The Conjunctiva: Diseases and Tumours

Published on 08/03/2015 by admin

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3 The Conjunctiva: Diseases and Tumours

The conjunctiva is the transparent mucous membrane lining the inner surfaces of the eyelids; it is reflected over the anterior episclera and sclera before terminating at the limbus, where it is continuous with the corneal epithelium. In the embryo the conjunctiva develops from the ectoderm covering the lids and surface of the globe, and is formed during the third month of intrauterine life as the eyelids grow together. A healthy conjunctiva is essential for normal ocular function and together with the eyelids it is critical in maintaining a suitable environment for the cornea to function as the primary refractive element of the eye. Its mucous and accessory lacrimal secretions are important components of the precorneal tear film, and their deficiency gives rise to tear film instability and poor wetting of the corneal surface, which may lead eventually to pathological changes in the cornea. It follows, therefore, that examination of the whole conjunctival surface is necessary in order to interpret signs in the cornea, and an understanding of conjunctival pathology is a prerequisite to the effective management of many corneal disorders. As part of its function in maintaining the corneal environment, the conjunctiva also has an important role in defending the eye against a variety of agents.

THE NORMAL CONJUNCTIVA

PATHOLOGICAL CHANGES OF THE CONJUNCTIVA

The conjunctiva may undergo a variety of changes as a result of disease. Recognition of the types of change may give valuable information about the aetiology of the disease, but the conjunctiva has a limited repertoire of pathological responses and great care should be exercised before attributing particular conjunctival signs to a diagnosis. It is rare for pathognomic findings to occur in the conjunctiva: diagnosis should always be based on the history and on examination of the adjacent tissues such as the lids and cornea in addition to the conjunctiva.

Causes of papillary and follicular conjunctivitis are listed in Table 3.1.

Table 3.1 Causes of papillary and follicular conjunctivitis

Papillae Follicles
Allergic conjunctivitis; atopic, vernal, seasonal or perennial Virus infection: adenovirus, herpes simplex, molluscum contagiosum
Topical preparations: drops, preservatives, ointments Cnlamydial infection: trachoma (subtypes A–D), paratrachoma (subtypes E–K)
Chronic 1irritation: giant papillary conjunctivitis, superior limbic conjunctivitis, dry eyes Drug induced: glaucoma, antiviral drops
  Lymphoid disease: reactive hyperplasia, conjunctival lymphoma

DRUG-INDUCED CHANGES

The normal conjunctiva and cornea can undergo changes in response to toxicity from topical or systemic drugs or other chemicals, in the absence of overt inflammation. Many old topical medications contained silver or mercury which were deposited in the conjunctiva; these are no longer used, but toxic changes can be seen with topical antibacterial or antiviral drugs if given for prolonged periods. In the cornea this is seen as punctate epithelial erosions or a persistent epithelial defect and in the conjunctiva as a papillary reaction, keratinization or punctal stenosis. Preservatives in eye drops can frequently induce problems, particularly in eyes with tear film abnormalities or when the drops are given for long periods of time. Such abnormalities normally resolve on ceasing treatment or substituting a preservative-free preparation.

Contact hypersensitivity reactions may arise in the conjunctiva or eyelids from a wide variety of agents but are commonly related to the use of cosmetic preparations, contact lens solutions or topical medications (eye drops or ointments). Substances may also be transferred to the periocular tissues from the hand by means of eye-rubbing or contact with bedlinen. The skin changes on the eyelids resemble those of a contact dermatitis, whereas in the conjunctiva there is primarily a follicular response. Symptoms include redness, irritation, discharge and, especially, itching of the eye. Patients may also complain that symptoms are most marked immediately after application of the drop or ointment. The pathogenesis involves delayed (cell mediated) hypersensitivity; the condition evolves gradually over several days or weeks unless previous sensitization has occurred, in which case its onset may be much more acute.

CHEMICAL BURNS

Acid burns coagulate and precipitate the superficial proteins of the conjunctiva and cornea; they do not penetrate the eye and so tend to cause only superficial scarring. In contrast, alkalis saponify the lipid in cell membranes and rapidly penetrate the eye producing devastating damage to both blood vessels and intraocular structures within minutes of contact. First-aid measures, especially prompt immersion of the eye in cold water or irrigation of the conjunctiva with bicarbonate solution to dilute and remove the alkali are vital in reducing damage. Alkali burns are followed by collagenase secretion from the limbal conjunctiva in the recovery phases; this may produce corneal melting and is compounded by destruction of the accessory lacrimal glands and goblet cells in the conjunctiva, producing tear film abnormalities. Corneal ulceration is the most serious complication of chemical injury and can lead to perforation. Decreased collagen synthesis is associated with local ascorbate deficiency and keratocyte loss. Damage to the limbus results in delayed epithelialization with conjunctiva-derived epithelium resulting in a vascularized opaque corneal surface and very poor prognosis with corneal transplantation. Surgical management of these eyes involves either autologous or donor limbal stem cell transplantation (see Ch. 6).

