Conditions affecting the external eye

This article gives an overview of conditions affecting the lashes, the lids and the lacrimal system. See the separate articles listed below for more detail:

• Allergic Conjunctivitis.

• Blepharitis.

• Blepharospasm.

• Chalazion.

• Congenital Nasolacrimal Duct Obstruction.

• Dacryocystitis and Canaliculitis.

• Dry Eyes.

• Epiphora.

• Eye Injuries.

• Orbital and Preseptal Cellulitis.

• Ptosis and Lid Lag.

Lashes

Trichiasis¹

Trichiasis is a lid margin disorder in which the eyelashes are misdirected and in contact with the ocular surface. It is a major cause of ocular morbidity and usually arises secondary to inflammation and scarring of the eyelash follicles. This may cause misdirection of a normal lash or the growth of metaplastic lashes. Rarely, congenital metaplastic eyelashes occur where there is a partial or complete second row of lashes (distichiasis) behind the meibomian gland orifices. It is often associated with cicatricial entropion.

With the exception of distichiasis, treatment is aimed at:

• Limiting scar formation (for example, through good control of meibomian gland inflammation or blepharitis).

• Removing the offending lash(es):

  • Epilation works for about 4-6 weeks.

  • Electrolysis and cryotherapy offer more permanent solutions.

  • Where there is a localised growth of lashes that are otherwise treatment-resistant, a wedge resection of that part of the lid may be performed.

• Other than epilation, these treatments are carried out in an oculoplastics unit and the success rate is around 70%.

• Distichiasis requires more involved oculoplastic surgery.

Poliosis²

This is premature, localised whitening of the lashes and eyebrows (it may involve any hairy area of the body). Microscopically there is decreased or absent melanin in the affected hair follicles. Classically, poliosis is associated with several genetic syndromes, including piebaldism, Waardenburg's syndrome and tuberous sclerosis. It may be associated with inflammation: ocular causes include chronic anterior blepharitis and sympathetic ophthalmitis. In rare cases, it has been seen as an early manifestation of conjunctival melanoma.

Madarosis³

Madarosis refers to loss of eyebrows or eyelashes. It is common, and occurs in association with both systemic and localised conditions. It may be scarring or non-scarring, depending on the aetiology. Causes include:

• Telogen effluvium is the most common cause of hair loss. Telogen effluvium is an increased shedding of otherwise normal telogen hairs. It usually occurs in response to systemic disease or altered physiological states such as severe emotional stress, etc. It can result in a diffuse loss of eyebrow hair.

• Infective disorders: chronic staphylococcal blepharitis, parasitic infection (eg, Demodex folliculorum), systemic fungal infections (eg, paracoccidioidomycosis) as well as HIV infection. Non-scarring eyebrow loss has been reported in secondary syphilis. It is incomplete and has been described as having a moth-eaten appearance. Madarosis is a hallmark of lepromatous leprosy.

• Autoimmune disorders: these include alopecia areata, discoid lupus erythematosus, systemic lupus erythematosus and scleroderma.

• Lichen planopilaris is a rare inflammatory condition that results in patchy progressive permanent hair loss, mainly on the scalp but eyebrows may be affected.

• Endocrine disorders: hypothyroidism, hyperthyroidism, hypopituitarism and hypoparathyroidism. Postmenopausal frontal fibrosing alopecia is a type of lichen planopilaris, a distinct form of scarring alopecia which consists of a receding hairline with scarring associated with a partial or complete loss of eyebrows. The loss of eyebrows may be the presenting sign.

• Drugs and toxins: long-term use of botulinum A injections and drugs such as miotics, anticoagulants, cholesterol-lowering drugs, antithyroid drugs, bromocriptine, propranolol, valproic acid and chronic epinephrine therapy have been reported to cause loss of eyelashes. Ciliary madarosis has been reported following cocaine use. Intoxication with arsenic, bismuth, thallium, gold, quinine and vitamin A can also cause loss of eyelashes.

• Tumours: both benign and malignant - see below.

• Dermatological conditions - eg, psoriasis, atopic dermatitis, seborrheic dermatitis, scleroderma.

• Dietary causes - eg, chronic zinc deficiency, hypoproteinaemia and, possibly, iron deficiency.

• Iatrogenic (following removal or due to trichotillomania).

• Metabolic disorders (eg, mitochondriopathy, malnutrition, sickle cell anaemia).

• Congenital (eg, Ehlers-Danlos syndrome and lid coloboma).

