Common causes of Leucocoria in Children

1. Other Differentials of
Leukocoria
Narciso F. Atienza, Jr, MD, DPBO
Section Chief Retina/Vitreous
Cardinal Santos Medical Center
Legaspi Eye center
St. Lukes Medical Center

2. pseudoretinoblastoma
212 / 500 cases - retinoblastoma
4 most common
Persistent hyperplastic primary vitreous - 28%
Coats’ disease - 16%
presumed ocular toxocariasis - 16%
retinopathy of prematurity - 4.7%
Shields, JA, Shields CL: DIfferential diagnosis of retinoblastoma
Intraocular tumors. A text and atlas. Philadelphia, wb saunders co, 1992

3. Persistent fetal
vasculature

4. Congenital anomaly of the eye resulting from failure of
the embryonic, primary vitreous, and hyaloid vasculature
to regress
unilateral, seen in full term infants with no systemic
findings

5. Subclasses
anterior PHPV
retro-lental fibroascular membrane, elongated ciliary
processes, cataract, and microphthalmia
posteriorPHPV
vitreous membrane and stalk, retinal fold, traction
RD, hypoplastic optic nerve and macula,
microphthalmia
combination of the 2

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9. clinical course
majority develops glaucoma, retinal detachment, intraocular
hemorrhage, and phthisis

10. contra-indications for
surgery
NLP vision
unrecordable visual evoked potential
intense afferent pupillary defect

11. results in surgical
treatment
surgical results are better in anterior type v.s. that of the
posterior type.
surgery for posterior type PHPV can result in functional
visual outcome
71% of patients may achieve 20/300 vision or better
Mittra, RA, Huynh LT, et. al. VISUAL OUTCOMES FOLLOWING LENSECTOMY
AND VITRECTOMY FOR COMBINED ANTERIOR AND POSTERIOR PERSISTENT
HYPERPLASTIC PRIMARY VITREOUS. ARCH OPHTHA 1998; 116: 1190-1194

12. 6/24 eyes maintained Snellens vision
50% will ultimately achieve useful vision
Dass AB, Trese, MT. Surgical results of persistent hyperplastic
primary vitreous. Ophthalmology 1999; 106:280 - 284.

13. Coats Disease

14. Coats’ disease
named afterMr. George Coats
“presence of extensive mass of exudation”
exudative retinitis / retinal telangiectasia.
characterized as an idiopathic, developmental retinal
vascular abnormality usually affecting one eye.

15. coats disease
sexual predilection: 3M : 1F
previously - no genetic predisposition
presently - somatic mutation of the NDP gene
CRB1 gene (55%)
“Norrin” hence there may be a correlation with Norrie
disease
related to idiopathic parafoveal telangiectasia
said to be the same spectrum as leber’s miliary aneurysm

16. Causes of loss of
vision
Macular exudation
vitreous hemorrhage
retinal detachment
macular ischemia

17. Classification by Gomez
Morales
isolated focal exudates
massive elevated exudation
partial retinal detachment
total retinal detachment
secondary complications such as uveitis, glaucoma, or
cataract

22. treatment and
prognosis
Indicated in the following
macular involvement of retinal detachment or
exudation
vitreous hemorrhage
Photocoagulation or surgery

23. treatment / prognosis
Spontaneous regression has been reported, but rare
treatment prior to formation to exudation is preferable
treatment is also usually applied also to the vascular,
rather than the exudative areas

24. shields and co-workers
124 eyes
median follow-up times = 6 mos to 24 years
observation - 22 eyes (18%)
photocoagulation - 16 eyes (13%)
cryotheraphy - 52 eyes (42%)
RD repair - 20 eyes (17%)
enucleation - 14 eyes (11%)

25. 60 eyes - second treatment
27 eyes - third treatment
exudation resolved in 46 eyes (45%) - median of 12
months
88 eyes - underwent surgical (primary, secondary or
tertiary procedure). 50 eyes (57%) - resolution of retinal
detachment

26. Results

27. risk factors for poor outcomes
post-equatorial in location
diffuse
superior
failed resolution of SRF on treatment
presence of retinal macrocysts
Shields JA, Shields CL, Honavar SG. CLassification and management
Shields JA, Shields CL, Honavar SG. CLassification and management
of coats disease: the 2000 Proctor LEcture.
Am J Ophthalmology 2001; 131:572-583.

