Bilateral anterior lenticonus: Scheimpflug imaging system documentation and ultrastructural confirmation of Alport syndrome in the lens capsule.
BACKGROUND
Alport syndrome is a combination of proteinuria, hematuria, and neurosensory high-frequency deafness. Bilateral anterior lenticonus may be a late sign. Diagnosis relies on characteristic electron microscopy changes of glomerular basement membranes in renal biopsy specimens.


METHODS
A 38-year-old man was seen for progressive visual acuity loss (20/400 OU; best-corrected visual acuity, 20/60 OD and 20/50 OS). Findings from slitlamp examination included bilateral anterior lenticonus and central posterior subcapsular cataract, documented using a modified Scheimpflug imaging system. Retinal pathology was not present. On detailed questioning, a history of microhematuria and proteinuria since childhood and progressive high-frequency deafness for years were discovered. The family history was negative for nephropathies, deafness, or eye diseases. Cataract extraction rehabilitated the patient's vision.


RESULTS
Electron microscopy of a fragile capsulorhexis specimen showed typical thinned basal lamina with basement membrane disruptions.


CONCLUSIONS
Anterior lenticonus is a rare bilateral progressive developmental anomaly. More than 90% of cases are associated with Alport syndrome. For diagnosis of Alport syndrome, the presence of 3 of 4 criteria is required: family history positive for Alport syndrome, progressive intra-auricular deafness, characteristic eye anomalies, and positive findings from glomerular ultrastructural examination. We believe that ultrastructural proof of anterior lenticonus may also be provided in the lens capsule. Arch Ophthalmol. 2000;118:895-897
Junk
A K
AK
Department of Ophthalmology, Columbia University, Eye Radiation and Environmental Research Laboratory (ERERL), Room 211, 630 W 168th St, New York, NY 10032. akj19@columbia.edu
Stefani
F H
FH
Ludwig
K
K
D000328:Adult; D000869:Anterior Eye Segment / Q000473:pathology; D001485:Basement Membrane / Q000648:ultrastructure; D002386:Cataract / Q000175:diagnosis; D002387:Cataract Extraction; D003941:Diagnostic Techniques, Ophthalmological; D006801:Humans; D007903:Lens Capsule, Crystalline / Q000648:ultrastructure*; D007905:Lens Diseases / Q000175:diagnosis*; D008297:Male; D009394:Nephritis, Hereditary / Q000175:diagnosis*; D014792:Visual Acuity