Document Detail

Clinical presentation of familial exudative vitreoretinopathy.
MedLine Citation:
PMID:  21868098     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To describe the clinical characteristics, staging and presentation of patients with familial exudative vitreoretinopathy (FEVR) in our clinical practice over the last 25 years.
DESIGN: Case series, retrospective review.
PARTICIPANTS: We included 273 eyes of 145 patients.
METHODS: Data collected from charts included gender, gestational age at birth, birthweight, age at presentation, referring diagnosis, family history, prior ocular surgery, and clinical presentation in each eye. Eyes with invasive posterior segment procedures before initial presentation were excluded.
MAIN OUTCOME MEASURES: Demographics on presentation and clinical staging.
RESULTS: Patients were slightly male predominant (57%) with a mean birthweight of 2.80 kg (range, 740 g-4.76 kg), mean gestational age of 37.8 weeks (range, 25-42), and mean age at presentation of almost 6 years (range, <1 month-49 years). A positive family history of FEVR was obtained in 18% of patients. A positive family history for ocular disease consistent with but not diagnosed as FEVR was obtained in an additional 19%. Stage 1 FEVR was identified in 45 eyes, stage 2 in 33 eyes, stage 3 in 42 eyes, stage 4 in 89 eyes, and stage 5 in 44 eyes. Radial retinal folds were seen in 77 eyes, 64 of which were temporal or inferotemporal in location.
CONCLUSIONS: The FEVR patient population is remarkable for the wide range of age at presentation, gestational age, and birthweight. Although a positive family history on presentation may support the diagnosis of FEVR, a negative family history is of little help. The majority of retinal folds extended radially in the temporal quadrants, but radial folds were seen in almost all quadrants. Fellow eyes demonstrated a wide variation in symmetry. The presentation of FEVR may mimic the presentation of other pediatric and adult vitreoretinal disorders, and careful examination is often crucial in making the diagnosis of FEVR.
FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any of the materials discussed in this article.
Tushar M Ranchod; Lawrence Y Ho; Kimberly A Drenser; Antonio Capone; Michael T Trese
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Publication Detail:
Type:  Journal Article     Date:  2011-08-25
Journal Detail:
Title:  Ophthalmology     Volume:  118     ISSN:  1549-4713     ISO Abbreviation:  Ophthalmology     Publication Date:  2011 Oct 
Date Detail:
Created Date:  2011-10-04     Completed Date:  2011-11-30     Revised Date:  2012-06-13    
Medline Journal Info:
Nlm Unique ID:  7802443     Medline TA:  Ophthalmology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2070-5     Citation Subset:  IM    
Copyright Information:
Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
Associated Retinal Consultants, Royal Oak, MI 48073, USA.
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MeSH Terms
Birth Weight
Child, Preschool
Exudates and Transudates
Eye Diseases / diagnosis*,  genetics,  surgery
Gestational Age
Infant, Newborn
Middle Aged
Retinal Diseases / diagnosis*,  genetics,  surgery
Retrospective Studies
Scleral Buckling
Vitreous Body / pathology*
Comment In:
Ophthalmology. 2012 May;119(5):1093   [PMID:  22551617 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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