PMID- 26299697
OWN - NLM
STAT- MEDLINE
DCOM- 20160204
LR  - 20240109
IS  - 1549-4713 (Electronic)
IS  - 0161-6420 (Linking)
VI  - 122
IP  - 11
DP  - 2015 Nov
TI  - Familial Exudative Vitreoretinopathy: Spectral-Domain Optical Coherence 
      Tomography of the Vitreoretinal Interface, Retina, and Choroid.
PG  - 2270-7
LID - S0161-6420(15)00762-9 [pii]
LID - 10.1016/j.ophtha.2015.07.024 [doi]
AB  - PURPOSE: The in vivo microstructural features of familial exudative 
      vitreoretinopathy (FEVR) have not been well described. We present new anatomic 
      features of FEVR with functional and genetic correlations. DESIGN: Consecutive, 
      retrospective, observational case series. PARTICIPANTS: Patients with FEVR 
      treated from 2009 to 2014. METHODS: We identified 346 patients with FEVR. Those 
      imaged with spectral-domain optical coherence tomography (SD OCT) with or without 
      enhanced depth imaging (EDI) were included, and images were correlated with 
      best-corrected visual acuity (BCVA), widefield angiography, fundus 
      autofluorescence (AF), and wnt signaling pathway mutations. MAIN OUTCOME 
      MEASURES: Exploratory SD OCT findings and BCVA. RESULTS: A total of 225 imaging 
      sessions were acquired in 74 eyes from 41 patients. Mean age was 19.0 years. 
      Sixty-seven eyes (91%) had interpretable images, of which 50 (75%) had anomalous 
      microstructural findings; all eyes with FEVR severity of stage 2 or greater had 
      abnormalities. A broad spectrum of features were identified: various forms of 
      posterior hyaloidal organization, vitreomacular traction (VMT), vitreopapillary 
      traction, vitreo-fold traction, vitreo-laser scar adhesion, diminished foveal 
      contour, persistent fetal foveal architecture, cystoid macular edema (CME), 
      intraretinal exudates and subretinal lipid aggregation, dry or edematous radial 
      folds, and disruption of the ellipsoid zone. Mean foveal, central macular, and 
      choroidal thicknesses were 305+/-145 mum, 337+/-160 mum, and 216+/-64 mum, respectively. 
      In stages 1 to 2, greater foveal and central macular thicknesses (Rho=0.493, 
      0.544, respectively; both P<0.001) correlated with poorer BCVA, but not choroidal 
      thickness (Rho=0.032; P=0.868). Posterior hyaloidal organization (P<0.001), VMT 
      (P<0.001), CME (P<0.001), exudation (P<0.001), and disruption of the ellipsoid 
      zone (P<0.001) were associated with poorer BCVA. Disruption of the ellipsoid zone 
      (beta=0.699; P<0.001) and posterior hyaloidal organization (beta=0.289; P=0.011) 
      retained significance in multivariate modeling (R2=0.627; P<0.001). 
      Spectral-domain OCT detected all cases of angiographic edema and areas of outer 
      retinal dysfunction that were hypoautofluorescent on AF. Microstructural-genetic 
      associations were not identified. CONCLUSIONS: Spectral-domain OCT imaging 
      identified microstructural anomalies in the majority of patients with FEVR.
CI  - Copyright (c) 2015 American Academy of Ophthalmology. Published by Elsevier Inc. 
      All rights reserved.
FAU - Yonekawa, Yoshihiro
AU  - Yonekawa Y
AD  - Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan.
FAU - Thomas, Benjamin J
AU  - Thomas BJ
AD  - Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan.
FAU - Drenser, Kimberly A
AU  - Drenser KA
AD  - Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan.
FAU - Trese, Michael T
AU  - Trese MT
AD  - Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan.
FAU - Capone, Antonio Jr
AU  - Capone A Jr
AD  - Associated Retinal Consultants, William Beaumont Hospital, Royal Oak, Michigan. 
      Electronic address: acaponejr@arcpc.net.
LA  - eng
PT  - Journal Article
PT  - Observational Study
PT  - Research Support, Non-U.S. Gov't
DEP - 20150820
PL  - United States
TA  - Ophthalmology
JT  - Ophthalmology
JID - 7802443
RN  - 0 (Eye Proteins)
RN  - 0 (FZD4 protein, human)
RN  - 0 (Frizzled Receptors)
RN  - 0 (NDP protein, human)
RN  - 0 (Nerve Tissue Proteins)
RN  - 0 (TSPAN12 protein, human)
RN  - 0 (Tetraspanins)
SB  - IM
CIN - Ophthalmology. 2016 May;123(5):e36-7. PMID: 27107365
CIN - Ophthalmology. 2016 May;123(5):e36. PMID: 27107366
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Child, Preschool
MH  - Choroid/*pathology
MH  - Eye Diseases, Hereditary
MH  - Eye Proteins/genetics
MH  - Familial Exudative Vitreoretinopathies
MH  - Female
MH  - Fluorescein Angiography
MH  - Frizzled Receptors/genetics
MH  - Humans
MH  - Macular Edema/diagnosis
MH  - Male
MH  - Middle Aged
MH  - Mutation
MH  - Nerve Tissue Proteins/genetics
MH  - Retina/*pathology
MH  - Retinal Diseases/diagnosis/genetics
MH  - Retrospective Studies
MH  - Tetraspanins/genetics
MH  - Tomography, Optical Coherence/*methods
MH  - Visual Acuity/physiology
MH  - Vitreous Body/*pathology
MH  - Wnt Signaling Pathway/genetics
MH  - Young Adult
EDAT- 2015/08/25 06:00
MHDA- 2016/02/05 06:00
CRDT- 2015/08/25 06:00
PHST- 2015/04/26 00:00 [received]
PHST- 2015/07/09 00:00 [revised]
PHST- 2015/07/22 00:00 [accepted]
PHST- 2015/08/25 06:00 [entrez]
PHST- 2015/08/25 06:00 [pubmed]
PHST- 2016/02/05 06:00 [medline]
AID - S0161-6420(15)00762-9 [pii]
AID - 10.1016/j.ophtha.2015.07.024 [doi]
PST - ppublish
SO  - Ophthalmology. 2015 Nov;122(11):2270-7. doi: 10.1016/j.ophtha.2015.07.024. Epub 
      2015 Aug 20.