Childhood cataracts

childhood cataracts
how to approach
differential diagnosis
treatment
glasses prescription
amblyopia

1. Panit Cherdchu , M.D. Ophthalmology Department Phramongkutklao Hospital Childhood Cataracts

2. CLASSIFICATION

3. Pediatric cataracts can be congenital or acquired The earlier the onset, the more amblyogenic the cataract will be Lens opacities that are visually significant before 2-3 months of age are the most likely to be detrimental to vision

4. Morphology and etiology (manifest in pattern) Cataract Etiology Other possible finding Spoke like Fabry syndrome Mannosidosis Corneal whorls hepatosplenomegaly Vacuolar Diabetes mellitus Increased blood glucose level Multicolored flecks Hypoparathyroidism Myotonic dystrophy Low serum calcium level Characteristic facial features, tonic “grip” Green sunflower Wilson disease Kayser -Fleisher corneal ring Thin disciform Lowe syndrome Hypotonia , glaucoma

5. Anterior polar cataracts (APCs) Less than 3 mm in diameter Small white dots in the center of Anterior lens capsule Congenital Sporadic opacities Unilateral or bilateral Nonprogressive and visually insignificant Unilateral APCs are associated with anisometropia  Amblyopiarefraction and F/U is important Anterior pyramidal cataracts = pyramidal shape and project into anerior chambermust be distinct from original APCs due to more progressive and amblyogenic factors Anterior pyramidal cataracts APCs

6. Nuclear Cataracts Opacities involve the center or nucleus 3 mm in diameter Irregularity of the lens fibers can extend more peripherally Stable in most cases bilateral>unilateral Inherited or sporadic Eyes with nuclear cataracts might be small and are at risk for glaucoma in childhood

7. Lamellar cataracts Discrete, round, lamellar affect 1 or more layers of cortex Larger than nuclear cataracts 5 mm or larger in diameter Bilateral>Unilateral Because onset is usually after the fixation reflex has been established, patients with lamellar cataracts have a better visual prognosis then those with earlier cataracts onset

8. Anterior Lenticonus This refers to a thinned-out central anterior capsule with or without anterior cortical opacities. Anterior lenticonus is said to be characteristic of Alport syndrome. Spontaneous rupture of the lens can occur, resulting in a hydrated total cataract.

9. Posterior lenticonus (lentiglobus) Progressive thinning of the central posterior capsule Thinning causes the deformation to have an oil droplet appearance on red reflex examination Outpouching of the lens Capsule can develops tear rapid total opacification will come along Almost always unilateral*** Normal eye size Posterior capsule weakness is congenital but cataracts will develop later Good visual prognosis after surgery

10. Posterior subcapsular cataracts (PSCs) Less common in children than adults Usually acquired and bilateral Progressive Corticosteroid, uveitis, retinal abnormalities, and radiation exposure are risk factors Association with NF type 2

11. Sectoral cataracts Wedge-shaped cortical cataracts are occasionally seen in children Idiopathic or associated with occult posterior segment tumor, previous blunt trauma, or retinal coloboma with fibrous bands***

12. Peripheral vacuolar cataract Premature infants Encounter during ROP examination Resolve over time

13. Persistent fetal vasculature (PFV) Last knowns as persistent hyperplastic primary vitreous Most common cause of unilateral cataract Isolated, sporadic malformation Bilateral cases may be associated with systemic or neurologic abnormalities Affected eye are smaller than the other PFV has a spectrum of severity Mild PFV are prominent hyaloid vessel remnants, a large mitendorf dot, and a bergmeister pailla End of the spectrum are microphthalmic eyes with dense retrolental plaques; a thick fibrous persistent hyaloid artery; elongated ciliary processes (classic for PFV); and a prominent radial iris vessels Traction on the optic disc may cause distortion of the posterior retina Retrolental plaque is densest centrally

14. Courtesy of Nasreen Syed, MD. Photomicrograph of persistent fetal vasculature. Note the prominent anterior fibrovascular plaque ( asterisk ). The posterior remnant of the persistent hyaloid artery is evident at the optic nerve head ( arrow ).

15. Courtesy of Jerry A. Shields, MD

16. Traumatic disruption of lens In children, traumatic anterior lens capsule rupture quickly results in a hydrated white cataract. However , in children, lens cortex in the anterior chamber may be well tolerated without an intraocular pressure (IOP) rise. Cataract surgery can often be delayed for a few days or up to 3 or 4 weeks to allow the traumatic iritis to subside before the cataract and IOL surgery.

17. Role of vision screening vision screening is mandatory to detect cataracts as soon as possible. Late detection may result in poor visual outcomes. All newborns must have red reflex screening, ideally followed by another red reflex examination at the 6-8 week neonatal checkup. Red reflex testing is done by using direct ophthalmoscope from a distance of 1-2 feet in a darkened room. Preschool vision screening (at 3 and 5 years) is often done in the community. Photo screeners are used in preverbal and verbal children. These may help the pediatrician save time in screening. They work by a computer analyzing the red reflex for inequality in color, intensity, or clarity. New screeners utilizing polarized laser light are more accurate at detecting decreased vision. The presence of any opacities, an absent red reflex, or leukocoria should prompt an urgent referral to a pediatric ophthalmologist.