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Fig. 3.35 In the same patient as Fig. 3.34, there is extensive conjunctival necrosis 3 weeks after the ammonia burn. The whole upper tarsal conjunctiva is pale, yellowish and swollen, with poorly defined vessels. The upper edge is starting to slough away from the underlying tissues. Healing will occur with fibrosis of the lid leading to cicatricial entropion, tear film deficiency and further corneal damage.

By courtesy of Professor R J Buckley.

NONPIGMENTED TUMOURS OF THE CONJUNCTIVA

Tumours of the conjunctiva occur infrequently and may cause diagnostic problems at presentation. The clinical differentiation between hypertrophic and neoplastic processes, and between benign and malignant conditions, may be extremely difficult on the basis of a single examination. Repeated observations over a period of time with the aid of serial photographs may be helpful in determining the evolution of the lesions; in many cases a biopsy will be required to make a pathological diagnosis.

Tumours may be classified, according to their tissue of origin, into those arising from the surface epithelium (or its associated glandular elements), connective tissue, vascular tissue, lymphoid tissue or peripheral nerves. Some common conjunctival tumours are listed in Table 3.2. Pigmented lesions of the conjunctiva are an important group of conditions that are considered in detail separately.

Table 3.2 Common conjunctival tumours

Cell/tissue of origin Benign Malignant
Epithelium Hyperplasia, papilloma, CIN* Squamous cell carcinoma
Vascular Haemangioma Kaposi’s sarcoma
Lymphoid system Reactive lymphoid hyperplasia Lymphoma
Nerve Neurofibroma  
Melanocytic Naevus, melanocytosis Melanoma

PIGMENTED LESIONS OF THE CONJUNCTIVA

The melanocytes of the conjunctiva are derived from the neural crest, as are those of the skin and uveal tract. A wide variety of pigmented conjunctival lesions is seen clinically. They can be difficult to diagnose and have varying degrees of malignant potential. Conjunctival naevi are usually thought of as congenital lesions, although they are frequently not apparent at birth and tend to become larger and more pigmented with age. Junctional and compound conjunctival naevi have a low malignant potential. About 60 per cent of conjunctival melanomas arise in a naevus or in an area of primary acquired conjunctival melanosis. On examination, junctional, compound and subepithelial naevi and primary acquired melanosis, move with the conjunctiva but deep conjuctival melanocytic lesions do not.

Conjunctival naevi are similar to cutaneous naevi but with two important differences: the conjunctiva has no dermis, hence ‘subepithelial’ replaces ‘dermal’ in the terminology; also naevus cells migrating down into the subepithelial tissue may be accompanied by surface epithelial cells and if goblet cells are present mucinous cysts may form. Most conjunctival naevi are compound which have a low malignant potential.

MELANOSIS

Congenital melanosis is common in eyes of black patients. It is either epithelial where it appears as localized pigment flecks or subepithelial where it is diffuse and may be associated with scleral and uveal pigmentation (ocular melanocytosis – melanosis oculi).

Ocular melanocytosis together with ipsilateral lid and periorbital skin pigmentation (blue naevus) comprises oculodermal melanocytosis (naevus of Ota). This is a unilateral slate grey discolouration of the sclera that is associated with increased pigmentation of the lid skin and uveal tract. There is no increased risk of malignancy in the lids or conjunctiva but patients with melanosis oculi do have an increased risk of uveal melanoma (1:400), probably due to increased numbers of melanocytes in the uveal tract.

Acquired melanosis may be due to a variety of causes such as exposure or drugs but primary acquired melanosis of the conjunctiva, which is akin to lentigo maligna of the skin, carries a high risk of the patient developing multiple malignant conjunctival melanomas. Although malignant melanomas of the conjunctiva will invade locally and metastasize to the regional lymph nodes, their growth tends to be slow; small lesions do well with local treatment, especially cryotherapy.

MALIGNANT MELANOMA OF THE CONJUNCTIVA