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Pediatric cataracts , case selection before surgery Dr.Sayed Ezatollah Memarzadeh

ophthalmologistEsfahan 1391EPIDEMIOLOGY

1 In every 250 newborn has lens opacitymost of them are not visually significantPrevalence of visually significant cataract is1.2 to 6 in 10000 live birth 10-20% of blindness in children worldwide Bilateral cataract is more common than unilateralMORPHOLOGY AND LOCATION

Three main types : zonular , polar , lenticonusZonular is the most common type Zonular subtypes : nuclear , lamellar , sutural Polar : anterior or posterior Lenticonus : anterior or posterior ANTERIOR POLAR

Small , not progressive , visually not significant 2/3 unilateral , 90% sporadic associated with corneal guttata and astigmatismmicrophthalmos or persistent pupillary membrane 1/3 anisometropia , strabismus , amblyopiaMorphology : Examples

ANTERIOR PYRAMIDAL Central cone projecting into AC Surrounding cortical cataract May progress and need surgery Bilateral , sporadicAnterior pyramidal

ANTERIOR SUBCAPSULAR

Often idiopathic

Trauma

Alports syndrome

NUCLEAR

Dense , congenital , visually significant Bilateral , often AD Associated with microphthalmos

SUTURAL

Involved Y sutures and fetal nucleus Progressive , visually not significant Bilateral , often ADLAMELLAR

Most common type Cortical , outside the Y sutures , clear nucleus and subcapsular area Progressive and eventually needs surgery Bilateral , often AD Galactosemia , hypoglycemiaZonular lamellar cataracts

TOTAL All layers are involved AD ,Downs syndrome , Metabolic disease, Trauma Progression of other types of cataractPERSISTENT HYPERPLASTIC PRIMARY VITREOUS

Failure of regression of hyaloid system Fibrovascular stalk and membrane over the posterior capsule Elongated ciliary processes Anterior displacement of lens lead to glaucoma Microphthalmos , occasional intralenticular hemorrhage Sporadic , unilateral(90%) , progressive Early surgery with vitrectomy instruments Visual prognosis depends on amount of microphthalmia and involvement of posterior pole

Bilateral PHPV : Norries disease , Trisomy13 POSTERIOR SUBCAPSULAR

Developmental Downs syndrome ,steroid, blunt trauma ,idiopathicBLUE DOT

Small scattered blue white opacities

Visually not significant

Downs syndromePOSTERIOR LENTICONUSThinning and bowing of central posterior capsule Unilateral Myopia , irregular astigmatism , amblyopia Progressive and eventually needs surgery Difficult surgery due to bowing of posterior capsule CHRISTMAS TREE Small flecks with various colors

Myotonic dystrophy ,hypoparathyroidismMEMBRANOUS End stage

Absorbed lens material ,fused capsules

Hallerman strieff , Rubella , Lowe ,Trauma IMPORTANT POINTS REGARDINGMORPHOLOGY

Visually significant : nuclear , lamellar ,posterior , total ,membranous

Progressive : posterior lenticonus , PHPV ,lamellar , subcapsular

Most common : lamellar

Better visual prognosis : anterior ,sutural , posterior lenticonus ETIOLOGY Hereditary isolated cataract Metabolic diseases Intra uterine infections Trauma IdiopathicChromosomal abnormalities Other causes ETIOLOGY

Bilateral : 45% idiopathic , 50% hereditary , 5% infectiousUnilateral : 85% idiopathic, 5% hereditary , 2% infectious , 8% trauma Associated with systemic disease : 25% in bilateral ,5% in unilateralOcular abnormality : 15% in bilateral , 50% in unilateralHEREDITARY ISOLATED CATARACT AD : most common , 25% new mutation variable expression ,examination of family members , associated with microphthalmos AR, XR ,rare INTRA UTERINE INFECTION

TORCHS : toxoplasma , rubella , CMV ,herpes , syphilis Usually bilateral , dense , and central IgM antibody titer is elevated Rubella is the most common Total cataract due to candida in premature infantsRUBELLA CATARACT

15% of patients with congenital rubella have cataract 80% bilateral Retinopathy ,strabismus ,microphthalmos ,optic atrophy ,glaucoma Systemic : congenital heart defects , hearing loss , mental retardation Prone to sever inflammation , post operative high dose steroid and pupillarydilationMETABOLIC DISORDERSCataract usually appears after birth Galactosemia , Hypoglycemia , DM Fabrys disease Hypoparathyroidism : multicolor cataract Wilsons disease :AD , 20% develop posterior subcapsular sunflower cataract

GALACTOSEMIA

Oil droplet cataract progress to lamellar and then total cataract AR , three types Transferase deficiency : early cataract , sever systemic disease vomiting ,diarrhea , hepatomegaly , jaundice Galactokinase deficiency : cataract later in infancy , few systemic signs , milk restriction reverse cataract Epimerase deficiency : no cataract Diagnosis : urine for reducing substance 2 hours after milk feedingHYPOGLYCEMIA Complicated pregnancy Small for gestational age Mental retardation Bilateral lamellar cataract More in boys Reversible in most casesFABRYS DISEASE

