Congenital & Infantile Cataract

Prof. Naimatullah Khan Kundi

Khyber Teaching Hospital

Peshawar

Congenital & Infantile Cataract

Congenital Cataract

When lens opacities that present at birth

Infantile Cataract

Lens opacities that develop during 1st yr of life

Fairly common (1 in 2000 live birth)

UL / BL

Classification

Morphology

Presumed aetiology

Metabolic disorders

Associated ocular anomalies

Systemic disorders

Congenital & Infantile Cataract

In General

1/3 of cataracts associated with other

syndromes

1/3 occurs as inherited trait

1/3 undetermined causes

Congenital & Infantile Cataract

1. Polar

2. Sutural

3. Nuclear

4. Capsular

5. Lamellar

6. Complete

7. Membranous

Morphological classification of congenital and infantile cataracts

Polar These lens opacities involve

sub capsular cortex lens capsule

Anterior and posterior Polar Anterior Polar:

BL, Small, Symmetric, Non progressive autosomal dominant (frequently) Less effects on vision Sometimes associated with other ocular anomalies like:

Micro-ophthalmos Persistent pupillary membrane Anterior lenticonus

Congenital and infantile cataracts

Polar

Posterior Polar cataracts:

Stable/Progressive (Occasionally) Sporadic/Familial

Sporadic:

UL,

May be associated with remnants of tunica vasculosa

Lentis,

Lenticonus,

Lentiglobus

Congenital and infantile cataracts

Polar (cont’d)

Familial:

BL

Autsomal dominant

Larger and positioned closer to the nodal point of

the eye

Produce more visual impairment

Congenital and infantile cataracts

Sutural (Stellate) Cataract

Opacification of ”Y” – sutures of fetal nucleus

BL

Symmetric,

Autosomal dominant pattern

Seldom impair vision

Congenital and infantile cataracts

Nuclear

Opacification of the:

Embryonic nucleus or

Embryonic + fetal nucleus BL

Opacity may involve the:

Complete nucleus

Discrete layers within the nucleus

Congenital and infantile cataracts

Capsular Cataract

Opacification of the:

Lens epithelium and

Anterior lens capsule

It spares the cortex

They protrude into the anterior chamber

(Cf. Anterior Polar cataract)

Vision not adversely affected

Congenital and infantile cataracts

Lamellar (zonular) cataracts

Most common type of congenital/infantile cataract

BL, Symmetrical

Effect on vision vary (size/density)

Causes:

Toxic influence

Hereditary (autosomal dominant)

Early toxic influence – opacity small and deeper

Opacities – in specific layers/zones of the lens

Congenital and infantile cataracts

Lamellar (zonular) cataracts

Clinically the cataract visible as a layer of

Opacification that surrounds a clear center and

itself surrounded by a layer of clear cortex

Disc shaped configuration when viewed from the

front

Additional arcuate opacities within cortex straddle

the equator of the lamellar cataract. These

Horseshoe shaped opacities are called Riders

Congenital and infantile cataracts

Complete (Total) Cataract

1. Opacification of all the lens fibers

2. Red reflex completely obscured

3. No retinal view obtained with direct /indirect

ophthalmoscope

4. UL/BL

5. Produce profound visual impairment

Congenital and infantile cataracts

Membraneous Cataract

1. When lens proteins are resorbed from:

• Intact lens

• Traumatized lens

2. The anterior and posterior lens capsules fuse into

one dense membrane

3. The resulting opacity and lens distortion causes

significant visual disability

Congenital and infantile cataracts

Rubella Cataract Maternal infection – RNA Toga Virus

Congenital Rubella Syndrome: Pearly white nuclear opacification

Sometimes complete cataract / cortex may liquefy

Histopathology:

Retention of nuclei deep within lens substance

Live virus may be recovered from the lens as late 3 yrs after birth

Cataract removal may be complicated by excessive posterior operative inflammation caused by release f these live virus

Congenital and infantile cataracts

Rubella Cataract (cont’d)

Other Ocular Manifestations:

1. Diffuse pigmentry retinopathy

2. Micro ophthalmos

3. Corneal clouding (Transient/Permanent)

4. Glaucoma

Although congenital rubella syndrome may cause

cataract or glaucoma, both conditions are not

characteristically present in the same eye

Congenital and infantile cataracts