Congenital cataract & ITS MANAGEMENT

CONGENITAL CATARACT

1. Congenital cataract By: Dr nikita jaiswal Pg 1 st year Ims and sum hospital

2. These cataracts are present at birth or that develop within the first year of life are called congenital /infantile cataract. Fairly occurring in 1 of every 2000 live births - some lens opacities do not progress and are usually insignificant - others can produce profound visual impairment

3. Congenital cataract -- unilateral -- bilateral in general these congenital cataract 1/3 rd extensive syndromes 1/3 rd inherited trait 1/3 rd undetermined cause

4. bAsed on etiology Bilateral Idiopathic Hereditary- autosomal dominant -autosomal recessive/x-linked genetic/metabolic disease -down syndrome,marfan syndrome - hypoglycaemia,hypoparathyroidism -Myotonic dystrophy Maternal infection - rubella,cmv,varicella,syphilis,toxoplasmosis Ocular anoamlies-aniridia Toxic – corticosteroids,radiation Unilateral Idiopathic Ocular anomalies - post.lenticonus -post pole tumors -persistent fetal vasculature Traumatic Rubella Masked b/l cataract

5. Congenital cataract in a variety of morphologic configuration lamellar polar sutural coronary cerulean capsular complete & membranous

6. Lamellar: it is also known as zonular cataract - these are autosomal dominant trait - effect on visual acuity with the size & density of the opacity - these are opacifications of specific layers/zones of the lens - visible as an opacified layer that surrounds a clearer center & is itself surrounded by a layer of clear cortex - disc shaped configuration -riders-these are horseshoe shaped opacities .

7. Polar cataract : lens opacity involves subcapsular cortex&capsule of anterior or posterior pole of the lens. Ant polar cat.-it is a.d small,b /l symmetric,non progressive opacities that do not impair vision. Post polar cat.-it produces more visual impairment because it tends to be larger in size they may be-familial-usually b/l sporadic-often unilateral

8. sutural: the sutural or stellate cataract is an opacification of the “y” sutures of the fetal nucleus -it doesnot impair vision -these opacities often have branches or knobs projecting from them.

9. CORONARY: A.D GROUP OF CLUB SHAPED CORTICAL OPACITIES THAT ARE ARRANGED AROUND THE EQUATOR OF LENS LIKE A CROWN --THEY CANT BE SEEN UNTILL THE PUPILS ARE DILATED --USUALLY DO NOT AFFECT THE VISUAL ACUITY

10. CERULEAN :SMALL BLUISH OPACITIES LOCATED IN THE LENS CORTEX --HENCE THEY ARE ALSO K/as BLUE DOT CATARACT --NON-PROGRESSIVE USUALLY DO NOT CAUSE VISUAL SYMPTOMS

11. CAPSULAR - THESE CATARACTS are small opacificationof the lens epithelium & anterior lens capsule that spare the cortex complete- also k/as total cataract all the lens fibres are opacified . The red reflex is totally obscured retina cant be seen by direct /indirect oph .

12. SPOKE LIKE FABRY’S DISEASE VACUOLES MANNOSIDOSIS DIABETES MULTICOLOR FLECKS HYPOPARATHYRODISM MYOTONIC DYSTROPHY GREEN “SUNFLOWER” WILSON’S DISEASE THIN DISCIFORM LOWE’S SYNDROME LAMELLAR GALACTOSEMIA HYPOGLYCEMIA

13. RUBELLA- CAUSED BY RUBELLA VIRUS CAN CAUSE FETAL DAMAGE ESPECIALLY IF THE INFECTION OCCURRS IN 1 st TRIMESTER OF PREGNANCY. PEARLY WHITE OPACIFICATIONS ENTIRE LENS IS OPACIFIED & CORTEX MAY LIQUEFY LIVE VIRUS PARTICLES MAY BE RECOVERED AS LATE AS 3 YRS AFTER BIRTH CATARACT REMOVAL MAY BE COMPLICATED BY EXCESSIVE POST-OP INFLAMMATION RELEASE BY THESE LIVE VIRUS

14. Management

15. -detailed history -careful clinical evaluation -basic assessment of child’s vision - iop -fundus examination under dilatation -B-scan for posterior segment A-scan to measure axial length of both the eyes

16. TIME OF SURGERY SURGICAL TECHNIQUE TYPE OF OPTICAL REHABILITATION POST-OP MANAGEMENT OF AMBLYOPIA

17. CONGENITAL CATARACTS:LABORATORY EVALUATION RESULT POSSIBLE DIAGNOSIS + REDUCING SUBSTANCE AMINOACIDURIA HEMATURIA , PROTEINURIA ‘’MALTESE CROSS” FIGURES GALACTOKINASE DEFICIENCY LOWE’S SYNDROME FABRY’S DISEASE ERYTHROCYTE ENZYMES GLUCOSE TORCH titres,VDRL test CALCIUM, PHOSPHORUS GALACTOKINASE DEFICIENCY HYPER/HYPO GLYCEMIA RUBELLA.TOXOPLASMOSIS,CMV,HERPES,SYPHILIS,HYPOPARATHYROIDISM urine Blood

