Congenital cataract

Congenital cataract develops as a result of intrauterine pathology and is often combined with different developmental malformations of both the eye and other organs.

Congenital cataract is the opacity of the lens, which is noted from birth or appears soon after it.

Congenital cataracts can be sporadic or can develop with chromosomal abnormalities, metabolic diseases (eg, galactosemia) or intrauterine infections (eg, rubella) or maternal diseases during pregnancy. Cataracts can be nuclear or can cover the substance of the lens under the anterior or posterior capsule. They can be one-sided or two-sided. Cataracts can be undetected if the child does not test a red reflex or at birth does not conduct an ophthalmoscopy. Just like with other types of cataracts, the turbidity of the lens disrupts vision. Cataracts can obscure the appearance of the optic disc and the vessels of the fundus and should be evaluated by an ophthalmologist.

Congenital cataracts can occur under the influence of various toxic substances on the embryo and fetus during the formation of the lens. Most often, this happens when the mother during the pregnancy endures viral infections: influenza, measles, rubella, and toxoplasmosis. Great influence is exerted by various endocrine disorders in women during pregnancy and insufficiency of the function of the parathyroid glands (for example, hypocalcemia, which leads to a violation of the development of the Fetus). Often, congenital cataracts are of a family nature. The most common congenital cataract is bilateral, but there is also a one-sided congenital cataract.

Congenital diseases of the lens are divided into cataracts, changes in shape, size, lens dislocation, coloboma and absence of the lens.

Congenital lens dislocations occur in hereditary disorders of connective tissue metabolism and anomalies in the bone system.

Marfan syndrome - a small lens with its subluxation is a manifestation of this syndrome. In addition, with it, there is a lesion of the cardiovascular system in the form of heart defects, aortic aneurysms. The musculoskeletal system changes in the form of arachnodactyly (long fingers and toes), dolichocephaly (increase in the longitudinal dimensions of the head), brittle bones, frequent dislocations, high growth, long limbs, scoliosis, funnel-shaped breasts, underdevelopment of the mouse and genital organs. Less common is a change in the psyche. The eye is affected in 50-100% of cases. Ectopia of the lens, changes in its shape are caused by underdevelopment of the ligaments supporting it. With age, the separation of the zinn ligament increases. At this point the vitreous body protrudes in the form of a hernia. A complete dislocation of the lens is also possible. There may be cataracts, strabismus, nystagmus and other eye syndromes, congenital glaucoma.

The Margetapi syndrome is a systemic hereditary lesion of the mesenchymal tissue. Patients with this syndrome have small growth, short limbs, increased head size (brachycephaly), limitation of joint mobility, changes in the cardiovascular system and other organs. From the lens - ectopia down, spherofakia, microfakia, etc .; myopia, retinal detachment, congenital glaucoma.

Change in the shape and magnitude of the lens - Lentikonus - a conical protrusion of one of the lens surfaces. In the lens there is a protrusion (as an additional small lens), which can be from the front and back surfaces, it is transparent. In transmitted light, against the background of the lens, one can see a rounded formation in the form of a drop of oil in water. This cut has a very strong refraction, always accompanied by myopia (maybe pseudo-myopia). In these protrusions there are seals - a core with a high refractive power.

Depending on the size of the lens distinguish:

• microphage (small lens, usually with a change in shape, the equator is visible in the pupil area, often there is a dislocation of the lens);
• Spherofakia (spherical lens), annular lens (in the center of the lens as a result of any reason has resolved). At a narrow pupil of a lens it is not visible. With an enlarged pupil under the cornea, a ring is retained, usually cloudy;
• bifukigo (two lenses), which is very rare. One lens is located temporally or nasally, or the lenses are located one above the other,

Coloboma of the lens - a defect in the lens tissue and the lower part, which is formed as a result of incomplete closure of the embryonic fissure during the formation of the secondary eyeball. This pathology is very rare and is usually combined with the coliboma of the iris, ciliary body and choroid.

