title: Novel optical coherence tomography classification of torpedo maculopathy.
abstract: BACKGROUND
  Torpedo maculopathy is a rare condition with a twofold clinical significance. Firstly, it is a differential of atypical congenital hypertrophy of the retinal pigment epithelium. Secondly, visual field loss has been reported. We demonstrate the spectrum of structural abnormality of torpedo maculopathy as seen on optical coherence tomography, and correlate this with age of presentation, fundus autofluorescence, retinal sensitivity loss and visual field abnormality.
  
  
  METHODS
  A retrospective, observational case series.
  
  
  METHODS
  Five Australian patients seen between 2008 and 2013.
  
  
  METHODS
  Fundus photography, optical coherence tomography, fundus autofluorescence and visual field analysis. One patient underwent fluorescein angiography.
  
  
  METHODS
  Lesion appearance on each imaging modality, and visual field analysis.
  
  
  RESULTS
  We consistently observed a flat, hypopigmented lesion located in the temporal macula, with a distinctive tip pointing toward the fovea. Optical coherence tomography demonstrated variable retinochoroidal features ranging from mild outer retinal disturbance (type 1) to outer retinal cavitation (type 2). Lesion appearance on short-wave autofluorescence showed varying degrees of hypo-autofluorescence. Near-infrared autofluorescence was performed in two patients and showed a well-defined region of hypo-autofluorescence. Microperimetry showed normal sensitivity over the lesion in one patient and a dense paracentral scotoma over the temporal portion of the lesion in another. On Humphrey field analysis, only one of two patients tested had a paracentral scotoma.
  
  
  CONCLUSIONS
  Two types of torpedo maculopathy lesions are described here with unique optical coherence tomography, demographic, fundus autofluorescence and retinal sensitivity features. These may represent different stages of the same disease that evolve over several decades.
authors.affiliation: Centre for Ophthalmology and Visual Science (Incorporating the Lions Eye Institute), The University of Western Australia, Perth, Western Australia.
authors.forename: Evan N
authors.initials: EN
authors.lastname: Wong
authors.affiliation: Discipline of Clinical Ophthalmology and Eye Health, University of Sydney, Sydney, New South Wales, Australia.|Chatswood Retina Service, Retina Associates, Sydney, New South Wales, Australia.
authors.forename: Samantha
authors.initials: S
authors.lastname: Fraser-Bell
authors.affiliation: Chatswood Retina Service, Retina Associates, Sydney, New South Wales, Australia.|Australian School of Advanced Medicine, Macquarie University, Sydney, New South Wales, Australia.
authors.forename: Alex P
authors.initials: AP
authors.lastname: Hunyor
authors.affiliation: Centre for Ophthalmology and Visual Science (Incorporating the Lions Eye Institute), The University of Western Australia, Perth, Western Australia.|Department of Ophthalmology, Royal Perth Hospital, Perth, Western Australia, Australia.
authors.forename: Fred K
authors.initials: FK
authors.lastname: Chen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D006984:Hypertrophy; D008297:Male; D008875:Middle Aged; D012164:Retinal Diseases / Q000145:classification* / Q000175:diagnosis; D055213:Retinal Pigment Epithelium / Q000473:pathology*; D012189:Retrospective Studies; D012607:Scotoma / Q000175:diagnosis*; D041623:Tomography, Optical Coherence / Q000145:classification*; D014792:Visual Acuity / Q000502:physiology; D058609:Visual Field Tests; D014794:Visual Fields
keywords: fundus autofluorescence; microperimetry; optical coherence tomography; torpedo maculopathy