Spontaneous Rupture of the Anterior Lens Capsule Combined With Macular Hole in a Child With Alport's Syndrome

Clear lens extraction in Alport syndrome with combined anterior and posterior lenticonus or ruptured anterior lens capsule

“…[6][7][8][9][10] Clear lens extraction should not be delayed in the presence of splitting or cracking in the anterior lens capsule as surgical results in the presence of a ruptured capsule (the tears can be large, uncontrolled, and peripheral) can be less than ideal. 6 In addition, the peripheral parts of the anterior lenticular capsule have multilayer epithelial cells with an increased propensity to proliferate, 13,14 unlike the peripheral capsule in normal eyes in which the epithelial cells are in a single layer and of uniform thickness.…”

“…However, this is very rare; to our knowledge, only 6 cases have been reported in the past 5 decades. [6][7][8][9][10] We present 4 Alport-syndrome patients (7 eyes) in whom CLE with implantation of an IOL was performed for visual rehabilitation. The anterior lens capsule spontaneously ruptured in 1 patient.…”

Retinal findings in patients with Alport Syndrome: expanding the clinical spectrum

“…A wide range of ocular manifestations have been described in the setting of AS including corneal posterior polymorphous dystrophy and anterior lenticonus as well as changes in the internal limiting membrane of the retina and macular holes 7 8 9 10. The classic ocular features include a central or perimacular dot and fleck retinopathy, which occurs in 85% of affected adult males, and anterior lenticonus, which is present in 25% 11.…”

Optical Coherence Tomography Findings in Alport Syndrome

“…However, detailed data supporting this hypothesis are lacking. 4 The degenerative features noted in the maculae of our cases may be the result of AS-associated abnormalities of the basement membrane network. Multifocal dehiscences along the inner retinal surface with varying levels of progressive retinal thinning could underlie our OCT findings and may predispose the eye to MH formation.…”

“…Spontaneous rupture of the lenticonus in association with MHs has been reported in AS. 4 Interestingly, transmission electron microscopy studies of the anterior capsule in AS have shown dehiscences encompassing up to two thirds of the lens epithelia. Some investigators have suggested that ultrastructural changes of the internal limiting membrane due to abnormal Col4 play a significant role in the pathogenesis of MH, as in the anterior capsule.…”