PMID- 26284746
OWN - NLM
STAT- MEDLINE
DCOM- 20160308
LR  - 20151125
IS  - 1879-1891 (Electronic)
IS  - 0002-9394 (Linking)
VI  - 160
IP  - 5
DP  - 2015 Nov
TI  - Spectral-Domain Optical Coherence Tomography in Wagner Syndrome: Characterization 
      of Vitreoretinal Interface and Foveal Changes.
PG  - 1065-1072.e1
LID - S0002-9394(15)00504-8 [pii]
LID - 10.1016/j.ajo.2015.08.012 [doi]
AB  - PURPOSE: To evaluate the spectrum of morphologic abnormalities in patients with 
      Wagner syndrome by spectral-domain optical coherence tomography (SD OCT). DESIGN: 
      Retrospective comparative case study. METHODS: Institutional study of patients 
      entered into the French Vitreoretinopathy Study Group database. Twelve eyes of 9 
      patients from 3 unrelated families with genetically confirmed Wagner syndrome and 
      28 eyes from 15 age- and sex-matched healthy family controls were scanned by SD 
      OCT. Morphology and layer thickness of the total retina, inner retinal layers, 
      outer retinal layers, and photoreceptor layer at different degrees of 
      eccentricity from the fovea were compared between the 2 groups. RESULTS: A thick 
      multilayered membrane adherent to the perifovea but completely detached from the 
      fovea, thus forming a bridge over the foveal pit, was observed in 84% of eyes 
      from patients with Wagner syndrome. At the equatorial area, SD OCT imaging 
      allowed visualization of the architecture of an avascular vitreous veil with 
      localized retinal traction. Most retinal layers were significantly thinner in 
      patients with Wagner syndrome compared to the control group, except at the foveal 
      center where abnormal persistence of 1 or more inner retinal layers could be 
      observed. CONCLUSION: SD OCT provides better structural insight into the range of 
      retinal defects at the vitreoretinal interface and fovea, which is not only 
      useful for improving diagnosis and management, but also for understanding the 
      pathogenesis of Wagner syndrome.
CI  - Copyright (c) 2015 Elsevier Inc. All rights reserved.
FAU - Rothschild, Pierre-Raphael
AU  - Rothschild PR
AD  - Assistance Publique-Hopitaux de Paris, Groupe Hospitalier Cochin-Hotel-Dieu, 
      Service d'ophtalmologie, Universite Paris Descartes, Sorbonne Paris Cite, Paris, 
      France; Institut National de la Sante et de la Recherche Medicale, Centre de 
      Recherche des Cordeliers, Unite Mixte de Recherche 1138, equipe 17, Paris, 
      France. Electronic address: rothschild.pr@gmail.com.
FAU - Burin-des-Roziers, Cyril
AU  - Burin-des-Roziers C
AD  - Institut National de la Sante et de la Recherche Medicale, Unite Mixte de 
      Recherche 1163, Institut Imagine, Laboratoire de Genetique Ophtalmologique, 
      Universite Paris Descartes, Sorbonne Paris Cite, Paris, France.
FAU - Audo, Isabelle
AU  - Audo I
AD  - Institut National de la Sante et de la Recherche Medicale, Unite Mixte de 
      Recherche _S968, Paris, France; Centre National de la Recherche Scientifique, 
      Unite Mixte de Recherche 7210, Paris, France; Universite Pierre et Marie Curie 
      Paris 6, Institut de la Vision, Paris, France; Centre Maladies Rares/Centre 
      d'Investigations Cliniques 503 Institut National de la Sante et de la Recherche 
      Medicale, Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, Paris, 
      France; Department of Molecular Genetics, Institute of Ophthalmology, London, 
      United Kingdom.
FAU - Nedelec, Brigitte
AU  - Nedelec B
AD  - Institut National de la Sante et de la Recherche Medicale, Unite Mixte de 
      Recherche 1163, Institut Imagine, Laboratoire de Genetique Ophtalmologique, 
      Universite Paris Descartes, Sorbonne Paris Cite, Paris, France.
FAU - Valleix, Sophie
AU  - Valleix S
AD  - Institut National de la Sante et de la Recherche Medicale, Centre de Recherche 
      des Cordeliers, Unite Mixte de Recherche 1138, equipe 17, Paris, France; 
      Universite Paris-Descartes, Sorbonne Paris Cite, Assistance Publique-Hopitaux de 
      Paris, Laboratoire de Biologie et Genetique Moleculaire, Hopital Cochin, Paris, 
      France.
FAU - Brezin, Antoine P
AU  - Brezin AP
AD  - Assistance Publique-Hopitaux de Paris, Groupe Hospitalier Cochin-Hotel-Dieu, 
      Service d'ophtalmologie, Universite Paris Descartes, Sorbonne Paris Cite, Paris, 
      France.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20150815
PL  - United States
TA  - Am J Ophthalmol
JT  - American journal of ophthalmology
JID - 0370500
RN  - 126968-45-4 (Versicans)
RN  - Hyaloideoretinal degeneration of Wagner
SB  - IM
MH  - Adult
MH  - Female
MH  - Fovea Centralis/*pathology
MH  - Humans
MH  - Male
MH  - Retinal Degeneration/*diagnosis/physiopathology
MH  - Retrospective Studies
MH  - Tomography, Optical Coherence/*methods
MH  - Versicans/*deficiency
MH  - *Visual Acuity
MH  - Vitreous Body/*pathology
EDAT- 2015/08/19 06:00
MHDA- 2016/03/10 06:00
CRDT- 2015/08/19 06:00
PHST- 2015/06/03 00:00 [received]
PHST- 2015/08/07 00:00 [revised]
PHST- 2015/08/08 00:00 [accepted]
PHST- 2015/08/19 06:00 [entrez]
PHST- 2015/08/19 06:00 [pubmed]
PHST- 2016/03/10 06:00 [medline]
AID - S0002-9394(15)00504-8 [pii]
AID - 10.1016/j.ajo.2015.08.012 [doi]
PST - ppublish
SO  - Am J Ophthalmol. 2015 Nov;160(5):1065-1072.e1. doi: 10.1016/j.ajo.2015.08.012. 
      Epub 2015 Aug 15.