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Congenital glaucoma

Congenital glaucoma
Congenital or infantile glaucoma is one of the most severe types of eye pathology in newborns leading to early loss of vision.

This is a relatively rare disease – one case for 10 000 children – but congenital glaucoma is the ground for loss of sight in 2.5-7% of blind children. This means that almost every 10th child gets blind because of congenital glaucoma.

Types of congenital glaucoma

Reason for the development of glaucoma:

  • Congenital glaucoma is present in 15% of cases. It is also often combined with other eye abnormalities.
  • Prenatally diagnosed glaucoma develops due to the effect of teratogenes on the fetus during the pregnancy period (overexposure to X-rays, oxygen deficiency, vitamin deficiency, toxicosis, infection diseases).

The factors affect abnormal shaping of the anterior chamber angle, surplus production of aqueous humor in the eye with its corrupted outflow leading to increase of intraocular pressure. In the neonatal period and up to one year tissues of the eye-ball can actively spread due to the increasing intraocular pressure which leads to enlarging of the eye-ball and straining and increasing of the diameter of cornea.

Three forms of congenital glaucoma:

  • simple congenital glaucoma (hydrophthalmos);
  • glaucoma combined with abnormalities in the development of eye (Sturge-Weber-Krabbe disease);
  • glaucoma combined with a constitutional congenital defect (microphthalmos, Weill-Marchesani syndrome, Marfan disease, Rubinstein-Taybi syndrome).

Congenital glaucoma divided by the state of intraocular pressure:

  • normal pressure – no swelling of cornea, Maklakov’s tonometry: up to 23 mm Hg;
  • moderate-high pressure - Maklakov’s tonometry: 23-30 mm Hg, no swelling of cornea, little swelling of cornea can appear occasionally;
  • high pressure - Maklakov’s tonometry: 31-40 mm Hg, constant cornea swelling, striated or partial blurring of cornea;
  • very high pressure - Maklakov’s tonometry: more than 40 mm Hg, stable cornea swelling, blurring of cornea up to raw central blurring.

Congenital glaucoma and course of the disease:

  • malign C. glaucoma – last stages of the process are evident already at the birth of the child or hydrophthalmos progresses in the first 1-2 months after the birth;
  • typical C. glaucoma – process brightly develops in the age of 3-4 months combined with photo-phobia, blepharism, watery eye characterizing the development of the disease;
  • benign C. glaucoma – slow development of clinical presentations, as a rule – between the first and second year of life, insignificant enlargement of eye-ball (intermediate state between the infantile and children’s form of congenital glaucoma);
  • abortion-triggered C. glaucoma – intraocular pressure normalizes spontaneously and progression of the disease stops.

Congenital glaucoma and its dynamics:

  • Stable C. glaucoma – pathological enlargement of the eye-ball terminates, no decrease of vision acuity. This can be the case only after a successful operation.
  • Progressing C. glaucoma – further pathological enlargement of the eye-ball, decrease of vision acuity. This state is seen before the operation or after the operation failed to reach positive results (i.e. when the intraocular pressure remains to be high). Progress of glaucoma after the operation provides grounds for possible additional surgery.

Diagnostic of glaucoma in children

One of the most important aspects is regular and thorough diagnostic of children’s eyes in order to confirm congenital glaucoma diagnosis.

Specialists of Yasny Vzor pediatric eye clinics conduct diagnostics of 5-6 year old children suspected to have congenital glaucoma only in the medical sleep, because it is a complex task to evaluate the structures of an eye while the child is awake.

Diagnostic measures obligatory include as follows:

  • measurement of intraocular pressure;
  • assessment of cornea and anterior chamber angle;
  • eye-ball length measurement;
  • fundus examination, examination of optic papilla (discus nervi optici).

In children older than 3 years we conduct optical coherence tomography to define the grade of the optic nerve damage and follow-up the dynamics of its changes.

It often happens that the child is mistakenly diagnosed with glaucoma on the basis of 1-2 indications. Our practice shows the examples when we managed to exclude congenital glaucoma diagnosis due to a proper dynamic follow-up after the full set of diagnostic procedures. One needs to understand that an incorrect diagnosis leads to unnecessary operation that could result in permanent damage of the optical nerve.

Etiology of congenital glaucoma

Abnormality in the intraocular fluid drainage leads to the increase of intraocular pressure. The causes leading to this disease are various pathological states of pregnant woman, especially during the first months of pregnancy (infections, poisonings etc.).

Symptoms of congenital glaucoma

We want to draw attention of parents to the fact that the major part of children (55.6%) experience first signs of glaucoma in the early age (starting from the first days of life and until the age of 5-6).

If you see any corneal opacity or the eyes of your child seem to be huge or “expressive”, it could be a first sign of congenital glaucoma. In 75% of such cases glaucoma develops in both eyes.

The main symptoms of congenital glaucoma are: watery eye, eye fissure narrowing and photo-phobia.

Congenital glaucoma is often combined with simultaneous development of defects in other systems and organs (microcephalism; heart defects; deafness; phacomatosis etc.) as well as in the eyes (microcornea; aniridia; cataract etc.).

Parents need to know that congenital or infantile glaucoma don’t provide for complaints in the majority of affected children. We see widening and thinning of the conjunctiva and other eye-ball coats on the late stages of the disease. Complicated cataract may also be the case. In the beginning of the development of congenital glaucoma the fundus of an eye remains regular. The worst complication of congenital glaucoma is loss of sight due to distortion of blood supply in the optic papilla.

Treatment of congenital glaucoma

In case of congenital glaucoma we tend to perform surgery whereby the selection of the surgery method is on the surgeon. It depends on the stage of the disease and involvement of eye structures. Only surgery can help to create the conditions for regular drainage of intraocular fluid.

  • Drainage of the anterior chamber angle. This is a unique technical approach that helps to stabilize the glaucoma process and normalize intraocular pressure in the majority of cases. Together with our American colleagues we are of the opinion that especially this operation can eliminate cause of glaucoma (in some cases even after only one operation) in children.
  • Trabeculotomy. It is applied only in case of an intense blurring of cornea: trabecula is dissected through the resected sclera. Trabecula is a part of the anterior chamber angle. It is dissected for a better drainage of intraocular fluid.
  • Trabeculectomy. A part of trabecula is deleted through the resected sclera. This creates a way for drainage of intraocular fluid under the conjunctiva thus leading to decrease of intraocular pressure.
  • Goniotomy. This operation is performed on early stages of glaucoma. A part of the anterior chamber angle is dissected through the cornea. The efficacy rate of the operation is 85%.
  • Integrated method. Trabeculotomy + trabeculectomy.
All operations on children are performed only under general anesthesia.

Expected response to treatment of glaucoma in children

Expected response to congenital glaucoma treatment depends on the age of the patient, combined eye defects and supplied treatment. Our statistic shows that favorable prognosis for normalization of intraocular pressure is 90% in children of 2-12 months.

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