Congenital or Developmental Glaucoma

These glaucomas occur in eyes in which an anomaly present at birth produces an intraocular pressure rise.

This type of glaucoma is extremely rare and it is often, though not always, inherited. This means that the affected child might be brought to the ophthalmologist by the parents because they are aware of the condition in the family. Children could be born with raised intraocular pressure and for these cases, the prognosis is not so good as in those where the pressure rise does not occur until after the first few months of life.

In primary developmental glaucoma, the glaucoma is caused by a defective development of the angle of the anterior chamber, and gonioscopy shows that the normal features of the angle are obscured by a pinkish membrane. Raised intraocular pressure in infancy has a dramatic effect because it causes enlargement of the globe. This can best be observed by noting an increase in the corneal diameter. The enlarged eye has given the condition the name of buphthalmos or "bull's eye" (Figure 12.8).

Figure 12.8. Congenital glaucoma: note the enlarged left cornea (with acknowledgement to Mr R. Gregson). CO

Other important signs are photophobia and corneal oedema. The diagnosis is confirmed by an examination under anaesthesia, which includes measuring the corneal diameters and the intraocular pressure. Surgical treatment is nearly always required and this involves passing a fine knife through the peripheral cornea so that the point reaches the opposite angle of the anterior chamber. Once in the angle, it is moved gently to and fro to open up the embryonic tissue that covers the trabecular mesh-work (goniotomy). The other (or secondary) developmental glaucomas include the rubella syndrome, aniridia, mesodermal dysgenesis, Peter's anomaly and the phacomatoses, where the intraocular pressure rise is associated with other ocular and systemic developmental anomalies.

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