Abstract
Purpose: :
To describe a case of hypotony secondary to spontaneous scleral rupture in a patient with a retinochoroidal coloboma and to present a pathogenic mechanism for this condition.
Methods: :
Case report. Clinical, fluorescein angiographic, ultrasonographic, and computed tomographic findings are presented.
Results: :
A 40–year old male had a three–week history of hyptony right eye. Acuity was counting fingers and intraocular pressure was 2 mm Hg. Examination of the right eye disclosed a diffuse conjunctival bleb superotemporally which transilluminated, 1+ anterior cell and flare, optic disc edema, choroidal folds, an area of myelinated nerve fibers along the superotemporal arcade, and a retinochoroidal coloboma with underlying base sclera and incarcerated vitreous strands. Fluorescein angiography, optical coherence tomography, ultrasound, and computed tomography were performed. The diagnosis of spontaneously ruptured sclera in association with retinochoroidal coloboma was made. The wound was surgically closed primarily with suturing and supported with a scleral buckle. The patient’s vision returned to 20/40 and the pressure to 18 mm Hg with resolution of choroidal folds.
Conclusions: :
This case of spontaneous scleral rupture in association with retinochoroidal coloboma includes several unique features not previously reported to our knowledge. First, the simultaneous presence of myelinated nerve fibers, retinochoroidal coloboma, and scleral coloboma in the same quadrant of the affected eye may be coincidental or may arise from a common ocular developmental abnormality. Second, persistent hypotony suggests continued flow of liquefied vitreous or aqueous through the defect. Lastly, the adjacent but distinct locations of the retinochoroidal coloboma and ectatic sclera provide convincing evidence for our proposed ontogenic mechanism.
Keywords: anatomy • retinal development • sclera