Chapter 6

Core Messages

■Differential diagnostic symptoms and signs in favor of a tumor of the lacrimal sac include a swelling above the medial canthal tendon, the presence of telangiectases in the skin overlying the mass, and the presence of serosanguinous discharge or a bloody reflux with atraumatic irrigation.

■All patients should be asked for a history of predisposing conditions, such as systemic diseases (e.g., lymphoma, Wegener granulomatosis, sarcoidosis) or neoplasms.

■External dacryocystorhinostomy (DCR) allows detailed inspection of the lacrimal sac and adequate tissue biopsy.

■Lacrimal sac biopsy should be considered selectively in patients with atypical clinical or intraoperative findings or in those with a history of predisposing systemic diseases.

■Biopsy results may help to define the degree of active specific inflammation requiring further chemoor immunotherapy postoperatively.

■Selective lacrimal sac biopsy permits early diagnosis of potentially life-threatening malignant tumors to determine further definitive management.

Table 6.2. Lacrimal sac tumors

I.Epithelial tumors

1.Squamous cell papilloma

2.Transitional cell papilloma

3.Mixed-cell papilloma (exophytic or endophytic)

4.Oncocytic adenoma (oncocytoma)

5.Squamous cell carcinoma

6.Transitional cell carcinoma

7.Adenocarcinoma

8.Mucoepidermoid carcinoma

9.Oncocytic adenocarcinoma

II.Nonepithelial tumors

1.Fibrous histiocytoma

2.Pyogenic granuloma

3.Neurilemmoma

4.Lymphoid tumors

5.Malignant melanoma

6.Angiosarcoma

Source: Modified from [6]

The recognition and proper management of such lifethreatening lesions require an understanding of the anatomy and general diagnostic techniques of the lacrimal drainage system.

6.2Surgical Anatomy of the Lacrimal Drainage System

On lid closure, tears are wiped to the nasal bulbar conjunctiva and tear meniscus and are then drained through the superior and inferior lacrimal puncta, which are open only with open eyes, and canaliculi into the lacrimal sac and by a sort of “lacrimal peristalsis” into the nose (Fig. 6.1). The canaliculi start with a 2-mm vertical component and continue with a horizontal portion 8–10 mm long. The common canaliculus, 1–2 mm long, leads into the lacrimal sac. Its entry into the sac at the internal ostium is often partially covered by a mucosal flap, which is based anteriorly and also called “the valve of

Fig. 6.1 Schematic illustration of the lacrimal drainage system with approximate measurements (Redrawn from [8])

Rosenmüller.” The lacrimal sac lies in the fossa between the anterior (frontal process of maxilla) and posterior (lacrimal bone) lacrimal crest and is surrounded by the anterior and posterior limbs of the medial canthal tendon. The body of the sac measures 10–12 mm in vertical height, and 3–5 mm of the sac (fundus) lie above the internal ostium. The suture line in the lacrimal fossa runs vertically between the thin lacrimal bone and the thicker frontal process of the maxilla. It is mostly located one half of the way from the anterior to the posterior lacrimal crest. The sac leads into the bony nasolacrimal duct, which measures 12–15 mm in length and travels within the wall of the maxillary sinus and the lateral nasal wall. The duct extends for about 5 mm below the bony portion and opens beneath the inferior turbinate in the lateral wall of the nose. A mucosal valve (Hasner) usually prevents retrograde passage of mucus or air upward. The nasal entry site of a DCR lies at the anterior tip of the middle turbinate. The ethmoid sinus may extend to the lacrimal sac fossa. Bony removal of the lacrimal sac fossa may result in entry into the ethmoid sinus rather than into the nasal vault [8, 20].

6.3Basic Diagnostics for Disorders of the Lacrimal Drainage System

Prior to clinical examination, it is helpful to ask the patient for severity, duration, and quality of symptoms. The most common symptoms indicating dysfunction of the lacrimal drainage system include epiphora, punctal discharge, and medial canthal swelling. Epiphora is

typically worse in the winter months and windy weather. The eye can be sticky due to an expressible mucocele or collected dried tears. The vision can be blurred secondary to an elevated tear meniscus (prismatic effect, especially on downgaze, for example, when reading) or tear-splat- tered glasses. Chronic epiphora can induce red, sore lower lid skin, with secondary anterior lamella (vertical) shortening (mild cicatricial ectropion). Excessive wiping away of tears can cause or exacerbate a medial ectropion.

Mucopurulent punctal discharge suggests stasis in the lacrimal sac or canaliculi, mostly secondary to nasolacrimal duct obstruction. Accumulation of inflammatory debris can result in dacryolithiasis in up to 15% of DCR surgeries. Lacrimal sac stones consist of dried mucus, lipid, and inflammatory cells and are more likely to be found in chronically inflamed sacs.

Medial canthal swelling may be caused by an abscess, a dacryolith, or a tumor in the lacrimal sac. But, not all masses in the medial canthal area arise from the lacrimal sac (acute skin infection, acute ethmoiditis, ruptured dermoid cyst). Swellings below the medial canthal tendon are typical of dacryocystitis. Differential diagnostic signs in favor of a tumor of the lacrimal sac include a mass above the medial canthal ligament (absent in dacryocystitis), the presence of telangiectases in the skin overlying the mass (instead of the diffuse erythema of dacryocystitis) and the presence of serosanguinous discharge or a bloody reflux with atraumatic irrigation (both of which are not usually observed in dacryocystitis).

All patients should be asked not only for their complaints, but also for the history of predisposing conditions, such as systemic diseases (e.g., lymphoma, Wegener granulomatosis, sarcoidosis), trauma, neoplasms, and dacryocystitis.

In addition to a comprehensive ophthalmic examination, particularly with regard to ocular surface disease and eyelid and punctum position, the assessment of the lacrimal drainage system must include inspection, palpation, digital expression of lacrimal sac contents, and standard irrigation and probing of the nasolacrimal system. Imaging studies (dacryocystography, computed tomography (CT), magnetic resonance imaging) are reserved for selected patients with atypical symptoms and signs. Detailed history taking and nasal endoscopy must be performed by an otorhinolaryngologist to rule out intranasal pathology [8].

6.4Selective Lacrimal Sac Biopsy in External Dacryocystorhinostomy

Although endonasal endoscopic DCR is gaining clinical popularity in the therapy of acquired dacryostenosis, the external DCR is regarded as the gold standard in terms of

Fig. 6.2 External DCR allows an excellent possibility for inspection of the lacrimal sac and for taking biopsy samples of abnormal-appearing findings

Table 6.3. Results of selective lacrimal sac biopsies