title: Surgical outcomes of progressive tractional retinal detachment associated with familial exudative vitreoretinopathy.
abstract: OBJECTIVE
  To evaluate various surgeries for treating retinal detachment (RD) associated with familial exudative vitreoretinopathy (FEVR).
  
  
  METHODS
  Retrospective, interventional case series.
  
  
  METHODS
  The charts of 22 patients who underwent surgery were reviewed. A complete ophthalmic examination was performed including wide-field fundus images with fluorescein angiography. The primary and secondary outcomes were fundus features (vascular activity of the fibrovascular proliferation and extent of tractional RD) and visual acuity (VA), respectively.
  
  
  RESULTS
  Thirty-one eyes were included (12 eyes underwent scleral buckling, 1 scleral buckling and vitrectomy, 7 vitrectomy alone, and 11 lensectomy and vitrectomy). Twenty-six eyes were reattached during 1 surgery. Scleral buckling resulted in cessation of fibrovascular proliferation and retinal reattachment; only 1 of these eyes required vitrectomy. Lens-sparing vitrectomy resulted in stabilized fibrovascular proliferation and retinal reattachment. Vitrectomy with lensectomy did not achieve retinal reattachment in 4 eyes. Fibrovascular proliferation has a rich vascular component in patients younger than 3 years, and collagen fibers were present mainly with more advanced age. The postoperative VA improved in 5 of 8 eyes examined, was unchanged in 1 eye, and decreased in 2 eyes with macular involvement.
  
  
  CONCLUSIONS
  FEVR-induced RDs are highly variable and require careful preoperative evaluation to determine the best surgical procedure. Vitrectomy with release of posterior traction is essential in younger patients with vascularly active fibrovascular proliferation, whereas scleral buckling may be important for cases with peripheral traction anterior to the equator. In all cases, peripheral thermal treatment applied to all ischemic areas contributed to reduced peripheral neovascularization.
authors.affiliation: Department of Ophthalmology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Yamane
authors.affiliation: Department of Ophthalmology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Yokoi
authors.affiliation: Department of Ophthalmology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Nakayama
authors.affiliation: Department of Ophthalmology, National Center for Child Health and Development, Tokyo, Japan.
authors.forename: Sachiko
authors.initials: S
authors.lastname: Nishina
authors.affiliation: Department of Ophthalmology, National Center for Child Health and Development, Tokyo, Japan. Electronic address: azuma-n@ncchd.go.jp.
authors.forename: Noriyuki
authors.initials: N
authors.lastname: Azuma
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015785:Eye Diseases, Hereditary; D000080345:Familial Exudative Vitreoretinopathies; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D005654:Fundus Oculi; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012160:Retina / Q000473:pathology / Q000601:surgery*; D012163:Retinal Detachment / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D012164:Retinal Diseases / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D012189:Retrospective Studies; D012591:Scleral Buckling; D016896:Treatment Outcome; D014792:Visual Acuity; D014821:Vitrectomy