BASICS

DESCRIPTION

• Cavernous hemangioma of the orbit is a benign proliferation of vascular channels which induces progressive ectasia.

– This may also occur in the central nervous system, liver, and thyroid

• It is also known as cavernoma

EPIDEMIOLOGY

Incidence

• Estimated 4–12% of orbital tumors – the most common orbital tumor in adults

• Rarely occurs in infants or children

• Female > male

PATHOPHYSIOLOGY

Although histologically benign, damage is through compression of the optic nerve, extraocular muscles, and the globe itself.

ETIOLOGY

Hamartomatous benign vascular growth

DIAGNOSIS

HISTORY

• Painless, progressive proptosis

• Hyperopic shift in refraction

• Pressure sensation

• Diplopia

• If advanced, vision loss due to optic neuropathy

• Often discovered on imaging for other reasons, i.e., headache

PHYSICAL EXAM

• Hertel exophthalmometry for baseline and comparison

• Resistance to retropulsion

• Dilated episcleral vessels

• Choroidal folds on funduscopic exam due to compression of globe

• Evaluate for compressive optic neuropathy:

– Relative afferent pupillary defect

– Vision loss

– Color testing

– Visual field testing

– Optic nerve edema or atrophy

DIAGNOSTIC TESTS & INTERPRETATION

Diagnostic Procedures/Other

Imaging of orbits – CT/MRI

DIFFERENTIAL DIAGNOSIS

• Thyroid eye disease

• Orbital tumor – schwannoma, hemangiopericytoma, solitary fibrous tumors

• Carotid–cavernous fistula

• Optic nerve meningioma or glioma

TREATMENT

• Surgical resection (orbitotomy) if compression of optic nerve, extraocular muscles, or the globe is evident. Surgery may also be indicated for severe proptosis or for pathologic diagnosis if the identity of the mass is in question. Surgical resection is usually uncomplicated unless located at the orbital apex.

ADDITIONAL TREATMENT

General Measures

If exposure keratopathy develops due to lagophthalmos, treat with lubrication and follow carefully to ensure stability or resolution.

SURGERY/OTHER PROCEDURES

As mentioned above

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

• Ophthalmologist

• Oculoplastic/orbital specialist

• If extends extraorbitally, may require neurosurgical or otolaryngological consult

PATIENT EDUCATION

For patients who are being observed, they must be aware of need for swift evaluation if a change in vision were to occur

PROGNOSIS

• Vast majority who are observed remain stable over time

• Majority with indications for surgery do well after resection – no risk of recurrence

COMPLICATIONS

Visual loss

ADDITIONAL READING

• Cheng JW, Wei RL, Cai JP, et al. Transconjunctival orbitotomy for orbital cavernous hemangiomas. Can J Ophthalmol 2008;43(2):234–238.

• Scheuerle AF, Steiner HH, Kolling G, et al. Treatment and long-term outcome of patients with orbital cavernomas. Am J Ophthalmol 2004;138(2):237–244.

• Weir RE, Evans S, Hajdu SD, et al. The convex retina: Optical coherence tomography in hypermetropic shift, without choroidal folds, from intraconal cavernous haemangioma. Orbit 2009;28(6):398–400.

CODES

ICD9

• 224.1 Orbital tumor, benign

• 228.09 Hemangioma of other sites