John S. Kennerdell

The nonspecific orbital inflammatory syndromes.

The nonspecific orbital inflammatory syndromes are a peculiar group of inflammations that may occur in acute or subacute forms and can become chronic. They may be diffuse or primarily localized to a specific tissue of the orbit. The nonspecific inflammations that are targeted toward specific tissues are myositis, dacryoadenitis, perineuritis, and periscleritis. Each of these syndromes has definite signs, symptoms, ultrasonic and radiologic findings. Therefore, this group of nonspecific inflammations should be more clearly classified for purposes of better understanding and better management. All of these inflammatory syndromes in the acute form respond well to high doses of oral corticosteroids tapered gradually over a period of months, but may be reoccurrent or become chronic. The subacute form responds less well. Occasionally, patients require radiation to stop the inflammation in the subacute or chronic state, but these patients are often left with a functional deficit. The cause, although presumed to be an immune disorder involving the orbital tissues, remains unknown.

A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapya

PURPOSE Comparison of treatment outcome in patients with primary optic nerve sheath meningioma (ONSM). DESIGN Retrospective, nonrandomized, comparative interventional case series. PARTICIPANTS Sixty-four patients with at least 50 months of well-documented follow-up. INTERVENTION Observation, surgery only, radiotherapy only, or surgery and radiotherapy. MAIN OUTCOME MEASURES Visual acuity expressed as ratio, radiographic progression, and treatment complications. RESULTS Sixty-four patients (55 women [85.9%] and nine men [14.1%]) were followed for a mean of 150.2 months (range, 51-516 months; standard deviation [SD], 74.7). The mean age at diagnosis was 47.1 years (range, 17-81 years; SD, 15.4). Fifty-eight patients had unilateral disease and six patients had bilateral disease. Of 59 patients with vision greater than no light perception at diagnosis, 13 patients were observed only, 12 had surgery only (four biopsies or partial resections, eight total resections), 18 received radiation alone, and 16 had surgery and radiation (14 biopsies or partial resections and radiation, two total resections and radiation). Irradiated patients received 4000 to 5500 cGy of conventional multiport or conformal external beam therapy, typically fractionated over 6 weeks. Visual acuity measurements at diagnosis among the four groups were not different (ratio, P = 0.186). Visual acuity at diagnosis was > or =0.5 in 56.3%, 0.4 to 0.050 in 12.5%, and <0.050 in 31.3%. Visual acuity measures at last follow-up were different among the four groups (ratio, P = 0.004). At last follow-up the acuity was > or =0.5 in 28.1%, 0.4 to 0.050 in 15.6%, and <0.050 in 56.3%. Visual acuity fell significantly for the observed only (ratio, P = 0.002), surgery only (ratio, P = 0.019), and surgery with radiation groups (ratio, P = 0.030). The radiation only group showed a decrease in visual acuity that was not significant (ratio, P = 0.301). Complication rate was 33.3% in radiation only cases, 66.7% in surgery only cases, and 62.5% in surgery with radiation cases. Twenty-one patients (32.8%) showed radiographic progression. Four patients who were observed, seven patients who had surgery alone, and eight patients who had surgery and radiation developed radiographic progression. Two patients who had radiation alone had radiographic progression before treatment. Only two patients treated with radiation only showed radiographic progression after radiotherapy, and both had at least one surgical procedure before the radiotherapy. CONCLUSIONS Patients with ONSM receiving radiation alone demonstrated the best visual outcome during the follow-up period. We recommend that fractionated external beam radiation (5000-5500 cGy) be considered as initial treatment in adults in selected cases of ONSM when preservation of visual function is a reasonable therapeutic goal.

Evaluation of orbital cellulitis and results of treatment.

Optimal management of patients with orbital cellulitis depends on how accurately the disease is classified and on the appropriateness with which antibiotics and surgery are used to treat the disease. Therapy must be adjusted on the basis of the extent of the disease. In order to determine the balance of treatment modalities which is most beneficial for certain disease presentations, we reviewed a series of 303 patients with orbital cellulitis. The anatomical and bacteriological etiology of the disease was determined in each case on the basis of the examination, visual acuity, results of sinus radiography, results of culture, ultrasonography, and computerized tomography. To avoid the 5% complication rate that occurred in this series, an evaluation and treatment protocol is recommended.

The Management of Optic Nerve Sheath Meningiomas

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 abandonded 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.

