Clinical Features

Orbital cavernous hemangioma can occupy an intra-conal or extraconal position in the orbit (Figures 14.1 and 14.2). When the cavernous hemangioma is located intraconally, it leads to a slowly progressive axial prop-tosis (Figure 14.1). When it is located extraconally in

TABLE 14.1. Frequency of Various Vascular Tumors of the Orbit.

No. of Cavernous

Capillary

Author

cases hemangioma

hemangioma

Lymphangioma

Varices

Hemangiopericytoma

Shields et al.1

645 20 (3.1%)

7 (1.1%)

4 (0.6%)

2 (0.3%)

5 (0.8%)

Sen5

266 16 (6.0%)

5 (1.9%)

4 (1.5%)

1 (0.3%)

Gunalp and Gunduz3

1092 35 (3.2%)

15 (1.3%)

3 (0.3%)

3 (0.3%)

1 (0.1%)

Henderson et al.2

1376 60 (4.4%)

30 (2.2%)

18 (1.3%)

4 (0.3%)

16 (1.2%)

Seregard and Sahlin4

300 27 (9%)

2 (0.7%)

2 (0.7%)

4 (1.3%)

1 (0.3%)

Vascular

Arteriovenous

Author

leiomyoma Angiosarcoma

fistula

Aneurysm

Hamartoma

Shields et al.1

2 (0.3%)

_

_

Sen5

_

Gunalp and Gunduz3

3 (0.3%)

3 (0.3%)

_

Henderson et al.2

1 (0.1%)

5 (0.4%)

2 (0.1%)

1 (0.1%)

Seregard and Sahlin4

_

_

the orbit, the displacement of the globe is opposite the position of the tumor. Rarely, a cavernous hemangioma presents as a lacrimal gland mass9 or as an intraosseous tumor.10,11

A palpable mass is rarely present. There are usually no significant inflammatory signs such as eyelid edema or conjunctival injection. Visual acuity is usu ally good unless the cavernous hemangioma compresses the optic nerve. When the tumor is located in the vicinity of the globe, it may induce hyperopia and choroidal folds. It is interesting that the hyperopia and choroidal folds may persist even after complete removal of the cavernous hemangioma (Figure 14.1).12 Ocular motility may be slightly limited. Amaurosis

FIGURE 14.1. (A-C) Typical cavernous hemangioma presentation with slowly progressive unilateral proptosis due to an intraconal lesion. (B,C) Axial Tl-weighted MR images with and without contrast reveal a well-encapsulated, oval lesion within the cone. (D) Histopathologically, the specimen consists of multiple cavernous spaces separated by fibrous septae; most of the caverns are filled with blood. (E) The gross specimen after surgical excision shows a reddish blue encapsulated lesion. (F) Fundus photograph shows the choroidal folds (arrow) secondary to the compression of the lesion.

fugax, in extreme positions of gaze, is probably related to ischemia of the optic nerve as it is compressed by the retrobulbar mass.13 The rare occurrence of cavernous hemangioma in a patient with hyperthy-roidism has been described.14

Ophthalmoscopic findings include choroidal folds, optic disk edema, and optic atrophy.15 The development of optic atrophy is related to the presence of a long-standing tumor. These tumors have been misdi-agnosed in the past as optic neuropathy. Such errors are rare now, since the advent of neuroimaging studies.

Cavernous hemangioma is typically a solitary unilateral tumor. However, rarely bilateral and multiple hemangiomas have been reported in the same patient (Figure 14.2).16 Multiple orbital cavernous hemangi-omas may occur as a sporadic lesion or in association with Maffucci syndrome (multiple enchondromas, soft tissue hemangiomas, and a generalized tendency to neoplasia) and blue rubber bleb nevus syndrome (bluish cutaneous and mucosal hemangiomas, soft tissue hemangiomas, enteric hemangiomas with gastrointestinal bleeding) (see Chapter 16).17

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