Spontaneous scleral rupture adjacent to retinochoroidal coloboma.

Background: Spontaneous scleral rupture in association with retinochoroidal coloboma is a rare and poorly understood event, with few reports in the literature.

Methods: Interventional case report.

Results: A 40-year-old man had a spontaneous decline in visual acuity with hypotony in the right eye. Photographic, fluorescein angiographic, optical coherence tomographic, ultrasonographic, and computed tomographic findings demonstrated that the cause was spontaneous rupture of ectatic sclera adjacent to a retinochoroidal coloboma. Surgical repair with primary suture imbrication and support with a segmental scleral buckle restored the intraocular pressure and baseline visual acuity.

Conclusions: The adjacent but distinct locations of the retinochoroidal coloboma and ectatic sclera in this case suggest that during embryogenesis a full thickness defect was present in the eye wall, but due to differential growth rates, the scleral and retinochoroidal defects ceased to be superimposed. Persistent hypotony implies continued flow of liquefied vitreous or aqueous through the defect. Suture imbrication and scleral buckling can be a successful treatment option.