Infestations

These cause itching, blepharitis and folliculitis. Treatment may be by manual removal or chemical delousing. All clothes and linen also need to be laundered at >50°C.

• Pediculosis. Heavy infestations of Pediculus humanus corporis or capitis ('head louse') may spread to involve the lashes. If there are only a few lice, manual removal may be appropriate. More extensive infestation requires chemical treatment. Options include malathion or permethrin. The whole body needs to be treated on two separate occasions one week apart.

• Phthiriasis palpebrarum. Phthirus pubis ('crab louse') infestation occurs in pubic hair but may also affect children living in poor hygienic conditions. However, it is more commonly seen in adults, in whom it is usually a sexually acquired infection. It causes irritation and itching and is managed by trimming of the lashes (and pubic hair), destruction of the lice and ova (eg, yellow mercuric oxide 1%) and delousing of the patient (all the body) and other family members.

Lids

Allergic disorders

There are several forms:

• Acute allergic oedema. This is usually caused by insect bites but is also seen where there is angio-oedema, urticaria and occasionally in response to drugs. There is painless pitting periorbital oedema in an otherwise well patient. Systemic antihistamines may help.

• Contact dermatitis. This can occur in response to topical medication (due to the active component or the preservative), particularly chloramphenicol, neomycin and dorzolamide. Treatment is withdrawal of the offending agent ± a short course of mild steroid cream.

• Atopic dermatitis (eczema). There is thickening, crusting and vertical fissuring of the lids. Treatment is with emollients and mild topical steroid creams.

Infections

A wide variety of infections can occur around the external eye:

• Preseptal or orbital cellulitis. This is discussed in the separate Orbital and Preseptal Cellulitis article.

• Varicella zoster. This causes a widespread vesicular rash, primarily affecting the head neck and trunk. The eyelids (including the lining conjunctiva) can be affected by this itchy rash. Cool compresses and, occasionally, artificial tears can help. Reactivation of the virus gives rise to shingles (see next bullet point).

• Herpes zoster ophthalmicus. Shingles occurring in the first division of the trigeminal nerve gives rise to the characteristic unilateral maculopapular rash, often associated with marked pain and systemic malaise (may last up to one week prior to the development of the rash) and typically occurs in the older patient population. The eyelid, conjunctiva, episclera, sclera, cornea and anterior chamber may all be involved. (Cutaneous involvement of the tip of the nose suggests an increased likelihood of ocular complications, as this area is served by the first division of the nerve: this is called Hutchinson's sign.) Treatment is with systemic antivirals (eg, famciclovir 750 mg od for 7-10 days). Management of the lid involves cool compresses and topical lubrication (topical antibiotics are used for secondary infections.) Patients should be reviewed by an ophthalmologist with a slit lamp the same day to rule out globe involvement - see the separate Shingles (Causes, Symptoms, and Treatment) article.

• Herpes simplex. Primary infection is usually not clinically apparent unless occurring in the neonate (see the separate Ophthalmia Neonatorum article) or when associated with atopic dermatitis or immunodeficiency. Secondary infection frequently manifests itself as a dendritic corneal ulcer which needs to be further assessed in the Eye Unit to rule out deep structure involvement. Simple lid involvement in the absence of any deeper manifestation may be treated symptomatically with cool compresses ± antibiotic ointment to prevent secondary vesicle infection. Secondary disease is also managed with oral aciclovir (200 mg-400 mg five times a day for 7-14 days).

• Impetigo. This superficial skin infection, caused by Staphylococcus aureus and Streptococcus pyogenes, most commonly occurs in children, and the lids may become involved where there is facial infection. Treatment involves both topical antibiotics and systemic flucloxacillin or erythromycin.

• Erysipelas. S. pyogenes causes this more unusual, acute expanding cellulitis. Lid involvement may be severe. It is treated with phenoxymethylpenicillin or erythromycin.

• Molluscum contagiosum. This is caused by a dsDNA virus of the pox group, which is transmitted by close contact. It is characterised by pearly umbilicated nodules which may be profuse in HIV-infected individuals. It mainly affects children and young adults, who tend to have far fewer lesions.

• Necrotising fasciitis. Periocular infection may be secondary to trauma or surgery. Periorbital redness and oedema with subsequent bulla formation and black gangrenous discolouration of the skin are characteristic and prompt treatment with intravenous antibiotics as well as surgical debridement is essential.

