Surgical Retina

Retinal Detachment

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Retinal breaks/degenerations

A full thickness defect in the neurosensory layer of the retina

  • Holes

    • Atrophic: not associated with vitreoretinal traction, therefore no increased risk of retinal detachment
    • Operculated: less risk compared to HST, but may be treated with laser if the patient is symptomatic, persistent traction, superior or large holes.
  • Tears

    • Horseshoe/flap: high incidence of progression to retinal detachment. Almost universal vitreous traction along the edge of the flap
    • Giant retinal tear: greater than 3 clock hours circumferentially, developing from vitreous base or lattice degeneration
  • Dialysis:

    • Break originating from the ora. Vitreous base is attached. 10% of RRDs.
  • Lattice degeneration: area of retinal thinning due to discontinuity of ILM with crossing white lines and small round holes. Associated with Stickler’s, Marfan’s, Ehlers-Danlos. Not treated prophylactically unless risk factors (see below)

  • White-without-pressure: may be treated if fellow eye has had a GRT

  • Retinoschisis: may be treated if double layer break

  • Benign degenerations:

    • Retinal hyperplasia
    • Microcystoid change
    • Honeycomb/reticular degeneration
    • Snowflake degeneration
    • Pavingstone degeneration
    • Peripheral drusen
    • Pars plana cyst

Indications to treat a retinal break

  • Symptomatic
  • Subclinical RD
  • Horseshoe tear (ie. U-shaped)
  • Large tear
  • Location: superotemporal/posterior
  • Only eye or fellow eye with previous RD
  • High myope
  • Vitreoretinopathy or systemic risk factor such as Marfan’s/Stickler’s

Hot Topic

Lincoff’s rules: four guidelines for locating retinal breaks causing RRD

Only apply to primary rhegmatogenous retinal detachments

  • Superior or temporal detachments: hole lies within 1.5 clock hours of the highest border (98% of the time)
  • Total or superior detachments that cross the 12 o’clock meridian: the primary hole is at the 12 o’clock position or within a triangle extending between the ora (apex) and 1 clock hour either side of the 12 o’clock position (93% of the time)
  • Inferior detachments: the higher side indicates the side of the break (95% of the time)
  • Bullous inferior detachments: the primary break lies above the horizontal meridian.

Other rules/guidelines

  • Inferior detachments with equal height on both sides: break is likely at 6 o’clock

Retinoschisis

  • Splitting of the retina
  • Degenerative retinoschisis:
    • More common in hypermetropes

    • Typically bilateral (50-80%)

    • Senile form (most common): split in the outer plexiform layer/inner nuclear junction

    • Reticular (less common) form: split in the nerve fibre layer, similar to X-linked juvenile retinoschisis

      • More likely to develop retinal detachment
    • Both inner and outer leaf breaks are needed for secondary RD to occur

Clinical features

  • Asymptomatic unless very posterior extension
  • Absolute field defect (compared to relative for RRD)
  • Usually found inferotemporally (in 72%)
  • Laser reaction (cp. Retinal detachment)
  • Smooth surface with absent pigments

Complications

  • Inner leaf breaks
  • Outer leaf breaks
  • Retinal detachment:
    • Low risk type due to outer leaf break only
    • High risk rhegmatogenous type: both inner and outer leaf break with full thickness retinal elevation

Retinal detachment types

Rhegmatogenous

  • Associated with retinal breaks
  • Corrugated surface
  • Pigment and/or blood in vitreous
  • Static fluid

Exudative

  • No breaks
  • Smooth surface
  • Inflammatory cells may be present in vitreous
  • Shifting fluid

Tractional

  • Preretinal fibrosis (no breaks)
  • Smooth surface
  • Clear vitreous
  • Minimal or no fluid

Proliferative vitreoretinopathy (PVR)

  • RPE cells migrate into vitreous through a retinal break

  • Convert to myofibroblasts and release transforming growth factor

  • Fibrosis and contracture occur leading to tractional RD

  • Risk factors

    • Multiple or large breaks
    • Vitreous haemorrhage
    • Recurrent detachment
    • Excessive cryotherapy or laser treatment
    • Silicone oil
    • Iris trauma

Retina society classification (1991)

  • A: vitreous haze and pigment clumps
  • B: retinal wrinkling, rolled edges, stiffness and vessel tortuosity
  • C (multiple sub-classes): subretinal strands

Management

  • Surgery +/- pharmacological adjuncts (5-FU).

  • Early surgery if macula preserved to protect vision

  • Silicone Study (1992)

    • Compared silicone oil vs gas for PVR associated with RRD
    • Oil was more effective than SF6 gas, but equal to C3F8.