18. Examination “Surgery is not definite” Anterior capsule opacities are not visually significant unless they occlude the entire pupil Central or posterior lens opacities greater than 3 mm in diameter are usually visually significant Strabismus+unilateral cataract and bilateral cataracts+nystagmus =visually significant

19. Preverbal children older than 2 months fixation behavior, fixation preference, and object occlusion Bilateral cataractsfixation behavior+family’s observation Older children (lamellar or PSCs), glare testing may be useful for detecting decreased vision Monitor posterior segment, B-scan ultrasonography if blocked

20. Work up Unilateral cataracts are not usually associated with occult systemic or metabolic disease Bilateral cataracts may be associated with many systemic and metabolic disease Family history+no associated systemic disease  work up is not necessary Geneticist is crucial in detecting associated disorders

21. Cataracts surgery in pediatric patients

22. Timing of the procedure

23. “The younger the child, the greater the urgency to remove the cataract” To reduce the risk of visual deprivation amblyopia For optimal visual outcome ***Visually significant unilateral cataract should be removed before age 6 weeks ***Visually significant bilateral cataracts, before age 10 weeks

24. For older children with bilateral cataracts, surgery should be done when the level of visual function interferes with the child’s visual needs Although BCVA with 20/70 is satisfied for kids but it is never enough later in life surgery is performed when BCVA < 20/40 Driver’s license** For older children with unilateral cataract, perform surgery when optical treatment and amblyopia therapy cannot improve VA beyond 20/40

25. Intraocular Lens Use in Children IOL implantation depends on age Children aged 1-2 years and older is widely accepted Younger infants is not the best candidate Compared with CL rehabilitation in aphakic patients, IOL implantation in infants aged 1-6 months is associated with a significantly higher rate of complication, but not to visual acuity outcome at age 1 year Surgical intervention with CL wear + patching of the uninvolved eye for treatment of amblyopia Infants with aphakic secondary IOL implantation can be performed after 1-2 years of age Infant aphakia treatment study showed that aphakic infants with mild PFV treated with CL had a higher incidence of adverse evnts after lensectomy compared with children with other forms of unilateral cataract  both groups had similar visual outcomes 1 years after surgery

26. IOL formula Method 1: emmetropia pros: no glasses needed after surgery and low risk of amblyopia in poor compliance patients Cons: myopia when grow up Method 2: undercorrection Pros: emmetropia when grow up Cons: marked hyperopia and glass needed after surgery+ risk of amblyopia in poor compliance patients

27. Intraocular Lens Use in Children Age Aim undercorrection อ.อาภัทรสา add < 3 mo 40% + 5% 3-6 mo 35% + 5% 6-12 mo 30% + 5% 12-24 mo 25% + 5% 24-60 mo 20% + 5% 60-72 mo 10-15% + 5% 7-8 yr 2 D   8-10 yr 1 D   >10 yr Emmetropia   Rupal H Trivedi et al. Ophthalmic pearls: Selecting intraocular lens power in children. www.aao.org

28. Rupal H Trivedi et al. Ophthalmic pearls: Selecting intraocular lens power in children. www.aao.org Intraocular Lens Use in Children Age Residual refraction <1.9 mo +10 D 2-3.9 mo +9 D 4-5.9 mo +8 D 6-11.9 mo +7 D 1-1.9 yr +6 D 2-3.9 yr +5 D 4-4.9 yr +4 D 5-5.9 yr +3 D 6-6.9 yr +2 D 7-7.9 yr +1.5 D 8-8.9 yr +1 D 10-13.9 yr +0.5 D >14 yr plano

29. BJO 2015 Intraocular Lens Use in Children Age Residual refraction (D) <6 mo +6 to +10 6-12 mo +4 to +6 1-3 yr +4 3-4 yr +3 4-6 yr +2 to +3 6-8 yr +1 to +2 >8 yr Plano to +1

30. Intraocular Lens Use in Children Dahan (JCRS 1997) <2 yr : 20% undercorrection 2-8 yr : 10% undercorrection Enyedi (AJO 1998) Rule of 7 Age + undercorrection = 7 Infant aphakia treatment study (IATS) 4-6 wk : 8 D undercorrection 7-26 wk : 6 D undercorrection IOL: age based undercorrection

31. Management of the Anterior Capsule Different technique with adult capsulorhexis Elasticity of the capsule is greatest in younger patients 2-incision push-pull technique

32. Lensectomy without IOL implantation Lensectomy is performed through a small limbal or pars plana incision with a vitreous-cutting instrument ( vitrector ) Irrigation can be provided by an integrated infusion sleeve or by a separate cannula Phacoemulsification is not required ( lens are soft in children) Remove all cortical material because of the propensity for reproliferation of pediatric lens epithelial cells Posterior capsule opacification occurs rapidly I nyoung children controlled posterior capsulectomy and anterior vitrectomy should be performed at the time of surgery in children who are unlikely candidates for awake Nd:YAG capsulotomy Clear view leads to better screening, refraction and follow up

