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Glaucoma

Miscellaneous Glaucoma

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Red cell glaucoma: secondary to hyphaema (usually traumatic)

  • Around 50% of patients with traumatic hyphaema have posterior segment injury
  • Raised IOP occurs in about 25%
  • Corneal blood staining can occur despite normal IOP and may persist for years
  • Black ball hyphaema indicates deoxygenation of blood and increased hypoxic risk to endothelium

Ghost cell glaucoma: secondary to vitreous haemorrhage

  • Tan-coloured cells seen in the angle having lost haemoglobin
  • Medical therapy but consider AC washout

Angle recession glaucoma

  • Post-traumatic angle recession and trabecular damage carries 10% risk of glaucoma at 10 years

  • A rupture in the face of the ciliary body that extends posteriorly between the longitudinal and circular fibres of the ciliary muscle

  • Ciliary processes are displaced posteriorly and the overall shape of the ciliary body changes from triangular to fusiform

  • Asymmetry of AC depth, pupil and angle seen

  • Gonioscopy

    • Torn iris processes
    • Variation in ciliary body width within same quadrants
    • Increased visibility of scleral spur

  • Patients should be screened at 6mo and yearly

Schwartz-Matsuo syndrome

  • AC inflammation and raised IOP associated with rhegmatogenous RD
  • Photoreceptor outer segments block the TM
  • Medical therapy until surgical repair of the RD

Raised episcleral venous pressure

  • Back-pressure reduces aqueous drainage
  • Seen in

    • Sturge Weber syndrome: causes two types of glaucoma

      • Under age 10, mechanism is due to abnormal angle formation
      • Over age 10, mechanism is due to increased episcleral pressure

    • Orbital varices

      • Rare vascular lesions that intermingle with orbital contents
      • Engorge depending on posture, straining or coughing leading to intermittent proptosis
      • Intracranial extension is rare
      • May be complicated by orbital hemorrhage
      • Surgery is indicated for complicated lesions but carries great risk of optic nerve compromise through ischaemia

    • Carotid-cavernous fistulae

    • SVC obstruction/mediastinal tumours

    • Cavernous sinus thrombosis

    • Retrobulbar tumours

    • Dysthyroid orbitopathy

    • Familial episcleral pressure elevation

Blood in Schlemm’s canal is seen in glaucomas affecting episcleral pressure

  • Sturge Weber
  • Carotid-cavernous fistula
  • Hypotony
  • 20% of malignant melanoma is associated with raised IOP

Iridoschisis: splitting and atrophy of the iris leaf can cause angle closure

  • Associated with nanophthalmos

Iridocorneal endothelial syndrome (ICE)

  • Rare unilateral** abnormality of corneal endothelium** migrating across the angle, TM and anterior iris

    • Abnormal endothelium behaves like epithelium in manner of proliferation

  • Significant anterior segment distortion

  • F>M, typically 20-40 years old

  • 50% risk of glaucoma (a progressive secondary closed angle glaucoma due to high peripheral anterior synechiae)

    • Glaucoma is worse in those with mainly Cogan-Reese or iris atrophy

  • Possibly related to HSV

  • Subtypes

    • Chandler syndrome (most common): mainly corneal (including corneal decompensation)
    • Essential iris atrophy: mainly iris changes eg correctopia
    • Cogan-Reese syndrome: diffuse iris naevus or pigmented nodules

  • Clinical features

    • Pain
    • Blurred vision
    • Corneal guttata
    • Corneal oedema
    • Corectopia: displaced pupil with iris atrophy
    • Pseudopolycoria
    • Broad PAS on gonioscopy

  • Management as for POAG. There is no role for laser trabeculoplasty.

