Capillary hemangioma (benign hemangioendothelioma; strawberry hemangioma) is an important cutaneous vascular tumor of childhood. In the ocular region, it usually begins on the eyelid (see section on eyelid tumors), but it can occasionally occur in the orbit (1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20). About 7% of ocular adnexal capillary hemangiomas arise in the orbit posterior to the orbital septum (12). In the authors’ series, orbital capillary hemangioma accounted for 1% of all biopsied orbital lesions in all age groups (2) and for 4% of all orbital biopsies in children (3). In the more recent clinical review of 1,264 cases, orbital capillary hemangioma accounted for 4% of all orbital masses (4). In the authors’ series of 1,264 consecutive space-occupying orbital lesions, the 36 capillary hemangiomas accounted for 17% of the 213 vascular lesions and for 3% of all lesions (4).

When it occurs in the orbit, capillary hemangioma is usually apparent shortly after birth as a soft mass deep to the eyelid. It is more pronounced when the child strains or cries. Occasionally, it occurs in the deeper portions of the orbit without eyelid signs. Associated hemangioma on the periocular skin facilitates making the orbital diagnosis. The main complications are amblyopia and strabismus. Lesions .1 cm in diameter are more likely to cause these complications, whereas lesions ,1 cm in diameter are considerably less likely to do so (18). The lesion usually shows enlargement for 1 to 2 years and then undergoes gradual regression, similar to eyelid capillary hemangioma. Orbital capillary hemangioma may be associated with large visceral hemangiomas, which can cause platelet entrapment and thrombocytopenia, a condition called the Kasabach-Merritt syndrome (1).

Orbital capillary hemangioma is rare in adults. However, an orbital tumor with similar histopathologic features has been reported to arise in the medial rectus muscle of a 73-year-old woman (14).

With computed tomography (CT) and magnetic resonance imaging (MRI), orbital capillary hemangioma varies from case to case. It can be a round or ovoid circumscribed mass or an irregular or diffuse mass. It shows marked homogeneous enhancement with contrast agents. It is important that rhabdomyosarcoma can have similar features and should be included in the differential diagnosis. However, rhabdomyosarcoma rarely is present in infancy but generally occurs in children aged between 5 and 15 years.

Histopathologically, orbital capillary hemangioma is composed of lobules of proliferating small vascular channels separated by thin fibrous septa. The pathogenesis of capillary hemangioma has been largely unknown. However, it has been recognized that placenta and capillary hemangiomas have unique immunohistochemical similarities. This has led to speculation that infantile hemangiomas could be of placental origin. Two theories have been proposed to explain this hemangioma- placental connection. First, angioblasts may proliferate toward placental tissue in the site where the hemangioma develops. A second intriguing theory is that cells of placental origin could embolize to the target areas (15,16).

Initial management of orbital capillary hemangioma should include refraction and occlusive patching treatment for associated amblyopia. If there is no threat to vision, the lesion can be cautiously observed. Oral or local injection of cortico steroids can hasten regression of the lesion. Ultrasound guidance has been used to accurately place the needle in the tumor (10). However, corticosteroids have a number of potential complications, including retinal artery obstruction, linear perilymphatic subcutaneous fat atrophy, eyelid depigmentation, eyelid necrosis, and adrenal gland suppression (7,18,19). Interferon-a-2b has also been reported to be effective in hastening tumor regression (9). Surgical excision is an appropriate treatment for select well-circumscribed tumors (8,13).

Orbital capillary hemangioma can occur in the deep orbit and produce proptosis or displacement of the globe or it can occur in the anterior portion of the orbit and present as a reddish-blue, fluctuant, subcutaneous eyelid mass. The more common cutaneous eyelid capillary hemangioma is discussed in this text.

Orbital capillary hemangioma can have a variety of clinical manifestations. A common feature of infantile capillary hemangiomas, however, is their tendency to grow rapidly in infancy and then to stabilize and gradually regress.

In some instances, a capillary hemangioma of the eyelid can be associated with orbital capillary hemangioma and extraocular cutaneous hemangiomas.

In a very young infant, surgical excision may be the best treatment of a large, anterior, well-circumscribed orbital capillary hemangioma producing significant ocular signs and symptoms and that is destined to show further growth and visual loss. A clinicopathologic correlation is shown.

Aldave AJ, Shields CL, Shields JA. Surgical excision of selected amblyogenic periorbital capillary hemangiomas. Ophthalmic Surg Lasers 1999;30:754-757.

