Types OF Cataract in Pediatric Patients به نام خداوند جان وخرد Kianersi F. M.D 1391/12/10

Classifications Cataracts in Pediatric Patients can be classified by: Age, Etiology, Morphology.

Classifications of Cataracts according to age

Cataracts in Pediatric Patients Congenital Infantile Juvenile

Cataract Congenital Lens opacity present at birth.

Infantile Cataract Lens opacities develop during the first year of life.

Juvenile Cataract Lens opacities develop before 10 years of age.

Congenital - Infantile Because some lens opacities escape detection at birth and are noted only on later examination, these terms are used interchangeably by many physicians.

Congenital - Infantile Congenital and infantile cataracts cover a broad spectrum of severity. Whereas some lens opacities do not progress and are visually insignificant, others can produce profound visual impairment. Congenital and infantile cataracts may be unilateral or bilateral.

Etiologic Classification

Etiologic Classification 1. Hereditary cataract 2. Metabolic cataract 3. Traumatic cataract 4. Secondary cataract 5. Cataract secondary to maternal infection during pregnancy 6. Iatrogenic cataract 7. Syndromes and congenital cataract

Associated with microphthalmos. Examination of family members. 1. Hereditary cataract AD: 75% (AR—XL) The affected individuals are usually perfectly well, and have no associated systemic illness. Associated with microphthalmos. Examination of family members.

2. Metabolic cataract Galactosaemia Hypoglycaemia Hypocalcaemia

2. Metabolic cataract (Galactosaemia) Galactosemia is a metabolic disorder in which the child’s body cannot metabolize galactose, a major component of milk and milk products. The baby develops typical ‘oil droplet’ cataracts which are easily seen by examining the red reflex. These are reversible, and the lens returns to normal on removing dairy products from the diet.

2. Metabolic cataract (Hypoglycaemia) Hypoglycaemia of whatever cause may give rise to lens opacities in a child. The majority of babies with hypoglycaemia will also have convulsions and may have permanent brain damage.

2. Metabolic cataract (Hypocalcaemia) Hypocalcemia may result in cataracts though these are usually functionally less significant than cataracts resulting from hypoglycaemia.

3. Traumatic cataract Trauma is the most common cause of unilateral cataract in children. Traumatic cataract is usually the result of a penetrating injury, though blunt trauma can also lead to cataract formation.

4. Secondary cataract (Uveitis) The most common type of secondary cataract seen in the pediatric ophthalmology clinic is as a result of Uveitis seen in conjunction with arthritis (juvenile chronic arthritis [JCA]). The cataract may be as a direct result of inflammation within the anterior segment, or can also result from the steroids used to treat the condition.

4. Secondary cataract (steroid INDUCED) Cataracts caused by steroid ingestion are usually posterior sub-capsular. Progression of the cataract will be halted following cessation of treatment although not reversible.

4. Secondary cataract (intraocular tumor) Less frequently, cataract may be seen secondary to an intra - ocular tumor such as Retinoblastoma.

5. Cataract secondary to maternal infection during pregnancy (TORCHS SYN.) TORCHS syndrom: Rubella Toxoplasmosis, Toxocariasis, Cytomegalovirus (CMV), Herpes, Syphilis. Usually bilateral, dense, and central.

5. Cataract secondary to maternal infection during pregnancy (Rubella) The most common maternal infection to cause congenital cataract in the child is Rubella. The cataracts caused by Rubella may be present at birth, or develop several months later.

5. Cataract secondary to maternal infection during pregnancy (Rubella) These children may also have: Microphthalmia, Glaucoma, Retinal pigmentary disease, Microcephaly, Deafness, Heart defects and Mental retardation.

6. Iatrogenic cataract Iatrogenic cataract is most commonly seen in children who have had: Total body irradiation for leukemia, Organ transplants, On long-term systemic steroid therapy. These children are usually older children and do very well after cataract surgery.

7. Syndromes and congenital cataract There are large variety of chromosomal and dysmorphic syndromes, in which the child will have a high risk of having congenital cataract. It is important to notice any abnormal features in children presenting with cataract, such as unusual facial features, extra digits, unusual skin, short stature, developmental delay, microcephaly or hydrocephaly.