Combined Clinical and Computed Tomographic Diagnosis of Orbital Glioma end Meningioma

The clinical information on 22 patients with orbital optic nerve gliomas and 47 patients with meningiomas was correlated with computed tomographic findings obtained in both axial and coronal studies. Most of the gliomas occurred in children, although 7 patients presented after 20 years of age. Among the patients with meningiomas, the majority were women in early middle age, although two tumors occurred in children less than 20 years of age. Low grades of proptosis (median, 2 mm for both tumors), frequent significant visual field obscurations with eye movements, and opto-ciliary shunt vessels pointed toward the diagnosis of an optic nerve tumor. Patients with gliomas generally manifested massively swollen fusiform optic nerves with clear-cut margins due to circumscription by an intact dura. Kinks and bucklings of the optic nerve as well as infarctive cysts distinguished the glioma CT-scan patterns from the meningiomas. Distinctive axial CT-scan features of the meningiomas not shared by the gliomas were narrowly and diffusely enlarged nerves with polar expansions either at the orbital apex or immediately behind the globe; calcification; irregular excrescent margins signifying extradural invasion into the orbital soft tissues; a negative optic nerve shadow running down the center of the lesion; and bone erosion near the orbital apex. Coronal studies often revealed irregular margins signifying transgression of the dura. A diffusely and narrowly enlarged optic nerve shadow with regular margins (intrasheath lesions) was the one morphologically overlapping pattern displayed by 11 meningiomas and three gliomas. In these cases there tended to be more profound visual loss in the gliomas compared with the meningiomas, as well as the more frequent presence of opto-ciliary vessels in the meningiomas. Arteriography may be helpful in this particular category by demonstrating a tumor blush for the meningiomas, whereas this finding is typically absent with optic nerve gliomas. Meningiomas may be very closely simulated by dural or intraneural inflammations.

Optic Nerves Sheath Meningiomas: Clinical Manifestations

A retrospective clinical study of optic nerve sheath meningiomas based on 22 patients showed that symptoms most commonly develop in women between the ages of 35 and 60 years. The most common presenting symptoms were decreased vision and transient visual obscurations. In the earliest stages, many patients presented with normal to mildly impaired acuity (despite subjectively decreased vision), optic disc edema and enlargement of the blind spot. Optic disc edema was frequently associated with refractile bodies indicative of chronic swelling. Optic disc edema preceded the development of optic atrophy. Another group of patients presented with a history of longstanding vision loss, visual acuity of 20/200 or worse and optic atrophy. Optociliary shunt vessels were late findings only seen in five patients. The most consistent visual field abnormality was peripheral constriction. Cecocentral scotomas were uncommon. Intracranial involvement was present in five patients. There were two patients with bilateral optic nerve sheath meningiomas without CT evidence of intracranial involvement. Computerized tomography was found to be indispensable in the diagnosis of optic nerve sheath meningiomas and the detection of intracranial involvement.

Acute and Subacute Orbital Myositis

Of 12 consecutive patients (nine women and three men ranging in age from 18 to 70 years) with orbital myositis, four had histories of ocular or systemic autoimmune disease. Five patients treated within two weeks of developing acute orbital pain and extraocular muscle dysfunction were classified as having acute myositis. They responded to corticosteroids within 72 hours although three had single recurrences during six- to 16-month follow-up periods. No patients were heterotropic or proptotic at the final examination. Seven patients with less severe or atypical symptoms who had delays of two or more months between onset and treatment were classified as having subacute myositis. All had recurrences with five having two or more. Three required supplemental radiation therapy during seven- to 20-month follow-up periods. At the final examinations, six patients had motility defects and one had proptosis, indicating that delays in treatment may lead to recurrences, extraocular muscle dysfunction, and proptosis.

An Orbital Decompression for Severe Dysthyroid Exophthalmos

A procedure has been developed for maximizing decompression in patients with nonspecific orbital inflammatory disease or dysthyroid orbitopathy that involves removal of parts of all four orbital walls through a lateral orbitotomy using a 30-mm incision combined with a canthotomy and an inferior cul-de-sac incision. The procedure has been performed on seven orbits of four patients who had greater than 30 mm of forward placement of the eyes as measured by Hertel exophthalmometry and/or a 10-mm difference in the forward placement of the two eyes, indications considered for this procedure. The procedure has resulted in 10-17 mm of reduction in proptosis in these patients, which is consistently greater than that obtained by any other procedure. A complication of spinal fluid leak resulted in the recommendation that the procedure not be performed in patients over 65 whose dura is considered too thin to prevent spontaneous leakage and minimal surgical trauma. In all circumstances, this procedure must be performed by an experienced neuro-ophthalmic orbital surgical team.

Orbital complications of acute sinusitis: evaluation, management, and outcome.

Acute orbital cellulitis is most commonly caused by sinusitis. This complication is based on the anatomy of the sinuses, orbit, and their venous connections. A series of 134 patients is reviewed to demonstrate the etiology and clinical course of orbital cellulitis. Antibiotic therapy alone does not prevent permanent complications. Surgical intervention is found necessary in all age groups and is required in 20% of the study population.

Malignant Gliomas of the Optic Nerve Pathways

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.