Cavernous hemangioma is the most common vascular tumor of the orbit (1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31). In the authors’ series of 1,264 orbital masses, the 77 cavernous hemangiomas accounted for 36% of vascular tumors and for 6% of all tumors in the series (3). In a series from Turkey, 41% of orbital vascular tumors were cavernous hemangiomas (16). In another report by the authors, it accounted for 8% of orbital tumors in patients $60 years of age (22).

Orbital cavernous hemangioma is a benign tumor that tends to occur in adulthood as a relatively stationary or slowly progressive lesion that can produce painless proptosis. Because it most often occurs in the muscle cone, it usually produces axial proptosis. It does not generally produce inflammatory signs. Rarely, a cavernous hemangioma can present as an intraosseous lesion in the orbital bones (6,8,23). Orbital cavernous hemangioma is rarely bilateral (9). Although cavernous hemangioma is generally a solitary lesion, it can sometimes be multiple, particularly when seen in the blue rubber bleb nevus syndrome (13,21). In rare instances, multiple confluent cavernous hemangiomas have involved the orbit and brain simultaneously (26).

Modern techniques of neuroimaging have been instrumental in making a more accurate diagnosis of orbital cavernous hemangioma (25). Since the advent of good imaging modalities like computed tomography and magnetic resonance imaging, this tumor is often being discovered more often as a coincidental finding when it is small and asymptomatic. As it slowly enlarges, it can produce marked proptosis, compression of the optic nerve, and choroidal folds. Imaging studies demonstrate a well-circumscribed round to ovoid mass, usually in the muscle cone. It shows mild enhancement with contrast agents. The differential diagnosis of a well-circumscribed, round to ovoid, solid orbital mass also includes peripheral nerve sheath tumors (schwannoma and neurofibroma), hemangiopericytoma, fibrous histiocytoma, solitary fibrous tumor, and melanoma. It has been suggested that orbital cavernous hemangioma can be differentiated from schwannoma by contrast-enhancement spread pattern methods on dynamic MRI (27).

Histopathologically, orbital cavernous hemangioma is composed of dilated, congested vascular channels separated by connective tissue that contains smooth muscle. The pattern is generally quite typical but on occasion lymphangioma can have similar histopathologic features.

Management of orbital cavernous hemangioma ranges from periodic observation for small, asymptomatic lesions to surgical excision for larger, symptomatic tumors. The surgical approach is determined by the size and location of the tumor. A conjunctival or cutaneous approach may be used for anterior lesions. We have had considerable success removing cavernous hemangiomas that are located in the anterior half of the orbit by a transconjunctival approach. Deep orbital tumors may require a lateral orbitotomy, sometimes with an osteotomy (Kronlein approach). Complete excision is important and meticulous surgery is necessary to avoid incomplete excision and recurrence. Techniques of transcranial approach are used for lesions located at the orbital apex (11).

With the advent of computed tomography and magnetic resonance imaging, some orbital cavernous hemangiomas are discovered at an early stage, when they are asymptomatic. Such lesions can usually be followed without treatment because they often remain asymptomatic for many years. The patient can be examined once or twice a year with pupil examination, visual acuity, color vision, exophthalmometry, ophthalmoscopy, visual fields, and CT or MRI studies. If the patient begins to develop findings that could cause visual loss, the surgical removal of the tumor is generally appropriate. Like other orbital tumors, they can produce fundus changes like choroidal folds, optic disc edema, and compression of the globe.

In cases of suspected orbital cavernous hemangioma, one must determine the best approach to surgical removal. If a portion of the tumor is located fairly anteriorly, a conjunctiva approach, rather than cutaneous approach, can be employed in selected cases. An example is shown of a relatively large cavernous hemangioma that was removed by a conjunctival approach. In such instances, a cryoprobe can facilitate removal of the tumor.

Depicted is a clinicopathologic correlation of a cavernous hemangioma with globe compression and optic discedema in which the tumor was removed through a horizontal forniceal conjunctival incision.

Larger tumors in the muscle cone or tumors located laterally in the posterior aspect of the orbit can be removed by a superolateral orbitotomy with a skin incision and extraperiosteal approach. An osteotomy (Kronlein approach) is not usually necessary.

In cases where the tumor is nasal to the optic nerve, a superonasal orbitotomy can be employed. Although a conjunctival incision could have been used in the case shown, the skin-extraperiosteal approach offers better surgical exposure. This patient was seen several years ago; today, we may have used a conjunctival approach in such case.