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).

This study is comprised of a description of a clinical case series, an investigation into the radius of anterior lens curvature, and a review of the relevant literature.

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 radius of anterior lens curvature (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).

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.