Management of nonspecific inflammatory and lymphoid orbital lesions.
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Biomedical subjects
Publications and source records attributed to J S Kennerdell.
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Five cases representing varied presentation and treatment of the sclerosing type of nonspecific orbital inflammation are presented. Rapid identification and vigorous, individualized treatment are recommended: for local lesions, removal; for diffuse lesions, debulking, and/or steroid treatment, followed by radiation therapy.
Orbital muscle and adipose tissues from seven Graves' orbitopathy patients were studied with in situ assays for IgE. The cases varied in disease severity and site biopsied. Two monoclonal and one polyclonal anti-IgE reagents produced similar results. Identically prepared monoclonal anti-IgM and anti-IgG antibodies and tissues obtained from five patients treated for unrelated orbital disorders were used as controls. Graves' tissues exhibited extravasated leukocytes and leukocyte-rich vessels. These leukocytes were mostly lymphoid. Some basophils and mast cells were identified and polymorphonuclear cells were abundant within vessels of adipose tissue. IgE-positive material was found in association with the majority of leukocytes and with muscle fibers. Parallel sections reacted with anti-IgM antibody were negative, whereas anti-IgG produced diffuse staining with no specific structures highlighted. Control, non-Graves' tissues had no evidence of immune cell activity and were either negative or displayed reactions with anti-IgE reagents that were in most cases different from those of Graves' tissue. Serum IgE was measured in six of the seven patients and was elevated in the two patients with fulminating disease.
We present one of three cases in which we have used the carbon dioxide laser to successfully perform subtotal surgical removal of plexiform neurofibromas of the lid and orbit.
Extraocular muscle recessions with adjustable sutures were performed on 32 muscles for diplopia secondary to dysthyroid-myopathy. Orthophoria in the primary position was achieved in 30 of the 32 (94%) 1 day after surgery. All remained orthophoric for at least 6 weeks. Of 20 patients followed for 6 months or more, 12 (60%) remained orthophoric for the full extent of their follow-up (6 months to 8 years), and another three (15%) were asymptomatic with prisms (3, 4, and 8 prism diopters). Thus, a total of 15 of 20 (75%) patients remained asymptomatic at least 6 months after surgery.
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Thirty-eight patients (39 eyes) with optic nerve sheath meningiomas were entered into a treatment plan and were followed up for at least three years. Eighteen eyes were simply observed because they had minimal functional deficit or the eye was blind. Radiation was used on six eyes with documented progressive visual loss that still had useful vision. Surgery was initially used in an attempt to remove optic nerve sheath meningiomas but was abandoned except in exceptional cases. Total surgical removal of the tumor was carried out in ten eyes with loss of vision and in which the meningioma was growing. Surgery for subtotal or complete removal of the tumor followed by radiation was carried out in five eyes exhibiting aggressive growth of the tumor. This treatment plan has helped us to treat patients with optic nerve sheath meningiomas in an orderly way.
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Three approaches as used with modern technology, magnification with an operating microscope, and self-retained retraction have been presented. These approaches have allowed satisfactory exposure of difficult retrobulbar orbital areas from the various directions and have allowed us to more comfortably microdissect and remove orbital tumors. They also provide good exposure for the use of the carbon dioxide laser, which has been an aid in removing the intraorbital extensions of meningiomas, plexiform neurofibromas, and difficult lymphangiomas that occupy the intraconal compartments of the orbit. These approaches should only be undertaken by a skilled ophthalmic orbital surgical team in the case of the anterior medial and lateral approach, and definitely by a well-experienced neurosurgical/ophthalmic orbital team for tumors involving the orbital cranial junction and the superior orbital compartment. Experience gained over 17 years has convinced us that a team approach is mandatory for the difficult deep intraorbital tumors, even with the anterior medial or lateral approach.
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Lymphangiomas of the ocular adnexa, especially those in the orbit, are difficult to treat because the unencapsulated tumor freely interdigitates with normal orbital tissue, obliterating any potential surgical plane. Because of the hemorrhagic and friable nature of the tumor, conventional surgical techniques are frequently complicated by bleeding. We used the CO2 laser to remove these lesions subtotally by controlled vaporization in six patients (four girls and two boys, 5 to 17 years old). Three pupils remained dilated postoperatively because of damage to the ciliary nerves and symblepharon formation occurred in one case. None of these has produced any symptoms. In one case, however, laser treatment may have produced corneal anesthesia.
When vasculitis, an angiocentric and angiodestructive process, occurs in the orbit, the clinical presentation and radiographic findings resemble those of idiopathic inflammatory pseudotumor. Three patients, two men and a woman, 28 to 72 years old, initially thought to have "pseudotumor" failed to response to corticosteroid therapy. Orbital biopsy specimens in all patients disclosed vasculitis. There was no evidence of systemic vasculitis. High-dose prednisone effectively eliminated pain and reduced inflammation but did not adequately control fibrosis formation leading to ultimate loss of function. Each patient eventually lost an eye to this process. Therapy with cyclophosphamide, a B-cell cytotoxic drug, produced a prompt response in terms of eliminating pain, inflammation, and formation of fibrous tissue. Cyclophosphamide therapy has been instrumental in preserving sight in each patient's remaining eye. In such cases we believe the benefits of cyclophosphamide therapy outweight the known risks.
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Mucoceles occupying the sella turcica with suprasellar extension are quite rare. A case of intrasellar mucocele is reported. The preoperative clinical and computed tomographic appearance could not be differentiated from that of pituitary adenoma.
Fine-needle aspiration was used as a primary orbital diagnostic technique in 156 patients. A 20-mm syringe, a 22-gauge, 3.75-cm needle, and a plastic pistol grip were used. Local anesthetic was not necessary. When the needle is in the lesion, small to-and-fro movements with a minimum of angulation are helpful. The aspirate is spread on slides fixed with alcohol. Positive cytologic identification was made in 125 of 156 cases. Commonly identified lesions included metastatic carcinoma, inflammatory lesions, and lymphoid lesions. Small posterior apical lesions are difficult to aspirate, but are often the most rewarding diagnostically. Fine-needle aspiration was not successful in tumors of fibrous consistency, in those located in the orbital apex, or in lymphocytic lesions with specimens inadequate for diagnosis.
Intrapapillary refractile bodies were observed in seven of 13 patients with chronic optic disc edema secondary to optic nerve sheath meningioma. The observation of refractile bodies preceded or coincided with decline in vision and disappeared as optic atrophy supervened. Intrapapillary refractile bodies appear to be a nonspecific sign of chronic optic disc edema.
Dysthyroid orbitopathy often presents with the classical signs and symptoms of hyperthyroidism with or without pretibial myxedema. However, it may start insidiously without a history of thyroid disease and without clinical signs of hyperthyroidism. The signs or symptoms may be confusing and mimic neurologic disease. The therapy of dysthyroid orbitopathy is becoming more accurately individualized, but is not yet directed at the immunologic cause.
A patient with the preoperative diagnosis of recurrent orbital apical meningioma was found to have a fibrotic capsule surrounding a silicone plate implanted 3 years previously to reconstruct the orbital roof. Caution is urged in using alloplastic orbital implants for reconstructive purposes in patients undergoing orbital surgery, because associated fibrous capsule formation may mimic tumor recurrence.