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
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.
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.
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
Causes of loss of visionMacular exudationvitreous hemorrhageretinal detachmentmacular ischemia
Classification by Gomez Moralesisolated focal exudatesmassive elevated exudationpartial retinal detachmenttotal retinal detachmentsecondary complications such as uveitis, glaucoma, orcataract
treatment and prognosisIndicated in the following macular involvement of retinal detachment or exudation vitreous hemorrhagePhotocoagulation or surgery
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
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%)
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
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.
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.
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
clinical presentationchronic endophthalmitisposterior pole granulomaperipheral granulomatous inflammatory masslarvae is not usually seen clinically
treatmentcontrol of intra-ocular inflammatory responsesurgery is reserved for complications of vitreousopacification, retinal detachment or membrane formationanti-helminthic agents benefits are unproven
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 Hagler WS, Pollard ZF et al. RESULTS OF SURGERY FOR OCULAR TOXOCARA CANIS. OPHTHALMOLOGY 1981; 88: P. 1081-1086
bilateral disorder of the peripheral retinal vasculardevelopment.most commonly associated with retinal tractionno systemic associations4% of total pseudo-retinoblastoma.
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)
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
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
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 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
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
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