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Other Differentials of    Leukocoria  Narciso F. Atienza, Jr, MD, DPBO         Section Chief Retina/Vitreous        Cardin...
pseudoretinoblastoma212 / 500 cases - retinoblastoma4 most common  Persistent hyperplastic primary vitreous - 28%  Coats’ ...
Persistent fetal vasculature
Congenital anomaly of the eye resulting from failure ofthe embryonic, primary vitreous, and hyaloid vasculatureto regressu...
Subclassesanterior PHPV  retro-lental fibroascular membrane, elongated ciliary  processes, cataract, and microphthalmiapos...
nnknmbnnnnnnnnnnnnnnnn
clinical coursemajority develops glaucoma, retinal detachment, intraocularhemorrhage, and phthisis
contra-indications for       surgeryNLP visionunrecordable visual evoked potentialintense afferent pupillary defect
results in surgical          treatmentsurgical results are better in anterior type v.s. that of theposterior type.surgery ...
6/24 eyes maintained Snellens vision50% will ultimately achieve useful vision                   Dass AB, Trese, MT. Surgic...
Coats Disease
Coats’ diseasenamed afterMr. George Coats  “presence of extensive mass of exudation”exudative retinitis / retinal telangie...
coats diseasesexual predilection: 3M : 1F  previously - no genetic predisposition  presently - somatic mutation of the NDP...
Causes of loss of        visionMacular exudationvitreous hemorrhageretinal detachmentmacular ischemia
Classification by Gomez           Moralesisolated focal exudatesmassive elevated exudationpartial retinal detachmenttotal ...
treatment and            prognosisIndicated in the following  macular involvement of retinal detachment or  exudation  vit...
treatment / prognosisSpontaneous regression has been reported, but raretreatment prior to formation to exudation is prefer...
shields and co-workers124 eyesmedian follow-up times = 6 mos to 24 years  observation - 22 eyes (18%)  photocoagulation - ...
60 eyes - second treatment27 eyes - third treatmentexudation resolved in 46 eyes (45%) - median of 12months88 eyes - under...
Results
risk factors for poor outcomes  post-equatorial in location  diffuse  superior  failed resolution of SRF on treatment  pre...
anti-VEGF may have a role in coats disease management  Stergiou PK, Symeonidis C, Dimitrakos SA. Coats disease: treatment ...
OcularToxocariasisToxocariasis
Associated with the common ascarid of dogs  Toxocara canis  not associated with visceral larvae migranshistory of pica or ...
infection is usually acquired thru ingestion of infectedsoil and not thru direct contact with dogs.hematogenous spread fro...
Life cycle
unilateral visual loss, strabismus, leukocoriano discomfort
evaluationperipheral eosinophilia is rare in patients with oculartoxocariasisfecalysis is usually negativeELISA testing fo...
clinical presentationchronic endophthalmitisposterior pole granulomaperipheral granulomatous inflammatory masslarvae is no...
Intra-oculartoxocariasis withsurrounding eosinophilicreaction
treatmentcontrol of intra-ocular inflammatory responsesurgery is reserved for complications of vitreousopacification, reti...
prognosisdepends on the macular involvement and the potentialfor amblyopia.
17 cases reported  12 cases (71%)- re-attached after surgery  15 cases (88%) - vision was stable after surgery            ...
Familial ExudativeVitreo-retinopathy
bilateral disorder of the peripheral retinal vasculardevelopment.most commonly associated with retinal tractionno systemic...
resembles that of retinopathy of prematurity11th chromosome. most of the time is a mutation of NorrieDisease.absent signif...
clinical stages - OLD     classificationstage 1 - mildest form of FEVR  patients are asymptomatic  non proliferative perip...
stage 2  proliferative and exudative changes  limited traction retinal detachment  border between perfused and non-perfuse...
stage 3  seen in most severely affected eyes  retinal detachment and dragging (20%-30%)  tangential traction on the macula
Stages - patterned after the          ICROPStage                     Clinical features           Avascular retinal periphe...
course and treatmentmanagement is similar to ROPperipheral retinal ablation may help in controlling thedisease  ultimate g...
half of patients with FEVR are asymptomaticpatients may present with poor fixation, true orpseudostrabismus, or nystagmus....
