42.1 Goals

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  To remove the affected tissue for cure of tumor, infection, or inflammation.

  
  
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  To minimize risk of complication.

  
  
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  To create a socket that will allow for an oculofacial prosthesis (if desired).

42.2 Advantages

Exenteration is performed as a life-saving procedure in high-grade malignancies involving the orbit for local disease control and to increase overall survival by decreasing the risk of metastasis. For patients with preexisting metastatic disease, exenteration can serve as a palliative measure to improve the quality of life by alleviating intractable pain in cases of significant mass effect or infiltrating disease. Exenteration for invasive orbital infections can decrease overall mortality by preventing or minimizing intracranial spread of the infection. ^{1 ,​ 2 ,​ 3 ,​ 4}

42.3 Expectations

When discussing the surgical procedure of exenteration and the postoperative recovery, the patient and family members must be prepared for the emotional and psychosocial aftermath of removal of the eye socket and its cosmetic sequelae. Patient’s expectations of the aesthetic rehabilitation period including the appearance of the ocular prosthesis and timing of the prosthesis fitting after surgery should be explicitly discussed. Exenteration also does not eliminate the possibility of tumor recurrence or metastatic spread, and the need for continued follow-up should be emphasized. The patient will live with lifelong monocular precautions, including the need for polycarbonate glasses to protect the remaining eye.

42.4 Key Principles

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  Exenteration involves removal of all soft-tissue contents of the orbit including the globe, extraocular muscles, periorbita, and ocular adnexa with part or all of the eyelids.

  
  
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  Depending on the clinical situation, the patient may need total exenteration, eyelid-sparing exenteration, subtotal exenteration, or exenteration in conjunction with additional intracranial or sinus surgeries.

  
  
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  Primary reconstruction strategies include healing by secondary intention, split-thickness skin graft, myocutaneous advancement flap, dermal substitute, or free flap.

42.5 Indications

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  With advancements in medical and surgical care, orbital exenterations occur less frequently than before. Globe-sparing resection, if possible, is always considered first. ²

  
  
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  Orbital exenteration is most commonly performed for orbital involvement of malignancies. Due to the anatomic limitations of vital structures being confined within a small space, and the formless nature of orbital fat making reliable identification of tumor margins difficult, complete resection of an infiltrating tumor of the orbit can be challenging. In such cases, exenteration can be the only option to ensure complete resection. Other scenarios include cases in which resection is expected to leave a blind, disfigured eye and ocular adnexa with limited function of the remaining orbital tissues, making exenteration the preferred surgery to achieve the best functional and cosmetic outcome. ^{1 ,​ 2 ,​ 3 ,​ 4}

  
  
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  A thorough history and physical examination should be performed, including consideration of the natural history of the tumor, tumor size, stage, histology, visual function, extent of orbital involvement, and the patient’s overall health.

  
  
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  Tissue diagnosis based on permanent histopathology must be confirmed prior to proceeding with exenteration. Under no circumstance should frozen sections be used as a surrogate to decide on exenteration. Frozen sections may be used intraoperatively for margin control during exenteration. ^{1 ,​ 2 ,​ 3 ,​ 4}

  
  
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  Examples of primary orbital tumors that may require orbital exenteration: adenoid cystic carcinoma, rhabdomyosarcoma, retinoblastoma, mucoepidermoid carcinoma. ^{1 ,​ 2 ,​ 3 ,​ 4 ,​ 5 ,​ 6 ,​ 7 ,​ 8}

  
  
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  Examples of eyelid or ocular adnexal tumors with invasion into the orbit: melanoma, basal cell carcinoma, squamous cell carcinoma, sebaceous cell carcinoma. ^{1 ,​ 2 ,​ 3 ,​ 4 ,​ 5 ,​ 6 ,​ 7 ,​ 8}

  
  
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  Examples of sinonasal tumors with invasion into the orbit: squamous cell carcinoma, esthesioneuroblastoma. ^{1 ,​ 2 ,​ 3 ,​ 4 ,​ 5 ,​ 6 ,​ 7 ,​ 8}

  
  
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  Exenteration may be indicated in severe life-threatening orbital fungal infections such as mucormycosis and aspergillosis with risk of intracranial extension. ^{1 ,​ 2 ,​ 3 ,​ 4 ,​ 5 ,​ 6 ,​ 7 ,​ 8}

  
  
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  Rare instances of severe periocular necrotizing fasciitis (group A β-hemolytic Streptococcus) unresponsive to medical therapy and debridement may undergo exenteration. ⁹

  
  
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  Benign tumors of the orbit or congenital deformities are rarely considered for palliative exenteration, in cases of diffuse disease resulting in irreversible vision loss, uncontrollable pain, or severe disfigurement. ¹⁰

  
  
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  Severe trauma with extensive involvement of the orbital bone or soft tissues may require an exenteration if attempts to salvage the remaining structures are not successful and not amenable to reconstruction. ¹¹

42.6 Contraindications

Given the permanent vision loss and facial disfiguration resulting from exenteration, all other alternatives need to be thoroughly discussed with the patient and his or her family. In some elderly, debilitated patients with additional medical comorbidities or limited life expectancy, a realistic discussion regarding the expected benefit of exenteration versus the physical and emotional burden of exenteration must be held. The postoperative wound care regimen can also be a barrier for patients with no family or nursing help. Each patient therefore needs to be considered on an individual basis to make a tailored treatment plan.

42.7 Preoperative Preparation

The patient must undergo a complete ocular examination including vision, motility, globe position, dilated fundus exam, and a thorough eyelid and ocular adnexa evaluation. Preexisting nasolacrimal system obstruction, lymphadenopathy, facial sensory defects, and involvement of adjacent external facial and sinonasal structures should be noted. A definitive pathological diagnosis must be made on permanent histological specimens from a biopsy instead of frozen sections. A metastatic work-up may be indicated depending on the tumor type. Orbital imaging with computed tomography and/or magnetic resonance imaging must be obtained to assess the location and extent of pathology.

If the disease involves the nasal cavity, sinuses, or the intracranial space, the patient should have a preoperative neurosurgery or otolaryngology evaluation to assess if transnasal or transcranial approaches may be required in addition to orbital exenteration. For patients with malignant lesions, medical oncology and radiation oncology are also part of the multidisciplinary team to coordinate neoadjuvant or adjuvant chemotherapy and radiation. For emotional preparation and expectation of possible cosmetic outcomes, patients may also wish to see an ocularist during the preoperative period.

Preoperative planning also includes surgical decision regarding the type of exenteration to be performed. For more posteriorly located tumors without skin or conjunctival involvement, skin-sparing technique can preserve the eyelids, which can be used to line the socket instead of requiring an additional skin graft. Lesions in the anterior orbit, involving the conjunctiva or eyelid skin, may only need a subtotal exenteration and partial excision of the surrounding skin, unless the disease is infiltrative and diffuse in nature (i.e., pagetoid spread of sebaceous cell carcinoma), in which case total exenteration is warranted. Extensive benign lesions can often be managed with an eyelid-sparing exenteration.