Orbital Exenteration
Bita Esmaeli
INTRODUCTION
Orbital exenteration is the surgical removal of the contents of the orbit and the periorbital soft tissue, including the eye, extraocular muscles, optic nerve, periorbita, orbital adipose tissue, eyelids, and periorbital skin. The most common indication for orbital exenteration is a malignancy of the eye, orbit, ocular adnexa, or periocular region for which salvage of the globe is not possible. In some cases, not only the orbital and periorbital soft tissue but also the bony orbital walls must be removed.
HISTORY
Orbital exenteration not only results in loss of the eye and its function but also causes significant facial disfigurement. Thus, this procedure should be reserved for patients in whom salvage of the globe is not possible because of the very aggressive nature of the malignancy and the need for wide margin of resection. In most instances, orbital exenteration is only appropriate for patients in whom the possibility of cure is likely enough to justify this radical procedure.
Given the dramatic changes in function and appearance caused by orbital exenteration, counseling of the patient prior to surgery is essential. Proper presurgical counseling and understanding of the patient’s expectations are important to minimize the patient’s distress. During the preoperative interview, the patient’s past medical and surgical history, current medications, coagulation status, and allergies should be ascertained. There should also be a review of systems focusing on symptoms or signs of cancer metastasis, which would be a relative contraindication to the procedure.
PHYSICAL EXAMINATION
During the physical examination in a patient being considered for orbital exenteration, the tumor should be carefully evaluated, and its exact location within the orbit, ocular adnexal structures, paranasal sinuses, or nasal cavity should be noted. This information is used to plan the skin incision and to decide which other adjacent periorbital structures to include in the resection, if indicated. When an “eyelid-sparing” orbital exenteration may be possible, the skin of the upper and lower eyelids should be carefully examined, and the degree of laxity and redundant skin should be noted as this skin will be used to line the orbit.
INDICATIONS
The indications for orbital exenteration can be divided into four general categories: tumors of the eyelid or conjunctiva for which salvage of the globe is not possible, intraocular tumors with extension to the orbital soft
tissue, orbital extension of tumors of the paranasal sinuses or nasal cavity, and orbital extension from intracranial processes.
tissue, orbital extension of tumors of the paranasal sinuses or nasal cavity, and orbital extension from intracranial processes.
Orbital exenteration can be required for treatment of squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma, conjunctival melanoma, uveal melanoma with extrascleral or orbital extension, epithelial cancers such as adenoid cystic carcinoma of the lacrimal gland, rhabdomyosarcoma, and other rare sarcomas.
Orbital exenteration may also be indicated for certain nonmalignant neoplasms, such as neurofibromatosis causing severe orbital displacement, immobility, and blindness or extensive lymphangioma causing disfigurement or orbital pain. Finally, inflammatory or infectious processes associated with refractory orbital pain (e.g., invasive fungal infections such as mucormycosis) may be indications for orbital exenteration.
CONTRAINDICATIONS
Orbital exenteration may not be appropriate in patients with widespread metastatic disease, patients with tumors expected to have a high likelihood of local-regional recurrence despite orbital exenteration, elderly patients with multiple medical comorbidities, or patients with a short life expectancy. For such patients, palliative radiation therapy, other palliative medical treatments, or various degrees of debulking may offer a better quality of life than orbital exenteration.
PREOPERATIVE PLANNING
Imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) of the orbit are an integral part of the preoperative evaluation and provide information about the gross anatomic extent of the cancer. For example, if there is extension of cancer into the paranasal sinuses or nasal cavity, a multidisciplinary approach may be more appropriate. Involvement of bony walls of the orbit on CT would help plan the appropriate bony resection and reconstruction. Or, if there is deeper orbital extension based on CT or MRI, a deeper orbital exenteration may be planned. A careful examination for signs of local or regional disease spread is essential because orbital exenteration may not be appropriate in patients with regional lymph node or distantorgan metastasis. The evaluation for systemic disease should be tailored to the type of cancer.
Because general endotracheal anesthesia is necessary for orbital exenteration, a preoperative anesthesiology consultation is important to rule out any cardiac or pulmonary contraindications to general anesthesia.
It is also important to discuss with the patient the types of reconstructive procedures available and to find out whether the patient is interested in wearing an orbital prosthesis, as this may affect the choice of reconstructive procedures. Whether postoperative adjuvant radiation therapy will be needed should be taken into account in surgical planning as the need for radiation therapy impacts the choice of tissue coverage for the orbital cavity.
SURGICAL TECHNIQUE
Orbital Exenteration