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Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and
histopathological correlation after successful clear lens extraction
#MMPMID24969069
Sonarkhan S
; Ramappa M
; Chaurasia S
; Mulay K
BMJ Case Rep
2014[Jun]; 2014
(?): ? PMID24969069
show ga
A 29-year-old woman presented with symptoms of gradual, progressive, painless
diminution of vision in both eyes for past 8-10?years. On examination,
uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye.
Anterior segment examination was essentially normal except for a conical
protrusion in the pupillary axis of the anterior lens capsule with a clear
underlying lens. During surgery, central protruded fragile lens capsule was
meticulously handled to create well-centered continuous curvilinear capsulorhexis
by using paediatric rhexis forceps. Histopathology of excised capsule showed
markedly thinned lens capsule with normal epithelium. Electron microscopy of the
anterior lens capsule showed multiple linear and irregular zones of dehiscence.
Few of these had fibrillar, irregular electron-dense material and vacuoles; and
adjacent cells were irregular suggestive of Alport syndrome. Postoperative course
was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction
is an effective means of rehabilitating visually symptomatic anterior lenticonus
of Alport syndrome.
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