[Unilateral congenital cataracts removed early: long-term refractive changes]. / Cataractes congénitales unilatérales opérées précocement: devenir réfractif à long terme.

PURPOSE: Predict long-term refractive changes after primary lens implantation for unilateral congenital cataract in order to know the intraocular lens power to use to obtain best functional results. METHODS: This retrospective study analyzed 53 children with unilateral congenital cataract operated on with primary intraocular lens implantation, with a mean follow-up of 6.8 years. The mean age at surgery was 2.98 years. RESULTS: The mean myopic shift per year was -0.83 D/year. Children operated on before the age of 2 years had a mean myopic shift of -9.15 D, and others presented -2.13 D. The variability of myopic shift decreased with age. The mean visual acuity was +0.25 logMAR. Four intraocular lenses were changed because of substantial anisometropia. CONCLUSION: Early surgery with primary implantation and intensive amblyopia treatment provide good long-term visual results. Long-term refractive changes of very young children operated on for unilateral congenital cataract is extremely variable. Changing the intraocular lens is conceivable when substantial anisometropia slows down amblyopia rehabilitation.

[A murine model of transitory optic neuropathy based on small interference RNA-induced OPA1 silencing in vivo (gene mutation associated with Kjer's disease)]. / Modèle murin de neuropathie optique transitoire par inhibition in vivo de l'expression d'OPA1 (gène impliqué dans la maladie de Kjer).

PURPOSE: Developing a murine model of OPA1 linked optic neuropathy. METHODS: Intravitreal injections (in adult C57BL/6J mice) of small interference RNA (siRNA) specific to OPA1 were performed in the left eye. The right eye served as control, injected with nonspecific siRNA (siRNA scramble). Visual evoked potentials and flash electroretinograms were performed 5 and 12 days after injection. Three months after injection, microscopy of optic nerve sections was performed. RESULTS: The electrophysiological tests showed a significant reduction in the VEP when the siRNA OPA1-injected eye was stimulated, compared with the control eye injected with siRNA scramble. The electroretinogram was normal in both eyes: no significant difference between the right and the left eye was found. Three months after injection, no measurable axonal degeneration was found in either eye. CONCLUSION: The reduced expression of OPA1 based on RNA silencing in adult mice could induce reversible dysfunction of retinal ganglion cells.

[Abrupt visual loss in children. Three case studies of ocular bartonellosis]. / Baisse d'acuité visuelle brutale chez l'enfant. A propos de 3 cas de bartonellose oculaire.

PURPOSE: To evaluate the severity and progression of neuroretinitis in children caused by Bartonella. PATIENTS AND METHODS: Retrospective study of three consecutive cases. Patients underwent ophthalmological, medical, and radiological evaluations. Bartonella serology was positive. Intravenous treatment was started immediately with antibiotics and steroids. Patients were re-evaluated after visual acuity recovery. RESULTS: Progression was quickly positive with fosfomycin and fluoroquinolones in all cases. CONCLUSION: Bartonella induces neuroretinitis even without systemic signs of cat-scratch disease, with sometimes sudden bilateral blindness. Long-term progression is good but urgent medical treatment is necessary.

[Corneoscleral involvement in congenital erythropoietic porphyria. Günther disease]. / Atteinte cornéo-sclérale au cours de la porphyrie érythropoïétique congénitale. Maladie de Günther.

Congenital erythropoietic porphyria (CEP) is an extremely rare autosomal recessively inherited disorder characterized by mutilating cutaneous photosensitivity and abnormal porphyrin heme synthesis in bone marrow. The present report describes a typical case of CEP with cornea involvement and scleromalacia in areas exposed to sunlight. The results obtained by conjunctival impression cytology are reported.