Leucocorias

By Dnyaneshwar Bhagwan potfode

1. PEDIATRIC CATARACT
AND LEUCOCORIA
Dnyaneshwar bhagwan potfode

2. objectives
•Objectives of this talk:
•Saying hello!!
•Interacting with all you wonderful people
•Introducing you all to pediatric cataract and its differential diagnosis

3. What do u think??
•What is “ leucocoria”
•When should we refer leucocoria for management?
•How is the visual prognosis after management of pediatric cataract?
•What is the mortality of retinoblastoma?

4. H V DESAI EYE HOSPITAL, PUNE

5. H V DESAI EYE HOSPITAL, PUNE

6. H V DESAI EYE HOSPITAL, PUNE

7. H V DESAI EYE HOSPITAL, PUNE

8. OUR AIM
H V DESAI EYE HOSPITAL, PUNE

9. Causes of Paediatric
cataract

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

11. Causes of cataract in healthy neonate
Hereditary
(usually dominant)
Idiopathic
With ocular anomalies
. PHPV
• Aniridia
• Coloboma
• Microphthalmos
• Buphthalmos

12. Aniridia with cataract

13. Cataract with Typical Iris Coloboma

14. Causes of cataract in unwell neonate
Intrauterine infections
• Rubella
• Toxoplasmosis
• Cytomegalovirus
• Varicella
Metabolic disorders
• Galactosaemia
• Hypoglycaemia
• Hypocalcaemia
• Lowe syndrome

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

16. Anterior polar cataract
May be dominant inheritance
Capsular Pyramid
With persistent pupillary
membrane
With Peters anomaly

17. Posterior polar cataract
Ocular associations
• Persistent hyaloid remnants
• Posterior lenticonus
• Persistent hyperplastic primary vitreous

18. Coronary (supranuclear) cataract
• Round opacities in deep cortex
• Surround nucleus like a crown
Usually sporadic

19. Cortical spoke-like cataract
Systemic associations
• Fabry disease
• Mannosidosis

20. Lamellar cataract
Systemic associations
• Galactosaemia
• Hypoglycaemia
• Hypocalcaemia
• Round central shell-like opacity
surrounding clear nucleus
• May have riders
Usually dominant inheritance

21. Central pulverulent cataract
• Spheroidal opacity within nucleus
• Relatively clear centre
• Non-progressive
Dominant inheritance

22. Sutural cataract
Opacity follows shape of Y suture
Usually X-linked inheritance

23. Focal dot opacities
• Blue dot cortical opacities
• Common and innocuous
• May co-exist with other opacities

24. Traumatic Cataract
Traumatic cataract with
full thickness cornea tear
Rosette cataract due
to blunt trauma

25. Complicated Cataract

26. Signs and Symptoms of Developmental Cataract
•Informant usually parents
•Usually a white spot in the pupillary area
•Child is usually brought with the history of diminuition of vision or
not able to recognize objects
•Unsteady eyes
•Deviation of eyes
•Associated symptoms of systemic disease , if present

27. Management of Paediatric Cataract
Laboratory Investigations
For Bilateral cases
•Full blood count , serum glucose
•Blood calcium and phosphorous
•Galactokinase levels
•TORCH test
•Urine Analysis
•For reducing substances in galactosaemia
•For amino acids to exclude Lowe’s Syndrome in suspected cases
For Unilateral Cases
•They are mostly idiopathic, elaborate laboratory workup is not needed

28. When to operate?
•Bilateral dense cataracts – require early surgery at the age of
4-6 weeks of age to prevent stimulation deprivation amblyopia.
•Bilateral partial cataracts - may not require surgery or may
require surgery at a later date
•Unilateral dense cataract – urgent surgery is advised.
Aggressive anti-amblyopia treatment should follow post surgery.
•Unilateral partial cataract – Need for surgery depends on the
•best corrected vision.

