4 Lacrimal Apparatus

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A. Congenital Anomalies of the Lacrimal

Gland

Complete absence of the lacrimal gland (aplasia), displacement (ectopia), lacrimal gland cysts (dacryops, A), or lacrimal gland fistulas are rare.

B. Dry Eye: Sicca Syndrome

Complex qualitative and quantitative disorders of the production and surface adhesion of the tear film.

Etiology/pathogenesis. Multifactorial, caused by ophthalmological and general diseases and by exogenous factors (B, Table 1).

Epidemiology. Very common. Symptoms in ca. 22% of women and 10% of men between 55 and 60 years. Signs of keratoconjunctivitis sicca in 20% of women and 15% of men between 45 and 54 years.

Clinical features. Dryness, sensation of pressure, pain, burning, scratching, foreign-body sensation, photophobia, lid swelling, corneal edema, corneal epithelial filaments, conjunctival folds parallel to the lid margins (LIPCOF), tear meniscus irregular and/or reduced <0.2 mm.

Diagnosis. The diagnosis is made clinically and by means of tests, including the Schirmer test <10mm/5min, Jones basal secretion test <10mm/5min, break-up time <10 s.

Differential diagnosis. Infection, asthenopia.

Treatment. Tear substitute, moisture chamber, glasses with side protection, punctum plug.

Treatment of the underlying disease. Reduction in exogenous factors (smoke, dust, night work, etc.).

Prognosis. Rarely major symptoms. Increased bacterial superinfection.

C. Acute Dacryoadenitis

Sudden unilateral tender swelling of the lacrimal gland.

Etiology/pathogenesis. See C, Table 2.

Epidemiology. Usually middle-aged women. Clinical features. Erythema, swelling, tenderness of the lateral third of the upper lid (S- shaped curve of the upper lid, C), conjunctival chemosis, palpable preauricular lymph nodes, fever, general malaise, leukocytosis. Diagnosis. The diagnosis is made clinically.

Differential diagnosis. Lid/orbital cellulitis, hordeolum, lid abscess, sinusitis, erysipelas.

Treatment. Antibiotic therapy. Incision and drainage.

Prognosis. The disease lasts 8–10 days.

D. Chronic Dacryoadenitis

Painless, slightly inflammatory swelling of the lacrimal gland, unilateral or bilateral.

Etiology/pathogenesis. Sequela of acute dacryoadenitis, granulomatous diseases (D, Table 3).

Epidemiology. Usually men over 40 years. Clinical features. Painless swelling of the lateral upper lid.

Diagnosis. The diagnosis is made clinically. Further investigations depending on the underlying disease. Biopsy.

Differential diagnosis. Tumor, dermoid, osteomyelitis, systemic diseases.

Treatment. Treatment of the underlying disease.

Prognosis. Depending on the underlying disease.

E. Pleomorphic Adenoma

(Benign Mixed-Cell Tumor)

Etiology/pathogenesis. Unknown. Benign tumor of epithelial and myoepithelial cells. Epidemiology. Commonest epithelial tumor of the lacrimal gland (50%). Age peak at 4th–5th decade. The ratio of men to women is 1.5:1 to 2:1.

Clinical features. Painless, slowly growing tumor. Visual changes and double vision are not reported. Displacement of the eyeball downward and nasally.

Diagnosis. CT shows a sharply demarcated tumor. Bone atrophy possible. On MRI the tumor appears hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging. Eyeball deformation is possible.

Differential diagnosis. Adenoid cystic carcinoma.

Treatment. Complete excision including the pseudocapsule. Incisional biopsy leads to a high rate of recurrence.

Prognosis. Five-year survival rate of 99%. Recurrences possible after incomplete excision, malignant transformation also possible.

A. Congenital Abnormalities of the

Lacrimal Gland

Lacrimal gland cyst

C. Acute Dacryoadenitis

S-shaped curve of the upper lid

Table 2 Etiology/pathogenesis of acute dacryoadenitis

D. Chronic Dacryocystitis

Table 3 Pathogenesis/pathogenesis of chronic dacryoadenitis

•Unhealed acute dacryoadenitis

•Chronic conjunctivitis

•Tuberculosis

•Syphilis

•Leprosy

•Sarcoidosis

•Actinomycosis

•Nocardiosis

•Trachoma

B. Sicca Syndrome

Table 1 Causes of sicca syndrome

•General diseases

–Diabetes mellitus

–Thyroid diseases: hypothyroidism, endocrine ophthalmopathy, Hashimoto thyroiditis

–Sjögren syndrome, rheumatoid arthritis, SLE (systemic lupus erythematosus), Wegener granulomatosis, sclerodema

–Skin diseases: neurodermatitis, acne rosacea, allergies

–Pregnancy

•External influences

–Smoke, dry heating, air conditioning

–Night work

–Contact lenses

–Cosmetics

•Medications

–Analgesics

–Antiarrhythmics

–Antihistamines

–Antihypertensives

–Hormones

–Lipid-lowering drugs

–Psychotropic medications

–Synthetic retinoids

–Virostatic drugs

–Cytostatic drugs

•Diseases of the eye

–Systemic, infectious, tumor, or surgical damage to the lacrimal gland

–Conditions of the conjunctiva: trachoma, pemphigoid, allergies, operations, Stevens–Johnson syndrome, burns, corrosive injury, radiation

–Conditions of the cornea: dystrophies, previous keratoplasty

–Lid deformities, lid closure deficit, blepharitis

E. Pleomorphic Adenoma

Diseases of the Lacrimal Gland

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4 Lacrimal Apparatus

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A. Adenoid Cystic Carcinoma

Etiology/pathogenesis. Not known.

