title: SPECTRAL DOMAIN VERSUS SWEPT SOURCE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF THE RETINAL CAPILLARY PLEXUSES IN SICKLE CELL MACULOPATHY.
abstract: OBJECTIVE
  To compare the spectral domain and swept source optical coherence tomography angiography findings in two cases of sickle cell maculopathy.
  
  
  METHODS
  A 53-year-old man and a 24-year-old man both with sickle cell disease (hemoglobin SS) presented with no visual complaints; Humphrey visual field testing demonstrated asymptomatic paracentral scotomas that extended nasally in the involved eyes. Clinical examination and multimodal imaging including spectral domain and swept source optical coherence tomography, and spectral domain optical coherence tomography angiography and swept source optical coherence tomography angiography (Carl Zeiss Meditec Inc, Dublin, CA) were performed.
  
  
  RESULTS
  Fundus examination of both patients revealed subtle thinning of the macula. En-face swept source optical coherence tomography confirmed the extent of the thinning correlating with the functional paracentral scotomas on Humphrey visual field. Swept source optical coherence tomography B-scan revealed multiple confluent areas of inner nuclear thinning and significant temporal retinal atrophy. En-face 6 × 6-mm spectral domain optical coherence tomography angiography of the macula demonstrated greater loss of the deep capillary plexus compared with the superficial capillary plexus. Swept source optical coherence tomography angiography 12 × 12-mm imaging captured the same macular findings and loss of both plexuses temporally outside the macula.
  
  
  CONCLUSIONS
  In these two cases of sickle cell maculopathy, deep capillary plexus ischemia is more extensive within the macula, whereas both the superficial capillary plexus and deep capillary plexus are involved outside the macula likely due to the greater oxygen demands and watershed nature of these areas. Swept source optical coherence tomography angiography clearly demonstrates the angiographic extent of the disease correlating with the Humphrey visual field scotomas and confluent areas of inner nuclear atrophy.
authors.affiliation: East Bay Retina Consultants Inc, Oakland, California.
authors.forename: Jesse J
authors.initials: JJ
authors.lastname: Jung
authors.affiliation: Silicon Valley Eyecare, Santa Clara, California.
authors.forename: Michael H
authors.initials: MH
authors.lastname: Chen
authors.affiliation: East Bay Retina Consultants Inc, Oakland, California.
authors.forename: Caroline R
authors.initials: CR
authors.lastname: Frambach
authors.affiliation: East Bay Retina Consultants Inc, Oakland, California.|Department of Ophthalmology, University of California San Francisco, San Francisco, California.
authors.forename: Soraya
authors.initials: S
authors.lastname: Rofagha
authors.affiliation: East Bay Retina Consultants Inc, Oakland, California.
authors.forename: Scott S
authors.initials: SS
authors.lastname: Lee
mesh_terms: D000208:Acute Disease; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000175:diagnosis; D002196:Capillaries / Q000473:pathology; D005451:Fluorescein Angiography / Q000379:methods*; D005654:Fundus Oculi; D006801:Humans; D008266:Macula Lutea / Q000473:pathology*; D008875:Middle Aged; D012164:Retinal Diseases / Q000175:diagnosis* / Q000209:etiology; D012171:Retinal Vessels / Q000473:pathology*; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity; D055815:Young Adult