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Photo Essay
August 2001

Successful Closure of Spontaneous Scleral Fistula in Retinochoroidal Coloboma

Arch Ophthalmol. 2001;119(8):1220-1221. doi:10.1001/archopht.119.8.1220

A 20-YEAR-OLD woman had sudden painless diminution of vision in the right eye of 20 days' duration. She had noticed a spontaneously disappearing blister in the same eye on waking from sleep and was aware of a hearing deficit from the left ear. Her best-corrected Snellen visual acuity at initial examination was 6/24 OD and 6/6 OS. The left eye was normal. Slitlamp examination of the right eye revealed minimal cellular inflammation in the anterior chamber, posterior subcapsular cataract, and intraocular pressure of 4 mm Hg with applanation tonometer. The fundus examination showed hypotonic retinopathy and a choroidal coloboma just temporal to the major vascular arcades (Figure 1). Interestingly, a fistula could be seen in the coloboma, which appeared to open and close on eye movements. B-scan ultrasonography also showed an area of choroidal excavation temporally with a communication into the orbit and adjoining hypoechoic area, corresponding to fluid collection from the leaking fistula (Figure 2). Magnetic resonance imaging of the head and orbit confirmed the presence of a posterior bulbar fistula of the right eye and also incidentally detected acoustic schwannoma of the left eighth nerve (Figure 3). The patient underwent repair of the ocular fistula with n-butyl cyanoacrylate glue (Nectacryl; Nectar Laboratories Limited, Hyderabad, India) and glycerine-preserved scleral graft. Intraoperatively, a 7 × 7-mm area of extreme scleral thinning that corresponded to the site of choroidal coloboma and had abnormal adhesions with the lateral rectus muscle was identified. A small fistula was noted 5 mm from the anterior edge of the scleral thinning (Figure 4). Postoperatively, the patient had visual acuity of 6/9, intraocular pressure of 14 mm Hg, resolution of the inflammatory reaction, closure of the fistula, and disappearance of hypotonic retinopathy (Figure 5).

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