Development of Posterior Lenticonus Following the Diagnosis of Isolated Anterior Lenticonus in Alport Syndrome.

Lubna M Halawani,Turki A Bin Dakhil,Hammam A Alotaibi,Mohammed F Abdulaal,Anoud F Alsaati

doi:10.7759/cureus.12970

Abstract

Alport syndrome is a genetic disorder that manifests as renal disease, hearing loss and ocular dysfunction. Lenticonus is one such ocular condition, in which the lens takes on an abnormal cone shape, with a protrusion either at the front or back of the lens. Both sides of the lens are rarely affected at the same time in the general patient population. Although anterior lenticonus is the type that is often reported in Alport syndrome, it is rare for such patients to have both anterior and posterior lenticonus. Here, the case of a 32-year-old male with Alport syndrome is described. The patient was diagnosed with a progressive posterior lenticonus, having been diagnosed eight years earlier with isolated anterior lenticonus. Examination of the eye revealed the typical indications of lenticonus with flecked retinopathy. The patient had co-presenting astigmatism and a refractive error, which could not be corrected by wearing contact lenses or spectacles. It is critical that such cases are anticipated and identified prior to performing surgery, so that an appropriate approach can be taken, thereby minimizing surgical complications.

Highlights

Lenticonus is an uncommon morphological abnormality in which the lens capsule thins and bulges, adopting a conical shape
In addition to anterior lenticonus, Alport syndrome can manifest as cataracts, corneal arcus juvenilis, posterior polymorphous dystrophy, and flecked retinopathy
Reports in the literature suggest it could be associated with Lowe’s syndrome [2]. This case report describes a case in which the patient diagnosed with a progressive posterior lenticonus, having been diagnosed eight years earlier with isolated anterior lenticonus

Summary

Introduction

Lenticonus is an uncommon morphological abnormality in which the lens capsule thins and bulges, adopting a conical shape. A 32-year-old male, Alport syndrome patient came to the ophthalmology clinic with a complaint of progressive loss of vision in both eyes, since childhood. Elsewhere, he had been diagnosed with bilateral isolated anterior lenticonus (Figure 1). The follow-up examination showed that in the intervening years between visits to the ophthalmology clinic, a posterior lenticonus had developed in his left eye (Figure 2). The same examination of the left eye revealed a clear cornea but a finding of anterior and posterior lenticonus, measuring three and four millimeters, respectively. The classic oil-droplet appearance that is associated with fleck retinopathy was observed (Figure 3)

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