Congenital cataract is a waterfall. Of all the birth defects found in 60% of cases, one in five for 100 thousand of the population.

[1], [2], [3], [4], [5], [6]

Capsular congenital cataract

Polar (front and back) cataracts. Anterior polar cataract is the cataract of a very early period of embryogenesis (the first month of fetal life in the embryo). Limited white opacity at the anterior pole of the lens occurs at the time when the anterior cerebral end forms eyeballs growing toward the outer ectoderm. From the side of the external ectoderm, during the same period, the lens begins to grow. The latter fills the eye vesicle, and an eye glass is formed. The development of anterior polar cataract is associated with a disorder in the lenticular lens eradication from the ectoderm. Anterior polar cataract can develop due to other intrauterine processes of inflammatory nature, and also after birth as a result of perforated ulcer of the cornea. In the forward polar cataract, a limited clouding of white not more than 2 mm in diameter located in the center of the lens surface is determined. The turbidity of this consists of highly altered, irregularly formed turbid lens fibers located under the lens bag.

The posterior polar cataract is a cataract of late embryogenesis. It is formed in the prenatal period. In fact, with such a cataract formed corn on the back capsule of the lens. It is considered that this is the remainder of the artery of the vitreous body, which undergoes reduction to the birth of the child.

Clinically defined limited turbidity of the rounded form is grayish white, located at the posterior pole of the lens. There may be diffuse cloudiness, which consists of individual points, or may be layered.

A variation is pyramidal cataract. In addition to turbidity, there is a projection in the region of the pole, that is, it proliferate into the vitreous body in the form of a pyramid. Since polar cataracts are always congenital, they are two-sided. Due to their small size, as a rule, they do not lead to a significant disruption of the central vision and are not subject to surgical treatment.

[7], [8], [9], [10], [11], [12]

Capsular lenticular cataract

Spindle-shaped cataract - turbidity - is located in the region of the posterior and anterior poles, which are connected by a bridge. The turbidity is presented in the form of a thin gray ribbon, resembling a spindle and consists of three thickenings. This clouding can also be located in the core region. Vision slightly decreases. Such cataracts usually do not progress. Since early childhood, patients are adapting to look through the transparent areas of the lens. Treatment is not required,

Lenticular cataract:

• anterior axial embryonic cataract. Occurs in 20% of healthy individuals. Very gentle opacification in the anterior part, anteroposterior axis of the lens, at the seam of the embryonic nucleus. The sight does not affect;
• stellate (cataract sutures) - turbidity along the seams of the embryonic nucleus. On the seams there are crumbs resembling semolina. Occurs in 20% of healthy individuals. The child retains a sufficiently high visual acuity;
• central nuclear cataract. There are three subspecies: powdered cataracts - diffuse turbidity of the embryonic nucleus, which consists of small-dot bullet-like opacities, eyesight does not decrease; saturated turbidity - with his vision not a more pointed cataract - many point cloudiness of grayish and bluish color in the region of the embryonic nucleus.

[13], [14]

Zonal (layered) cataract

Zonular cataract (laminated) is the most common form of congenital cataracts - 60%. More common bilateral cataracts. The turbidity is presented in the form of a cloudy disk, which is located on the border of the adult and embryonic nuclei. For this cataract is characterized by the alternation of transparent and turbid layers of the lens. At the equator in the adult nucleus there is a second cloud of opacity, separated from the first by a layer of transparent fibers. The turbidity of the second layer is wedge-shaped, and its intensity is not uniform.

In the transmitted light, a red reflex is seen on the periphery, and in the center there is a gray haze. The edge of the turbid disk is uneven, loopy. It has been established that zonal cataracts are not only congenital. It can also occur in the postnatal period in children suffering from spasmophilia against the background of hypocalcemia that have suffered rickets, with hypoglycemia. Vision suffers depending on the degree of clouding of the lens.

Diskovidnaya congenital cataract is similar to zonal, but there is no unevenness and looseness of the edge of the murky disc.