29. Aphakic correction in children
•Spectacles
•Contact Lenses
•Intraocular Lenses ( IOLs)

30. Selection of IOL
•Both the biometry and age of the child determine the power of the IOL
•Foldable acrylic IOLs are preferred
•Large myopic shift expected in younger children.
•Aim for undercorrection.

31. Prognosis
•Visual Outcomes depend on -Type of cataract
- Time of intervention
- Amblyopia management
•Follow up for aphakic and pseudophakic children should be
throughout childhood and preferably throughout life.
REPEAT REFRACTION , AMBLYOPIA THERAPY – THE SINGLE MOST THING OF
IMPORTANCE TO COMPLEMENT A GOOD PEDIATRIC CATARACT SURGERY FOR A
GOOD VISUAL PROGNOSIS IN CHILDREN

32. •It is very very important to try and diagnose any visually significant
lenticular opacities at the earliest
•and to refer them to the pediatric specialty at the earliest
• so that the child gets the perfect treatment at the earliest to prevent
AMBLYOPIA

33. LEUCOCORIA
CONGENITAL CATA

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37. Retinoblastoma
•Retinoblastoma is the most common and rapidly developing
intraocular tumor of childhood, accounting for 1% of childhood
cancer deaths and 5% of blindness in children.
•Develops in the cells of the retina in childhood(1-4) year.
Approximately 1in 20,000 birth
•The disease is bilateral in approximately 30% of cases.
•Overall mortality from retinoblastoma is now decreased.
•With modern diagnostic and therapeutic advances, the mortality
rate from metastatic or recurrent retinoblastoma has been as low
as 5%.

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

40. •retinoblastom
a

41. RETINOBLASTOMA

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43. Congenital retinal telangiectasis (Coats' disease)
•Idiopathic Retinal vascular disorder
•Usually affects young male patients unilaterally in their first or
second decade of life.
•Up to 1/3
rd
of patients are >30 years at time of presentation.
•No defined familial inheritance.
•Presentation- patients may present with decreased vision, as well as
strabismus or leukocoria in children.
•The hallmark feature of congenital retinal telangiectasis is localized
fusiform aneurysmal dilations of the retinal vessels reminiscent of
tiny light bulbs

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45. •Coats
disease

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47. Persistent hyperplastic primary vitreous (PHPV)
•Congenital anomaly in which the primary vitreous fails to
regress
in utero.
•Highly vascular mesenchymal tissue forms a mass behind the
lens.
•A grey-yellow retro-lental membrane may produce leukocoria.
•The globe is white and slightly micro-ophthalmic.
•PHPV are mostly unilateral and non-hereditary. When bilateral,
PHPV may follow autosomal recessive or autosomal dominant
inheritance pattern.

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55. COLOBOMA•Greek word koloboma meaningmutilated or curtailed.
•Occurs due to failure of closure of choroidal fissure
Coloboma of optic disc:
Coloboma of choroid and retina:
Coloboma of macula:
•Associations
CHARGE Syndrome,Trisomy 13 (Patau syndrome) Trisomy 18 (Edwards
syndrome),Cat-eye syndrome
•Posteriorly located coloboma can involve the optic nerve, retina, and choroid.
•If the retina is involved, it appears as an area of whitening often with pigment
deposition at the junction of the coloboma and normal retina.
•patients with coloboma have increased risk for retinal detachment.

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57. Retinal detachment in childhood
•Retinal detachment in childhood can be confused with retinoblastoma, and vice versa.
•The possibility of an underlying retinoblastoma should always be considered when a child
presents with retinal detachment and vitreous haemorrhage, even when a history of
trauma is obtained.

58. All children with newly discovered leukocoria should be referred promptly to
an ophthalmologist to exclude retinoblastoma and other life- or sight-
threatening conditions

59. Now - What do u think??
•What is “ leucocoria”
•When should we refer leucocoria for management?
•How is the visual prognosis after management of pediatric cataract?

60. 07/11/2018