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PEDIATRIC CATARACT

PEDIATRIC CATARACTPresenter: Dr. Ajay Kumar SinghModerator: Dr. Wangchuk Doma

Venu Eye Institute & Research Centre, New DelhiINTRODUCTIONPediatric cataract

Congenital cataract(present at birth)Developmental cataract(develops soon after birth)Traumatic cataract INTRODUCTIONIncidence - 6/10,000

10% of childhood blindness

Congenital or AcquiredVisually significant or notStable or ProgressiveUnilateral or BilateralPartial or Complete

Developmental opacity are usually partial & stationary ANATOMYLens - A transparent,biconvex,crystalline structure between iris & vitreous in a saucer shaped depression called patellar fossa.

ComponentsAnterior capsuleAnterior capsular epitheliumCortexNucleusPosterior capsule

CONGENITAL VERSUS DEVELOPMENTALCongenital & developmental cataract not synonymous

In widest sense, most forms of cataract (even senile cataract) may be considered as developmental, for human lens grows until late in life

Criteria for congenital cataractpresent at birthsituated within fetal nucleusdiameter of opacity less than 5.75mm (diameter of newborn lens)

If size of opacity more than 5.75mm,cataract must be postnatalGenetic MutationAutosomal Dominant

MetabolicGalactosaemiaLoweHypoparathyroidismFabry

InfectiveTORCH organismsCongenital rubella

ChromosomalTrisomy 21 (Down)Trisomy 18 (Edward)Trisomy 13 (Patau)

SkeletalHallerman-Streiff-Francois syndromeNance-Horan syndrome

Ocular anomaliesAniridiaAnterior segment dysgenesis syndrome

Idiopathicin 50%

Congenital cataracts: BilateralCongenital cataracts: UnilateralSporadicno family history/systemic disease

Cause identified in only 10%

Ocular anomaliesPersistent fetal vasculature (most common)

Affected infants usually full term & healthy

MORPHOLOGYTYPESPunctate (blue dot) cataractAnterior Capsular cataractAnterior polar cataractPyramidalReduplicatedPosterior polar cataractEmbronal (nuclear) cataractCoronary cataract/Fusiform cataractZonular (Lamellar) cataractTotal cataractSoftMembranous MORPHOLOGICAL TYPES

PUNCTATE(BLUE DOT) CATARACTMost common type,universal

Cataracta coerulea/blue dot cataract

Multiple tiny blue spots scattered all over lens esp in cortex

Variant cataracta centralis pulverulenta dominantly inherited,non progressive central spheroidal/biconvex opacity consisting of powdery fine white dots within embronic /fetal nucleus

Non progressive&visually insignificant

ZONULAR (LAMELLAR) CATARACTAccounts for 50% of visually significant cataractMay be congenital or occurs at a later stage as development interfered at a later stageZone around embryonic nucleus (usually in area of fetal nucleus) become opacified,extent depending on duration of inhibiting factorOpacity sharply demarcated . Area of lens within & around opaque zone is clear

Linear opacities like spokes of a wheel (called riders) may run outwards

Usually bilateral,formed just before/shortly after birth

Fill pupillary aperature when pupil undilated, thus affecting vision

Often hereditary (autosomal dominant)Associated with hypovitaminosis D (with evidence of ricket) or hypocalcemia (with tetany or defective tooth enamel)&maternal malnutrition

ZONULAR (LAMELLAR) CATARACTCORONARY CATARACTAround pubertySituated in deeper layers of cortex &superficial layers of adolescent nucleusCorona or club shaped opacities near periphery of lens ,usually hidden by iris while axial region &extreme periphery remain clearNon progressive & does not interfere with vision

NUCLEAR CATARACTAssociated with rubellaIncidence more if infection contracted in 2nd monthDevelopment of lens inhibited at very early stageEmbyonal nuclear cataractProgressive ,becomes total cataractAssociated microphthalmos, nystagmus,strabismus,glaucoma,iris hypoplasia & pigmentary retinopathy

ANTERIOR CAPSULAR CATARACTMay be due to Delayed formation of anterior chamber (Developmental)

Acquired (commonly)-follows contact of capsule with cornea, usually after perforation of ulcer in ophthalmia neonatorum

Three types-Anterior polar (flat type)Anterior pyramidalReduplicatedFlat type white plaque forms in central pupillary area less than 3mm in diametervisually insignificantPyramidal type sometimes central plaque projects forwards into anterior chamber like a pyramidFrequently surrounded by area of cortical opacitymay affect vision

