Aim: To report 3 cases of Barded Biedl syndromes (BBS) with their clinical characteristics ophthalmic and therapeutic aspects. Observations:We report 3 cases of children with a BBS. It was a descriptive and analytical study. The average age was 14 years. They were 2 boys and one girl. All the patients had an eye examination, a general pediatric examination and paraclinical exams. According to BEALES criteria, they all presented a BBS. The treatment was: diet advice; insulin therapy; corrective lenses; vitamin therapy A and E; renal protection, clinical and biological monitoring especially renal, ophthalmological and blood sugar monitoring. A child (case 3) died after 18 days in hospital of renal failure. Discussion & Conclusion:BBS is a rare genetic disease. It is a part of a large group of syndromes called ciliopathies. The ophthalmological involvement determines the functional prognosis and the renal impairment, affects the vital prognosis. An early and correct treatment is necessary. The genetic counselling plays an important role in the prevention of this disease.

Aim: To describe clinical and prognostic characteristics of irido-chorio-retinal colobomas through 3 cases. Observations:The average age of the three patients was 12,66 years. The sex-ratio was 0,5 with female predominance (66,7%). The three patients were admitted for a severe visual impairment and automated refraction test did not work for the two eyes. They all presented a nuclear cataract and an inferior iris coloboma for the two eyes. All the patients had bilateral chorio-retinal colobomas including papilla. The pediatric examination found one case of cryptorchidism but was normal for the two other patients. B-scan ocular ultrasound found a chorio-retinal defect facing the optical nerve for one patient. We managed the low vision and prescribed sunglasses to avoid photophobia. A cataract surgery was realized, and we also gave a genetic counselling. No patient had a retinal detachment. Discussion & Conclusion:Chorio-retinal coloboma is a rare and serious disease, because of its evolution and complications. It requires early, appropriate, and multidisciplinary treatment.