Lenticular hydrops in lenticonus: a new concept

Abstract

A 25-year-old male patient presented with biopsy-proven Alport's syndrome with bilateral cataracts (history of unstable refractive error over past 6 years with recent-onset diminution of vision in either eye for the past 1 year; best-corrected visual acuities of 20/100 OD and 20/200 OS; (Fig. 1A, B). On optical coherence tomography the patient showed right eye lenticonus (Fig. 1C) with a communicating hyperreflectivity (fibrosed hydration track) between the anterior capsular and subcapsular opacities (Fig. 2A). The left eye showed a flatter anterior curvature with hyperreflective dots in the anterior chamber suggestive of leaked lens matter or low-grade inflammation (Fig. 1D) with volume loss in the anterior subcapsular region (Fig. 2B) and a localized area of capsular thinning (Fig. 2C). It was hypothesized that microruptures in the anterior capsule (secondary to thinning in the lenticonus) with aqueous hydration could have caused the bilateral cataract and lenticular volume loss in the left eye, thus called hydrops.