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Ocular cicatricial pemphigoid

Ocular cicatricial pemphigoid is chronic cicatrising conjunctivitis. It is a rare, idiopathic, chronic, progressive autoimmune disease. Although this vesiculobullous disease primarily involves conjunctiva it also affects other mucous membranes including the mouth and oropharynx (80 per cent), genitalia and anus as well as skin in 15 to 25 per cent of cases. It is twice as common in women as men.

Patients frequently present with recurrent attacks of mild and non-specific conjunctival inflammation with occasional mucopurulent discharge. Early signs are insidious conjunctival hyperaemia. Later, subconjunctival bullae may form and then rupture causing ulceration followed by subepithelial fibrosis.

Ocular complications include trichiasis, cicatrising entropion, symblepharon (localised adhesions between the conjunctiva over the eye and back of the eyelid), dry eye (damage of the lacrimal ductules), ankyloblepharon (adhesion of the edges of the upper and lower eyelids at the outer canthus), corneal vascularisation and keratinisation. Recurrent, unresponsive mild and non-specific conjunctival inflammation ­ if associated with conjunctival shrinkage ­ suggests OCP.

Thyroid eye disease

Thyroid eye disease (TED) is an autoimmune orbitopathy associated with thyroid dysfunction (usually Grave's disease) and is the most common cause of unilateral or bilateral proptosis in adults.

An active inflammatory phase and painful eyes (sometimes for two-three years) leads eventually to a burnt-out/quiescent phase where eyes are white but there are residua of active phase (lid retraction, proptosis, motility disturbance).

The more severe the active phase the greater the residual disfigurement/ dysfunction. TED most commonly occurs between the ages of 25 and 50. It is five times more common in women, although it is more frequently severe in men and the elderly. Smoking is proven to exacerbate TED and reduces the efficacy of treatment.

Depending on the severity of the disease, symptoms will range through: starey appearance, grittiness, red eyes, puffy eyelids, painful eye movements, double and poor vision. Its five main clinical manifestations are: eyelid retraction (90 per cent); periorbital soft tissue swelling (80 per cent); proptosis (60 per cent); restricted eye movements (50 per cent) and optic nerve compression (5-10 per cent).TED is not limited to Grave's disease.

Endophthalmitis

Although endophthalmitis refers to intraocular inflammation involving the vitreous cavity and the anterior chamber of the eye, it is usually used to describe an infectious process.

Infection can be exogenous or endogenous and can occur at any age in men and women. Causes include any intra-ocular surgery ­ cataract surgery, glaucoma surgery, retinal surgery ­ or penetrating ocular trauma. Typically acute endophthalmitis usually develops within a few days of surgery but can occur much later. It is also caused by blood-borne spread of infection during septicaemia (infected intravenous lines, endocarditis, pyelonephritis and osteomyelitis).

A rapid drop in vision with or without pain in a postoperative period is highly suggestive of infectious endophthalmitis. Pain is usually severe but not invariably so.

Features include a discharge, photophobia and lid swelling. Signs are red, tender eye with swollen lids, injected conjunctiva, and a hazy cornea with or without hypopyon (fluid level of pus in anterior chamber).

Carotid-cavernous fistulae

Carotid-cavernous fistulae are arterial communications between the carotid artery or one of its branches and the cavernous sinus (lying behind the orbits and around the pituitary gland). High-flow fistulae occur at any age while low-flow fistulae are more common in the elderly, especially postmenopausal hypertensive women.

Most high-flow fistulae result from severe trauma, car accidents, sports injuries and falls. Most low-flow fistulae are idiopathic. High-flow fistulae typically occur suddenly and are characterised by a florid clinical picture while low-flow fistulae are more insidious in onset but characterised by similar albeit more subtle features.

Symptoms are blurred vision, headache, double vision, and ocular or orbital pain and a subjective bruit. Signs include a bruit heard over the vertex of the skull (high-flow fistulae), characteristic tortuous epibulbar vessels, conjunctival swelling and haemorrhage, pulsating proptosis, reduced vision and restricted ocular movements.

Bilateral conjunctival vascular engorgement may be confused with chronic conjunctivitis and associated proptosis with thyroid eye disease.

Most fistulae are not life-threatening and the major organ at risk is the eye. Interventional radiologists can treat patients with visual deterioration, severe exposure keratopathy, intolerable bruit or headache.

Acute angle closure glaucoma

Acute angle-closure glaucoma is due to sudden blockage of the trabecular meshwork ­ through which aqueous humour normally exits the eye and is located in the angle of the anterior chamber ­ by the iris. Patients may complain of eye pain, headache, blurred vision, rainbow-coloured halos around lights, nausea and vomiting typically coming on over a few minutes to a few hours. Pain may be poorly localised so patients may present with nausea, vomiting and headache symptoms similar to acute abdomen. Signs include red eye, corneal oedema (hazy cornea ­ ground glass appearance/lack of lustre), semi-dilated and unreactive pupil and hard eye on digital pressure (compare with your own).

Acute primary angle closure glaucoma is rare before age 40 but becomes increasingly common thereafter as the depth and volume of the anterior chamber angle decrease. This type of glaucoma is more common in women. Hypermetropic or longsighted people have shallower anterior chambers and are at greater risk.

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