title: Outer retinal deformity detected by optical coherence tomography in eyes with foveal hypoplasia.
abstract: OBJECTIVE
  To investigate the relationship between vision and foveal maturity, especially in foveal hypoplasia exhibiting severe structural immaturity.
  
  
  METHODS
  This retrospective observational case series included 42 eyes of 23 patients (mean age, 7.0 ± 5.0 years; 9 patients with foveal hypoplasia as an isolated entity and 14 patients with aniridia). A complete ophthalmic examination included measurement of best-corrected visual acuity (BCVA) and spectral-domain optical coherence tomography (SD-OCT). The sensory retina, ganglion cell complex (GCC), and outer retinal layers, including Henle's fiber layer (HFL), were measured and analyzed.
  
  
  RESULTS
  Using SD-OCT images, eyes were classified as having a differentiated (6 eyes), diffuse (19 eyes), or no HFL (17 eyes), based on the appearance of the HFL around the foveal region. The logMAR BCVA was significantly worse (p < 0.0001) in eyes with diffuse HFL and those with no HFL than in those with differentiated HFL. Outer retinal thickness (outer plexiform layer + HFL + outer nuclear layer) was less (p = 0.0051) in eyes with no HFL than in those with differentiated HFL. The logMAR BCVA, GCC thickness, and outer retinal thickness in eyes with foveal hypoplasia with aniridia were significantly worse (p = 0.0083), thicker (p = 0.0039), and thinner (p = 0.0001), respectively, than in eyes with foveal hypoplasia as an isolated entity.
  
  
  CONCLUSIONS
  In eyes with foveal hypoplasia with severe structural immaturity, diffuse HFL or no HFL was associated with worse vision. There was greater foveal immaturity in eyes with foveal hypoplasia with aniridia compared with foveal hypoplasia as an isolated entity.
authors.affiliation: Department of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.|Department of Ophthalmology, The Jikei University School of Medicine, Tokyo, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Katagiri
authors.affiliation: Department of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yokoi
authors.affiliation: Division of Biostatistics, Center for Clinical Research and Development, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Masashi
authors.initials: M
authors.lastname: Mikami
authors.affiliation: Department of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Nishina
authors.affiliation: Department of Ophthalmology and Laboratory for Visual Science, National Center for Child Health and Development, 2-10-1, Okura, Setagaya-ku, Tokyo, 157-8535, Japan. azuma-n@ncchd.go.jp.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Azuma
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005124:Eye Abnormalities / Q000175:diagnosis*; D005260:Female; D005584:Fovea Centralis / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008297:Male; D055214:Retinal Photoreceptor Cell Outer Segment / Q000473:pathology*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity
keywords: Aniridia; Foveal hypoplasia; Henle’s fiber layer; Isolated; Optical coherence tomography