Optic nerve sheath meningioma.
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Biomedical subjects
Publications and source records attributed to J S Kennerdell.
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Computed assisted tomography (CT) of seven cases of dysthyroid optic neuropathy showed moderate to severe thickening of the extraocular muscles at the orbital apex, white CT of two control cases of dysthyroid orbitopathy without neuropathy showed minimal or fusiform thickening. The consequences include probable apical compression with optic nerve neuropathy either by direct pressure on the nerve or its blood supply. The management of these cases is either by oral administration of corticosteroids in high dosage with gradual tapering or radiation therapy to the orbital apex (approximately 3,000 rad over a ten-day period). If surgical decompression is to be attempted, it should be designed to decompress the optic nerve at the orbital apex.
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A patient with Klippel-Trenaunay-Weber (KTWS), pneumosinus dilatans, and familial Adie's syndrome who developed bilateral perioptic meningiomas is described. This case demonstrates the efficacy of fine-needle aspiration biopsy for tissue diagnosis of optic nerve tumors in blind eyes.
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Thirty-four patients with childhood Graves' disease were examined to determine the incidence and severity of Graves' ophthalmopathy in children and adolescents. Twenty-three of the 34 patients formed a population of childhood Graves' disease unselected for ocular disease; 11 were unaffected and 12 (52.2%) had findings considered to be of mild functional importance. The remaining 11 patients were referred for evaluation because of a history of Graves' ophthalmopathy; eight (72.7%) exhibited findings of mild severity and three the more meaningful complications of ophthalmoplegia. There were no cases of dysthyroid optic neuropathy or sight-threatening corneal involvement. Both the presence of asymmetric ocular manifestations and increasing severity of disease seem to correlate with increasing age. Although ophthalmoplegia does occur infrequently, Graves' ophthalmopathy in children and adolescents seems to be a substantially more benign process than that seen in adults.
In three patients we used B-scan ultrasonography to guide the placement of an aspirating needle, which allowed rapid and accurate cytologic diagnosis of retrobulbar orbital neoplasms. This technique spares the patient the greater costs, inconvenience, and morbidity of more invasive procedures.
We compared the clinical course and histopathologic findings in five patients with malignant gliomas originating from the optic nerve pathways with those in previously reported cases to elucidate further the clinical features of this tumor and facilitate future diagnoses. Patients with malignant optic nerve pathway gliomas had unilateral or bilateral visual dysfunction, which was often accompanied by periorbital discomfort in an otherwise asymptomatic adult. Regardless of origin, the clinical course invariably was bilateral blindness rapidly followed by death. Occasionally, the diagnosis was made preoperatively and was confirmed by surgical exploration and biopsy.
A 25-year-old woman experienced acute visual loss after injection of methylprednisolone suspension into the concha nasalis inferioris. Results of ophthalmoscopic examinations were normal throughout the observation period, a finding not reported previously in cases of visual loss after head or neck injections. An afferent pupillary defect, which diminished as visual acuity improved, implicated otpic nerve damage, as did the visual field defect which also improved slightly. This is, to our knowledge, the first case report of acute visual loss caused by optic nerve neuropathy after injection to the head or neck.
Biopsies were done for seven patients who had blinding optic nerve tumors or inflammations, using the fine needle aspiration technique guided by the CT scan. Cytologic material was recovered in this manner in all cases, which allowed the rendering of diagnoses. This allowed for therapeutic planning with minimal therapeutic invasion.
A patient with simultaneous involvement of the right medial rectus by dysthyroid and myasthenic myopathy is presented. These abnormalities were confirmed by a positive Tensilon test, computerized tomography demonstrating an enlarged right medial rectus, and a right medial rectus muscle biopsy demonstrating histopathology typical of both diseases.
A simple instrument, called the Phaco Elevator, is recommended for elevation of the nucleus into the anterior chamber, particularly when the nucleus is soft.
Severe medial rectus injury occurred in two cases following intranasal sinus surgery. In the first case a scarred medial rectus muscle was found at the time of corrective surgery. Reexamination of the surgical specimen in the second case confirmed our finding of medial rectus transection. Both patients had good cosmetic and functional improvement by means of vertical recti transposition to correct their large angle, fixed exotropias. A review of the literature indicates that this is, to our knowledge, a previously unreported complication of intranasal sinus surgery.
Fifteen orbital tumors have been evaluated with the fine-needle aspiration biopsy (FNAB) technique. The best indication for FNAB is supposed malignant orbital disease. The technique has not been helpful in tumors or inflammatory disease with a high fibrous content. Lesions that are suspected of being pseudotumors are not recommended for FNAB since, even in histologic sections, they are notoriously difficult to distinguish from well-differentiated lymphocytic malignant lymphoma. Benign encapsulated tumors should not be subjected to FNAB.
This presentation demonstrates the advantages of a fourth generation CT scanner in prognosis and localization of orbital space taking lesions, including its use as a localization guide for fine needle aspiration biopsy. It also provides an accurate means of follow-up of orbital lesions which have been either left in situ, partially excised, or excised. Six patients with difficult orbital problems of various types have been described to demonstrate how this high resolution CT scanner was utilized to improve the recognition and management of these problems.
We think the use of the operating microscope in deep orbital surgery is essential for a safer and more accurate removal of the tumors that occupy this difficult surgical location.
Ten patients with biopsy-proved lymphoid hyperplasia (pseudotumor) of the orbit were treated with radiation therapy individed sose ranging from 2,500 to 3,000 rads. Five of the ten patients had prior treatment with corticosteroids in high dosages with only partial or no response. All ten patients had clinical resolution of the lymphoid hyperplasia orbital mass within three weeks following radiation therapy. A follow-up period of six months to five years has demonstrated no recurrences. There were no discernible complications from the radiation therapy. We believe that radiation therapy should be considered as a primary treatment of benign lymphoid hyperplasia of the orbit.
Neurodiagnostic techniques now permit precise localization of intraorbital tumors relative to the optic nerve and intracranial compartment. With such localization, surgical procedures may be planned along more anatomical lines. Those circumscribed tumors may be planned along more anatomical lines. Those circumscribed tumors lateral, superior, and inferior to the optic nerve may well be approached by a lateral orbitotomy using microdissecting techniques. Those tumors medial to the optic nerve deep in the apex and those with possible intracranial extension should be approached transfrontally. In both surgical approaches, precise anatomical knowledge and microdissecting techniques are needed.