title: Peripheral nonperfusion and tractional retinal detachment associated with congenital optic nerve anomalies.
abstract: OBJECTIVE
  To report an association of congenital optic nerve anomalies with peripheral retina nonperfusion and to describe the clinical manifestations and treatment.
  
  
  METHODS
  Retrospective, observational case series.
  
  
  METHODS
  Fifteen patients with congenital optic nerve anomalies referred for pediatric retina consultation were studied. Sixteen eyes of 9 patients with optic nerve hypoplasia and 8 eyes of 6 patients with other congenital optic nerve anomalies, including optic nerve coloboma, morning glory disc, and peripapillary staphyloma, were included.
  
  
  METHODS
  All patients underwent examinations under anesthesia. Wide-angle retina photographs and fluorescein angiograms were reviewed. The severity of nonperfusion was graded. The presence of fibrovascular proliferation (FP), vitreous hemorrhage (VH), and tractional retinal detachment (TRD) were documented. Anatomic outcome after treatment was recorded.
  
  
  METHODS
  Severity of nonperfusion, occurrence of secondary complications, and the anatomic outcome of patients who underwent laser treatment.
  
  
  RESULTS
  In patients with optic nerve hypoplasia, 12 of 16 eyes (75%) had severe peripheral nonperfusion, 12 of 16 eyes (75%) had FP, 3 of 16 eyes (19%) had VH, and 10 of 16 eyes (63%) had TRD. Six of these eyes with severe nonperfusion received laser photocoagulation to the nonperfused retina; laser-treated retinas remained attached in all 6 eyes. In patients with the other optic nerve anomalies, 7 of 8 eyes (88%) had mild to moderate nonperfusion, 2 of 8 eyes (25%) had FP, 1 of 8 eyes (12%) had VH, and 2 of 8 eyes (25%) had TRD. Six of 9 patients (67%) with optic nerve hypoplasia and 1 of 6 patients (17%) with other anomalies had a coexisting congenital brain disease.
  
  
  CONCLUSIONS
  Congenital optic nerve anomalies may be associated with peripheral retina nonperfusion and the secondary complications of FP, VH, and TRD. In this select group of patients, the nonperfusion associated with optic nerve hypoplasia seemed to be more severe and associated more frequently with secondary complications. Peripheral retina examination in eyes with optic nerve anomalies may identify nonperfusion or FP. Laser treatment of the avascular retina may have helped prevent complications from proliferative retinopathy in eyes clinically observed to have progressed or considered at risk for progression to proliferative retinopathy.
authors.affiliation: Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois; Retina Consultants, Ltd., Des Plaines, Illinois. Electronic address: michaelj.shapiro@gmail.com.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Shapiro
authors.affiliation: Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois.
authors.forename: Clement C
authors.initials: CC
authors.lastname: Chow
authors.affiliation: Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois; Retina Consultants, Ltd., Des Plaines, Illinois; Section of Ophthalmology and Section of Surgery, University of Chicago, Chicago, Illinois.
authors.forename: Michael P
authors.initials: MP
authors.lastname: Blair
authors.affiliation: Ophthalmic Consultants of Long Island, Westbury, New York.
authors.forename: Daniel F
authors.initials: DF
authors.lastname: Kiernan
authors.affiliation: Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois.
authors.forename: Lawrence M
authors.initials: LM
authors.lastname: Kaufman
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003103:Coloboma / Q000150:complications*; D005260:Female; D005355:Fibrosis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009900:Optic Nerve / Q000002:abnormalities*; D012163:Retinal Detachment / Q000209:etiology*; D012171:Retinal Vessels / Q000473:pathology; D012189:Retrospective Studies; D014823:Vitreous Hemorrhage / Q000209:etiology; D055815:Young Adult