CONGENITAL CATARACT

Prepared by: Muhammad Shukri Bin Johar 070100441 J2 2012

Department Of

Outlines

Lens Anatomy Definition Epidemiolgy Pathophysiology Etiology Clinical features Investigations Differential diagnoses Management Prognosis

Lens Anatomy

Lens Anatomy
The crystalline lens : Function: refract light to be focus on the retina. Transparent, elips biconvex structure in the eye Anterior to the lens: iris & Posterior to the lens: vitreous body. The lens is suspended in place by the suspensory ligaments and connects to the ciliary body. The anterior surface is less curved than the posterior. By nine weeks into human development, the lens is surrounded and nourished by tunica vasculosa lentis (derived from the hyaloid artery). Beginning in the fourth month of development, the hyaloid artery begin to regres , the lens receives all its nourishment from the aqueous humor.

Definition

term any opacity of the lens or capsules causing visual impairment is called cataract. Congenital cataract (Developmental Cataract) means that cataract that presents at birth, or develop soon after birth

Epidemiology

Opacity in lens

Can be: Visually significant or not Stable or Progressive Congenital or Acquired Unilateral or Bilateral Partial or Complete
Congenital: incidence 6/10 000 10% of childhood blindness

Pathophysiology

At birth, the embryonic and fetal nuclei make up most of the lens. Postnatally, cortical lens fibers are laid down from the conversion of anterior lens epithelium into cortical lens fibers Any insults to the nuclear/lenticular fiber can result opacity

Etiology

Clinical Features
History: Painless loss of vision Refraction disturbance and glare Physical examination: Several types of congenital cataract are recognized with slit-lamp biomicrosocpe. (Morphologically)

Morphology : Examples

Investigations

Screen newborns with red reflex test History : Family Maternal infections Examination: systemic diseases or syndromes
Workup: Bilateral cases without known hereditary basis TORCH screen s-glucose s-calcium, phosphate Urine: reducing substances (galactosaemia) amino acids ( Lowe syndrome) haematuria (Alport syndrome)

Ocular examination

Formal estimate of vision not possible in neonate Special tests: Preferential looking test, visually evoked potentials Density and position of cataract

Morphology
Associated ocular pathology Indicators of severe visual impairment : No fixation Nystagmus Strabismus

Differential Diagnose

Retinoblastoma Ablatio retina

surgery (treatment of choice) should be performed btwn 6w6m. Most ophthalmologists opt for surgery much earlier, ideally when patients are younger than 2 months. If in dense cataract (bilateral/ unilateral) or partial cataract with vision less than 6/18, the surgery ASAP Bilateral cases: 1 week apart Non visually significant cases : careful observation, possible pupillary dilation Conventional aphakic glasses: only useful for bilateral aphakia, but wearing heavy spectacles (+15D/>) should be considered Extended-wear soft contact lens: especially for uniocular aphakia but require commitment of the parents.

Surgical Techniques Pars plana lansectomy with vitrectomy: most suitable for cengenital cataract. Planned extracapsular cataract extraction (ECCE): a posterior chamber intraocular lens (PCIOL) may be considered ECCE if child older than 3 years age. The incidence of posterior ocular opacification (PCO) after ECCE with PCIOL is very high in young children. A posterior capsulorhexis with/without vitrectomy is helpful to prevent PCO in younger group

Prognosis

Uni/Bilateral (unilateral congenital cataracts, 40% develop visual acuity of 20/60 or better while bilateral congenital cataracts, 70% develop visual acuity of 20/60 or better). Associated with systemic or other ocular defects (poor prognosis) Commitment of the parents Sometimes patient can develop with complications such as loss of vision even with aggressive surgical and optical treatment, amblyopia, glaucoma, strabismus and retinal detachment

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Prepared by: Muhammad Shukri Bin Johar 070100441 J2 2012

Department Of