Anomalies in the development of the lens

Anomalies in the development of the lens may have different manifestations. Any changes in the shape, size, and location of the lens cause severe disturbances in its function.

Congenital aphakia - the absence of the lens - is rare and, as a rule, is combined with other malformations of the eye.

[1], [2], [3]

Symptoms of lens development anomalies

Microfakia is a small lens. Usually this pathology is combined with a change in the shape of the lens - spherofakia (globular lens) or a violation of the hydrodynamics of the eye. Clinically, this is manifested by high myopia with incomplete correction of vision. A small round crystalline lens suspended from long, weak filaments of the circular ligament has a much greater mobility than normal. It can be inserted into the pupillary lumen and cause a pupillary block with a sharp increase, intraocular pressure and pain syndrome. To release the lens, you need to expand the pupil by medicamentous means.

Microfakia in combination with a subluxation of the lens is one of the manifestations of the Marfan syndrome, a hereditary malformation of the entire connective tissue. Ectopia of the lens, changes in its shape are caused by hypoplasia of its supporting ligaments. With age, the separation of the zinn ligament increases. At this point the vitreous body protrudes in the form of a hernia. The equator of the lens becomes visible in the pupil area. A complete dislocation of the lens is also possible. In addition to eye pathology, Marfan syndrome is characterized by the defeat of the musculoskeletal system and internal organs.

It is impossible not to pay attention to the features of the appearance of the patient: high growth, disproportionately long limbs, thin, long fingers (arachnodactyly), poorly developed muscles and subcutaneous fatty tissue, curvature of the spine. Long and thin ribs form a thorax of unusual shape. In addition, they reveal the developmental defects of the cardiovascular system, vegetative-vascular disorders, dysfunction of the adrenal cortex, the violation of the daily rhythm of excretion of glucocorticoids in the urine.

Microspherophacia with a subluxation or complete dislocation of the lens is also noted in the Marchezani syndrome - a systemic hereditary lesion of mesenchymal tissue. Patients with this syndrome, in contrast to patients with Marfan syndrome, have a completely different appearance: low growth, short arms, with which it is difficult to grab their own head, short and thick fingers (brachidactyly), hypertrophied muscles, asymmetric compressed skull.

Coloboma of the lens - a defect in the lens tissue along the midline in the lower part. This pathology is extremely rare and is usually combined with the coliboma of the iris, ciliary body and choroid. Such defects are formed due to incomplete closure of the embryonic fissure during the formation of a secondary eye glass.

Lenticonus - a conical protrusion of one of the surfaces of the lens. Another type of pathology of the lens surface is lentiglobus: the anterior or posterior surface of the lens has a spherical shape. Each of these developmental anomalies is usually marked on one eye, can be combined with opacities in the lens. Clinically, the lentikonus and lentiglobus are manifested by increased refraction of the eye, i.e. The development of high degree myopia and hard-to-correct astigmatism.

Treatment of lens development anomalies

With anomalies in the development of the lens, not accompanied by glaucoma or cataract, special treatment is not required. In those cases when due to the congenital pathology of the lens there arises an uncorrected anomaly of refraction, the changed lens is removed and replaced by an artificial one.