VASCULAR AND HEREDITARY RETINAL DISEASE
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VASCULAR AND HEREDITARY RETINAL DISEASE

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VASCULAR AND HEREDITARY RETINAL DISEASE VASCULAR AND HEREDITARY RETINAL DISEASE Presentation Transcript

  • DISORDERS OF THE VITREOUS AND RETINAL DETACHMENT Dr Russell J Watkins
  • Vitreous
    • Virtually acellular
    • Viscous
    • Collagen framework, reinforces with hyaluronate
    • 98% water; volume = 4.5ml in emmetropic eye
    • Condensations of vitreous
      • Anterior hyaloid membrane
      • Posterior hyaloid membrane
      • Tracts in gel
      • Cloquet’s canal
  • Vitreous
    • Attachments of vitreous
      • Vitreous base
        • Strong, 3-4mm annular attachment, extending across ora serrata
      • Weigert’s ligament
        • 8-9mm annular attachment to posterior lens surface (anterior end of Cloquet’s)
      • Vitreopapillary adhesions
        • Posterior end of Cloquet’s canal  Weiss ring
      • Vascular adhesions (hence bleeds with PVD)
      • Areas of vitreoretinal degeneration
        • e.g. lattice degeneration, cystic retinal tufts
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  • Vitreous
    • Aging changes
      • Dissociation of hyaluronate from fibrils
      • Pooling of hyaluronate
      • Fibril degeneration &  elasticity
      • Drainage of hyaluronate into retrovitreal space (  posterior vitreous detachment [PVD])
  • Vitreous Opacities
    • Muscae volitantes: remnants of hyaloid system
    • Syneresis
    • Weiss ring
    • Haemorrhage
    • Tobacco dust: pigment cells
    • Inflammatory cells
      • Pars planitis
      • Chorioretinitis
      • AC spillover
  • Vitreous Opacities
    • Synchisis scintillans (settles inferiorly with rest)
    • Asteroid hyalosis
      • Seen in 1:200 eyes; more common in DM
      • Ca 2+ -soaps, adherent to fibrils
      • Does not settle at rest
    • Neoplastic cells
    • Amyloid
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  • Vitreous
    • Vitreous degeneration
      • Syneresis
        • Vitreous liquefaction, fibril aggregation & condensation
        • Associated with floaters
        • Caused by myopia, senescence, trauma, inflammations, hereditary causes
      • Posterior vitreous detachment
  • Posterior Vitreous Detachment
    • Collapse of vitreous gel
    • Associated with photopsia & floaters
    • Causes
      • Senile
      • Myopic
      • Post-inflammatory
      • Post vitreous haemorrhage
      • Diabetes mellitus
    • Read Chignell et al (2000) Optometry in Practice 2(1);97 et seq
  • Posterior Vitreous Detachment
    • PVD with gel collapse
      • Without vitreous haemorrhage, 4% develop retinal breaks
      • With vitreous haemorrhage, 20% develop breaks
    • PVD without gel collapse
      • Associated with future retinal hole or vitreous haemorrhage
      • Scaffold for proliferative new vessels
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  • Vitreous Haemorrhage
    • Proliferative retinopathies
      • DM
      • Retinal vein occlusion
      • Sickle cell retinopathy
      • ROP
      • Eale’s disease
    • PVD
    • Trauma
  • Vitreous Haemorrhage
    • Disciform macular degeneration
    • Blood dyscrasias
    • Subarachnoid haemorrhage (Terson’s syndrome)
    • Complications of vitreous haemorrhage
      • Syneresis; Fibrosis  traction RD; Haemosiderosis; Haemolytic (ghost cell) glaucoma; Synchisis scintillans; Ochre membrane
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  • Retinal Detachment
    • Retinal detachment can be:
      • Rhegmatogenous
      • Tractional
      • Exudative
  • Rhegmatogenous RD
    • RD occurring in association with hole formation; incidence 1:10,000 per year
    • Predisposing conditions
      • Acute PVD
      • Myopia (esp. High myopia)
      •  Age
      • Trauma
      • Aphakia (1% of ICCE; 0.1% of ECCE with intact PC)
      • Vitreoretinal degenerations
  • Rhegmatogenous RD
    • Pathogenesis
      • Following PVD, dynamic vitreoretinal traction occurs at abnormal adhesions
        • This can  vitreous haemorrhage
      • Traction  energy transmission to retina
      • To relieve traction, a break forms
      • RD caused by collection of subretinal fluid
  • Rhegmatogenous RD
    • Pathogenesis
      • Sensory retina detaches from RPE
        • Pigment cells may be avulsed into vitreous  tobacco dust
        • This predisposes to proliferative vitreoretinopathy (PVR)
