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Pediatric cataracts , case selection before surgery Dr.Sayed Ezatollah Memarzadeh ophthalmologist Esfahan 1391

EPIDEMIOLOGY 1 In every 250 newborn has lens opacity most of them are not visually significant Prevalence of visually significant cataract is 1.2 to 6 in 10000 live birth 10-20% of blindness in children worldwide Bilateral cataract is more common than unilateral

MORPHOLOGY AND LOCATION Three main types : zonular , polar , lenticonus Zonular 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 astigmatism microphthalmos or persistent pupillary membrane 1/3 anisometropia , strabismus , amblyopia

Morphology : Examples

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

Anterior pyramidal

ANTERIOR SUBCAPSULAR Often idiopathic Trauma Alport’s 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 AD

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

Zonular lamellar cataracts

TOTAL All layers are involved AD ,Down’s syndrome , Metabolic disease, Trauma Progression of other types of cataract

PERSISTENT 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 : Norrie’s disease , Trisomy13

POSTERIOR SUBCAPSULAR Developmental Down’s syndrome ,steroid, blunt trauma ,idiopathic

BLUE DOT Small scattered blue white opacities Visually not significant Down’s syndrome

POSTERIOR LENTICONUS Thinning 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 ,hypoparathyroidism

MEMBRANOUS End stage Absorbed lens material ,fused capsules Hallerman strieff , Rubella , Lowe ,Trauma

IMPORTANT POINTS REGARDING MORPHOLOGY 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 Idiopathic Chromosomal abnormalities Other causes

ETIOLOGY Bilateral : 45% idiopathic , 50% hereditary , 5% infectious Unilateral : 85% idiopathic, 5% hereditary , 2% infectious , 8% trauma Associated with systemic disease : 25% in bilateral ,5% in unilateral Ocular abnormality : 15% in bilateral , 50% in unilateral

HEREDITARY 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 infants

RUBELLA 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 pupillary dilation

METABOLIC DISORDERS Cataract usually appears after birth Galactosemia , Hypoglycemia , DM Fabry’s disease Hypoparathyroidism : multicolor cataract Wilson’s 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 feeding

HYPOGLYCEMIA Complicated pregnancy Small for gestational age Mental retardation Bilateral lamellar cataract More in boys Reversible in most cases

FABRY’S DISEASE Alpha galactosidase deficiency XR Posterior spoke like cataract in 50% Not visually significant Whorl like sub epithelial corneal opacity Tortuosity of ocular vessels

RENAL DISORDERS Lowe’s 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 significant

OCULAR DISEASES ASSOCIATED WITH 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 vitrectomy

IMPORTANT POINTS Reversible in early stages : galactosemia , steroid induced , hypoglycemia cataract and glaucoma : Lowe, Rubella, Aniridia, Anterior segment dysgenesis

DIAGNOSIS LEUKOCORIA White reflex in anterior or diffuse opacities of lens STRABISMUS NYSTAGMUS PHOTOPHOBIA

SIZE AND DENSITY OF CATARACT Evaluation with direct ophthalmoscope or retinoscope More than 3mm dense central opacity is significant and need surgery In incomplete bilateral cataracts , density is more important than the size of opacity If major retinal vessels can not be seen through the cataract , surgery is indicated Semi 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 Coat´s disease ROP PHPV Retinal detachment Coloboma Retinal dysplasia Norrie´s disease

STRABISMUS 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 , VA<20/200 Presents in 50% of children with dense bilateral cataract Rarely seen in unilateral cataract Cataract surgery may dampen the nystagmus

EVALUATION OF A CHILD WITH BILATERAL CATARACT Family history : critical , exam of parents and siblings History : age of onset General Ocular Laboratory tests : IgM for TORCH , VDRL , Urine for reducing agent after milk Optional laboratory tests : Urine amino acid (Lowe’s syndrome) and protein( Alport’s syndrome) ( , RBC galactokinase) , Blood sugar Ca and Ph , Copper

The visually significant cataract In central visual axis, bigger than 3mm Posterior cataract No clear zones in between Retinal details not visible with direct ophthalmoscope Nystagmus or strabismus present Poor central fixation after 8 weeks

DECISION FOR SURGERY IS DEPENDENT IN THESE FACTORS Laterality Visual behavior of the infant (FIXATION) Presence of associated ocular and systemic abnormalities

LATERALITY If a child with unilateral or bilateral cataract develops strabismus, surgery must be done as soon as possible In partial unilateral cataract , pupillary dilation combined with amblyopic therapy is an alternative for surgery If a child with bilateral cataract develops nystagmus , surgery is indicated although visual prognosis is generally poor

VISUAL BEHAVIOR Visual attention Pupillary reflex : RAPD is poor prognostic sign Ability to pick up small objects

TIMING OF SURGERY dense cataract , surgery must be done before age of 6 weeks in unilateral cases dense cataract , surgery must be done before age of 10 weeks in bilateral cases Interval between the surgery of the two eyes with should be minimized Surgery before 4 weeks of life will increases the risk of glaucoma and pupillary membrane Some authors advocate surgery on both eyes simultaneously in selected cases

BILATERAL PATCHING Total bilateral patch for unilateral or bilateral cataract Temporary , less than 2 weeks It may extend the critical period of visual development Contraindicated in unilateral cataract with poor prognosis like optic nerve or macular pathology

Evaluation 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)

CATARACT IN 4-6 MONTHS BABY Is it truly congenita? Previous photographs to see red reflex Best prognosis if there is no strabismus and nystagmus Surgery must be done even in presence of strabismus or nystagmus VA>20/60 in 40% of unilateral and 60% of bilateral cases

PARENTS 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 therapy

POST SURGICAL TREATMENT Evaluation of fixation behavior Refraction in each visit Periodically IOP measurement under GA In unilateral cases , occlusion of the fellow eye 50 to 70% of waking hours

NONSURGICAL 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 therapy

PROGNOSTIC 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 membrane IOL implantation will increase risk of reoperation Nystagmus is a poor prognostic factor Amblyopia is more advanced in unilateral cataracts

VISUAL OUTCOME Surgery before 2months’’ Unilateral : VA 20/60 ( 20/800 to 20/30 ) Bilateral : 80% VA more than 20/50 gross stereopsis may develop on rare occasions Surgery after 2 months or with nystagmusm’’ Unilateral : VA HM to 20/160 Bilateral : VA less than 20/100

Considerations regarding surgery Intraocular lens : Power of lens – Myopic shift of the growing eye Surgical technique Postoperative intraocular inflammation (uveitis) Glaucoma and retinal detachment may develop

Ideal” 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 design a. 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 the nozzle diameter smaller than 2,0 mm.

IOL POWER 1 - 2 YEARS = IOL FORMULA - 20% UNDERCORRECT IF RESULTS QUESTIONABLE

Postoperative considerations Clear vision for distance and near Intraocular lens: regular refraction Spectacles Contact lenses Treatment of amblyopia : Occlusion therapy

Conclusion: Congenital cataracts Correct management essential to prevent permanent visual loss Team effort ophthalmologist, paediatrician, geneticist,family Early detection within the first month of life is very important Knowledge of systemic conditions associated with cataract Glaucoma may develop

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