White Pupillary reflex in children

1. White Pupillary reflex in children Dr. Mariya Nazish Memon MBBS,FCPS,Fellow Pead Ophth & strabismus(ASEH) Senior Registrar , Head of Unit Pediatric Ophthalmology and Strabismus Liaquat university Eye hospital, Hyderabad

2. OBJECTIVES • Enlist common causes of white pupil in children • Identify the child with serious visual and life threatening problem • Understand the immediate need of referral to Ophthalmologist

3. Leucokoria • White pupillary reflex • “amaurotic cat’s eye” • Greek word “leucos” (white) and “korê” (pupil)

5. Causes of White Pupil in children • Cataract • Retinoblastoma • Retinopathy of prematurity • Persistent fetal vasculature • Coats disease • Toxocariasis • Coloboma (fissure or cleft) of choroid or optic disc • Retinal dysplasias • Uveitis • Vitreous hemorrhage

6. Importance Infancy and early childhood is an important time for visual development. • The eyes grow and emmetropise • Vision improves • Stereopsis matures • Accommodation develops

7. Congenital cataract Opacification of the crystalline lens present at the time of birth or develop after birth during maturity period of the lens

8. Important facts • 33% - idiopathic - may be unilateral or bilateral • 33% - inherited - usually bilateral • 33% - associated with systemic disease - usually bilateral • Other ocular anomalies present in 50%

9. Classification of congenital cataract Anterior polar Posterior polar Coronary Cortical spoke-like Sutural Focal dots Lamellar Central pulverulent

10. Causes of cataract in healthy neonate • Hereditary • (usually dominant) • Idiopathic • With ocular anomalies • . PHPV • Aniridia • Coloboma • Microphthalmos • Buphthalmos • Iatrogenicpediatriccataract • Laser photoablation for ROP or tumor • External beam radiation • steroid therapy • Damage to posterior capsule due to • posterior vitrectomy

11. Causes of cataract in unwell neonate • Intrauterine infections • Rubella • Toxoplasmosis • Cytomegalovirus • Herpes simplex • Varicella • Metabolic disorders • Galactosaemia • Hypoglycaemia • Hypocalcaemia • Lowe syndrome • Chromosomal abnormalities • Down syndrome (trisomy 21) • Patau syndrome (trisomy 13) • Edward syndrome (trisomy 18)

12. Management OCULAR EXAMINATION • Visual behavior • Density of cataract • Morphology • Associated ocular pathology • Pupillary reflex • Ocular Ultrasound(B Scan)

13. Systemicinvestigations Serology: • TORCHS titre and VDRL Urine analysis: • for amino acids(lowesyndrom) and reducing substance after drinking milk(galactosaemia) Blood test: • Fasting blood sugar,serum calcium and phosphours, red-cell GPUT and galactokinase level

14. Indications for Surgery • Bilateral dense Cataracts • Unilateral dense Cataracts • Partial unilateral /bilateral cataract

15. Management • Surgery: • Lens matter aspiration, posterior capsulotomy, anterior vitrectomy +/_ IOL implantation • Visual rehabilitation: • Spectacles • Contact lenses • IOL implantation • Ambyopia therapy

16. Retinoblastoma • Most common intraocular tumour of childhood • May be heritable(40%) or non-heritable(60%) • Located chromosome- 13q14  • malignant transformation of primitive retinal cells before final differentiation. • As these cells disappear in the first few years of life, the tumour is seldom seen after 3 years of age

17. Presentations of Retinoblastoma • Leukocoria - 60% • Strabismus - 20% • Secondary glaucoma • Anterior segment invasion • Orbital invasion • Orbital inflammation

18. Signs/Growth pattern Endophytic Exophytic

19. Investigations Ultrasound C T Scan

20. InvestigationsMRI

21. Poor Prognostic Factors • Optic nerve involvement • Choroidal invasion • Large tumour • Anterior location • Poor cellular differentiation • Older children

22. MANAGEMENT Depends on size, location and staging of tumour Treatment of small (3 mm diameter) tumours • Photocoagulation • Cryotherapy • Chemotherapy Medium sized (upto 12 mm) tumours • Chemotherapy • External beam radiation Large tumours • Chemotherapy • Enucleation

23. Treatment Extraocular extension Chemotherapy Radiotherapy Metastatic Disease High dose chemotherapy Intra-thecal chemotherapy Total body radiotherapy

24. Follow-up • Heritable Retinoblastoma patients can develop recurrences and need to be followed up regularly • Examine the patients every 6-8wks till 3yrs,every 6 months till the age of 5 yrs and then annually till the age of 10 years.

