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White Pupillary reflex in children

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Presentation on theme: "White Pupillary reflex in children"— Presentation transcript:

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)

4

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 Lamellar Central pulverulent Sutural Focal dots

10 Causes of cataract in healthy neonate
Hereditary (usually dominant) Idiopathic With ocular anomalies . PHPV Aniridia Coloboma Microphthalmos Buphthalmos Iatrogenic pediatric cataract 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 Systemic investigations
Serology: TORCHS titre and VDRL Urine analysis: for amino acids(lowe syndrom) 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 inflammation Orbital invasion

18 Signs/Growth pattern Endophytic Exophytic

19 Investigations Ultrasound C T Scan

20 Investigations MRI

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 External beam radiation Large tumours Enucleation

23 Treatment Extraocular extension Chemotherapy Metastatic Disease
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, Intraventicular haemorrhage, 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 ora serrata,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 Toxocara canis
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 endophytic Rb.

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:cochlear deafness,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


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