title: Diversity of retinal vascular anomalies in patients with familial exudative vitreoretinopathy.
abstract: OBJECTIVE
  To describe the diversity of clinical findings associated with familial exudative vitreoretinopathy (FEVR) using wide-field angiography and to update the current classification system.
  
  
  METHODS
  Retrospective case series at a single tertiary referral vitreoretinal practice.
  
  
  METHODS
  A total of 174 eyes of 87 subjects were studied.
  
  
  METHODS
  A retrospective chart review was conducted of patients with a diagnosis of FEVR between January 2011 and January 2013 at a single tertiary care retina practice. Data were collected from patient charts, including sex, gestational age at birth, age at presentation, referring diagnosis, family history, prior ocular surgery, clinical presentation, and diagnostic imaging in each eye. Inclusion criteria included clinical diagnosis of FEVR in patients referred to our clinic for evaluation of decreased vision. Patients were excluded if a diagnosis of FEVR could not be made.
  
  
  METHODS
  Clinical and angiographic findings.
  
  
  RESULTS
  A total of 87 subjects met the inclusion criteria for this study. A broad spectrum of previously undescribed clinical and angiographic findings were associated with FEVR on wide-field angiography. These findings can be grossly divided into anatomic and functional changes. Anatomic changes include aberrant circumferential peripheral vessels, venous and arterial tortuosity, late-phase disc leakage, central and peripheral telangiectasias, capillary anomalies, and capillary agenesis. Functional changes include venous-venous shunting, delayed arteriovenous transit, and delayed or absent choroidal perfusion on fluorescein angiography.
  
  
  CONCLUSIONS
  Familial exudative vitreoretinopathy has a wide range of unrecognized or under-recognized clinical and angiographic findings that are easily identified using wide-field fluorescein angiography. These novel findings have led to an update of the original FEVR classification scheme and more complete characterization of early stages of FEVR.
authors.affiliation: Department of Ophthalmology, University of Southern California, Los Angeles, California.
authors.forename: Amir H
authors.initials: AH
authors.lastname: Kashani
authors.affiliation: School of Medicine, Wayne State University, Detroit, Michigan.
authors.forename: Kevin T
authors.initials: KT
authors.lastname: Brown
authors.affiliation: Retina and Vitreous of Texas, Houston, Texas.
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Chang
authors.affiliation: Associated Retinal Consultants, P.C., Royal Oak, Michigan; Department of Ophthalmology, William Beaumont Hospital, Royal Oak, Michigan.
authors.forename: Kimberly A
authors.initials: KA
authors.lastname: Drenser
authors.affiliation: Associated Retinal Consultants, P.C., Royal Oak, Michigan; Department of Ophthalmology, William Beaumont Hospital, Royal Oak, Michigan.
authors.forename: Antonio
authors.initials: A
authors.lastname: Capone
authors.affiliation: Associated Retinal Consultants, P.C., Royal Oak, Michigan; Department of Ophthalmology, William Beaumont Hospital, Royal Oak, Michigan. Electronic address: mgjt46@aol.com.
authors.forename: Michael T
authors.initials: MT
authors.lastname: Trese
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015785:Eye Diseases, Hereditary; D000080345:Familial Exudative Vitreoretinopathies; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009898:Optic Disk / Q000098:blood supply*; D012161:Retinal Artery / Q000473:pathology*; D012164:Retinal Diseases / Q000145:classification / Q000175:diagnosis; D012166:Retinal Hemorrhage / Q000175:diagnosis*; D058456:Retinal Telangiectasis / Q000175:diagnosis*; D012169:Retinal Vein / Q000473:pathology*; D012189:Retrospective Studies