A clinical case of fitting scleral contact lenses for congenital bilateral eyelid coloboma complicated by exposure keratopathy

Abstract

Introduction. Eyelid coloboma is a rare craniofacial pathology, that usually affects the patient congenitally. Eyelid coloboma in addition to being a cosmetic disfigurement, also affects the cornea, vision and if associated with other systemic abnormalities can cause severe morbidity. Complications of eyelid coloboma are mainly due to corneal exposure from large upper eyelid defects resulting in exposure keratopathy and corneal ulceration if left untreated.The aim of case study: to analyze a clinical case of bilateral upper eyelid coloboma complicated by exposure keratophathy and irregular astigmatism, resulting in low visual acuity in a 4-year-old girl.Description of the case. The patient was born with Manitoba-oculo-trichio-anal (MOTA) syndrome, which was accompanied by bifid nose and eyelid coloboma. The patient had multiple reconstructive surgeries. She was referred to the Miami Contact Lens Institute (MCLI) for Scleral Contact Lens fit (SCL). The examination revealed bilateral acquired infantile corneal opacities of mild density, which occluded the visual axis; complex hypermetropic astigmatism 6.5 diopters; high-grade amblyopia in both eyes -best corrected visual acuity was OD 0.13 and OS 0.05. Scleral contact lenses were fitted to prevent progressive symblepharon, protect the ocular surface, restore vision and to provide comfort due to the severity of dry eye. SCL’s had ideal clearance in the center and at the periphery and provided visual acuity OD 0.2 and OS 0.1.Conclusion. Patients with congenital anomalies are often difficult to treat, especially after multiple reconstructive eyelid surgeries. The mechanical effect of the eyelids on the cornea and the high risk of developing symblepharon and severe amblyopia influenced our decision to fit the patient with SCL. These lenses create a fluid reservoir that provides hydration, comfort, and protection to the exposed surface of the eye and restores vision. Ophthalmologists and pediatricians should pay more attention to the advantages of SCL specifically for patients that require corneal protection from exposure and mechanical damage.