Benign lumps

• Chalazion. A chalazion is caused by non-infectious meibomian gland occlusion, with focal secondary granulomatous inflammation.

• Hordeola (styes). A hordeolum (stye) may be external or internal and is typically staphylococcal. Most hordeola are external and result from obstruction and infection of an eyelash follicle and adjacent glands of Zeis or Moll glands. Follicle obstruction may be associated with blepharitis:

  • Internal hordeolum. This is uncommon and results from infection of a meibomian gland. The swelling develops and points on the inside of the eyelid.

  • External hordeolum (stye).⁴ This is an acute staphylococcal abscess of a lash follicle. It presents as a tender lid margin swelling pointing anteriorly (there may be multiple small lesions):
    

    • Treat with lash removal if painful: this facilitates drainage; then warm compresses and 'milking' (gentle massage to try to express contents through the follicle).

    • Systemic antibiotics may be useful if it is very large.

    • Patients shouldn't attempt to puncture the stye themselves. However, you may incise the stye using a fine sterile needle - this procedure is not appropriate for children.

    • Symptoms resolve rapidly following stye rupture/drainage.

    • Consider referral if there are symptoms/signs of associated cellulitis (preseptal or orbital) or if the patient is systemically unwell. Also, consider referring if the stye is persistent or particularly large and painful and has not responded to conservative treatment.

    • If the stye has an atypical appearance or recurs in the same location, think of the possibility of malignancy.

• Cysts. Various cysts can arise around the eye. Common ones include sebaceous cysts, cysts of Moll (benign, non-tender translucent lesions arising from the apocrine sweat glands) and cysts of Zeis (similar to cysts of Moll but containing oily secretions). Cysts can be removed in a simple minor operative procedure, under local anaesthetic.

Tumours^{5 6}

Eyelid tumours may be benign, precancerous or malignant. 5-10% of all skin cancers occur in the eyelids. Malignant neoplasms represent the leading cause of plastic reconstruction in the orbital region.

Benign tumours

• Benign lesions. A number of benign lesions can occur around the orbit, including seborrhoeic keratosis, actinic keratosis (20% of cases may progress to squamous cell carcinoma), cutaneous horn formation, keratoxanthoma formation and melanocytic naevi.

• Papilloma. These very common lesions are derived from the squamous cells and may be broad-based (sessile) or narrow-based (pedunculated). Some are related to the human papillomavirus.

• Pyogenic granuloma. This is a pink, vascular, often pedunculated lesion growing out from the inside of the lid, which usually arises following surgery or trauma. If large or symptomatic, it can be excised.

• Capillary haemangioma (strawberry naevus). This is rare (although one of the most common tumours of infancy) and, when it occurs around the eye, tends to form on the upper lid. The tumour usually grows rapidly in the first year of life before receding. If the vision is threatened (a large tumour may close the eye or dent the cornea, so giving rise to astigmatism and/or amblyopia), treatment may be warranted. This may involve laser treatment, local steroid injection (there are a number of complications) or systemic steroids if there is associated visceral involvement. Only a few cases need surgical intervention.

Malignant tumours ^{5 6}

The presenting signs of eyelid skin cancers are highly variable. Given their infiltrative growth pattern, they often present with a scar-like appearance or texture.They may also present as mass, ulceration, or sore, altered appearance, a red spot and trichiasis, or ingrown eyelashes. Tumour types include:

• Basal cell carcinoma. This accounts for >90% of all neoplastic eye lesions: look for the shiny, firm, pearly, umbilicated nodule. It most frequently occurs on the lower lid (followed by the medial canthus, upper lid and lateral canthus). There is a sclerosing, non-nodular type, which is less common and is difficult to diagnose, but look for an indurated plaque ± lid distortion and lash abnormalities. The latter may imitate chronic inflammation/scarring (eg, chronic blepharitis). Younger patients predisposed to basal cell carcinoma include those with xeroderma pigmentosum and Gorlin-Goltz syndrome. Treatment is with wide local excision.

• Squamous cell carcinoma. This accounts for around 2-5% of lid malignancies and may arise from pre-existing actinic keratosis. The tumour tends to occur on the lower lid at the margin, commonly in fair-skinned elderly people with a history of sun exposure. It may be plaque-like (rough, scaly erythematous patch), nodular or ulcerating with a sharply defined base and everting borders. All types may ulcerate, show lymphatic and perineural spread and metastasise. It can be aggressive.

