Abstract

Aims: To investigate the lens biometric parameters in congenital lens deformities, using a novel technique of swept-source anterior segment optical coherence tomography (SS-ASOCT).Methods: This prospective study included patients with microspherophakia (MSP), coloboma lentis (CL), and posterior lenticonus (PL). For this cohort, 360-degree high-resolution lens images were obtained using the latest SS-ASOCT (CASIA2, Tomey Corp, Nagoya, Japan). The lens biometric parameters were calculated by the CASIA2 built-in software for anterior lens radius (ALR), posterior lens radius (PLR), anteroposterior distance (APD), anterior chamber depth (ACD), equatorial diameter (Eq Dia), rear projection length (RPL), and maximum diameter of the lesion (MDL).Results: This study included two eyes each with MSP and CL and one eye with PL. The lens of MSP was spherical and posteriorly dislocated, with decreased ALR and PLR, Eq Dia, but increased APD. In patients with CL, the coloboma was isolated, bilateral, inferior, and located toward the maldeveloped ciliary body. High astigmatism was mainly lenticular, and this was calculated by the ALR and PLR. Regarding the site of coloboma, a significant decrease in ALR was observed, while the PLR and APD were not affected. The PL eyes had a cone-shaped protrusion of the posterior lens surface with a subtle cataractous region around the apex. An extremely high posterior surface curvature was observed with a mean PLR of 1.67 mm. The RPL and MDL were about 1.80 and 0.4 mm, respectively, which were homogenous at different sections.Conclusions: The CASIA2 is a valuable option for in vivo crystalline lens measurement for congenital lens deformities, enabling the accurate diagnosis and providing illuminating insights into the pathogenesis of MSP, CL, and PL

Highlights

  • The crystalline lens originates from the surface ectoderm overlying the optic vesicle from the 4th week of intrauterine life [1]

  • Any abnormal condition during embryonic development may lead to the occurrence of the congenital lens deformity, which can result in the anomaly of the crystalline lens shape since or before birth [2]

  • The absence of tissues in the equatorial region of the lens was diagnosed as coloboma lentis (CL) [5], whereas a cone-shaped protrusion of the posterior lens surface was defined as posterior lenticonus (PL) [8]

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Summary

Introduction

The crystalline lens originates from the surface ectoderm overlying the optic vesicle from the 4th week of intrauterine life [1]. Any abnormal condition during embryonic development may lead to the occurrence of the congenital lens deformity, which can result in the anomaly of the crystalline lens shape since or before birth [2]. The regional weakness of the posterior capsule and subsequent herniation of the cortical lens fibers are the pathogenesis of posterior lenticonus (PL) [7, 8]. Due to their scarcity, the progress, diagnosis, and treatment of these conditions are poorly understood. Ordinary OCT imaging may be insufficient to image the entire area of the deformed lens due to limited axial range and a relatively low resolution of a posterior lens [9]. For the diagnosis of congenital lens deformities, a more accurate and efficient biometry measurement device is warranted

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