      • Retina becomes opaque due to oedema
      •  Photoreceptor degeneration
  • Types of Break
    • Horseshoe or U-shaped tear
    • Atrophic hole
    • Operculated
    • Dialysis
      • Involves splitting of vitreous base
      • Usually inferotemporal
      • Can be spontaneous or traumatic
    • Macular hole
      • Idiopathic; myopia; Commotio retinae
  • Types of Break
    • Giant retinal tear
      • 90-360° tear; may fold back
      • Associated with PVR
      • Trauma; myopia; Stickler’s syndrome
    • Bucket handle tears
      • Usually superonasal
      • Avulsion of vitreous base
      • Always traumatic
  • Rhegmatogenous RD
    • Symptoms
      • Photopsia
      • Floaters
      • Shadow
      • 60% of patients with RD have ALL symptoms
  • Rhegmatogenous RD
    • Examination
      • VA
      • Visual fields
      • Peripheral retinal evaluation
        • BIO + 3-mirror
      • Tobacco dust
      • Mild anterior uveitis
      • Low IOP
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  • Rhegmatogenous RD
    • Natural history
      • Progression to total detachment
      • Spontaneous reattachment (can happen)
      • Retinal & RPE atrophy
      • “ High water mark” - RPE hyperplasia
      • Viscous subretinal fluid
      • Intraretinal cyst formation
      • PVR
      • Rubeosis iridis
      • Phthisis bulbi
  • Rhegmatogenous RD
    • Principles of treatment
      • Localisation & closure of breaks
      • Relief of vitreoretinal traction
        • Buckling or vitrectomy
      • Neuroretinal-rpe adhesion
        • Photocoagulation or cryotherapy
      • Internal tamponade
        • Air; longer acting gases [SF 6 , C 3 F 8 ]; Silicone oil
  • Complications of RD Surgery
    • Anterior or posterior segment ischaemia
    • Infection
    • Perforation or erosion of plomb into eye
    • Extrusion of plomb
    • Muscle imbalance
    • Refractive changes
    • Macular pucker
    • Cataract
    • Glaucoma
    • Redetachment
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  • Vitreoretinal Degenerations
    • Predisposing to RD
      • Lattice degeneration - present in 40% of RD
      • Snail track degeneration
      • “ White without pressure”
    • Benign
      • “ White with pressure”; Pigment clumping; Diffuse chorioretinal atrophy; Peripheral microcystoid changes; Snowflake degeneration; Pavingstone degeneration; Honeycomb degeneration; Drusen; Oral pigmentary degeneration
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  • Tractional RD
    • Penetrating ocular trauma
    • Proliferative retinopathies
      • DM
      • Sickle cell retinopathy
      • ROP
      • Retinal vein occlusion
      • Eale’s disease
    • Persistent hyperplastic primary vitreous
    • Toxocariasis
    • Pars planitis
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  • Exudative RD
    • Uveitis e.g. Vogt-Koyanagi-Harada syndrome
    • Choroidal tumours
      • Malignant melanoma
      • Metastatic
    • Low blood protein levels
    • Hypertension
    • Eclampsia
    • Hypothyroidism
    • Choroidal effusion syndrome
  •  
  • Retinoschisis
    • Splits or cysts within the neurosensory retina
    • Retinoschisis can be
      • Primary
        • Senile (level of OPL)
        • Juvenile/congenital (level of RNFL)
      • Secondary to other conditions
  • Senile Retinoschisis
    • Bilateral in 33%
    • Usually inferotemporal
    • Usually hypermetropic
    • Dome elevation of inner retinal layers
    • White dots (snowflakes) on inner limiting membrane
    • Inner leaf has beaten metal appearance
    • Sheathing of peripheral vessels
  • Senile Retinoschisis
    • Round holes can occur in inner leaf
    • Larger holes can occur in outer leaf
    • 1% progress to rhegmatogenous RD
    • Peripheral field defect though often asymptomatic
    • Need periodic observation with visual fields
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  • Juvenile Retinoschisis
    • X-linked recessive
    • Splits in NFL
    • Bilateral
    • Foveal (cystoid changes BUT not CMO) or peripheral
    • Associated with Favre-Goldmann & Wagner’s diseases
    • Poor visual prognosis
    • Predisposes to RD, esp inferotemporally
  • Juvenile Retinoschisis
    • Present with
      •  vision (may be vitreous haemorrhage)
      • Maybe asymptomatic
      • Even though congenital, may not be detected for years
  •  
  • Secondary Retinoschisis
    • Proliferative retinopathies
    • Trauma
    • Vitreous traction