title: [Leber-Coats' disease: A retrospective study of 10 patients].
abstract: BACKGROUND
  Retrospective study analyzing clinical characteristics and treatment of children affected by Leber-Coats' disease.
  
  
  METHODS
  Children with Leber-Coats' disease seen in Edouard-Herriot Hospital, Lyon, between January 2004 and September 2013 were included. The following clinical characteristics were reported: sex, age at diagnosis, presenting symptom, Shields' stage, treatment used, and visual outcomes.
  
  
  RESULTS
  Ten children were included in our study. Mean age at diagnosis was 5.5 years. The disease was unilateral in 9/10 cases. The presenting symptom was loss of vision in 7/10 cases, strabismus in 3/10 cases, and microphthalmos in 1 case. The diagnosis was made fortuitously during a follow-up of familial congenital cataract in 2 patients. No cases of leukocoria were seen. Shields' stages were distributed as follows: stage 1, 2A and 2B: 1 case each, stage 3A1: 7 cases, stage>3A1: no cases. Laser photocoagulation was used up to stage 2B, combined with cryotherapy in stage 3A. Stage 1 and 2A were associated with relatively good final visual acuity. Significant macular lipid exudate (stage>2A) was associated with a worse prognosis.
  
  
  CONCLUSIONS
  These epidemiological data help us to better characterize these patients' prognosis. Early diagnosis seems to improve visual outcomes.
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France. Electronic address: bouchersophie@live.fr.
authors: S
authors: S
authors: Boucher
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France; Faculté de médecine Lyon Est, 8, avenue Rockefeller, 69003 Lyon, France.
authors: C
authors: C
authors: Rodier Bonifas
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France.
authors: T
authors: T
authors: Mathis
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France.
authors: H
authors: H
authors: Janin-Manificat
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France.
authors: J
authors: J
authors: Fleury
authors: Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France; Faculté de médecine Lyon Est, 8, avenue Rockefeller, 69003 Lyon, France; Faculté de médecine Lyon Sud, 165, chemin du Petit-Revoyet, BP 12, 69921 Oullins, France.
authors: C
authors: C
authors: Burillon
mesh_terms: D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D017679:Cryotherapy; D005260:Female; D006801:Humans; D007223:Infant; D017075:Laser Coagulation; D008297:Male; D029242:Optic Atrophy, Hereditary, Leber / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D058456:Retinal Telangiectasis / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D012189:Retrospective Studies; D014792:Visual Acuity
keywords: Anévrisme miliaire de Leber; Coats’ disease; Cryoapplication; Cryotherapy; Leber multiple miliary aneurysms; Maladie de Leber-Coats; Photocoagulation; Telangiectasia; Télangiectasies