summaryrecognitionearly detectionprompt referral
thank you for your       time
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Common causes of Leucocoria in Children

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  1. 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. 2. pseudoretinoblastoma212 / 500 cases - retinoblastoma4 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. 3. Persistent fetal vasculature
  4. 4. Congenital anomaly of the eye resulting from failure ofthe embryonic, primary vitreous, and hyaloid vasculatureto regressunilateral, seen in full term infants with no systemicfindings
  5. 5. Subclassesanterior PHPV retro-lental fibroascular membrane, elongated ciliary processes, cataract, and microphthalmiaposteriorPHPV vitreous membrane and stalk, retinal fold, traction RD, hypoplastic optic nerve and macula, microphthalmiacombination of the 2
  6. 6. nnknmbnnnnnnnnnnnnnnnn
  7. 7. clinical coursemajority develops glaucoma, retinal detachment, intraocularhemorrhage, and phthisis
  8. 8. contra-indications for surgeryNLP visionunrecordable visual evoked potentialintense afferent pupillary defect
  9. 9. results in surgical treatmentsurgical results are better in anterior type v.s. that of theposterior type.surgery for posterior type PHPV can result in functionalvisual 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
  10. 10. 6/24 eyes maintained Snellens vision50% will ultimately achieve useful vision Dass AB, Trese, MT. Surgical results of persistent hyperplastic primary vitreous. Ophthalmology 1999; 106:280 - 284.
  11. 11. Coats Disease
  12. 12. Coats’ diseasenamed afterMr. George Coats “presence of extensive mass of exudation”exudative retinitis / retinal telangiectasia.characterized as an idiopathic, developmental retinalvascular abnormality usually affecting one eye.
  13. 13. coats diseasesexual 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 diseaserelated to idiopathic parafoveal telangiectasiasaid to be the same spectrum as leber’s miliary aneurysm
  14. 14. Causes of loss of visionMacular exudationvitreous hemorrhageretinal detachmentmacular ischemia
  15. 15. Classification by Gomez Moralesisolated focal exudatesmassive elevated exudationpartial retinal detachmenttotal retinal detachmentsecondary complications such as uveitis, glaucoma, orcataract
  16. 16. treatment and prognosisIndicated in the following macular involvement of retinal detachment or exudation vitreous hemorrhagePhotocoagulation or surgery
  17. 17. treatment / prognosisSpontaneous regression has been reported, but raretreatment prior to formation to exudation is preferabletreatment is also usually applied also to the vascular,rather than the exudative areas
  18. 18. shields and co-workers124 eyesmedian 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%)
  19. 19. 60 eyes - second treatment27 eyes - third treatmentexudation resolved in 46 eyes (45%) - median of 12months88 eyes - underwent surgical (primary, secondary ortertiary procedure). 50 eyes (57%) - resolution of retinaldetachment
  20. 20. Results
  21. 21. 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.
  22. 22. 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.
  23. 23. OcularToxocariasisToxocariasis
  24. 24. Associated with the common ascarid of dogs Toxocara canis not associated with visceral larvae migranshistory of pica or exposure to puppies
  25. 25. infection is usually acquired thru ingestion of infectedsoil and not thru direct contact with dogs.hematogenous spread from the small intestines
  26. 26. Life cycle
  27. 27. unilateral visual loss, strabismus, leukocoriano discomfort
  28. 28. evaluationperipheral eosinophilia is rare in patients with oculartoxocariasisfecalysis is usually negativeELISA testing for T.canis at 1:8 dilution os 90% specific and91% sensitive and is highly suggestive (not diagnostic)anterior aqueous cytology may have a role in the diagnosis
  29. 29. clinical presentationchronic endophthalmitisposterior pole granulomaperipheral granulomatous inflammatory masslarvae is not usually seen clinically
  30. 30. Intra-oculartoxocariasis withsurrounding eosinophilicreaction
  31. 31. treatmentcontrol of intra-ocular inflammatory responsesurgery is reserved for complications of vitreousopacification, retinal detachment or membrane formationanti-helminthic agents benefits are unproven
  32. 32. prognosisdepends on the macular involvement and the potentialfor amblyopia.
  33. 33. 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
  34. 34. Familial ExudativeVitreo-retinopathy
  35. 35. bilateral disorder of the peripheral retinal vasculardevelopment.most commonly associated with retinal tractionno systemic associations4% of total pseudo-retinoblastoma.
  36. 36. resembles that of retinopathy of prematurity11th chromosome. most of the time is a mutation of NorrieDisease.absent significant neonatal and perinatal historyinheritance pattern has been noted autosomal dominant autosomal recessive x-linked (seen only in males)
  37. 37. clinical stages - OLD classificationstage 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
  38. 38. 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
  39. 39. stage 3 seen in most severely affected eyes retinal detachment and dragging (20%-30%) tangential traction on the macula
  40. 40. Stages - patterned after the ICROPStage 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
  41. 41. course and treatmentmanagement is similar to ROPperipheral retinal ablation may help in controlling thedisease ultimate goal is to present retinal detachment or secondary macular exudationhas a more chronic course that ROP
  42. 42. half of patients with FEVR are asymptomaticpatients may present with poor fixation, true orpseudostrabismus, or nystagmus.visual outcome is strongly influenced by age of onsent ofsigns or symptoms deterioration is greatest in the younger population
  43. 43. summaryrecognitionearly detectionprompt referral
  44. 44. thank you for your time

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