title: Republished review. [corrected] Late-onset retinal macular degeneration: clinical insights into an inherited retinal degeneration.
abstract: OBJECTIVE
  This study describes, in detail, the phenotype of late-onset retinal macular degeneration (L-ORMD) an inherited condition affecting both the retina and anterior segment. A staging based on clinical characteristics is proposed, and the relevance of this condition to current understanding of age-related macular degeneration is discussed.
  
  
  METHODS
  A systematic review of the literature regarding this condition supports a detailed description of the natural history. Clinical experiences in identifying, monitoring and managing patients are also presented.
  
  
  RESULTS
  L-ORMD is a rare fully penetrant autosomal dominant condition resulting from a mutation in the C1QTNF5 gene on chromosome 11. Affected individuals develop bilateral loss of vision, dark-adaptation abnormalities, fundus drusen-like yellow spots, midperipheral pigmentation, choroidal neovascularisation, chorioretinal atrophy and long anteriorly inserted lens zonules. Patients may benefit from treatment with high-dose vitamin A.
  
  
  CONCLUSIONS
  Raised awareness of L-ORMD should lead to earlier diagnosis and improved care for patients. New antivascular endothelial growth factor treatment may provide a new possibility for management. A deeper insight into molecular and genetic mechanisms of L-ORMD may suggest avenues to explore new treatments of this disorder.
authors.affiliation: Princess Alexandra Eye Pavilion, Chalmers Street, Edinburgh EH3 9HA, UK. shyamanga@aol.com
authors.forename: S
authors.initials: S
authors.lastname: Borooah
authors.forename: C
authors.initials: C
authors.lastname: Collins
authors.forename: A
authors.initials: A
authors.lastname: Wright
authors.forename: B
authors.initials: B
authors.lastname: Dhillon