• Sebaceous gland carcinoma. This rare (1-2% of lid malignancies), slow-growing but aggressive tumour usually affects the elderly and commonly arises from the meibomian glands. It tends to occur on the upper lid. It is aggressive and has a poor prognosis with an overall mortality rate of 10% (although 67% in patients with metastases). A history of recurrent presumed chalazion or chronic unilateral blepharoconjunctivitis should raise suspicions. Depending on tumour extent, wide local excision, regional lymph node clearance and even exenteration (widespread removal of the eye and periocular tissues) may be considered.

• Malignant melanoma. These represent 5% of cutaneous cancers and less than 1% of malignant lesions of the eyelid. They may develop de novo, from existing melanocytic naevi or from lentigo maligna. The typical characteristics of malignant melanoma are variable pigmentation, irregular borders, ulceration and bleeding. Eyelid melanomas involving the conjunctiva are usually more aggressive than those confined to the eyelid skin.

• Other tumours. Other more rare tumours occurring around the lids include melanoma (<1% of eyelid neoplasms: irregular pigmentation, inflammation, bleeding), Kaposi's sarcoma (arising from HHV8, relatively common in people with AIDS - look for a vascular purple-red nodule) and Merkel's cell carcinoma (very rare tumour of the elderly, usually presenting as a non-tender purple nodule and often on the upper lid).

Ectropion⁷

• Description. Ectropion is an eversion of the lid margin. Problems arise due to conjunctival and (particularly) corneal exposure. Severe cases may develop conjunctival keratinisation:

  • Involutional ectropion (which is age-related) is the most common form.

  • Paralytic ectropion occurs following a seventh cranial nerve palsy and cicatricial ectropion can occur following burns, trauma and chronic dermatitis.

  • Mechanical ectropion can occur whereby a mass (eg, a tumour) displaces the lid from the globe.

  • Congenital ectropion is rare and tends to be associated with other malformations.

• Presentation. It may be asymptomatic or the patient may complain of irritation, tearing (eye rubbing will make the ectropion worse) and irritation ± a red eye. Rarely, there will be complaints relating to exposure keratopathy (dry cornea).

• Assessment. Examine the lids, their apposition to the globe (note whether the tear meniscus lies between the lid margin and the globe or is down in the fornix) and the position of the puncta with regards to the globe - the punctum should not normally be visible: if it is, there is punctal ectropion. Assess the cornea with fluorescein staining to rule out exposure keratopathy. See the separate Dry Eyes article.

• Management. Lubricants and artificial tears may be helpful but surgery is the only corrective measure. This can be performed under local or general anaesthetic. If the ectropion is severe (assess by laying the patient in a supine position and asking them to close the eyes), taping the lids overnight can provide a temporary solution whilst waiting for surgery.

Entropion

• Description. This is an eversion of the lid margin. As with ectropion, it may be involutional or cicatricial (which can cause upper lid entropion - eg, trachoma) but it may also occur as a result of acute muscle spasm in response to ocular irritation (eg, infectious, inflammatory or traumatic) and, rarely, it may be congenital. Again, the main issue is its effect on the cornea.

• Presentation. Ocular irritation, foreign body sensation, blepharospasm, tearing and redness.

• Assessment. Assess the eyelashes to distinguish entropion from trichiasis (see 'Trichiasis', above) and the cornea for evidence of damage. Severe or recalcitrant cases may need a conjunctival biopsy if ocular mucous membrane pemphigoid is thought to be the underlying cause.

• Management. Temporising measures may include taping or, in the case of muscular spasm, botulinum injection (the effects last for about three months) but, ultimately, these patients will need corrective surgery.⁸

Lagophthalmos^{9 10}

• Description. Lagophthalmos is the inability to close the eyelids completely over the globe. The inability to effectively close the eyes leads to corneal exposure and excessive evaporation of the tear film. The main cause is facial nerve paralysis (paralytic lagophthalmos) but it also occurs after trauma or surgery (cicatricial lagophthalmos) or during sleep (nocturnal lagophthalmos). The main cause for paralytic lagophthalmos is Bell's palsy but it may be secondary to trauma, infections, tumours and other conditions.

• Presentation. The patient may complain of problems associated with exposure keratopathy: discomfort, redness, (compensatory) tearing and, if severe, photophobia and decreased visual acuity.