29. anti-VEGF may have a role in coats disease management
Stergiou PK, Symeonidis C, Dimitrakos SA. Coats' disease: treatment with intravitreal bevacizumab and laser photocoagulatio
Acta Ophthalmol. 2008 Oct 30.

30. Ocular
Toxocariasis
Toxocariasis

31. Associated with the common ascarid of dogs
Toxocara canis
not associated with visceral larvae migrans
history of pica or exposure to puppies

32. infection is usually acquired thru ingestion of infected
soil and not thru direct contact with dogs.
hematogenous spread from the small intestines

33. Life cycle

34. unilateral visual loss, strabismus, leukocoria
no discomfort

35. evaluation
peripheral eosinophilia is rare in patients with ocular
toxocariasis
fecalysis is usually negative
ELISA testing for T.canis at 1:8 dilution os 90% specific and
91% sensitive and is highly suggestive (not diagnostic)
anterior aqueous cytology may have a role in the diagnosis

36. clinical presentation
chronic endophthalmitis
posterior pole granuloma
peripheral granulomatous inflammatory mass
larvae is not usually seen clinically

37. Intra-ocular
toxocariasis with
surrounding eosinophilic
reaction

40. treatment
control of intra-ocular inflammatory response
surgery is reserved for complications of vitreous
opacification, retinal detachment or membrane formation
anti-helminthic agents benefits are unproven

41. prognosis
depends on the macular involvement and the potential
for amblyopia.

42. 17 cases reported
12 cases (71%)- re-attached after surgery
15 cases (88%) - vision was stable after surgery
Hagler WS, Pollard ZF et al. RESULTS OF SURGERY FOR OCULAR
TOXOCARA CANIS. OPHTHALMOLOGY 1981; 88: P. 1081-1086

43. Familial Exudative
Vitreo-retinopathy

44. bilateral disorder of the peripheral retinal vascular
development.
most commonly associated with retinal traction
no systemic associations
4% of total pseudo-retinoblastoma.

45. resembles that of retinopathy of prematurity
11th chromosome. most of the time is a mutation of Norrie
Disease.
absent significant neonatal and perinatal history
inheritance pattern has been noted
autosomal dominant
autosomal recessive
x-linked (seen only in males)

46. clinical stages - OLD
classification
stage 1 - mildest form of FEVR
patients are asymptomatic
non proliferative peripheral retinal vascular
abnormalities
differs from ROP is that FEVR never vascularize
over time, unlike ROP

47. stage 2
proliferative and exudative changes
limited traction retinal detachment
border between perfused and non-perfused retina is a
common feature
vascular changes affects visual acuity by retinal
dragging or detachment, exudation or hemorrhage

48. stage 3
seen in most severely affected eyes
retinal detachment and dragging (20%-30%)
tangential traction on the macula

50. Stages - patterned after the
ICROP
Stage Clinical features
Avascular retinal periphery without extraretinal
1
vascularization
Avascular retinal periphery with extraretinal
2
vascularization (with or without exudation)
Retinal detachment - subtotal, no foveal involvement
3
(Primary exudative or tractional)
Retinal detachment - subtotal, foveal involvement
4
(Primary exudative or tractional)
Retinal detachment - total
5
Open funnel / Closed funnel

51. course and treatment
management is similar to ROP
peripheral retinal ablation may help in controlling the
disease
ultimate goal is to present retinal detachment or
secondary macular exudation
has a more chronic course that ROP

52. half of patients with FEVR are asymptomatic
patients may present with poor fixation, true or
pseudostrabismus, or nystagmus.
visual outcome is strongly influenced by age of onsent of
signs or symptoms
deterioration is greatest in the younger population

53. summary
recognition
early detection
prompt referral

54. thank you for your
time