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[Leber-Coats' disease: A retrospective study of 10 patients]
S Boucher1, C Rodier Bonifas2, T Mathis1
1Service d'ophtalmologie, hôpital Edouard-Herriot, hospices civils de Lyon, 5, place d'Arsonval, 69003 Lyon, France.
Insights
Early diagnosis of Leber-Coats disease in children improves visual outcomes. This study analyzed clinical characteristics and treatment, finding that earlier stages correlate with better prognoses.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Retinal Diseases
Background:
- Leber-Coats disease is a rare condition affecting children.
- Understanding its clinical characteristics and treatment is crucial for prognosis.
Purpose of the Study:
- To analyze the clinical characteristics and treatment of pediatric Leber-Coats disease.
- To evaluate factors influencing visual outcomes in affected children.
Main Methods:
- Retrospective study of 10 children diagnosed with Leber-Coats disease between 2004 and 2013.
- Data collected included demographics, presenting symptoms, Shields' stage, treatments, and visual outcomes.
Main Results:
- Mean age at diagnosis was 5.5 years; 90% of cases were unilateral.
- Common presenting symptoms included vision loss and strabismus; leukocoria was not observed.
- Higher Shields' stages (greater than 2A) and macular lipid exudate were associated with poorer visual prognosis.
Conclusions:
- Epidemiological data aid in characterizing Leber-Coats disease prognosis.
- Early diagnosis and intervention appear to significantly improve visual outcomes in children.
Introduction:
Retrospective study analyzing clinical characteristics and treatment of children affected by Leber-Coats' disease.
Method:
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.
Conclusion:
These epidemiological data help us to better characterize these patients' prognosis. Early diagnosis seems to improve visual outcomes.
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