- Book Chapter
- 10.1093/oso/9780195340211.003.0030
Orbital Exenteration
- Oct 13, 2011
- Adam Hsu + 1 more +1
Orbital exenteration is a surgical procedure that removes varying degrees of the orbital contents and periorbital soft tissue, including periorbital skin, the globe, extraocular muscles, optic nerve, the periorbita, and the orbital fat. In some instances, the disease process may necessitate removal of the bony orbital walls as well. Orbital exenteration is reserved for highly malignant and potentially fatal neoplasms originating from the ocular adnexal structures; ocular 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. Tumors necessitating orbital exenteration may include squamous cell carcinoma, basal cell carcinoma, sebaceous carcinoma, melanoma of the conjunctiva, uveal melanoma with extrascleral or orbital extension, epithelial cancers such as adenoid cystic carcinoma of the lacrimal gland, rhabdomyosarcoma, and other rare tumors. The indications for orbital exenteration in a number of previously published series are summarized in Table 23-1. In addition to cancers, nonmalignant neoplasms—such as neurofibromatosis causing severe orbital displacement, immobility, and blindness or extensive lymphangioma compromising function and cosmesis—may also lead to orbital exenteration. Nonneoplastic diseases of rapidly infiltrative nature, either inflammatory or infectious, and nonneoplastic diseases associated with refractory orbital pain may also be indications for orbital exenteration. Examples include invasive fungal infections of the orbit such as mucormycosis; orbital socket contracture after enucleation causing severe pain; and extreme cases of Graves orbitopathy unresponsive to other treatment modalities. Orbital exenteration results in complete loss of vision in the affected eye as well as significant disfigurement of the upper face; thus, counseling of the patient prior to surgery 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, with a focus on symptoms of cancer and metastasis. The physical examination should include careful inspection for signs of local or regional extension of disease. Regional lymph node and distant-organ metastasis must be ruled out prior to the decision to proceed with an orbital exenteration.
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