• Assessment. Patients should be asked to close their eyes: look for the Bell's phenomenon (the globe should roll up when the lid closes) - if it is poor or absent, there is an increased risk of corneal damage. Examine the cornea and assess the patient for any of the underlying causes outlined above.

• Management. Underlying causes should be managed (think of orbital diseases in proptosis: thyroid eye disease, orbital tumours, lacrimal gland tumours, orbital inflammatory pseudotumour) in addition to intensive lubrication ± taping at night. Irreversible causes may warrant referral for more permanent surgical intervention.The main purpose is to prevent exposure keratitis and to re-establish eyelid function. It is equally important for the patient to regain a cosmetically acceptable appearance.

Acquired disorders of the lid

• Dermatochalasis. Redundant and lax eyelid skin and muscle is known as dermatochalasis. Dermatochalasis is a common finding in elderly people and occasionally in young adults. Gravity, loss of elastic tissue in the skin and weakening of the connective tissues of the eyelid contribute; the upper eyelid is most often affected. Some systemic diseases may predispose patients to develop dermatochalasis. These include thyroid eye disease, renal disease, eyelid trauma, cutis laxa and Ehlers-Danlos syndrome.

• Blepharochalasis. This rare condition involves repeated episodes of painless non-pitting oedema of both upper lids which spontaneously resolve. Episodes tend to start during puberty and decrease in frequency with age. There may be an end result of dermatochalasis..

• Floppy eyelid syndrome. This self-describing problem tends to occur in obese men and is often associated with sleep apnoea and snoring. The loose lids tend to part during sleep so resulting in an exposed cornea and chronic papillary conjunctivitis. Treatment is with lubricants and, in severe cases, surgery. Patients with other symptoms and signs relating to sleep apnoea may need to be referred to a respiratory physician.

• Lid retraction. This describes the situation where the upper lid margin rests above the superior corneal limbus (where the cornea meets the sclera) and can arise from a number of causes:
  

  • Thyroid eye disease.

  • Neurogenic causes (eg, facial nerve palsy, hydrocephalus, sympathomimetic drops).

  • Mechanical causes (eg, over-correction of ptosis, scarring of upper lid skin).

  • Congenital causes (eg, Down's syndrome).

Congenital disorders of the lid

• Epicanthic folds. These are bilateral vertical folds of skin at the medial canthi which may give rise to the impression that the baby has an esotropia (inward turning squint). This can be ruled out by looking at the position of the light reflection of a pen torch with respect to the position of the pupil. Where there is uncertainty, orthoptist assessment can confirm or refute this.

• Epiblepharon. This is the phenomenon where there is an extra fold of skin on the anterior lid margin which causes the lashes to be directed vertically rather than out. Manual correction restores the normal position. This is common and the majority of cases resolve with age.

• Telecanthus. This is the wide separation of the medial canthi despite normally positioned orbits (contrast this with hypertelorism: the actual orbits are widely separated). This may be an isolated phenomenon or part of a syndrome.

• Coloboma. This is the uncommon condition of a partial or full-thickness defect of the lid and is associated with systemic conditions such as Treacher Collins' syndrome. Treatment is surgical.

• Cryptophthalmos. This is a failure of lid development. There is residual surface ectoderm covering the (often poorly developed) eye. This may be autosomal dominantly inherited.

Lacrimal system¹¹

Blocked lacrimal ducts

• Description. Lacrimal duct obstruction may occur at any level from the puncta to further down the nasolacrimal duct. Idiopathic stenosis is the most common. Other aetiology includes trauma, granulomatosis with polyangiitis, infiltrating nasopharyngeal tumours and stones (dacryoliths). Congenital nasolacrimal obstruction usually refers to delayed canalisation of the duct.

• Presentation. Infants present with a sticky non-infected and non-red eye. Both sides may be affected. In older children and adults, presentation is with epiphora (excess tearing) and there may be an associated ectropion due to constant rubbing away of the tears.

• Assessment. Babies are left alone until at least 2 years of age. In adults and older children the canal may be probed: this will require anaesthetic in children. If this is inconclusive, a dacryocystography (DCG) may be useful. This involves taking plain film images after injection of radiopaque contrast into the system.

• Management - if the symptoms are problematic, surgical correction can be performed under a general anaesthetic. Traditionally, an external approach has been used, opening the obstruction from the side of the nose; however, more recently, an endoscopic procedure is favoured.

Dacryocystitis

See the separate Dacryocystitis and Canaliculitis article.

Dr Mary Lowth is an author or the original author of this leaflet.