Cavernous Hemangioma of the orbit
A cavernous hemangioma is the most common vascular lesion (Most common vascular tumor) in the orbital region, seen in adults. These tumors are slow growing and benign. Usually, they present as asymptomatic or painless lesions / Orbital mass, with associated proptosis or exopthalmos (forward displacement of eye – Bulging eyes). These lesions are usually unilateral. Very rarely, bilateral cases have been reported. It is a non-infiltrative vascular neoplasm surrounded by well-delineated fibrous capsule.
Most prevalent neoplasm seen in adults, especially those in middle age (30-50 years of age).
Female preponderance is also seen in this type of lesions.
Orbital cavernous hemangioma accounts for approximately 5-7% of all the orbital tumors.
Cavernous hemangioma is the most common benign neoplasm of the orbit. It is not a neoplasm in the general sense, because it is not derived from a single, proliferating cell. Cavernous hemangiomas are composed of a network of vascular channels, which are separated by fibrous tissue stroma. Growth of the tumor is secondary to budding of vascular channels into surrounding tissue.
No evidence of established risk factors for cavernous hemangioma. Nevertheless, Pregnancy has been said to be linked with increased growth of pre-existing cavernous hemangiomas.
It classically presents as a slow progressing or growing orbital mass, causing forward displacement of the eye ( ). Visual field defects as a result of compression of Optic nerve and Diplopia (double vision) may also take place.
Most prevalent signs include:
-axial proptosis (exopthalmos)
-optic nerve swelling
It rarely produces any inflammatory signs.
Patients usually come up with complaints such as an asymptomatic, progressive proptosis. There is involvement of extraocular muscles, optic nerve and globe as the tumor advances in size, leading to double vision (diplopia) or decreased vision.
The diagnosis can be established with the help of history and clinical examination. But it is confirmed with orbital imaging (CT scan). Indications like increased intra-ocular pressure, papillary abnormalities, optic nerve swelling and choroidal folds should point towards suspecting a possible mass in the orbital region.
Orbital imaging with CT (computed tomography) and MRI (magnetic resonance imaging) point towards the diagnosis of cavernous hemangioma.
A-scan and B-scan ultrasound scan are also found to be beneficial. B-scan ultrasound shows a smooth, round to oval lesion. A-scan reveals a regular internal structure, with high internal reflectivity.
CT scan exhibits cavernous hemangioma as a well-circumscribed, homogeneous mass which is slightly thicker to muscle located in the intra-conal region (within the eye muscles). Calcification may be found in long-standing tumors.
- Solitary fibrous tumor
- Fibrous histiocytoma
- Peripheral nerve sheath tumors ( Schwannoma and Neurofibroma)
Management of cavernous hemangioma depends on the presence or absence of symptoms. For asymptomatic small lesions, periodic observation with papillary examination once or twice in the year, visual acuity, color vision, exophthalmometry, dilated fundoscopy, visual fields and CT or MRI studies is indiacted. Surgical removel of the neoplasm or excision is the treatment of choice for larger long-standing lesions. Currently, there is no role of Radiotherapy.
The treatment is done only via surgical removal (surgery) of the neoplasm. No medical therapies are available currently for treating cavernous hemangiomas.
The location and the size of the lesion is helpful in deciding the approach to surgical removal of a cavernous hemangioma (orbitotomy or orbital surgery).
Anterior Orbitotomy using Transconjunctival or Transcaruncular approach – This approach is used when there is involvement of anterior two-thirds of the orbit.
– This approach is useful in cases where the lesions are located more posteriorly.
Transcranial approach Orbitotomy – This type of approach, through the skull and the brain, is useful in case of lesions involving the apex of orbit.
For removal of the tumor with minimal blood loss, a cryoprobe may be useful.
Injury to central retinal artery may lead to visual loss which is considered to be a complication of surgery. As the tumor is encapsulated, blood loss is usually minimal. As is the case with any orbital surgery (Orbitotomy), there is always a risk of injury to optic nerve, extraocular muscles and the surrounding adnexal structures. Surgery must be performed by the the , adequately trained in .
Visual prognosis is excellent, with complete surgical removal of the tumor. In case of incomplete excision of the tumor, chances of recurrence are more. Hyperopia (farsightedness) may persist even after surgically removing the tumor as a result of the long-standing mass effect (pressure effect) against the posterior globe (posterior portion of eye).
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