Orbital Tumors Treatment & Management

Updated: Sep 04, 2019
  • Author: Michael Mercandetti, MD, MBA, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Treatment

Medical Therapy

Medical therapy is tailored to the diagnosis obtained by biopsy or excision. Certain situations do not require a biopsy or excision to initiate treatment. Conditions such as orbital cellulitis often are treated medically with various antimicrobial agents. Surgical intervention is warranted if there is no response to treatment or clinical worsening is evident on examination. Orbital inflammatory disease (pseudotumor) usually is treated medically with systemic steroids. Capillary hemangiomas also can be treated with nonsurgical modalities, such as steroid injections.

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Surgical Therapy

With little wasted space and a lexicon of anatomical structures occupying the orbit, surgical intervention remains a challenge. Maximal operative exposure of the lesion with ginger and minimal manipulation of the orbital contents must be well-orchestrated for successful surgical outcomes. Advances in computer-assisted imaging devices are facilitating the operative experience, but widespread utilization is limited. [18]

Many cutaneous and bony approaches to the orbit have been described. The surgical approach used is reliant upon the location and size of the tumor with the surgeon's skill and experience lending itself to the choice of surgical entry into the orbit. This discussion is not meant to be a comprehensive tome on orbital surgery but merely an overview of commonly described orbitotomies.

Positions that minimize an increase in intraorbital (venous) pressure are preferred during the surgical procedure. This can be accomplished with the reverse Trendelenburg position and hypotensive anesthesia to the degree that it can be tolerated medically. Orbitotomies often are described by their anatomical location or in relation to the anatomy they transgress. Approaches can be from the periocular skin or more remote locations elsewhere on the face and scalp. Additional approaches can emanate from the conjunctival plane. Intracranial approaches also can be used.

The location of the lesion directs the surgeon toward selecting the most appropriate type and location of the orbitotomy. Concerns over the facility of using a given approach and ultimately the postoperative cosmetic appearance are considerations that assume variably weighted significance in any given situation. Indubitably, if equivalent approaches are feasible, that which produces the more appealing cosmetic result most often is preferred.

Anterior orbitotomy most commonly is performed transcutaneously or transconjunctivally. Often, the transconjunctival approach denotes an incision in the vicinity of the inferior forniceal area of the lower lid with or without a canthotomy and/or cantholysis. However, in the broadest sense, it encompasses transcaruncular and transbulbar conjunctival approaches with or without the release of the recti muscles. An example of such an approach is the medial orbitotomy via the medial bulbar conjunctiva with release and then reattachment of the medial rectus. For medially located lesions, such as those encroaching on the nasal orbital apex, this approach is possible. The optic nerve also can be reached from this approach.

Those lesions located superiorly are delimited further by their medial to lateral and anterior to posterior localization. Most often, a supertemporal lesion is from the lacrimal gland and a lateral orbitotomy or one of its modifications is an acceptable approach. Those lesions more centrally located and those in a superonasal orientation can be approached from a Lynch-type incision.

Alternatively, a coronal dissection can be used. In more posteriorly situated superior lesions, an intracranial approach may be warranted. In certain cases, medial and lateral approaches can be combined to access this area.

Inferiorly, the orbital lesion is approachable from a transconjunctival or a transcutaneous approach. In either situation, it may be required to perform a canthotomy and cantholysis to achieve adequate exposure. If the contiguous maxillary sinus is involved a Caldwell-Luc approach also can be used.

Laterally located lesions are approached from a lateral orbitotomy, such as the Berke, Reese, Stallard, or Wright approach.

Transnasal, transantral, and transethmoidal endoscopic approaches are being used more frequently to gain access to orbital lesions. [19]

Left lateral lid crease incision to access the sup Left lateral lid crease incision to access the superolateral orbit.

Transcranial approaches can be used for tumors involving the orbital-cranial regions. [20] . Skull-based tumors can also be approached via a transorbital approach. [21]

An interesting approach referred to as "round-the-clock" overlays a clock face on the orbit centered on the optic nerve. From this perspective, different approaches are used for certain clock hours. As an example, for the right medial orbit involving clock hours from 1 to 6, a medial transconjunctival approach is useful. Alternatively, an endonasal endoscopic approach can give access to the orbit at the apex, as well as to the mid and posterior orbit for clock hours 1 to 7. [22]

Core needle biopsy (CNB) of orbital tumors has been used with and without ultrasound guidance, as well as CT guidance. [23, 24]

Three-dimensional (3D) imaging can also be used in determining the approach to the tumor. [25]

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Preoperative Details

A systematic review and documentation of the patient's medical status is essential.