33. Lensectomy with IOL implantation Single-piece acrylic foldable IOLs is usually used Single-piece PMMA lenses are also still used Silicone lenses have not been well studied in children Primary IOL implantation Leave the posterior capsule intact then NdYAG capsulotomy later (mostly 5 yrsold or older) Primary capsulectomy is usually prefered for younger children due to opacification of lens capsule after surgery might occur in 18-24 months

34. Technique with posterior capsule intact Cortex is aspirated Clear corneal or scleral tunnel incision is enlarged to allow placement of the IOL Place in capsular bag or ciliary sulcus fixation is an acceptable choice Remove all viscoelastic material to prevent post operative rising of IOP Closure of 3-mm incision with absorbable suture has been shown to be safe and does not induce astigmatigsm

35. Techniques for primary posterior capsulectomy Posterior capsulectomy /vitrectomy before IOL placement After lensectomy , the vitrector (low suction/high-cutting rate) is performed to remove posterior capsule promtly with anterior vitrectomy The anterior capsule is enlarged Lens is implanted in the capsular bag +/- ciliary sulcus The capsulotomy must not extend/ IOL haptics do not pass the posterior opening Vitreous must not entangle with IOL Posterior capsulectomy /vitrectomy after IOL placement Place the IOL in the capsular bag Close the anterior incision Approach the posterior capsule through the pars plana Small conjunctival opening over the pars plana Sclerotomy by microvitreoretinal blade 2.5-3.0 mm posterior to the limbus Wide anterior vitrectomy can be performed

36. IOL implantation issues Complicated IOL calculation Variable growth of the eye Inaccurate keratometry Inaccurate axial length measurement Formulas is mostly for an adult Studies have shown RF error of aphakic pediatric eyes undergoes a variable myopic shift of approximately 7.00-8.00 D from age 1-10 with a wide SD RF at age 10 years is up to -8.00 D or greater RF change below age 1 year is even more unpredictable

37. 2 factors should be considered for IOL implantation in children Age Target refraction at the time of surgery IOL power for adulthood but mostly undercorrected and requires hyperopic spectacles or CL of decreasing powers until the teenaged years Aim for emmetropia (widely selected in unilateral cases) to reduce the risk of amblyopia and encourage binocular function

38. Postoperative care Medical therapy Topical ATB Topical corticosteroids Topical cycloplegics Intracameral corticosteroids Intracameral ATB

39. Amblyopia management Amblyopia therapy start as soon as possible after surgery For aphakic patients, corrective lenses—in general, contact lenses for unilateral or bilatreal aphakia , spectacles for bilateral aphakia start within 1 week of surgery For infants with bilateral aphakia , spectacles are the safest and simplest For infants with unilateral aphakia , cnotact lenses are the most popular method After optical correction of aphakia patching better eye (unilateral cataract/asymmetrical VA in bilateral cataract)

40. complication Incidence of postoperative infections and bleeding is similar to adults Strabismus is very commonly associated with cataracts Risk of glaucoma is increased in children who have surgery in infancy Glaucoma often develops many years after lens extraction

41. Visual outcome after cataract extraction Depends on many factors Age of onset Type of cataract Timing of surgery Choice of optical correction Treatment of amblyopia

42. TAKE HOME MESSAGE Central or posterior lens opacities greater than 3 mm in diameter are usually visually significant Strabismus+unilateral cataract and bilateral cataracts+nystagmus =visually significant ***Visually significant unilateral cataract should be removed before age 6 weeks ***Visually significant bilateral cataracts, before age 10 weeks Although BCVA with 20/70 is satisfied for kids but it is never enough later in life surgery is performed when BCVA < 20/40

43. REFERENCE Gilbert C. Worldwide causes of blindness in children. In: Wilson ME, Saunders RA, Trivedi RH, eds.  Pediatric Ophthalmology: Current Thought and a Practical Guide . Heidelberg, Germany: Springer; 2009: 47-60. Haargaard B, Wohlfahrt J, Fledelius HC, Rosenberg T, Melbye M. Incidence and cumulative risk of childhood cataract in a cohort of 2.6 million Danish children.  Invest Ophthalmol Vis Sci . 2004;45(5):1316-1320. Xu LT, Traboulsi EI. Genetics of congenital cataracts. In: Wilson ME, Trivedi RH, editors.  Pediatric Cataract Surgery: Techniques, Complications and Management . Philadelphia: Lippincott Williams & Wilkins; 2014: 1-8. Gillespie RL, O'Sullivan J, Ashworth J, Bhaskar S, Williams S, Biswas S, et al. Personalized diagnosis and management of congenital cataract by next-generation sequencing.  Ophthalmology . 2014;121(11):2124-2137 e1-2. Serafino M, Trivedi RH, Levin AV, Wilson ME, Nucci P, Lambert SR, et al. Use of the Delphi process in paediatric cataract management.  Br J Ophthalmol . 2015. doi : 10.1136/bjophthalmol-2015-307287. [ Epub ahead of print].

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