Posterior polymorphous dystrophy

  • Bilateral corneal endothelial adhesions with the iris causing angle closure
  • Presents similarly to ICE but is inherited (ie. presents in childhood and is usually autosomal dominant) and bilateral (cp with ICE which is unilateral)
  • 15% risk of glaucoma

Epithelial downgrowth

  • Migration of corneal epithelium through a wound (from trauma or surgery) which can then spread over the endothelium and TM
  • Difficult to treat with poor visual prognosis.

Aqueous misdirection syndrome aka malignant glaucoma aka ciliary block

  • Misdirection of aqueous into the vitreous causes anterior displacement of the vitreous, ciliary processes and lens with secondary angle closure

  • Risk factors

    • Short axial length
    • Chronic angle closure
    • Previous AACG
    • Nanophthalmos
    • Uveal effusion syndrome
    • Post-operative especially cataract or glaucoma surgery
    • Post-laser PI
    • Rarely after miotic therapy

  • Clinical features

    • Blurred vision
    • Raised IOP
    • Shallow/flat AC both centrally and peripherally
    • No pupillary block (cp. PACG): therefore can occur despite PI
    • No posterior choroidal/suprachoroidal cause
    • May occur post-trabeculectomy: flat bleb, high IOP, shallow AC, patent PI

  • Management

    • Half can be managed medically
    • Mydriasis (cp. PACG): cycloplegia pulls the lens-iris diaphragm posteriorly
    • Systemic and topical IOP-lowering therapy
    • Ensure patent PI
    • Laser disruption of the anterior vitreous face (hyaloidotomy)
    • Argon laser to the ciliary processes through the patent (usually surgical) PI
    • Trans-scleral cyclodiode
    • Surgery: peripheral zonulo-hyaloidectomy to create a single-chamber eye
      • Cataract extraction with PPV to debulk the vitreous

Post-operative raised IOP

  • Post-cataract surgery: consider a post-operative short course of diamox prophylactically

    • Retained viscoelastic
    • Retained lens fragment
    • Post-op inflammation
    • Suprachoroidal hemorrhage
    • Phacoanaphylaxis (see above)

  • Post-vitreoretinal surgery

    • Gas overfill: short-term medical therapy may suffice but some gas may need removal

    • Scleral buckle: secondary angle closure typically resolves spontaneously

      • Mechanism: obstruction of venous outflow produces choroidal effusions which cause anterior rotation of the ciliary body and secondary angle closure.
      • Alternatively: neovascular glaucoma caused by buckle-induced ischaemia

    • Silicone oil: can obstruct the TM. Managed with medical therapy first. Early oil removal (<6 months) can help but beyond that, removal makes little difference

    • Vitrectomy: may cause ghost cell glaucoma and increase risk of rubeosis

Plateau iris

  • Anteriorly positioned and larger ciliary processes push iris anteriorly in the periphery

  • AC may have normal depth centrally but can be much narrower peripherally

  • Iris has a flat contour

  • On dilation, the peripheral iris bunches up and occludes the TM

  • Demographics

    • Younger patients
    • Less hyperopic than primary ACG
    • Asian or caucasian

  • Plateau iris configuration refers to those who have normal IOP

  • Angle does not respond to indentation on gonioscopy as easily

    • “Double hump” sign on indentation gonioscopy: posterior displacement of the mid-peripheral iris depicts persistently anterior peripheral iris
    • Plateau configuration can persist despite PI
    • Peripheral anterior synechiae develop from Schwalbe’s line and then extend posteriorly (cp. With pupil block wherein PAS develop in a posterior to anterior direction)

  • Laser PI must be done but is not adequate

    • NB: a diagnosis of plateau iris cannot be made until a patent PI is performed to eliminate any component of pupillary block
    • Laser peripheral iridoplasty is more definitive
    • Chronic miotic therapy

Tilted disc syndrome

  • Superotemporal disc is elevated

  • Inferonasal disc is posteriorly displaced

  • Associated with superotemporal field loss

    • Does not respect vertical midline
    • Improves with myopic correction (‘refractive scotoma’)

  • May mimic a bitemporal hemianopia if bilateral

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