Blue cataracts are multiple haze bluish tones. The turbidity is localized between the nucleus and the capsule.

Complete cataract is the diffuse cloudiness of the entire lens, which can develop from layered cataracts. Sometimes under the capsule, plaques are visible.

Half-dissected cataract - the lens as if drying up, becomes flat. The diagnosis is ultrasound. Reduces the thickness of the lens to 1.5-2 mm, instead of it can remain a film.

Falsate cataract is a cloudy capsule and a small amount of lens material.

Atypical and polymorphic cataract. The turbidity differs in size and shape. For example, cataract with measles rubella is characterized by white cloudiness with a pearly hue. With the dilated pupil against this background, an eccentrically located rod is visible, in which a virus can live for a long time (several dozen years). The peripheral parts of the lens are more transparent.

Classification of congenital cataracts

Hereditary cataracts, their causes.

1. Genetic inferiority and heredity. Most often occur according to the dominant type, but can also be in the recipient type (especially with related incest).
2. Hereditary disorders of carbohydrate metabolism are galactose and cataract.
3. Disturbance of calcium metabolism - tetanic cataract.
4. Hereditary skin lesions.

At the heart of hereditary cataracts is the defeat of the gene and chromosome apparatus.

Intrauterine cataracts:

1. Embryopathy, which occur during pregnancy for up to eight weeks;
2. Fetopathies that occur after eight pregnancy cycles.

Their clinic is similar,

Causes of intrauterine cataracts:

1. maternal diseases (measles rubella, cytomegatia, chicken pox, herpes, influenza and toxoplasmosis);
2. intoxication mother (alcohol, ether, contraceptives, some abortifacients);
3. cardiovascular diseases of the mother, which lead to oxygen starvation and the occurrence of cataracts;
4. hypovitaminosis A and E, lack of folic acid;
5. Rhesus-conflict;
6. toxicosis of pregnancy.

Classification of congenital cataracts:

1. one- or two-sided defeat;
2. partial or complete;
3. localization of opacities (capsular, lenticular, capsulolenticular).

Clinical forms:

1. as a concomitant disease of the eye;
2. on visual acuity (I st.> 0.3, II st. 0.05-0.2, III st. - 0.05)

Classification according to EI Kovalevsky:

1. by origin (hereditary and intrauterine);
2. by leading localization (polar, nuclear, zonal, diffuse, polymorphic, coronal);
3. by the degree of vision loss (I st.> 0.3; II st. 0.05-0.2; 1P st. -0.05);
4. for complications and concomitant changes:
   • without complications and changes in visual function;
   • cataract with complications (nystagmus, etc.);
   • Cataract with concomitant changes (microphage, etc.).

Clinical forms of congenital cataracts:

1. capsular and bagged cataracts;
2. capsulolenticular (both capsule and lens substance are affected);
3. lenticular;
4. cataracts of embryonic sutures;
5. zonal or layered.

[15], [16], [17], [18], [19], [20], [21], [22]

Treatment of congenital cataracts

Removal of congenital cataracts within 17 weeks after birth provides development of vision and visual pathways of the cerebral cortex. Cataracts are removed by aspirating them through a small incision. Many children can implant an artificial lens. Postoperative vision correction with glasses, contact lenses, or both, is necessary in order to achieve a good vision.

After removing one-sided cataracts, the quality of the image from the operated eye proves to be worse than the unoperated eye (assuming that the second eye is normal). Since preference is given better to the seeing eye, the brain suppresses the image of inferior quality, and amblyopia develops (see earlier). Thus, the treatment of amblyopia is necessary for the development of normal vision in the operated eye. Some children, in spite of this, do not develop a good visual acuity. In contrast, in children with bilateral cataracts, whose image quality is the same from both eyes, the same vision in both eyes is more often developed.

Some cataracts are partial (posterior lenticone) and turbidity develops during the first 10 days of life. Partial congenital cataract has the best prognosis for vision.