Retinal detachment surgery

Overarching principles

  • Identify all breaks: can begin preoperatively, informed by Lincoff’s rules
  • Seal breaks: cryotherapy or laser
  • Drain subretinal fluid: not always necessary as long as break and traction addressed
  • Relieve vitreoretinal traction: buckle, vitrectomy, or pneumatic retinopexy

Cryotherapy

  • Freezing treatment (probe is between -40 and -70) turns water to ice causing cell death

  • Sterile inflammatory reaction

  • Retinal adherence fully develops over 2 weeks (note: retinopexy is faster)

  • Indications

    • To seal retinal breaks, especially where laser difficult due to

      • Small pupil
      • Far-peripheral retina
      • Media opacity
    • To treat pathological blood vessels eg. Coat’s or proliferative DM

      • Similar indications to above
    • Cyclodestruction (ciliary body) in glaucoma

    • Certain lid or intraocular tumours

  • Complications

    • Pain
    • Conjunctival fibrosis: risk of bleb failure in trab patients
    • Inflammation: vitritis, CMO
    • PVR
    • Muscle injury
    • Scleral damage/necrosis
    • Epiretinal membrane

Scleral buckles

  • Types

    • Placement

      • Radial
      • Segmental
      • Encircling
    • Material

      • Silicone
      • Hydrogels: more expensive and not used due to risk of complications
      • Gelatin: temporary
  • Indications

    • Radial buckles: large U-shaped tear or posterior breaks
    • Segmental buckles: multiple breaks
    • Encircling band: breaks over >two quadrants, complex RD, mild PVR, previous failed segmental buckle
  • Complications

    • Intraoperative: perforation, muscle damage, fish-mouthing, remaining break
    • Early: anterior segment ischaemia, angle closure, re-detachment, endophthalmitis, vitritis (from cryotherapy)
    • Late: extrusion/exposure or buckle infection, epiretinal membrane, PVR, diplopia, glaucoma

Vitrectomy

  • Indications

    • RRD (uncomplicated and certain complicated cases)

      • RD with vitreous haemorrhage
      • Severe PVR
      • Giant retinal tear
    • Tractional RD

    • Advanced diabetic retinopathy

    • Trauma with intraocular foreign body

    • Epiretinal membrane

    • Macular hole

    • Post-operative endophthalmitis

    • Dropped nucleus

    • Aqueous misdirection

    • Diagnostic: chronic inflammation

  • Complications

    • Intraoperative

      • Iatrogenic break
      • Vitreous haemorrhage
      • Suprachoroidal haemorrhage
      • Lens trauma
    • Postoperative

      • Vitreous haemorrhage
      • Choroidal haemorrahge
      • PVR
      • Recurrent detachment
      • Glaucoma
      • Cataract: almost inevitable
      • Band keratopathy
      • Endophthalmitis
  • Intraocular gases

    • Uses:

      • Vitreous replacement after vitrectomy since they are optically clear, inert and highly buoyant (provide greater force than silicone oil).
      • Retinal tamponade eg. pneumoretinopexy
    • Non-expansile: air, nitrogen, helium

    • Expansile:

      • SF6: persists for 2 weeks. Expansion volume is x2 (nonexpansile concentration is 20%)
      • C2F6: persists for 3 weeks. Expansion volume is x3 (nonexpansile concentration is 18%)
      • C3F8: persists for 6-8 weeks. Expansion volume is x4 (nonexpansile concentration is 15%)
    • Silicone oil

      • Has similar properties to gases however may emulsify due to lower surface tension. Heavy oil can be used for inferior detachments (heavier than water)

      • Uses

        • Longer lasting volume replacement eg. PVR, GRT, control of vitreous haemorrhage (longer lasting tamponade), patients who cannot posture
      • Provides better immediate post-operative vision

      • Complications

        • Glaucoma
          • Angle closure from pupil block (iridectomy usually needed inferiorly)
          • Open angle from emulsified oil
        • Cataract
        • Band keratopathy
        • Inflammatory reaction to oil: uveitis, retinal toxicity
        • Epiretinal membrane
        • Recurrent detachment

Pneumoretinopexy

  • Tamponade of break with intravitreal expansile gas
  • Indicated for superior breaks only
  • Avoids need for admission or complications of other procedures
  • However may fail and carry risks of CRAO, gas migration, and new breaks

X-linked juvenile retinoschisis

  • XLRS1 gene
  • Abnormal intercellular adhesion molecule leading to retinal splitting at the NFL
  • Poor visual prognosis: presents in early childhood with stellate/cystoid maculopathy which progresses to a macular scar
  • Foveal schisis with spoke-like folds
  • Non-specific atrophy
  • Vitreous haemorrhage
  • Retinal detachment in 5%
  • Associated with hypermetropia
  • Electronegative ERG: reduced b wave
  • No systemic findings

Stickler’s syndrome

  • Autosomal dominant abnormality of type II collagen production (COL2A1)

    • An autosomal recessive form is also recognised
  • Aka ‘hereditary arthro-ophthalmopathy’

  • Ocular features

    • Myopia
    • Empty vitreous
    • Lattice-like pigmentary perivascular changes
    • Strabismus
    • Retinal tears, giant retinal tears and retinal detachment
    • Cataract: comma-shaped cortical opacities
    • Ectopia lentis
    • Open angle glaucoma
  • Systemic

    • Epiphyseal dysplasia: degeneration of large joints
    • Cleft palate
    • Abnormal uvula (bifid)
    • Mid-facial flattening
    • Pierre-Robin sequence: micrognathia, glossoptosis
    • Sensorineural deafness
    • Mitral valve prolapse
    • Marfanoid habitus
  • Clinical diagnosis with molecular genetic testing

  • Management

    • Prophylactic retinopexy for extensive lattice
    • Refractive correction for myopia to avoid amblyopia

Wagner syndrome

  • Autosomal dominant vitreoretinopathy
  • Optically empty vitreous
  • Myopia
  • Retinal detachment

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