title: OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY OF CHOROIDAL NEOVASCULARIZATION IN FOUR INHERITED RETINAL DYSTROPHIES.
abstract: OBJECTIVE
  To demonstrate the clinical utility of optical coherence tomography (OCT) angiography (OCT-A) in inherited retinal dystrophies complicated by choroidal neovascularization (CNV).
  
  
  METHODS
  Optical coherence tomography angiography and structural OCT were performed using a 70-kHz spectral domain OCT system using the split-spectrum amplitude-decorrelation angiography algorithm. Semiautomated image processing software was used to segment and measure the CNV.
  
  
  RESULTS
  Four participants were enrolled to study the following inherited retinal dystrophies complicated by CNV: choroideremia, EFEMP1-related retinopathy, Best vitelliform dystrophy, and adult-onset vitelliform dystrophy. Interpretation of fluorescein angiography was difficult because of abnormal retinal architecture but suggested the presence of CNV. Structural OCT revealed subretinal or subretinal pigment epithelium fibrovascular tissue, within which flow signal was observed on OCT-A. The CNV morphology varied from dense capillary networks in active lesions to asymptomatic large caliber loops. Baseline CNV vessel areas ranged from 0.07 mm to 0.98 mm. After treatment with intravitreal bevacizumab, the CNV in choroideremia decreased in the vessel area then rebounded, whereas the one in EFEMP1-related retinopathy remained largely unchanged.
  
  
  CONCLUSIONS
  Optical coherence tomography angiography enables morphologic characterization and quantification of CNV in patients with retinal dystrophies despite distorted retinal architecture, can assess response to treatment, and may facilitate differentiation between active and regressed lesions.
authors.affiliation: Casey Eye Institute, Department of Ophthalmology, Oregon Health & Science University, Portland, Oregon.
authors.forename: Rachel C
authors.initials: RC
authors.lastname: Patel
authors.forename: Simon S
authors.initials: SS
authors.lastname: Gao
authors.forename: Miao
authors.initials: M
authors.lastname: Zhang
authors.forename: Talal
authors.initials: T
authors.lastname: Alabduljalil
authors.forename: Abdullah
authors.initials: A
authors.lastname: Al-Qahtani
authors.forename: Richard G
authors.initials: RG
authors.lastname: Weleber
authors.forename: Paul
authors.initials: P
authors.lastname: Yang
authors.forename: Yali
authors.initials: Y
authors.lastname: Jia
authors.forename: David
authors.initials: D
authors.lastname: Huang
authors.forename: Mark E
authors.initials: ME
authors.lastname: Pennesi
mesh_terms: D000293:Adolescent; D000368:Aged; D020256:Choroidal Neovascularization / Q000000981:diagnostic imaging*; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D008297:Male; D008875:Middle Aged; D058499:Retinal Dystrophies / Q000150:complications / Q000000981:diagnostic imaging*; D041623:Tomography, Optical Coherence / Q000379:methods*; D014792:Visual Acuity