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CHAPTER II

LITERATURE REVIEW
2.1. Anatomy of the Lens
The adult human lens is an asymmetric oblate spheroid that does not possess nerves,
blood vessels, or connective tissue. It is a transparent, biconvex structure enclosed in a
capsule. The lens is posterior to the iris and anterior to the vitreous humor of the vitreous
body. The anterior surface is in contact with the aqueous on the corneal side; the
posterior surface is in contact with the vitreous. The anterior pole of the lens and the
front of the cornea are separated by approximately 3.5mm.
The lens is held in place by the zonularfibers (suspensory ligaments), which run between
the lens and the ciliary body. These zonularfibers(collectively constituting the
suspensory ligament of the lens), which originate from the region of the ciliary
epithelium, are a series of fibrillin-rich fibers that converge in a circular zone on the lens.
Both an anterior and a posterior sheet meet the capsule 12mm from the equator and are
embedded into the outer part of the capsule (12m deep). It also is thought that a series
of fibers meets the capsule at the equator.

Figure 2.1.The iris separates the anterior and posterior chambers of the eye.
Histologically the lens consists of three major components capsule, epithelium, and
lens substance

Figure 2.2.Gross anatomy of the adult human lens

2.2. Congenital Cataract
The crystalline lens is a transparent structure. Its transparency may be disturbed due to
degenerative process leading to opacification of lens fibres. Development of an opacity in the
lens is known as cataract. These occur due to some disturbance in the normal growth of the
lens. When the disturbance occurs before birth, the child is born with a congenital cataract.
Therefore, in congenital cataract the opacity is limited to either embryonic or foetal nucleus.
Congenital cataract is a term used to define lenticular opacities at birth. Infantile cataract
encompasses all lens opacities that develop within the first year of birth. The terms are used
interchangeably due to some of these opacities being missed at birth only to be discovered
later in life by ophthalmologists. They vary in severity from being non-progressive and
visually insignificant to causing profound visual impairment.












