Congenital glaucoma

August 12, 2017 17:51 | Genetic Diseases

Congenital glaucoma - one of the most severe types of eye disease in newborns, early leads to blindness.The disease is relatively rare, its incidence is 1 in 10,000 newborns.However congenital glaucoma is the cause of vision loss in children 2.5-7%, t. E., Almost every 10 minutes a child goes blind from congenital glaucoma.In 15% of patients the disease is hereditary (family) or due to various disorders in utero.Most of the disease affects boys.The ratio of boys and girls with primary congenital glaucoma about 3: 2. In general, congenital glaucoma refers to a pathology of the 1st year of life, since at this age it appears in more than 90% of cases.

In congenital glaucoma, there are signs of grave violations of the organ of vision, so she soon appears and progresses rapidly, resulting in permanent loss of vision for 2-3 weeks.In this regard the special role played by early diagnosis and early surgical treatment, which is aimed at eliminating obstacles to current ocular fluid and the creation of artif

icial outflow tract.

Early diagnosis is crucial for successful treatment.However, it is possible only with a good knowledge of the early signs of the disease obstetricians, neonatologists and neonatologist of maternity hospitals, pediatricians and nurses performing nursing small children at home.This is due to the fact that children's ophthalmologist examines all children in order to avoid congenital abnormalities in only 2-4 months of age.

Hereditary glaucoma is noted in 15% of cases of this disease.I sent it in an autosomal recessive inheritance type, and often combined with other abnormalities of the eye.Intrauterine glaucoma develops under the influence of factors such as frequent X-ray examinations, chronic fetal hypoxia, avitaminosis, toxicosis, infectious diseases.

In normal body view has two cameras, front and rear chamber of the eye.In these chambers contains a fluid that is a refracting medium to focus all beams entering the human eye, to create a certain pattern visibility.Intraocular fluid circulates constantly, t. E. In the body of existing drainage system.The fluid is constantly produced in the choroid plexus of the eye, and the excess drain on certain structures of the front and rear camera eye.When glaucoma disorders occur, leading to violation of the outflow of fluid, its excessive accumulation and increased intraocular pressure.Congenital glaucoma is the result of incorrect and incomplete breakdown of tissues in the corner of the front of the camera in the process of fetal development.At this point in time and rate as the path must be the outflow of ocular fluid.Anomalies of the anterior chamber angle in congenital glaucoma is clearly expressed.Changes in the anterior chamber angle can be very diverse, up to its complete lack of unsplit and many parts of the outflow tract.Such dramatic changes in the drainage system does not leave hopes for the success of medical treatment and require urgent surgical intervention.

glaucoma stages denoted by Roman numerals, and they are classified according to certain criteria.The outer membrane of the eye in children thin, elastic, therefore increasing your eyes is the first and important symptom of glaucoma.In children it is easier to control the visual acuity than the field of view, so it is the second criterion.The third criterion of glaucomatous process are the changes of the optic nerve.

Intraocular pressure is the second characteristic of the pathological process in glaucoma.It may be normal, moderately high and high.Normal intraocular pressure is up to 27 mm Hg.Art.Moderately elevated ranges from 28-32 mm Hg.Art.The high intraocular pressure is 33 mm Hg.Art.and more.

Another characteristic process occurring in glaucoma, is its stability.By stable glaucoma include cases without increasing the eye, reduced vision, negative dynamics in the optic nerve as a result of systematic observations.In the absence of negative dynamics in 6 months or more the process is considered stable.For the unstable glaucoma include cases with negative dynamics: a marked increase in the size of the eye, reduced vision, tunnel vision.

In congenital glaucoma, the outflow of intraocular fluid dramatically hampered, fluid accumulates in excess, increased intraocular pressure.Dramatically increasing the load on the outer membrane of the eye causes it to stretch.The child's eyes early in the disease beautiful: large, expressive, anterior chamber deepens, the sclera of the stretching becomes bluish.Prolonged tension eyeball increases sharply ( "bull's eye"), cloudy, sclera thins dramatically and as stafilom uneven bulges outwards.Blind eyes fluctuate nystagmus.

In this disease requires urgent surgical treatment.

congenital glaucoma, secondary glaucoma different from adults and a variety of forms are the result of other medical conditions.

Secondary congenital glaucoma with ocular anomalies of the anterior segment of the eye:

1) aniridia (absence of the iris) in 50% of cases may be complicated by an increase in intraocular pressure.Glaucoma is often seen in adolescence.Children with aniridia should be under medical supervision with regular monitoring of intraocular pressure;

2) ectopia (wrong position) of the lens often leads to the development of glaucoma;

3) Rieger syndrome is the underdevelopment of the iris and the cornea.It is a hereditary disease, which is transmitted by dominant inheritance type.Glaucoma usually develops after the first decade of life in connection with which the eyeball is usually not increased;

4) Frank-Kamenetz syndrome only occurs in men.The disease is transmitted by recessive engaged with sex chromosome inheritance type.In this two-color iris anomalies: the area around the pupil grayish, blue or brown, and the peripheral part of the (broader) looks chocolate brown.Glaucoma develops in the second decade of life.

Secondary syndromic congenital glaucoma:

1) kernel-Weber syndrome.The cause of this disease is not fully elucidated, but a great place withdrawn heredity.The syndrome manifests itself by capillary tumors (hemangiomas) persons and focal vascular changes in the brain.Capillary hemangioma of the face is localized along the branches of the trigeminal nerve in the form of large purple spots.

The degree of vascular tumors in the brain depends on neurological symptoms.There are epilepsy, hydrocephalus (accumulation of excess liquid cavity of the skull), paresis, mental disorders, mental retardation.Glaucoma develops on the side of a birthmark in the defeat of the eyelids, especially the upper, and the conjunctiva.Such people need to be systematic monitoring of intraocular pressure;

2) neurofibromatosis.Congenital glaucoma can be when it is generalized (most severe) form that occurs with skin lesions, bone, brain, endocrine system, especially at the location in the area of ​​the upper eyelid and temple;

3) and Marfan syndrome Weill-Marchesani syndrome - a genetically caused diseases, which occur with damage to connective tissue, cardiovascular, musculoskeletal, endocrine and other systems of the body.Accompanied by incorrect position of the lens and, as a result, secondary glaucoma.