title: Correlation of visual acuity with foveal hypoplasia grading by optical coherence tomography in albinism.
abstract: OBJECTIVE
  To develop a new foveal hypoplasia grading system using optical coherence tomography (OCT) and to determine the correlations of visual acuity to the iris transillumination, macular transparency, and foveal hypoplasia grades, respectively, in young albinism patients.
  
  
  METHODS
  Observational case series.
  
  
  METHODS
  Thirteen patients who were clinically diagnosed with oculocutaneous albinism.
  
  
  METHODS
  The authors obtained refraction and correction for participants, who then underwent anterior segment photography and fundus photography. Foveal hypoplasia was evaluated using a fast macular cross-hair scan with StratusOCT (Carl Zeiss Meditec, Dublin, CA). The authors developed a new grading system based on foveal hyporeflectivity, the degree of choroidal transillumination, the presence of the tram-tract sign, and foveal depression as determined from OCT images. Correlations of Snellen visual acuity to the iris transillumination, macular transparency, and foveal hypoplasia grades were calculated, and areas under receiver operating characteristic curves (AROCs) were measured to evaluate the predictive value of foveal hypoplasia grading using OCT.
  
  
  METHODS
  The AROCs of foveal hypoplasia grading by OCT.
  
  
  RESULTS
  Thirteen patients were enrolled. Correlations of visual acuity to the iris transillumination (P<0.001), macular transparency (P = 0.014), and foveal hypoplasia (P = 0.004) grades were significant. The AROC values for foveal hypoplasia measurement by OCT, iris transillumination, and macular transparency measurements were 0.98, 0.91, and 0.87, respectively.
  
  
  CONCLUSIONS
  The prognostic value of foveal hypoplasia as assessed by OCT in young patients with albinism is superior to grades based on iris transillumination or macular transparency.
authors.affiliation: Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Je Hyun
authors.initials: JH
authors.lastname: Seo
authors.forename: Young Suk
authors.initials: YS
authors.lastname: Yu
authors.forename: Jeong Hun
authors.initials: JH
authors.lastname: Kim
authors.forename: Ho Kyung
authors.initials: HK
authors.lastname: Choung
authors.forename: Jang Won
authors.initials: JW
authors.lastname: Heo
authors.forename: Seong-Joon
authors.initials: SJ
authors.lastname: Kim
mesh_terms: D000293:Adolescent; D000328:Adult; D016117:Albinism, Ocular / Q000175:diagnosis; D016115:Albinism, Oculocutaneous / Q000175:diagnosis*; D002648:Child; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000175:diagnosis*; D005260:Female; D005584:Fovea Centralis / Q000002:abnormalities*; D006801:Humans; D007499:Iris Diseases / Q000175:diagnosis*; D008297:Male; D010781:Photography; D010857:Pigment Epithelium of Eye / Q000473:pathology; D012372:ROC Curve; D041623:Tomography, Optical Coherence / Q000379:methods*; D014786:Vision Disorders / Q000175:diagnosis*; D014792:Visual Acuity