title: Optical coherence tomography angiography of foveal hypoplasia.
abstract: To discuss foveal development in the context of detailed retinal vasculature imaging in foveal hypoplasia using optical coherence tomography angiography.
  
  
  
  In this case series, the optical coherence tomography angiography results of four patients with idiopathic foveal hypoplasia and two patients with foveal hypoplasia secondary to oculocutaneous albinism are presented.
  
  
  
  Cases with intact visual acuity demonstrated lower grades of foveal hypoplasia on optical coherence tomography, while those with poor vision demonstrated high grades of foveal hypoplasia. The superficial retinal capillary plexus was intact in the foveal area in all cases, with no demonstrable foveal avascular zone. The deep retinal capillary plexus was absent to variable degrees in most cases, but was most persistent in those cases with reduced vision.
  
  
  
  The superficial retinal capillary plexus is present in cases with foveal hypoplasia, while the deep retinal capillary plexus is absent to varying degrees. Our findings support the hypothesis that an intact foveal avascular zone of the deep capillary plexus allows for outer retinal photoreceptor specialisation to occur unimpeded, resulting in preserved visual acuity, while this process may be inhibited by an absent deep capillary foveal avascular zone with resultant poor vision.
authors.affiliation: Department of Medical Retina, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
authors.forename: Kaivon
authors.initials: K
authors.lastname: Pakzad-Vaezi
authors.affiliation: Department of Medical Retina, Moorfields Eye Hospital NHS Foundation Trust, London, UK.|Institute of Ophthalmology, University College London, London, UK.
authors.forename: Pearse A
authors.identifier: 0000-0002-9239-745X
authors.initials: PA
authors.lastname: Keane
authors.affiliation: Department of Medical Retina, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
authors.forename: João Nobre
authors.initials: JN
authors.lastname: Cardoso
authors.affiliation: Department of Medical Retina, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
authors.forename: Catherine
authors.initials: C
authors.lastname: Egan
authors.affiliation: Department of Medical Retina, Moorfields Eye Hospital NHS Foundation Trust, London, UK.
authors.forename: Adnan
authors.initials: A
authors.lastname: Tufail
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016115:Albinism, Oculocutaneous / Q000175:diagnosis*; D005260:Female; D005451:Fluorescein Angiography / Q000379:methods*; D005584:Fovea Centralis / Q000098:blood supply*; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D012171:Retinal Vessels / Q000473:pathology*; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence / Q000379:methods*; D055815:Young Adult
keywords: Diagnostic tests/Investigation; Embryology and development; Imaging; Macula; Retina