Epidemiology. Commonest malignant, second commonest epithelial lacrimal gland tumor (25–30%). Age peak in women about 40 years. Clinical features. Rapidly growing. Pain with tumor infiltration of nerves or bone.

Diagnosis. On CT, roundish poorly demarcated space-occupying lesion with irregular surface and bone destruction. MRI shows cystic isointense or hyperintense changes in the T1weighted image.

Differential diagnosis. See A, Table 1.

Treatment. Radical resection. Radiotherapy. Prognosis. Five-year survival rate of 21%. Recurrences and metastasis to the lungs. The prognosis depends on the histological grade.

B. Diseases of the Lacrimal Ducts

The hallmark of all diseases of the lacrimal ducts is epiphora. Epiphora immediately postnatally is due to atresia or stenosis of the lacrimal ducts, especially of Hasner’s valve. Signs of inflammation point to infection, which can be acute or chronic and appear as canaliculitis or dacryocystitis. Tumors are a rarer cause of epiphora. Signs suggestive of malignancy are:

●Development of mass above the medial palpebral ligament

●Telangiectasia overlying lacrimal sac swelling

●Bloody secretion, nosebleed, bloody reflux after irrigation of the lacrimal duct

C. Acute Dacryocystitis

Etiology/pathogenesis. Partial or complete obstruction of the nasolacrimal duct with inflammation due to infection, tumor, foreign bodies, after trauma or due to granulomatous diseases.

Epidemiology. Adults between 50 and 60 years. Clinical features. Epiphora; acute, unilateral, painful inflammation of the lacrimal sac; pus emerging from the lacrimal punctum; fever; general malaise. Pain radiates to the forehead and teeth.

Diagnosis. Diagnosis is made clinically. Swab and culture with antibiotic sensitivity.

Differential diagnosis. Orbital cellulitis, inflammation of the paranasal sinuses, erysipelas. Treatment. Conservative: see C, Table 2. For decompression, freeze the skin (with ethyl chlo-

ride), then make the incision. Dacryocystorhinostomy is often necessary later.

Prognosis. Spontaneous discharge of the pus into the ethmoid cells, conjunctival sac, and nose is possible. This can lead to chemosis, lid edema, and erysipelas, more rarely to orbital cellulitis.

D. Chronic Dacryocystitis

Etiology/pathogenesis. Congenital or idiopathic obstructions of the nasolacrimal duct, e.g., due to unhealed acute dacryocystitis, dacryoliths, foreign bodies, tumors, diseases of the surrounding structures (sinusitis, tumors), trauma.

Epidemiology. See acute dacryocystitis. Clinical features. Epiphora; signs of inflammation are absent; large amounts of mucopurulent secretion drain on pressure over the lacrimal sac.

Diagnosis. Swab and culture, lacrimal duct probing.

Differential diagnosis. None.

Treatment. See C, Table 2. Dacryocystorhinostomy.

E. Trauma of the Lacrimal Ducts

These injuries often occur together with lid and facial injuries, particularly with injuries of the nasal canthus. The canaliculi are affected in 70%, more rarely the lacrimal sac (20%) and the nasolacrimal duct (10%). The defect and the canaliculus can be located by thorough inspection under the operating microscope and irrigation with methylene blue or fluorescein. If fractures or foreign bodies are suspected, radiography of the orbit in two planes or CT is indicated. Therapeutically, the earliest possible reconstruction with silastic intubation of the lacrimal ducts should be attempted. The prognosis depends on the extent of the injury. Lid deformities and stenosis of the lacrimal duct system are possible.

A. Adenoid Cystic Carcinoma

Table 1 Differential diagnosis of tumors in the upper temporal quadrant of the orbit

•Benign

–Dacryops

–All forms of dacryoadenitis

–Pleomorphic adenoma

–Dermoid cyst

–Benign lymphoid lacrimal gland tumor

–Eosinophilic granuloma

–Aneurysmal bone cyst

–Cholesterol granuloma

•Malignant

–Adenoid cystic carcinoma

–Pleomorphic adenocarcinoma

–Mucoepidermoid carcinoma

–Squamous epithelial carcinoma

–Oncocytoma

–Malignant lymphoma

–Metastases

C. Acute Dacryocystitis

Table 2 Treatment of acute canaliculitis/dacryocystitis

•Bacterial

–Local: gentamicin eye drops 5 times daily and gentamicin eye ointment at night for 14 days; disinfectant dressings with Rivanol solution 1:1000; xylometazoline eye drops t.i.d. to reduce swelling

–Systemic: e.g., dicloxacillin p.o. For 10–14 days

–Actinomycetes: tetracycline eye ointment t.i.d.

•Chlamydia

–Local: tetracycline, ofloxacin, erythromycin eye ointment t.i.d. for 6 weeks

–Systemic: tetracycline, erythromycin, doxycycline, or sulfamethoxazole p.o. for 3 weeks

•Fungi

–E.g., natamycin eye ointment 1–2 hourly

•Viruses

–Varicella zoster: aciclovir 800 mg 5 tabs daily for 7 days, aciclovir eye ointment 5 times daily continued for 3 days after healing

B. Diseases of the Lacrimal Ducts

Occlusion of the superior and inferior lacrimal canaliculus

Occlusion of the common canaliculus

Occlusion of the nasolacrimal duct

Stenosis of the lacrimal ducts

D. Chronic Dacryocystitis

Lacrimal duct stenosis due to papilloma

E. Trauma of the Lacrimal Ducts

Avulsion of lacrimal duct

Acute dacryocystitis with persistent Hasner membrane

Lacrimal Gland Tumors, Lacrimal Ducts

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