congenital & infantile cataract
DESCRIPTION
This presentation is orginaly uploaded to http://kpkmedicalcolleges.tk by Dr.SulemanTRANSCRIPT
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