7. Syndromes and congenital cataract (Dwan syndrom)

7. Syndromes and congenital cataract (Dwan syndrom) Cataracts, Keratoconus, Glaucoma, Brushfield spots.

Morphology Classification of Congenital Cataracts

Morphology Classification of Congenital Cataracts Congenital cataracts occur in a variety of morphologic configurations, including: Polar, Sutural, Coronary, Cerulean, Nuclear, Capsular, Lamellar, Complete, and Membranous. Each of these categories encompasses a range of severity.

Polar Cataract Lens opacities that involve the subcapsular cortex and lens capsule of the anterior or posterior pole of the lens.

Cataract Anterior Polar Small, bilateral, symmetric, non progressive opacities that do not impair vision. Inheritance: AD pattern. Sometimes seen in association with other ocular abnormalities, including: Microphthalmos, Persistent pupillary membrane, and Anterior lenticonus.

Anterior Polar Cataract

Posterior Polar Cataracts Produce more visual impairment than anterior polar cataracts because they tend to be larger and are positioned closer to the nodal point of the eye. Capsular fragility has been reported.

Posterior Polar Cataracts Posterior polar cataracts are usually stable but occasionally progress. They may be sporadic or familial. Familial posterior polar cataracts are usually bilateral and inherited in an AD pattern.

Sutural Cataract (Stellate Cataract) Opacification of the Y-sutures of the fetal nucleus that usually does not impair vision.

Sutural Cataract (Stellate Cataract) These opacities often have branches or knobs projecting from them.

Sutural Cataract (Stellate Cataract) Bilateral and symmetric, sutural cataracts are frequently inherited in an AD pattern.

Coronary Cataract Consist of a group of club-shaped opacities in the cortex that are arranged around the equator of the lens like a crown, or corona.

Coronary Cataract They cannot be seen unless the pupil is dilated, and they usually do not affect V/A. Inheritance: AD pattern.

Cerulean Catract (Blue-dot Cataract) Small bluish opacities located in the lens cortex.

Cerulean Catract (Blue-dot Cataracts) They are non progressive and usually do not cause visual symptoms.

Nuclear Cataract Opacities of either the embryonic nucleus alone or both the embryonic and fetal nuclei. They are usually bilateral, with a wide spectrum of severity.

Nuclear Cataract Lens opacification may involve the complete nucleus or be limited to discrete layers within the nucleus.

Nuclear Cataract Eyes with congenital nuclear cataracts tend to be small.

Capsular Cataract Small opacifications of the lens epithelium and anterior lens capsule that spare the cortex. They are differentiated from anterior polar cataracts by their protrusion into the anterior chamber.

Capsular Cataract Capsular cataracts generally do not adversely affect vision.

Lamellar Cataract (Zonular Cataract) The cataract is visible as an horseshoe- shaped opacity that surrounds a clearer center and is itself surrounded by a layer of clear cortex.

Lamellar Cataract (Zonular Cataract) The most common type of congenital/infantile cataract. They are characteristically bilateral and symmetric. Their effect on V/A varies with the size and density of the opacity.

Lamellar Cataract (Zonular Cataract) Lamellar cataracts may be the result of a transient toxic influence during embryogenic lens development. The earlier this toxic influence occurs, the smaller and deeper is the resulting lamellar cataract. Lamellar cataracts may also be inherited as an AD trait.

Complete Cataract (Total Cataract) All of the lens fibers are opacified. The red reflex is completely obscured, and the retina cannot be seen with either direct or indirect ophthalmoscopy.

Complete Cataract (Total Cataract) Some cataracts may be subtotal at birth and progress rapidly to become complete cataracts.

Membranous Cataract Membranous cataracts occur when lens proteins are resorbed from either an intact or traumatized lens, allowing the anterior and posterior lens capsules to fuse into a dense white membrane.

EPIDEMIOLOGY

EPIDEMIOLOGY 1 in every 250 newborn has lens opacity. All of them are not visually significant. Prevalence of visually significant cataract is 1.2 to 6 in 10000 live birth. 10-20% of blindness in children worldwide. Bilateral cataract is more common than unilateral one.

EPIDEMIOLOGY 1/3 of congenital or infantile cataracts are associated with other disease syndromes, 1/3 occur as an inherited trait, and 1/3 result from undetermined causes.

EPIDEMIOLOGY Unilateral Cataract in childhood: Evaluation of other Ocular abnormality. Bilateral Cataract in childhood: Familial & Systemic evaluation, It is important that all children with congenital cataract are examined by a pediatrician to exclude any underlying systemic disorder.