Alpha galactosidase deficiency XR Posterior spoke like cataract in 50% Not visually significant Whorl like sub epithelial corneal opacity Tortuosity of ocular vesselsRENAL DISORDERS Lowes syndrome : XR , rare , congenital cataract in all , glaucoma ,miotic pupil, mental retardation , death in 2nd decade , amino acid in urine Alport : XD , anterior lenticonus , hematuria , proteinuria , deafness , myopia ,cataract is not visually significantOCULAR DISEASES ASSOCIATEDWITH PEDIATREIC CATARACT

Microphthalmia: the most common Aniridia

PHPV

ROP STREOID INDUCED CATARACT

Posterior sub capsular cataract

Reversible in initial stage

Slow progression in children

IATROGENIC PEDIATREIC CATARACT

Laser photoablation for ROP or tumor External beam radiation steroid therapy Damage to posterior capsule due to posterior vitrectomyIMPORTANT POINTS Reversible in early stages : galactosemia , steroid induced , hypoglycemia cataract and glaucoma : Lowe, Rubella, Aniridia, Anterior segment dysgenesisDIAGNOSIS

LEUKOCORIAWhite reflex in anterior or diffuse opacities of lensSTRABISMUSNYSTAGMUSPHOTOPHOBIA SIZE AND DENSITY OF CATARACT Evaluation with direct ophthalmoscope or retinoscopeMore than 3mm dense central opacity is significant and need surgeryIn incomplete bilateral cataracts , density is more important than the size of opacityIf major retinal vessels can not be seen through the cataract , surgery is indicatedSemi transparent opacities should be treated conservatively

DIAGNOSIS

Early detection and treatment of cataract in all infants is the aim This aim is difficult to achieve Screening is mandatory in, nursery , at 6 weeks , and in 6 months of age for all infants by assessment of red reflex with direct ophthalmoscope Photophobia more in zonular type Causes of Leukocoria

DIFFERENTIAL DIAGNOSIS OF LEUKOCORIA Cataract Retinoblastoma Toxocariasis Coats disease ROP PHPV Retinal detachment Coloboma Retinal dysplasia Norries diseaseSTRABISMUS

Common May develop even after surgery Up to 50% of children with cataract will develop strabismus More in unilateral cataract ET is more common in congenital cataract XT is more common in later onset and traumatic cataracts NYSTAGMUS Develop in 2 to 3 months of life Sign of early visual deprivation Searching nystagmus Poor visual prognosis , VA20/60 in 40% of unilateral and 60% of bilateral casesPARENTS CONSULTATION Parents should understand that treatment of the child starts only after surgery Need to regular follow up visits Need for wearing glasses or contact lenses even despite IOL implantation Need for occlusion therapyPOST SURGICAL TREATMENT

Evaluation of fixation behavior Refraction in each visit Periodically IOP measurement under GAIn unilateral cases , occlusion of the fellow eye 50 to 70% of waking hoursNONSURGICAL TREATMENTNS

For peri central or small semi transparent central cataracts Pupil dilation with 2.5% phenylephrine and sometimes tropicamide

Part time occlusion of good eye FACTORS INFLUENCING VISUAL OUTCOME Age of the onset of cataract Age of the surgery Associated ocular and systemic conditions Compliance with optical an occlusion therapyPROGNOSTIC FACTORS The most posterior and the most central the cataract, the greater the visual impact Surgery before 6-8 weeks of life is the most important prognostic factor

Surgery before 4 weeks of life is associated with increased risk of glaucoma and pupillary membraneIOL implantation will increase risk of reoperation Nystagmus is a poor prognostic factor Amblyopia is more advanced in unilateral cataractsVISUAL OUTCOME

Surgery before 2monthsUnilateral : VA 20/60 ( 20/800 to 20/30 )Bilateral : 80% VA more than 20/50gross stereopsis may develop on rare occasions Surgery after 2 months or with nystagmusm Unilateral : VA HM to 20/160 Bilateral : VA less than 20/100Considerations regarding surgery Intraocular lens : Power of lens Myopic shift of thegrowing eye Surgical technique Postoperative intraocular inflammation (uveitis) Glaucoma and retinal detachment may developIdeal IOL for the implantation in children No anterior chamber IOLs IOL haptic size - 10,0 11 mm Haptics angulation - 0 . Foldable optic sized 5,75 6,0 mm (rigid - 5x6 mm) Biconvex Material - hydrophilic acryl HYDROPHOBIC IOL designa. Single piece b. Possibility of haptics adaptation without IOL decentration in response to capsular growth or its contraction (no posterior vaulting) - probably complex, closed loop haptic c. Very good torsional and rotational stability of IOL in the capsul d. Square edges 8. Blue filter ? 9. Possibility of implantation with the use of injector with thenozzle diameter smaller than 2,0 mm.IOL POWER 1 - 2 YEARS = IOL FORMULA - 20% 2 - 4 YEARS = IOL FORMULA - 15% 4 - 8 YEARS = IOL FORMULA - 10% > 8 YEARS = IOL FORMULA UNDERCORRECT IF RESULTSQUESTIONABLEPostoperative considerations Clear vision for distance and near Intraocular lens: regular refraction Spectacles Contact lenses Treatment of amblyopia : Occlusion therapyConclusion: Congenital cataracts Correct management essential to prevent permanentvisual loss Team effort ophthalmologist, paediatrician,geneticist,family Early detection within the first month of life is veryimportant Knowledge of systemic conditions associated with cataract Glaucoma may develop