CONGENITAL CATARACT

Prepared by: Muhammad Shukri Bin Johar070100441

J22012

Department Of Ophthalmology

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 glarePhysical 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

Management

•surgery (treatment of choice)•should be performed btwn 6w-6m.•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

•surgery (treatment of choice)•should be performed btwn 6w-6m.•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

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

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

•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.

•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.

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

THANK YOU

Prepared by: Muhammad Shukri Bin Johar070100441

J22012

Department Of Ophthalmology