Figure 2.3. Morphology of Cataract

2.2.1. Etiology
Exact etiology is not known. Some factors which have been associated with certain types of
cataracts are described below:
I. Heredity.
Genetically-determined cataract is due to an anomaly in the chromosomal pattern of the
individual. About one-third of all congenital cataracts are hereditary. The mode of
inheritance is usually dominant. Common familial cataracts include: cataracta
pulverulenta, zonular cataract (also occurs as non-familial), coronary cataract and total soft
cataract (may also occur due to rubella).
II. Maternal factors
1. Malnutrition during pregnancy has been associated with non-familial zonular cataract.
2. Infections. Maternal infections like rubella are associated with cataract in 50 percent of
cases. Other maternal infections associated with congenital cataract include
toxoplasmosis and cytomegalo-inclusion disease.
3. Drugs ingestion. Congenital cataracts have also been reported in the children of mothers
who have taken certain drugs during pregnancy (e.g., thalidomide, corticosteroids).
4. Radiation. Maternal exposure to radiation during pregnancy may cause congenital
cataracts.
III. Foetal or infantile factors
1. Deficient oxygenation (anoxia) owing to placental haemorrhage.
2. Metabolic disorders of the foetus or infant such as galactosemia, galactokinase
deficiency and neonatal hypoglycemia.
3. Cataracts associated with other congenital anomalies e.g., as seen in Lowe's syndrome,
myotonia dystrophica and congenital icthyosis.
4. Birth trauma.
5. Malnutrition in early infancy may also cause developmental cataract.
IV. Idiopathic.
About 50 percent cases are sporadic and of unknown etiology.
2.2.2. Clinical types
Congenital and developmental cataracts have been variously classified. A simple
morphological classification of congenital and developmental cataract is as under :
I. Congenital capsular cataracts
1. Anterior capsular cataract
2. Posterior capsular cataract
II. Polar cataracts
1. Anterior polar cataract
2. Posterior polar cataract
III. Nuclear cataract
IV. Lamellar cataract
V. Sutural and axial cataracts
1. Floriform cataract
2. Coralliform cataract
3. Spear-shaped cataract
4. Anterior axial embryonic cataract
VI. Generalized cataracts
1. Coronary cataract
2. Blue dot cataract
3. Total congenital cataract
4. Congenital membranous cataract
I. Congenital capsular cataracts
1. Anterior capsular cataracts are nonaxial, stationary and visually insignificant.
2. Posterior capsular cataracts are rare and can be associated with persistent hyaloid artery
remnants.
II. Polar cataracts
1.Anterior polar cataract.
It involves the central part of the anterior capsule and the adjoining superficial-most
cortex. It may arise in the following ways:
i. Due to delayed development of anterior chamber.
In this case the opacity is congenital usually bilateral, stationary and visually insignificant.
ii. Due to corneal perforation. Such cataracts may also be acquired in infantile stage and
follow contact of the lens capsule with the back of cornea, usually after perforation due to
ophthalmia neonatorum or any other cause.
Morphological types: Anterior polar cataracts may occur as any of the following
morphological patterns:
i. Thickened white plaque in the centre of capsule.
ii. Anterior pyramidal cataract. In it the thickened capsular opacity is cone-shaped with its
apex towards cornea.
iii. Reduplicated cataract (double cataract).
Sometimes along with thickening of central point of anterior capsule, lens fibres lying
immediately beneath it also become opaque and are subsequently separated from the
capsule by laying of transparent fibres in between. The burried opacity is called imprint
and the two together constitute reduplicated cataract.
2. Posterior polar cataract.
It is a very common lens anomaly and consists of a small circular circumscribed opacity
involving the posterior pole. Posterior polar cataract may be associated with :
- Persistent hyaloid artery remnants (Mittendorf dot),
- Posterior lenticonus, and
- Persistent hyperplastic primary vitreous (PHPV).
Types. Posterior polar cataract occurs in two forms:
- Stationary form and
- Progressive form which progresses after birth.
III. Nuclear cataracts
i. Cataracta centralis pulverulenta (Embryonic nuclear cataract). It has dominant genetic
trait and occurs due to inhibition of the lens development at a very early stage and thus,
involves the embryonic nucleus. The condition is bilateral and is characterised by a small
rounded opacity lying exactly in the centre of the lens. The opacity has a powdery
appearance (pulverulenta) and usually does not affect the vision.
ii. Total nuclear cataract. It usually involves the embryonic and fetal nucleus and
sometimes infantile nucleus as well. It is characterized by a dense chalky white central
opacity seriously impairing vision. The opacities are usually bilateral and non
progressive.
IV. Lamellar cataract
Lamellar or Zonular cataract refer to the developmental cataract in which the opacity
occupies a discrete zone in the lens. It is the most common type of congenital cataract
presenting with visual impairment. It accounts for about 40 percent of the cases.
Etiology. It may be either genetic or environmental in origin. Genetic pattern is usually
of dominant variety. Environmental form is associated with deficiency of vitamin D.
Sometimes maternal rubella infection contracted between 7th and 8th week of gestation
may also cause lamellar cataract.
Characteristic features. Typically, this cataract occurs in a zone of foetal nucleus
surrounding the embryonic nucleus.












Figure 2.4. Lamellar Cataract

V. Sutural and axial cataracts
Sutural cataracts are comparatively of common occurrence and consist of a series of
punctate
opacities scattered around the anterior and posterior Y-sutures. Such cataracts are usually
static, bilateral and do not have much effect on the vision. The individual opacities vary
in size and shape and have different patterns and thus are named accordingly as under:
1. Floriform cataract. Here the opacities are arranged like the petals of a flower.
2. Coralliform cataract. Here the opacities are arranged in the form of a coral.