18. CONGENITAL CATARACT:DIAGNOSTIC EVALUATION CONDITION LABORATORY TEST GALACTOSEMIA URINE REDUCING SUBSTANCE RUBELLA ANTIBODY TITERS SYPHILIS VDRL TEST HYPOPARATHYROIDISM SERUM CALCIUM,PHOSPHORUS,ALKALINE PHOSPHATASE WILSON’S DISEASE SERUM CERULOPLASMIN HYPERGLYCEMIA/HYPOGLYCEMIA BLOOD GLUCOSE FABRY’S DISEASE URINE”MALTESE CROSS”(POLARIZED IIGHT) LOWE’S SYNDROME URINE AMINO ACIDS

19. Treatment is indicated only if the vision is considerably impaired --medical --surgical

20. medical If the patient has small opacities in whom the red reflex is not considered significantly impaired In some patients with small central opacity{3 mm or less} Patching Dilatation with tropicamide 0.5%or cyclopentolate 0.5% If vision improves 6/18 then no surgery required Who requires chronic cycloplegic agents to maintain dilation & in visual acuity has improved –surgical optical iridectomy should be considered. Classic eg .-peter anomaly –central cataract + corneal opacity but has a clear peripheral lens & cornea optical iridectomy better than corneal transplant & cataract extraction.

21. surgical If dense unilateral or bilateral critical period appears to be within the first 2 months. First 6 wks – precortical stage 6-8 wks -cortical stage Unilateral cat.--operated on by age 6 wks Bilateral cat.—slightly larger window 8--10 wks

22. Historical landmarks

23. Before 1960 – most congenital cataracts were removed by an extracapsular technique. in 1960- scheie introduced discission & aspiration technique in 1972 - machemar et al developed a new instrument { visc } vitreos infusion suction cutter current surgical technique: vitrectomy cutting instruments, irrigation/ aspiration,phaco or some combination of this technique

24. Current surgical technique Incision _ usually the incisions we take are self healing but in children the corneal tissue is less likely to heal thus suture closure of tunnel wounds re advised. Anterior capsulorhexis : a 1.4% sodium hyluronate is recommended for paediatric surgery to maintain the A.C stability abd increased vitreous upthrust.the ant. Capsulotomy shape,size and integrity are important to long term centration of iol .{the fugo plasma blade is a new tool for performing ant capsulotomy in children. Hydrodissection :to ensure maximum removal of lens cortex and lens epithelial cells, may be a single or multiple site --------- prerformed by injecting RL or balanced salt solution inn 2 ml disposable syringe avoided in cataract with post. Lenticonus or post polar cataract

25. Cataract removal -lens material may be removed with phacoaspirationor irrigation and aspiration. Posterior cont.. Curvilinear capsulorexhis { pccc }: we perform this at the age less than 6-8 years & any children with nystagmus where future yag may be difficult it is done to prevent the pco as it is amblyogenic and the surgeon is defeated in achieving the target use of high viscosity viscoelastic helps to achieve pccc.the desirable size of post rhexis is 3-3.5 mm. ant.vitrectomy . Iol lens implantation: capsular bag implantation is the best choice as iol & uveal tissue contact is lesser& centration is achieved{ aios advice it to be done by paediatric ophthalmologists}

26. Iol selection: pmma iols were the only choice the single piece hydrophobic acrylic iols are ideal for implantation now multifocal iol are gaining grounds as it gives the good compatibility with near and far vision of child limitations : iol power predictability visual development incision closure

27. Birth 34.4 0-1yr 28.7 1-2yr 26.4 2-3yr 23.0 3-4yr 22.1 4-5yr 20.9 5-6yr 19.5 INTRAOCULAR LENSES POWER TO ACHIEVE EMMETROPIA

28. Undercorrecting biometry by 10% in 2-8 yrs for children younger than 2 yrs under correct by 20% 1 year +6D 2 year +5 D 3 year +4 D 4 year +3 D 5 year +2 D 6 year +1 D 7year PLANO 8 year -1 TO -2 D 21MM 22.00D 20MM 24.00D 19MM 26.00D 18MM 27.00D 17MM 28.00D AXIAL LENGTH POWER

29. Visual rehabilitation Glasses { aphakic spectacles} Contact lens Epikeratophakia Intraocular lens

30. Aphakic spectacles ADVANTAGES: THEY CAN EASILY BE UPDATED TO MATCH THE RAPIDLY CHANGING REFRACTIONS IN YOUNG CHILDREN DISADVANTAGES: LENS THIKNESS & WEIGHT AS WELL AS OPTICAL DISTORTIONS IN NEW BORNS LENS POWER OF +24 TO +26D Which can be accomplished with very thick bubble shaped lens in older children the thinner high ensity aphakic specs can be used . Patching of normal eye is necessary when the child is using aphakic specs

31. CONTACT LENS MOST COMMON METHOD FOR BOTH BILATERAL AND UNILATERAL APHAKIA. ADVANTAGES: OPTICAL QUALITY IS GOOD *SOME CL CAN BE WORN THROUGHOUT 24 HOURS A DAY DISADVANTAGES- - RELATIVELY THICK -CAN BE WASHED OR RUBBED OUT EASILY -TIDIOUS FOR PARENTS -ASSOCIATED WITH CORNEAL COMPLICATIONS AS INFECTIONS & ULCERS LENS : SILICONE – HIGH O2 PERMEABILITY CHILDREN YOUNGER THAN 6 MONTHS-36 D Gas permeable lens can also be used

32. Epikeratophakia in 1980’s first performed because of problem in specs & c.l’s Procedure :- removing a central half thickness of the cornea & then suturing predetermined corneal donor tissue. Disadvantages: persistent hazinessespecially at the interface between host & the graft that could take up an year to clear. Late myopia & astigmatism in many eyes

33. Thank you