25. Retinopathy of Prematurity Proliferative retinopathy affects low birth weight premature infant.

26. RISK FACTOR Major Risk Factor: • Prematurity < 32 weeks gestation (< 30 weeks) • Low birth weight < 1500 gm (<1250 gm) • Supplemental Oxygen. Minor Risk Factor: • Maternal: Complications of pregnancy, use of beta blockers. • Fetal: Hypercarbia, Sepsis, Vitamin E deficiency, Intraventicularhaemorrhage, Recurrent apnea, RDS, Indomethacin treatment for PDA.

27. RETINAL VASCULARIZATION

28. STAGING

29. STAGING Stage:5. Funnel shaped Total retinal detachment

30. SYMPTOMS Symptoms of severe ROP include: • Nystagmus (Abnormal eye movements) • Amblyopia (Lazy eye) • Strabismus (Crossed eyes) • Myopia (Severe near sightedness) • Leucocoria (White-looking pupils ) • Glaucoma • Cataract • Retinal detachment

31. Screening for ROP • All pre mature born at or before 32 weeks of gestation • All premature with birth weight of 1500 gms or less • Screening should start 4 weeks after birth

32. Management • In 80% of infant ROP will regress spontaneously • Treatment is indicated in stage 3 disease • Argon laser in the periphery • Cryotherapy (trans-scleral) • Anti VEGF intravitreal injection • RD surgery for stage IV and V

33. Persistent fetal vasculature(PFV/PHPV) • Unilateral • Failure of regression of primary vitreous/hyaloid system • Typically present with leukocoria,squint or Nystagmus • Persistent anterior fetal vasculature • Persistent posterior fetal vasculature • Visual prognosis depends on amount of microphthalmia and involvement of posterior pole

34. Persistent anterior fetal vasculature • Retrolental mass with elongated ciliary processes • Advanced cases ass:with Cataract formation

35. Persistent posterior fetal vasculature • Confined to posterior segment • Dense white membrane or prominent retinal fold extends from optic disc to oraserrata,ass:with retinal detachment.

36. COATS DISEASE • Idiopathic retinal vascular talengiectasia with intraretinal and sub retinal exudation and retinal detachment • Unilateral • Seventy-five percent are male • Presents in 1st decade(avg:5yrs) with unilateral visual loss, strabismus and leucokoria

37. Ocular Toxocariasis • infestation of dog with Toxocaracanis • Human infestation:accidental ingestion of soil or food contaminated with ova shed in dog faeces • Very young children who eat dirt or are in close contact with puppies are at risk • In human intestine ,ova develop into larva ,penetrate intestinal wall and travel to various organs.liver,lungs,skin,brain and eyes. • Larva die,disintegrate and cause an inflamatory reaction and granuloma formation.

38. Ocular Toxocariasis • Presents as strabismus, leukocoria or unilateral visual loss • Ch:Endophthalmitis: (2-9yrs)mey cause cyclitic membrane and white pupil. Posterior pole granuloma in an otherwise quiet eye.(6-14yrs) may resemble endophyticRb.

39. Coloboma of Choroid/Optic disc • Incomplete closure of the embryonic fissure • Unilateral /bilateral • Sharply circumscribed, white area devoid of blood vessels in the inferior fundus • Large Coloboma may involve the disc and give rise to leucokoria • Complication: Retinal detachment

40. Retinal Dysplasia • Faulty differentiation of retina and vitreous • Isolated or ass:with systemic conditions such as Norrie disease and incontinentia pigmenti • Presents with congenital blindness with roving eye movement • Pink or white retrolental masses resulting in leucokoria • microphthalmos,shallow anterior chamber and elongated ciliary processes

41. Retinal Dysplasia • Norrie disease: • XL reccessive • Males are blind at birth or infancy • Sys:cochleardeafness,mental retardation • Incontinentia pigmenti • XL Dominent • Affecting girls and lethal in utero for boys • One third children develop retinal detachment in 1st yr of life • Vesiculobullous rash on trunk and extremities • Malformation of teeth,hair,nails,bones and CNS

42. Conclusion • Family physician play crucial role in the management of eye problem in children • Vision screening even with limited equipments can identify most important causes of visual loss

43. THANK YOU