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Treatment

     Treatment should be initiated promptly after the diagnosis is established. Medical treatment will often decrease the intraocular pressure, but will rarely control it satisfactorily.  Surgery is hence indicated to effect permanent reduction of the intraocular pressure.

     Medical treatment consists of agents that decrease the rate of aqueous humor formation. Acetazolamide given by mouth (15mg/kg/day) is the most effective initial therapy.  Dorzolamide, a topical carbonic anhydrase inhibitor, also deserves consideration; one drop is given two to three times per day.  Topical beta blockers also suppress aqueous formation and may be of help:they are given in addition to the topical or systemic carbonic anhydrase inhibitor.

     Goniosurgery, filtration surgery, and implant surgery are the three options for the management of congenital glaucoma.  In goniosurgery (goniotomy, trabeculotomy), the abnormal trabeculum is incised to enhance the passage of fluid into the filtering channels. For many patients with congenital glaucoma this can be a very effective operation. Children with more severe defects of the filtration channels often do not respond to goniosurgery, and filtration procedures may be required to bypass the malfunctioning system, creating a new egress passage for the aqueous humor.  The results of this type of surgery may be disappointing due to the failure of the new passage to remain patent.  When the filtration angle defect is seen on gonioscopy to be mild to moderate, with a visible ciliary body band, angle surgery  is effective in 75% of cases. Repeat surgery in an unoperated area may be necessary to achieve an adequate lowering of the eye pressure.  In case of failure of classic filtration surgery, implants (Molteno, Ahmed, others) may be used to drain the aqueous fluid from the eye by way of a silicone tube to an episcleral reservoir.

      Treatment of congenital glaucoma is difficult, specialized, and sometimes disappointing.  With persistent careful monitoring, however, the outcome of therapy can be viewed with guarded optimism.  Attention to occlusion therapy and correction of optical defects are important to prevent amblyopia.  Genetic counseling is essential and should rely on the individual family structure and general available statistics.