Lymphangioma of the Orbit

Computed Tomography (CT) shows a large lymphangioma (arrow), pushing the eye out of the orbit.

Lymphangioma is rare (less than 7% of childhood orbital tumors). Patients can present with acute proptosis (bulging eye) after minor head trauma, as a gradual proptosis, or after an upper respiratory infection.

Symptoms
Lymphangioma tends to start in the superior and nasal orbital quadrants. More than 50% affect anterior (conjunctival and adnexal) structures. Typically, the lymphangioma bleeds into itself causing cysts of blood (called chocolate-cysts) within the tumor. If the cyst forms behind the eye, it pushes the eye forward. If the tumor forms in the eyelid or structures around the eye "adnexa", blood filled lymphatic channels called "lymphangiectasias" can be seen beneath the conjunctiva.

A lymphangiectasia is seen beneath the conjunctival surface.

Diagnosis
Lymphangioma is usually diagnosed by an eye cancer specialist. A careful history may reveal sudden painful proptosis (bulging of the eye), facial trauma or that the tumor or proptosis started right after a upper respiratory infection.

Physical examination may reveal bluish discoloration of or blood vessels within the eyelid skin. Should the vessels extend under the conjunctiva, they are called lymphangiectasias.

A lymphangiectasia is seen beneath the conjunctival surface.

Severe cases can be associated with corneal exposure, ulceration and optic nerve damage.

Treatments
Though lymphangioma patients can present with a history of sudden proptosis (due to bleeding within the tumor), orbital lymphangioma is typically a slow growing tumor. Therefore, most lymphangiomas are followed by observation for growth (clinical and radiographic studies) prior to considering intervention.

Treatment of lymphangioma is typically indicated when it is associated with growth, optic nerve compression, corneal exposure problems (keratitis sicca), glaucoma or evidence of vision loss.

When treatment of lymphangioma is considered, the goal is rarely complete removal. This is because the edges of most orbital lymphangiomas are poorly defined. Most patients undergo several debulking surgeries to relieve acute optic nerve compression or corneal exposure. In rare cases, orbital lymphangioma patients may require exenteration of the orbit, or radiation therapy for relief of pain.

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