retinal detachment in the right eye, which was repaired 10 years previously. There was no history of trauma.
Her visual acuity was 20/60 OD and 20/25 OS. Hertel exophthalmometry was 15 mm on the right and 18 mm on the left. Supraorbital bruits were present bilaterally. Episcleral vessels were tortuous in the left eye. The right fundus had dry macular pigmentary changes. The left fundus was normal. Orbital Doppler imaging revealed arterialization of blood flow in both superior ophthalmic veins; the arterial pulse wave was consistent with bilateral, low-flow dural cavernous sinus fistulas.
She returned 1 week later with worsened diplopia. There was now arterialization of the conjunctival vessels in both eyes along with a new 40–prism diopter esotropia. Repeat orbital Doppler ultrasonography revealed greater dilation and pulsatile flow in both superior ophthalmic veins. Cerebral angiography confirmed bilateral dural cavernous sinus fistulas. She was treated conservatively.
Two months later, the patient returned urgently after noticing an acute decrease in vision in the left eye. Her visual acuity had declined to 20/200 OS. Fundus examination revealed a large serous macular detachment in the left eye. There were no choroidal detachments or retinal breaks present. Fluorescein angiography showed mild, late, diffuse choroidal-based leakage in the region of the serous detachment (Figure 1). Optical coherence tomography confirmed an isolated neurosensory retinal detachment of the macula (Figure 2A). Attempted embolization was unsuccessful because the involved vessels could not be accessed. Repeat Doppler studies and arteriography performed 2 days later showed no filling of the superior ophthalmic veins, indicating spontaneous thrombosis of these veins.
Early-phase (A) and late-phase (B) fluorescein angiography photographs showing mild, late, diffuse choroidal-based leakage in the region of the serous detachment.
A, Ocular coherence tomography showing a neurosensory retinal detachment of the macula. B, Follow-up ocular coherence tomography showing complete resolution of the macular retinal detachment with normalization of the foveal contour.
Two weeks later, the visual acuity improved to 20/40 OS, and the retinal detachment had completely resolved (Figure 2B). Repeat Doppler ultrasonography showed further spontaneous improvement in orbital flow bilaterally. During the ensuing 2 months, the remainder of the ocular signs and symptoms completely resolved.
Our patient had the unique finding of an isolated exudative macular detachment, in addition to many of the classic external ocular features of a spontaneous dural cavernous sinus fistula. There have been reports of peripheral exudative retinal detachments in patients with cavernous sinus fistulas, but these were associated with choroidal detachments.1-3
We propose the following mechanism to explain the pathogenesis of this serous macular detachment. Patients with cavernous sinus fistulas develop arterialization of the orbital veins. This arterialization causes venous stasis, which then leads to hypoxia of the choriocapillaris and subsequent impairment of retinal pigment epithelial cell function. Dysfunction of the choriocapillaris and retinal pigment epithelium has been shown to lead to retinal detachment.4 On normalization of orbital venous outflow, the choriocapillaris and retinal pigment epithelial function returns to normal, and the serous retinal detachment resolves.
To our knowledge, this is the first reported case of serous macular detachment initially seen without clinically evident choroidal detachment in a case of a dural cavernous sinus fistula. Dural cavernous sinus fistulas should be included in the differential diagnosis of isolated exudative macular detachments. Resolution of the macular detachment with visual improvement is possible if the hemodynamic abnormality normalizes.
Correspondence: Dr Regillo, The Retina Service of Wills Eye Hospital, 840 Walnut St, Suite 1020, Philadelphia, PA 19107 (firstname.lastname@example.org).
Financial Disclosure: None reported.
Garg SJ, Regillo CD, Aggarwal S, Bilyk JR, Savino PJ. Macular Exudative Retinal Detachment in a Patient With a Dural Cavernous Sinus Fistula. Arch Ophthalmol. 2006;124(8):1201–1202. doi:10.1001/archopht.124.8.1201