title: Familial exudative vitreoretinopathy (FEVR). Clinical profile and management.
abstract: OBJECTIVE
  To report our experience with the diagnosis and management of Familial Exudative Vitreoretinopathy (FEVR) in a predominantly older Indian population..
  
  
  METHODS
  This prospective interventional non-comparative case series included 38 patients of FEVR and their 23 family members. The diagnosis was established by clinical examination, fluorescein angiography and family screening. Prophylactic photocoagulation/cryotherapy or surgical treatment was done depending on the severity of the disease.
  
  
  RESULTS
  The mean age of the patients was 23.6 years. The fundus/fluorescein angiographic findings in 116 eyes of our 61 patients (6 eyes phthisical) were as follows: forty eight (41.4%) eyes had only peripheral avascular zone, 8 (6.9%) eyes had peripheral new vessels, and 35 (30.1%) eyes had retinal detachments (RD)--10 (8.6%) exudative, 5 (4.3%) tractional and 20 (17.2%) rhegmatogenous. Prophylactic photocoagulation or cryotherapy was done in 34 eyes for retinal holes, local exudative detachments and bleeding new vessels. All the eyes retained stable vision over a mean follow-up of 16 months. Only 14 RDs were suitable for surgery: scleral buckling, vitrectomy or both. The reattachment rate was 85.7% (12 of 14) and the best-corrected visual acuity (BCVA) improved to 5/60 or better in 50% of these eyes over a 2-year follow-up.
  
  
  CONCLUSIONS
  FEVR appears to be more common than reported. Timely diagnosis and intervention is essential in view of the lifelong progression of the disease, late exacerbations, frequent involvement of family members, and poor surgical results. A high index of suspicion, family screening and early prophylaxis are recommended to prevent avoidable blindness from this underdiagnosed disease.
authors.affiliation: Retina Vitreous Service, Aravind Eye Hospital & Postgraduate Institute of Ophthalmology, Madurai, Tamil Nadu, India. daksh@aravind.org
authors.forename: Dhananjay
authors.initials: D
authors.lastname: Shukla
authors.forename: Jatinder
authors.initials: J
authors.lastname: Singh
authors.forename: G
authors.initials: G
authors.lastname: Sudheer
authors.forename: Manoj
authors.initials: M
authors.lastname: Soman
authors.forename: Rajesh K
authors.initials: RK
authors.lastname: John
authors.forename: Kim
authors.initials: K
authors.lastname: Ramasamy
authors.forename: Namperumalsamy
authors.initials: N
authors.lastname: Perumalsamy
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D017679:Cryotherapy; D005122:Exudates and Transudates; D015785:Eye Diseases, Hereditary / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D017075:Laser Coagulation; D008297:Male; D008875:Middle Aged; D010539:Permeability; D011446:Prospective Studies; D012163:Retinal Detachment / Q000175:diagnosis / Q000601:surgery; D012164:Retinal Diseases / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D012166:Retinal Hemorrhage / Q000175:diagnosis / Q000601:surgery; D012591:Scleral Buckling; D014821:Vitrectomy; D014822:Vitreous Body / Q000473:pathology*