سلام خوشحالم در جمع شما اساتید محترم حضور دارم

1. سلام خوشحالم در جمع شما اساتید محترم حضور دارم

2. Pediatric cataracts , case selection before surgery Dr.SayedEzatollahMemarzadeh ophthalmologist Esfahan 1391

3. 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

4. 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

5. 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

6. Morphology : Examples

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

8. Anterior pyramidal

9. ANTERIOR SUBCAPSULAR • Often idiopathic • Trauma • Alport’s syndrome

10. NUCLEAR Dense , congenital , visually significant Bilateral , often AD Associated with microphthalmos

12. SUTURAL Involved Y sutures and fetal nucleus Progressive , visually not significant Bilateral , often AD

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

14. Zonular lamellar cataracts

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

16. 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

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

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

19. 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

20. CHRISTMAS TREE Small flecks with various colors Myotonic dystrophy ,hypoparathyroidism

21. MEMBRANOUS End stage Absorbed lens material ,fused capsules Hallermanstrieff , Rubella , Lowe ,Trauma

22. 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

23. ETIOLOGY • Hereditary isolated cataract • Metabolic diseases • Intra uterine infections • Trauma • Idiopathic • Chromosomal abnormalities • Other causes

24. 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

25. HEREDITARY ISOLATED CATARACT AD : most common , 25% new mutation variable expression , examination of family members , associated with microphthalmos AR, XR ,rare

26. 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

27. 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

28. 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

30. 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

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

32. 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

33. 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

34. OCULAR DISEASES ASSOCIATEDWITH PEDIATREIC CATARACT Microphthalmia: the most common Aniridia PHPV ROP

35. STREOID INDUCED CATARACT Posterior sub capsular cataract Reversible in initial stage Slow progression in children

36. IATROGENIC PEDIATREIC CATARACT Laser photoablation for ROP or tumor External beam radiation steroid therapy Damage to posterior capsule due to posterior vitrectomy

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

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

39. 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

41. 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

42. Causes of Leukocoria DIFFERENTIAL DIAGNOSIS OF LEUKOCORIA Cataract Retinoblastoma Toxocariasis Coat´s disease ROP PHPV Retinal detachment Coloboma Retinal dysplasia Norrie´s disease

43. 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

44. 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

45. 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

46. 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

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

48. 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

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

50. 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