Abstract / Summary
Purpose: To report a familial case series of anterior lentiplane with congenital cataract-microcornea syndrome (CCMC) and describe the imaging characteristics of anterior lentiplane using anterior segment optical coherence tomography (AS-OCT). Methods: This study is comprised of a description of a clinical case series, an investigation into the radius of anterior lens curvature (RAL), and a review of the relevant literature. Results: A 34-year-old woman was referred to the hospital for cataract and glaucoma evaluation. Eye examination revealed a posterior polar cataract, microcornea, and goniodysgenesis. AS-OCT demonstrated a markedly flat anterior lens surface (anterior lentiplane) and an increased central corneal thickness (CCT) in both eyes. A diagnosis of anterior lentiplane with CCMC and secondary glaucoma was made. Cataract surgery was performed on both eyes, and the glaucoma was controlled with medications. Her 15-year-old son showed similar bilateral microcornea with goniodysgenesis on eye screening. AS-OCT also revealed anterior lentiplane, increased CCT, and early cataract in the posterior lens of both eyes. Bilateral CCMC with anterior lentiplane was diagnosed. The RAL was calculated from AS-OCT images using a geometric approach (16.41 mm OD and 20.99 mm OS for the mother, and 37.66 mm OD and 38.30 mm OS for the son). Conclusions: To the best of the authors’ knowledge, this is the first clinically documented report of anterior lentiplane in cataract-microcornea syndrome as a familial case, and the first study to provide a quantitative description of its imaging characteristics using AS-OCT.