Benign lacrimal gland disease

The lacrimal gland is liable to inflammation, cysts and benign tumours, but these conditions can present in a similar fashion to malignancy and this complicates the clinical management of these patients. Inappropriate management of benign conditions can lead to serious consequences - as with, for example, malignant recurrence after biopsy of a benign pleomorphic adenoma.

Dacryocoele (Dacryops)

Dacryocoele is a retention cyst of a gland ductule and often presents in young adults, with a variable swelling in the supero-temporal conjunctival fornix and bursts of apparent lacrimation. The clinical diagnosis is absolute and imaging is not required.

Microsurgical opening of the cyst, to allow free drainage of the affected ductule, is indicated only where the cyst is large and persistent. Surgery should be with an operating microscope and the greatest of care taken to avoid damage to the normal lacrimal gland ductules - for fear of a post operative dry eye.

Very rarely the affected ductule will become infected with Actinomyces, this causing a slightly inflamed and chronically discharging eye.

Pleomorphic adenoma

Pleomorphic adenomas account for about 5% of all orbital tumours, 25% of lacrimal fossa masses and 50% of all epithelial tumours of the lacrimal gland. Most affect the orbital lobe and become evident in the fourth and fifth decade as a slow onset of painless proptosis and infero-medial displacement of the globe; the much rarer palpebral lobe lesions present in young people with a shorter history of a hard, mobile mass above the lateral part of the upper tarsus.

Orbital lobe tumours show a smooth expansion of the lacrimal gland fossa by an oval lesion in which calcification is rare, the mass causing displacement of orbital structures and often flattening of the globe (Figure 12.10); it is unusual for these tumours, even when large, to extend anterior to the orbital rim. In contrast, the rare palpebral lobe tumours show a normal gland with an enlarged, rounded anterior surface extending outside the orbital rim on CT scan.

The key to treatment of pleomorphic adenomas is recognition, on the basis of

Lacrimal Tumor Pictures
Figure 12.10 CT scan of a typical pleomorphic adenoma, showing displacement and flattening of the globe by a round lesion that may cause scalloping of the bone in the lacrimal fossa.

clinical history and radiological signs, with avoidance of biopsy. Because of the long-term risk of spontaneous malignant transformation, tumours of the orbital lobe should be excised intact through a lateral orbitotomy and breach of the "pseudocapsule" of compressed tissues avoided; to this end, the tumour is handled at all times with a malleable retractor and not with any form of forceps.

Palpebral lobe pleomorphic adenomas, sometimes mistaken for large chalazia and curetted, are excised intact through an upper eyelid skin-crease incision.

Breach of the pseudocapsule of these tumours risks a pervasive recurrence of tumour (sometimes malignant) throughout the orbit, this necessitating orbital exenteration. Although there are advocates of fine-needle aspiration biopsy of these tumours, there is no logical reason for undertaking this in the presence of clinically and radiologically characteristic disease.

Keratitis sicca can be troublesome in a few cases, although the incidence of this condition is lower with preservation of the palpebral lobe during excision of these tumours. It is treated with topical lubricants and, where necessary, occlusion of the lacrimal drainage canaliculi.

Dacryoadenitis

The lacrimal gland may be affected by an acute polymorphic inflammation, which may be due to bacterial infection, or a chronic, predominantly lymphocytic, dacryoadenitis which may be due to underlying systemic diseases such as sarcoid or Wegener's granulomatosis.

Acute dacryoadenitis presents with painful, red swelling of the upper eyelid - with an "S"-shaped ptosis (Figure 12.11) - and tenderness of the underlying lacrimal gland; systemic malaise is unusual.

Painless swelling of one or both lacrimal glands is the usual manifestation of chronic dacryoadenitis and the gland often shows

Figure 12.11 Slightly "S"-shaped lateral ptosis due to lacrimal gland enlargement.

diffuse enlargement on CT, with extension of changes outside the limits of the gland and with moulding of the abnormal tissue around the globe (Figure 12.12) - unlike the compressive flattening of the globe seen with pleomorphic adenoma.

Although most acute dacryoadenitis is probably not bacterial, it is usual to treat such cases with a course of systemic antibiotics and non-steroidal anti-inflammatory medications. If inflammation persists or worsens, orbital

Lacrimal Gland Abscess

Figure 12.12 Diffuse enlargement of the lacrimal gland on CT scan, due to dacryoadenitis: (a) axial view, (b) coronal view.

Figure 12.12 Diffuse enlargement of the lacrimal gland on CT scan, due to dacryoadenitis: (a) axial view, (b) coronal view.

CT scan should be performed with a view to surgical drainage of an abscess or biopsy of a lacrimal gland mass. The patient should be followed for several months, until there is clear evidence of resolution of any mass; if there is a persistent lacrimal gland mass, the patient should be scanned with a view to biopsy, as malignancy of the lacrimal gland may present as subacute dacryoadenitis.

Chronic dacryoadenitis requires CT scan of the orbit, chest x ray and blood tests for sarcoid and other systemic inflammatory diseases. If CT demonstrates lacrimal gland enlargement with moulding to the globe, then biopsy is indicated. If the mass is fixed at the orbital rim and palpable, then biopsy may be achieved under local anaesthesia, but otherwise general anaesthesia should be used as it can be difficult to locate mobile intraorbital masses under local anaesthesia.

General method for anterior orbitotomy and incisional biopsy

A skin incision is placed in a suitably hidden position, generally the upper eyelid skin-crease or the lower eyelid "tear trough", and for most incisional biopsies should be about 3cm long. The underlying orbicularis muscle is cauterised and divided at the midpoint of the skin incision, the points of a pair of scissors inserted through the defect and the scissors opened widely along the line of the muscle fibres, to separate them by blunt dissection; any remaining bridging tissues are diathermied and divided to reveal the underlying orbital septum. The septum is likewise divided along the line of incision, to expose the orbital fat, and the direction of the mass to be biopsied ascertained by analysis of the imaging and by palpation.

A closed pair of blunt-tipped scissors is gently directed through the orbital fat towards the site to be biopsied and the scissors opened widely to reveal the depths of the tissues; before withdrawing the scissors, a 12-16mm malleable

retractor is inserted alongside the opened scissors to maintain the plane and depth of exploration. This manoeuvre is then repeated until the abnormal tissue is reached, the surgical assistant maintaining as large a space as possible with the use of a pair of malleable retractors. Meticulous haemostasis is essential, as it can otherwise be almost impossible to recognise subtly abnormal orbital tissues - such as oedematous or infiltrated orbital fat.

When the abnormal tissue is located, which can be very difficult, then a relatively large biopsy should be taken using a number 11 blade; the tissue should preferably be gripped once only, to avoid crush artefact, with a single larger piece being more diagnostic than small fragments.

Bipolar cautery should be used to establish complete haemostasis and the orbicularis and skin closed with a running 6/0 nylon suture; if the biopsy site is post-equatorial, then a drain (corrugated or vacuum) should be placed.The drain is generally removed on the day after biopsy and the skin/muscle suture removed at seven to ten days.

Severe acute dacryoadenitis may be accompanied by a marked secondary keratitis and, if bacterial, may rarely form an abscess alongside the gland. Chronic dacryoadenitis typically results in loss of glandular tissue and secondary fibrosis, with a sicca syndrome in occasional cases.

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