ANTERIOR CAPSULAR CATARACTSubcapsular epithelium may grow in between capsule & cortical opacity

Clear lens fibre subsequently growing from there lay down a transparent zone between two opacities

Buried opacity is called imprint & two together constitute Reduplicated cataract AssociationPersistant pupillary membraneAniridiaPeters anomalyAnterior lenticonus

ANTERIOR CAPSULAR CATARACTPOSTERIOR CAPSULAR ( POLAR) CATARACTAssociated with persistent hyaloid remnants(mittendorf dots),posterior lenticonus & persistent anterior fetal vasculature

Common in minimal degree & visually insignificant

With persistent hyloid artery , lens deeply invaded by fibrous tissue leading to total cataract

OTHER TYPESSUTURAL CATARACTOpacity follows anterior /posteriorY sutureMay occur in isolation or association with other opacityMEMBRANOUS CATARACTRareAssociated with Hallermann-Streiff-Francois syndromeoccurs when lenticular material partially /completely reabsorbs leaving behind residual chalky white lens matter sandwiched between anterior & posterior capsule

Symptoms of Developmental CataractInformant usually parents

History of white spot in pupillary area

Child is usually brought with history of diminution of vision / does not recognize objects and parents

Unsteady eyes

Deviation of eye

Associated symptoms of systemic disease, if present

SignsDiminished vision (at times it is difficult to establish in very young children)

Lenticular opacity

Nystagmus

Deviation of eye

There may be other ocular and systemic abnormalities in cases of rubella nuclear cataract

Management of Developmental Cataract InvestigationsDetailed historyComplete ocular examination- UCVA, BCVA, pupillary reaction intra-ocular tension, fundus examinationB scan ultrasonography to exclude posterior segment abnormality like growth/ retinoblastomaA scan to determine axial length of the eyeRetinoscopy, to deremine the refractive statuscover test to exclude squintEarly photographs to know onset of cataractLaboratory investigations: (For bilateral cases)Blood TestFull blood count,Blood glucose(FBS/RBS)Serum calcium and phosphorusRBC transferase and Galactokinase levelsTORCH testHepatitis B virusUrine analysis:For reducing substance for galactosaemiaFor amino acids (to exclude Lowe syndrome in suspected cases)

U/L Pediatric cases are mostly idiopathic.No need of lab investigationManagement of Developmental Cataract Cataract in childhood not only reduce vision but also interfere with normal visual development

Timing of surgery ,surgical technique,choice of aphakic correction & amblyopia management are of utmost importance to achieve good &long standing results

The inflammatory response to surgical results are more pronounced in children

Post operative amblyopia management forms an integral part of visual rehabilitation in childrenManagement of Developmental Cataract ??????To operate or not?If yes, when to operate?If no, how to manage?If operating, should one place the IOL?If not placing IOL, how to manage and follow the child?

Non-surgical treatmentUsed for partial cataractsPartial cataract less than 3 mm and pericentral cataracts respond Pupillary dilatation with 2.5% phenylephrine and part time occlusion of good eyeCyclopentolate can be added once or twice a day if requiredProlonged cyclopegia (daily atropine) can induce amblyopiaPupillary dilatation is reserved for preverbal (1-6 years) with partial cataracts and borderline amblyopiaIf significant amblyopia persists, cataract extraction should be performed

26WHEN TO OPERATE??Bilateral dense cataracts : - require early surgery at 4-6 weeks of age to prevent development of stimulus deprivation amblyopia

Bilateral partial cataracts -may not require surgery or require only at a later date 27 3.Unilateral dense cataract -urgent surgery is advised within days -aggressive anti-amblyopia therapy should follow surgery -results are often poor -if detected after 16 weeks of age then surgery is inadvisable because amblyopia is refractory

4.Partial unilateral cataract may be treated non surgically28General techniquesDeep general anaesthesia is requiredPediatric cataracts are soft lens material can be aspirated through incisions that are 1-1.5mm long at the limbus ; can be subjected to lensectomy through pars planaA larger wound is needed to introduce IOLTunnel should be securely sutured to prevent dehiscence of wound with iris incarceration 29Specific techniquesThere are two approachesPars plana approachLimbal approach

Pars plana approach is being abandoned gradually in favour of limbal approach as limbal approach allows better preservation of the capsular bag for in-the-bag IOL placement.30Pars plana approachIndications :

neonates and infants