Clinicians from across the eye care professions, along with academics and key industry stakeholders, convened recently in Pula in northern Croatia to attend Pula 2018, a joint conference between the European Academy of Optometry and Optics (EAOO) and the Croatian University of Applied Sciences Velika Gorica. Representing more than 30 countries worldwide, delegates were invited to discuss and share the latest research, education and clinical practice in optometry, optics and dispensing, and vision science.
Opening the proceedings, Dr Thomas Freddo (Adjunct Professor at the MCP Health Sciences University in Worcester, Massachusetts) offered an overview of conjunctival disease and its management, based on his long career in optometric clinical practice.
He began by saying that he had always found it useful to categorise conjunctival disease into four groups; limbal, cystic, salmon-coloured or pigmented. Each was considered in turn and clinical pearls were drawn from real life cases the speaker had dealt with over his long career. Here are a few individual lesions discussed which often are not mentioned in discussions of anterior eye disease.
Limbal lesions
The first lesion considered was the rare limbal dermoid. As this rare lesion is present from birth, the speaker reminded the audience that its presence is most likely only to be known by optometrists as post-surgical scar tissue around the limbus. What is perhaps less well known is that the condition is sometimes part of a wider systemic syndrome.
An example of such would be Goldenhar syndrome, a rare congenital defect characterised by incomplete development of the ear, nose, soft palate, lip and mandible. It is associated with anomalous development of the first branchial arch and second branchial arch, the area around the developing pharynx of the human foetus.
The presence of phlyctenules, or phlyctenulosis, is an inflammatory condition characterised by discrete pale inflammatory lesions at the limbus. Though rare, the condition is worth remembering in cases of persistent red eye.
This was illustrated by the case of a 15-year-old girl, a daughter of a colleague of the speaker, whose red eye seemed unresponsive to the usual interventions. Careful questioning revealed that the girl had been sharing eye make-up with her friend over the preceding months.
This prompted the speaker to order a culture and the presence of Staphylococcal species was confirmed. The condition itself is not an infection as such, but the body’s response to toxins released by dead bacteria. Knowing this, topical steroids were introduced and the condition soon resolved.
Appropriate advice was also given regarding hygienic use of eye make-up. On a somewhat worrying note, the speaker explained that phlyctenules are sometimes associated with tuberculosis, so their presence should always prompt questions about any recent foreign travel.
Salzmann’s nodular degermation was next considered. This is again a rare condition and presents as a sequela to longstanding chronic ocular surface diseases that have since ‘burned out’. It is characterised by bluish-white to grey nodules without indications of inflammation. The epithelium is intact so staining is unlikely.
Bitot’s spots are an indication of ocular dryness, or xerophthalmia, associated with vitamin A deficiency so are common in areas where malnutrition occurs. Interestingly, the raised patches on the interpalpebral conjunctiva represent a protective response to severe dryness, with the exposed conjunctiva becoming keratinised to appear as raised pale dry areas of hard skin or leucoplakia. Their presence may cause discomfort but will resolve if vitamin A supplementation is offered.
The speaker then discussed the differences between the commonly found pterygium and the less common but more concerning conjunctival interepithelial neoplasm (or CIN). The former represents excess conjunctival growth, triggered by factors such as UV exposure, and the growth is clearly seen to move across the limbus.
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Where conjunctival growth extends around the limbus but does not cross, then CIN must be considered as a possible diagnosis. CIN is significant as it represents a pre-malignant stage of growth and involves the stem cells found in high concentration at the limbus. Subsequent malignancy is likely to relate to squamous cells as basal cell carcinomas are not found on the conjunctiva.
Cystic
A cyst will form when epithelial cells are pushed into a surrounding area, such as stromal conjunctiva or even sclera, where they divide to form a sphere filled with clear fluid. A useful confirmation of a cyst is to shine a bright thin slit onto them and they will be seen to scatter the light and glow. Where a cyst forms in dense or firm tissue, such as the stroma, they may not form a complete sphere (as in the conjunctiva) but instead appear hemispheric.
On a point of anatomical interest, the speaker described how goblet cells are found in their highest concentration in the fornices but are only sparsely concentrated at the limbus. Similarly, there are usually significant numbers of usually invisible lymphatic vessels throughout the conjunctiva.
These can become blocked and swollen. Conjunctival lymphangiectasis is a condition in which conjunctival swelling occurs as a result of dilated conjunctival lymphatic channels, most notably on the bulbar conjunctiva. The appearance at the slit lamp will show cystic lesions appearing clear or yellowish, representing elevated conjunctival lymphatic channels separated by translucent septate walls.
Although the actual cause is unknown, the presumed underlying aetiology is obstructed lymphatic channels. The condition is thought of as benign, but can be associated with local inflammation, disruption of the tear film (as with dellen), or secondary to haemorrhage (haemorrhagic lymphangiectasia of the conjunctiva).
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Generally speaking, the condition resolves once the source of mechanical impact is removed. This has been known to be caused by poorly fitting soft contact lenses.
The speaker described a case from some years back which had initially puzzled both him and the consultant ophthalmologist he was working with at the time. It concerned a middle-aged female who had presented with diffuse redness of the conjunctiva. On careful inspection, there appeared to be a widespread scatter of multiple tiny yellow bumps across the conjunctiva within the red area. Not sure what they were, he initially treated with topical steroid and the bumps got smaller but persisted.
He then decided to undertake a biopsy. Histological assessment of the lumps showed them to be granulomatous in nature. Where the granuloma is found to be hollow in the middle, then TB might be considered. Where cells grow through the middle, as in this case, sarcoidosis is a possible diagnosis. Sarcoidosis of the salivary glands presents as painless and persistent enlargement of the parotid glands and is present in around 6% of patients with the disease. This was subsequently confirmed in this patient and the diagnosis was then confirmed.
In cases where a follicular conjunctivitis is persistent and unresponsive to usual interventions, then molluscum contagiosum might be involved. As the lesions in this condition continually shed virus, then they must be managed first if any viral conjunctivitis is to resolve. Molluscum contagiosum is often thought of as a neonatal condition but may arise in anyone with immunocompromise. This includes those undergoing transplantation therapy and those with AIDS.
For this reason, the conjunctival condition is rife at present throughout Southern Africa, as the speaker was able to personally testify. In severe cases in remote areas, management includes abrading of each lesion with a hard utensil, such as a wooden toothpick, until they bleed. Phenol is then applied and the lesions might then resolve.
Lid malignancy
Though rare, sebaceous gland carcinomas of the lid, which might include involvement of either the meibomian glans or the gland of Zeiss, are important to know about because of their very high associated mortality rate. Even small lesions have a mortality of as great as 25%.
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Differential diagnosis is important and one might always be suspicious where there is asymmetric and persistent blepharitis or where a chalazion persists after topical management. Excision of a suspect chalazion will confirm malignancy and the speaker hopes most ophthalmologists would order a biopsy of any surgically excised chalazion. The problem is due to the readiness of these glandular cells to metastasise, something he described as ‘pagetoid spread’.
A useful clinical tip when assessing any elevated lesion on the lid is to look at the line of the lashes. Where the lash line is diverted, consider malignancy, or in the words of the speaker, ‘worry’.
Look out for further reports of clinical interest in the coming weeks along with an interview with the president of EAOO.