Ординатура / Офтальмология / Английские материалы / Tumors of the Eye and Ocular Adnexa_Char_2001

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The intraconal space (the volume surrounded by the extraocular muscles) is a common site of orbital neoplasms. Cavernous hemangioma is the most common adult intraconal tumor. Less frequently, other benign mesenchymal tumors, primary optic nerve neoplasms, metastases, benign lymphoid proliferations, and many less uncommon peripheral nerve and fibrous lesions can involve this area of the orbit, as can some rarer primary malignancies.

ORBITAL CAVERNOUS HEMANGIOMA

Cavernous hemangioma accounts for between 3 and 7 percent of orbital mass lesions and occurs most often in middle-aged women.1–3 Proptosis is the most common presenting sign (Figure 17–1); however, diminished vision, an afferent pupil defect, visual field defects, lid swelling, a palpable mass, and diplopia can occur. The proptosis is usually axial, but the clinical appearance is not diagnostic.

Few large clinical series have been reported. Ruchman and Flanagan reported 12 cases, Wright 10, and Harris and Jakobiec reviewed 66 cases, which had been managed by Reese and others over a 60-year

period at Columbia.4–6 In the latter series, 70 percent of patients were females, and the mean age was 42 years.6 McNab reported on the London experience of 85 patients managed between 1968 and 1988. In that report, cavernous hemangiomas accounted for approximately 7 percent of orbital cases.7

The author has managed about 35 orbital cavernous hemangiomas. Most patients were female; the mean patient age was 52 years (range 15 to 78 years). The interval between symptoms and diagnosis averaged 3.8 years with a range of 2 months to 10 years. All but one patient had a unilateral orbital lesion, and one had bilateral cavernous hemangiomas.

Usually, these tumors occur in the intraconal space; in approximately 20 percent, they are either both intraand extraconal or entirely extraconal in location (Figure 17–2). Less commonly, this tumor involves the orbital apex, the optic nerve canal, or

both and may present with profound loss of vision (Figure 17–3). Extraconal tumors are discussed in the next few chapters.

Visual acuity and color vision were diminished in more than one-half of the cases I have operated on. In a few patients, the initial symptoms were loss of peripheral visual field. Several patients had limitations of extraocular muscle movement.

Fundus abnormalities consisting of choroidal folds and/or disc edema occur in about one-half of patients with large tumors. Figure 17–4 demonstrates a typical pattern of horizontal choroidal folds. Figure 17–5 shows marked disc edema associated with a hemangioma compressing the optic nerve; neither finding is diagnostic for hemangioma. Almost all

patients have some degree of proptosis ranging from 0.5 to 8 mm; in a minority of patients, it was ≥ 6 mm. Occasionally, the location of the tumor relative to the optic nerve can result in decreased vision on medial or lateral gaze (Figure 17–6).

All cavernous hemangiomas had a number of common characteristics identifiable on computed tomography (CT) or magnetic resonance imaging (MRI) scans. Lesions are rounded, homogeneous, encapsulated, and usually do not involve the orbital apex (Figure 17–7).8,9 Most commonly they involve the temporal aspect of the intraconal space. One of our patients had bilateral cavernous hemangiomas (Figure 17–8).10 Others have reported bilateral tumors in association with Maffucci’s syndrome.11