A thorough explanation of the procedure and the risks, benefits, and alternatives should be clearly explained and documented. The patient should be cognizant of the exact procedure and if a biopsy will be performed on the mass or if an attempt for total excision will be made. It is imperative that the patient be informed of the possibility of enucleation or exenteration if indicated.

Preoperative documentation of visual acuity, degree of ptosis, lagophthalmos, proptosis, pupillary and extraocular muscle function, and the amount of diplopia in all fields of gaze is necessary. External photos are strongly suggested for documentation and later review.

A meticulous review of imaging with a neuroradiologist, if necessary, is essential for planning the surgical approach and identifying the mass and impingement of surrounding orbital structures.

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Intraoperative Details

During surgical intervention, periodic assessment of pupillary function is prudent. Assessing the pupil size prior to general anesthesia, after general anesthesia is induced, and after any periorbital injections containing epinephrine (prior to manipulating the globe) is worthwhile. Narcotics can cause pupillary constriction (miosis), and epinephrine can cause pupillary dilation (mydriasis). If not assessed before the orbital manipulation is undertaken, assessment as to the cause of a dilated pupil can be obscured when the pupil is checked during surgery.

Intraoperative manipulation of the globe must be adequate to allow for sufficient exposure of the operative site, yet manipulation must be gentle enough not to put undue pressure on the globe compromising vascular flow.

Extraocular muscle manipulation can trigger the oculocardiac reflex, with resultant bradycardia. The anesthesia staff should be aware of any extraocular muscle manipulation, so that the patient's heart rate can be assessed.

Bipolar cautery is preferred to avoid channeling of the current and injury to the optic nerve.

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Postoperative Details

Postoperatively, the patient must be assessed with regard to vision, bleeding, and pain.

Visual insult could occur in the intraoperative period and must be assessed postoperatively at 15-minute intervals for the first hour following surgery and every 30 minutes in the second hour. Additionally, pupillary and extraocular muscle function should be evaluated in the postoperative period.

Hemorrhage can occur in the orbit and may be potentially blinding and must be scrutinized for.

Pain may be variable, but if nausea and vomiting occurs as a result of pain medications or surgery, treatment must be given to avoid this and decrease venous pressure. Increased venous pressure can cause orbital congestion and lead to compression of the optic nerve.

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Follow-up

The patient is examined the day after surgery. Vision, extraocular motility, and pupillary function are evaluated and any dressing changes are performed. In sighted patients, the authors do not patch the eye for fear that a hemorrhage would remain unrecognized and result in increased orbital pressure, with resultant compromise of an essential intraorbital structure.

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Complications

The most feared complication in orbital tumor surgery is loss of vision. This can be due to excess pressure with retraction the globe. Compression of the central retinal artery can lead to irrevocable blindness.

Hemorrhage can occur operatively and postoperatively with resultant compression of the optic nerve and occlusion of the central retinal artery. Hemorrhage also can occur from laceration of the anterior or posterior ethmoidal arteries.

Monopolar cauterization should be used sparingly in the orbit because the current can channel through the optic nerve and lead to visual loss.

Because of the close proximity to the anterior cranial fossa, inadvertent intracranial injury can result.

Direct perforation of the globe is possible, especially if adequate protection, such as a corneoscleral shield, is not used.

Diplopia or other extraocular muscle disturbances can be the result of neurologic or direct muscular injury.

Paresthesia is a potential complication if there is injury to the infraorbital, supraorbital, or supratrochlear nerves.

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Outcome and Prognosis

The outcome and prognosis ultimately depend on the pathological diagnosis.

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Future and Controversies

Improved diagnostic modalities will enhance the ability of diagnosing orbital tumors. Stereotactic computer-guided imaging can help localize posterior lesions. Positron emission tomography (PET) is being investigated to identify the metabolic activity of orbital tumors. [26] . Gene therapy is showing promise in a multitude of cancers, and this "precision medicine" is being researched for treatment in orbital cancers such as lymphoma. [27]

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