Figure 2.5. Total Congenital Cataract

2.2.3. Differential diagnosis
Congenital cataracts presenting with leukocoria need to be differentiated from various other
conditions presenting with leukocoria such as retinoblastoma, retinopathy of prematurity,
persistent hyperplastic primary vitreous (PHPV), etc.,
2.2.4. Management of congenital and developmental cataract
A. Clinico-investigative work up.
A detailed clinicoinvestigative work up is most essential in the management of paediatric
cataract. It should aim at knowing the prognostic factors and indications and timing of
surgery.
1. Ocular examination should be carried out with special reference to:
Density and morphology of cataract
Assessment of visual function is difficult in infants and small children. An idea may be made
from the density and morphology of the cataract by oblique illumination examination and
fundus examination. Special tests like fixation reflex, forced choice preferential looking test,
visually evoked potential (VEP), optic-kinetic nystagmus (OKN) etc. also provide useful
information. Associated ocular defects should be noted (which include microphthalmos,
glaucoma, PHPV, fovealhypoplasia, optic nerve hypoplasia, and rubella retinopathy etc.
2. Laboratory investigations should be carried out to detect following systemic associations
in nonhereditary cataracts: Intrauterine infections viz. toxoplasmosis, rubella, cytomegalo
virus and herpes virus by TORCH test. Galactosemia by urine test, for reducing substances,
red blood cell transferase and glactokinase levels. Lowe's syndrome by urine chromatography
for amino acids. Hyperglycemia by blood sugar. Hypocalcemia by serum calcium and
phosphat levels and X-ray skull.
B. Prognostic factors
which need to be noted are:
Density of cataract,
Unilateral or bilateral cataract,
Time of presentation,
Associated ocular defects, and
Associated systemic defects
C. Indications and timing of paediatric cataract surgery,
1. Partial cataracts and small central cataracts which are visually insignificant can safely be
ignored and observed or may need non-surgical treatment with pupillary dilatation.
2. Bilateral dense cataracts should be removed early (within 6 weeks of birth) to prevent
stimulus deprivation amblyopia.
3. Unilateral dense cataract should preferably be removed as early as possible (within days)
after birth. However, it must be born in mind that visual prognosis in most of the unilateral
cases is very poor even after timely operation because correction of aphakia and prevention
of amblyopia in infants is an uphill task.
D. Surgical procedures. Childhood cataracts, (congenital, developmental as well as acquired)
can be dealt with anterior capsulotomy and irrigation aspiration of the lens matter or
lensectomy.
E. Correction of paediatric aphakia. It is still an unsolved query. Presently common views are
as follows:
Children above the age of 2 years can be corrected by implantation of posterior chamber
intraocular lens during surgery. Children below the age of 2 years should preferably be
treated by extended wear contact lens. Spectacles can be prescribed in bilateral cases. Later
on secondary IOL implantation may be considered. Present trend is to do primary
implantation at the earliest possible (2-3 months) specially in unilateral cataract.
Paediatric IOL: size, design and power. The main concerns regarding the use of IOL in
children are the growth of the eye, IOL power considerations, increased uveal reaction and
long-term safety. Present recommendation are :
- Size of IOL above the age of 2 years may be standard 12 to 12.75-mm diameter for in
the bag implantation.
- Design of IOL recommended is one-piece PMMA with modified C-shaped haptics
(preferably heparin coated).
- Power of IOL. In children between 2-8 years of age 10% undercorrection from the
calculated biometric power is recommended to counter the myopic shift. Below 2
years on undercorrection by 20% is recomended.


Bilateral congenital cataract accounts for 15%
7
of blindness in children worldwide.
Idiopathic
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cataracts are the most common. Underlying and associated causes of congenital
cataract vary worldwide. Isolated hereditary cataracts account for 25% of cases, the most
common being autosomal dominant, then autosomal recessive or X-linked.
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Down, Patau,
Edward, Turner and Cri du chat syndromes along with systemic diseases like galactosemia,
Lowe, Fabry, Alport, Dystrophia myotonica, hypoglycemia, hypoparathyroidism and Marfan
syndrome are frequent associations. Maternal infections like rubella, toxoplasma,
cytomegalovirus, herpes simplex and varicella (TORCH) may be causative.
1,

Morphologically cataracts may be classified into fibre-based and non-fibre based. These
include anterior or posterior polar cataracts, lamellar (round, grey shell surrounding a clear
nucleus), nuclear or cataracta centralis pulverulenta, sutural or stellate, floriform (flower
shaped), coralliform (coral-shaped), blue dot (punctate cerulean cataract), coronary
(supranuclear), subcapsular, total white, disciform, oil-droplet, spear and membranous
cataracts. Lamellar cataract is the commonest. (Nadeem et al, 2013)