Lens and vitreous
Alport syndrome

Author: Nat Pernick, M.D. (see Authors page)

Revised: 10 May 2018, last major update February 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Alport syndrome [title] review[ptyp]

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Cite this page: Pernick, N. Lens and vitreous: Alport syndrome. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyelensalport.html. Accessed March 23rd, 2019.
Definition / general
  • Also called hereditary nephritis
  • Inherited disorder of type IV collagen in basement membranes
  • Hematuria, progressive nephritis with proteinuria and declining renal function, deafness (55%), ocular abnormalities (15 - 30%)
Ocular abnormalities
  • Anterior lenticonus (forward central protrusion of anterior surface of lens due to weakness in type IV collagen, relatively specific for Alport)
  • Also keratoconus, spherophakia (small, spherical lens), myopia, retinal flecks, cataracts, retinitis pigmentosa, amaurosis (blindness without an apparent ocular cause)
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