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Emmanuel L. Bui Quoc, Emilie Costantini, Monika Voigt; Primary Congenital Glaucoma. Invest. Ophthalmol. Vis. Sci. 2011;52(14):1579.
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To evaluate our management of primary congenital glaucoma, which is a rare disease whose incidence is estimated at about 1/20000 to 30000 births. Its treatment is an emergency. Surgical treatment (goniotomy, trabeculotomy, deep sclerectomy or trabeculectomy) is required, though long term management is necessary, for relapses may occur, but also amblyopia must be detected and treated.
We have retrospectively studied the files of 8 children with congenital glaucoma requiring surgery before the age of 1 year old, between 2006 and 2009.
8 children were recruited. 15 eyes were operated: deep sclerectomy +/-trabeculotmoy was performed in all cases. 7 cases were bilateral. Follow up varied between 4 to 55 months. The mean number of examination under general anaesthesia was 7.5 (3 to 13). Relapses occurred in 7 eyes (46.7% of the cases): early relapses in 5 eyes (within 3 months after initial surgery) and late relapses in 2 eyes of 2 different patients (at least 3 years after initial surgery). 1.9 surgical procedure per eye (including diode laser cyclophotocoagulation) were performed (1 to 5). Cataract requiring surgery occurred in 4 eyes (26.7% of the cases). At the end of follow up, 14 eyes (93.3%) had a normal intraocular pressure (i.e. ≤ 9 mmHg under anaesthesia or ≤ 18 mmHg without anaesthesia). 5 eyes out of 15 still had local treatment. 7 out of 11 phakic eyes were myopic ( SE> -3 D). Amblyopia of one eye occurred in 5 children.
The diagnosis of congenital glaucoma depends on: corneal transparency, increased pachymetry, increases axial length. The value of measured intraocular pressure is not always pertinent, for elasticity of the cornea is affected by corneal oedema. Surgical treatment is compulsory, though it is not defined which is the most appropriate, for goniotomy, trabeculotomy, deep sclecetromy and trabeculectomy all showed their efficacy n the literature. It is striking, for the precise location of the obstacle to the resorption of aqueous humor is still unknown: internal or external ? It also could be hypothesised that any surgery allowing a transitory diminution of intraocular pressure could be efficient, allowing the trabeculum to mature. But it doesn’t explain late relapses. Management of congenital glaucoma remains a challenge, and a close follow up of the patients, with several examination under anaesthesia, is required, in order to diagnose relapses, evaluate the precise refraction and detect and treat amblyopia.
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