Congenital glaucoma

Congenital glaucoma (also known as infantile glaucoma) may be inherited or develop as a result of the fetus’s exposure to various damaging factors.

There are three recognized types of innate glaucoma:

• primary congenital glaucoma (hydrophthalmia);
• glaucoma combined with abnormal development of the eye;
• glaucoma combined with a system congenital pathology.

According to medical reports, a big number of children (55.6%) display the first symptoms of glaucoma at an early age (5-6 years).

Causes of innate glaucoma

The disorder is based on congenital anomalies in the development of the angle of the anterior chamber and the drainage system of the eye which prevents or hampers the outflow of the aqueous humor, thus raising intraocular pressure. The cause of this anomaly lies in various pathological states of a mother, particularly in the first months of pregnancy. These pathological factors are caused by various reasons: infections (rubella, influenza, etc.), intoxication, alcoholism, ionizing radiation, etc.

Basic signs of congenital glaucoma

Congenital glaucoma is characterized by peculiar clinical manifestations depending on the age of a child.

• growing intraocular pressure;
• photophobia and lacrimation;
• increased size of the eyeball (the child is born with big and ‘expressive’ eyes) and its further rapid growth;
• increased diameter of the cornea (at the later stages the diameter of the cornea may reach 20 mm and more) and the width of limbus;
• corneal edema (it looks dimmed);
• retarded reaction of the pupils;
• changes in the optic nerve disc.

Congenital glaucoma is quite often combined with simultaneous development of defects in other systems and organs (microcephaly, heart diseases, deafness, phacomatosis, etc.), including the eye (microcornea, aniridia, cataract, etc.).

In 75% of observed cases, congenital glaucoma develops in both eyes. Specialists report that congenital and infantile glaucoma in most cases does not lead to complaints with the exception of cases when glaucoma is caused by the corneal syndrome. In other words, the disorder develops as open angle glaucoma. At the later stages of glaucoma, there may appear staphyloma, sclera holes, and also tension and thinning of the conjunctiva. A complicated cataract often occurs. In the initial stages of congenital glaucoma, the fundus of the eye is normal. With the disease progression, the optic nerve head shows dystrophic changes due to poor blood circulation.

Congenital glaucoma is characterized by progressive aggravation of optic functions. At the early stages, loop of the vision is mainly due to changes in the cornea (edema, dimness), refraction anomalies, hyperopia. The development of congenital glaucoma leads to deterioration of the optic functions since the optic nerve and the retina are affected.

Treatment for congenital glaucoma

Surgery is the basic method for the treatment of congenital glaucoma. It is only surgery that can eliminate obstacles to the outflow of aqueous humor, which are created by structural anomalies in the drainage zone.

The basic surgical treatment methods are:
trabeculotomy, trabeculoectomy, combined methods (trabeculotomy + trabeculoectomy), gonyotomy, at a later period following gonyotomy there may be indications for additional gonyopuncture.

Medication therapy plays an important role in the complex treatment of the disorder and includes:

• reduction of intraocular pressure using complex of hypotensive drugs;
• prevention of post-surgical coarse cicatrisation ;
• application of neurotrophic medications aimed at preserving and improving the optic functions;
• desensibilizing and restorative therapy.

Functional treatment – ametropia correction, pleoptic treatment (children with congenital glaucoma often have medium and high myopia).

Outpatient observation

Children suspected to have congenital glaucoma or with diagnosed glaucoma are placed on the out patient list with obligatory medical examinations once a month: measurements of intraocular pressure, diameter of the cornea, width of the limbus, evaluation of vision functions (if possible).