Congenital Cataract

The Cataract is an ocular pathology characterized by the clouding of the eye’s natural lens; the clouding is usually a physiological condition linked to aging.

In some cases the cataract occurs in infants t and takes the name of congenital cataract, whose incidence is 3:10,000 infants and in two thirds it is bilateral. Congenital cataracts develop in infants for many reasons that can include hereditary tendencies, infections, metabolic problems, diabetes, traumas, inflammations or reactions to drugs.

Many systemic pediatric conditions can be associated to the presence of congenital cataract: inter alia the galactosemia, Lowe Syndrome (ocular cerebral renal syndrome), the hyperthyroidism, pseudohypertyroidism, Fabry disease, hypoglycemia and hyperglycemia.

The intrauterine infections mainly involved are rubella, toxoplasmosis, chickenpox, Cytomegalovirus infection and Herpes Simplex virus contracted during the intrauterine life.

Chromosome anomalies are also involved such as the Down Syndrome, Patau and Edwards  Syndrome and syndromes characterized mainly by abnormalities of the skull and facial bones, characteristic facial features, sparse hair, eye abnormalities etc. such as the Hallermann-Streiff-François syndrome and the Nance-Horan syndrome.

 

DIAGNOSIS

Without an early surgery, based on the clouding degree of the lens, the congenital cataract can determine a serious visual impairment or be the cause of amblyopia, the so called “lazy eye” defect that permanently prevents the patient from having a clear vision. This condition can also lead to other problems such as nystagmus, strabismus and the absence of central visual fixation.

Such conditions can deeply affect the learning ability and personality of a child. An early diagnosis appears, therefore, necessary for avoiding these serious complications. Notwithstanding the impossibility to make an objective evaluation of the visual defect in infants, the density and potential impact on the visual acuity are assessed principally on the basis of the aspect of the red reflex and the visibility of the eye fundus through the direct and indirect Ophthalmoscopy, often supplemented by an examination under narcosis for acquiring the anatomic parameters that are necessary for planning the surgery.  

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