Kimberly P. Cockerham

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.

Closed-Eye Ocular Injuries in the Iraq and Afghanistan Wars

Comprehensive ophthalmic evaluation was conducted in 46 veterans hospitalized because of traumatic brain injury after blast exposure in Iraq or Afghanistan. Evidence of closed-eye injury was found in 20 of these patients.

Clinicopathologic evaluation of the Mueller muscle in thyroid-associated orbitopathy.

Purpose: To study the histopathologic features of the Mueller muscle in chronic eyelid retraction caused by thyroid-associated orbitopathy. To investigate if the degree of eyelid retraction correlates with any histopathologic finding. Methods: A prospective case series of 23 consecutive patients with thyroid-associated orbitopathy was studied. Specimens were obtained during a standard muellerectomy. Formalin-preserved specimens were studied with the use of hematoxylin-eosin, periodic acid–Schiff, Masson trichrome, and Giemsa stains. Immunostaining against leukocyte common antigen, L26, CD3, and KP-1 was performed. Three control specimens were also evaluated in a similar fashion. Fresh tissue was placed in cold glutaraldehyde overnight, postfixed, dehydrated, and infiltrated with epoxy resin. Silver (70 nm) sections were cut and stained with uranyl acetate and lead citrate for electron microscopic examination. Results: On light microscopy, fibrosis and mast cell infiltration was present in all 23 specimens. Fat infiltration was noted in 16 of 23 specimens and did not correlate with increasing age of the patient. Interstitial edema and lymphocytic infiltration were not observed. On immunohistochemistry, leukocyte common antigen was positive, confirming the presence of inflammation. L26, CD3, and KP1 were negative. Electron microscopy demonstrated fibrosis, mast cells, and abundant contracting Mueller cells. The degree of clinical retraction in millimeters did not correlate with fibrosis, inflammation, or fat infiltration. The control specimens demonstrated rare fat and mast cell infiltration and no fibrosis. Conclusions: Contrary to previous reports, the Mueller muscle is involved in the inflammation and fibrosis that characterizes thyroid-associated orbitopathy. The Mueller muscle is grossly enlarged. On histopathologic inspection, fibrosis, fatty infiltration, and increased mast cell presence accompany focal atrophy of the Mueller muscle. In concordance with prior descriptions, many Mueller cells are in an actively contracting state on electron microscopy.

The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases.

Schwannomas (neurilemomas) are benign tumors that arise from Schwann cells in the peripheral nervous system. The most commonly involved nerves that cause neuro-ophthalmic manifestations are cranial nerves V and VIII. In this series of three women, schwannomas presented as intraconal masses that mimicked a cavernous hemangioma, a superior orbital mass transgressing the superior orbital fissure, and an expansive frontal lobe mass with clinical symptoms and signs of increased intracranial pressure. Although all three complained of visual blurring, none of our patients presented with Vth or VIIIth cranial nerve dysfunction. Histopathologic studies demonstrated well-circumscribed, encapsulated spindle-cell lesions with classic Antoni A and B patterns. Histopathologic examination is essential to confirm the diagnosis of a schwannoma that may be otherwise clinically confusing. Direct optic nerve compression, globe indentation with induced hyperopia, or increased intracranial pressure with optic nerve compromise may be responsible for visual symptoms. A multidisciplinary approach is often required because of the size and location of schwannomas.

Treatment of failed Adult Chiari Malformation decompression with CSF drainage: observations in six patients

Summary. Objective: We report the use of CSF drainage for the management of failed Adult Chiari Malformation (ACM) decompression. Methods: All patients with more than one year follow-up after treatment of their failed ACM were included in this study. They underwent initial decompression between September 1998 and April 2000. Clinical and radiological data were collected initially and at recurrence. Lumbar punctures (LP) were done at recurrence for diagnostic and therapeutic purposes. Opening pressures and symptomatic relief were recorded. Therapeutic options included intermittent LP and ventriculo-peritoneal shunting (VPS). Results: There were 6 patients (5 females and one male). Their age ranged from 19 to 43 years. Tonsillar descent ranged from 5 to 21 mm. The symptoms recurred 1.5 to 9 months postoperatively (average 5.6 months). Postoperative imaging revealed the presence of CSF flow behind the tonsils and the formation of a retrotonsillar neocistern in all patients. On LP, the opening pressure ranged from 17 to 31 cm of water (average 23 cm). All patients improved after CSF drainage, and four patients underwent VPS. The other patients were treated with repeat LP±Acetazolamide. There was significant improvement in all patients, with 18 months follow-up after CSF drainage (range 16–21 months). Conclusion: Our results suggest a role for CSF drainage in the treatment of some patients with failed ACM surgery. Possible explanations for the failure of ACM surgery in this subgroup include: surgical complications leading to neural hydrodynamic alteration, inadequate initial surgery, and coexistence with another pathology, possibly a mild form of intracranial hypertension. More prospective and hydrodynamic studies are needed to further clarify these issues.

Radiosensitive orbital inflammation associated with temporal arteritis.

A 75-year-old woman developed acute loss of vision in the OD, ipsilateral periocular pain, an afferent pupillary defect, sectoral optic disc edema, and later ipsilateral proptosis and an intraconal mass. She denied any symptoms of temporal arteritis, and a sedimentation rate was normal. Orbital biopsy demonstrated chronic granulomatous inflammation with perivasculitis. A temporal artery biopsy disclosed findings consistent with temporal arteritis. Following 2000 cGy of external beam radiation, her visual function and orbitopathy completely resolved. This unusual presentation of orbital inflammation in association with temporal arteritis demonstrates that pathologic findings of temporal arteritis may be clinically nonspecific and that external beam radiation may be an effective therapy in this setting.

Liposarcoma of the orbit : A management challenge

Summary: A previously healthy 3 5-year-old man was experiencing slowly progressive, painless proptosis of the right eye. Visual function was normal, but supraduction was limited. Computed tomography revealed a superior, extraconal orbital mass. Subtotal excision was performed, and a diagnosis of liposarcoma was rendered only with expert analysis. Despite subsequent orbital exenteration and postoperative radiation, a local recurrence developed 5 years later. The clinical features that predict recurrence, and management options that may promote longevity, are discussed.

Adult Chiari Malformation

Learning Objectives: After reading this article, the participant should be able to:1. Identify the epidemiologic and pathophysiologic aspects of adult Chiari malformation.2. Describe the clinical presentation and diagnostic workup of patients with adult Chiari malformation.3. Explain the indications for and complications of surgical treatment of adult Chiari malformation.

Visual field deficit caused by vascular compression from a suprasellar meningioma: case report.

OBJECTIVE AND IMPORTANCE Suprasellar meningiomas typically cause bitemporal hemianopsia by direct compression of the chiasm, its blood supply, or both. We report another mechanism for visual loss in suprasellar meningiomas, whereby compression by the suprajacent vascular complex is the offending agent. CLINICAL PRESENTATION A 78-year-old woman with a suprasellar meningioma was diagnosed incidentally. During the follow-up period, she developed an inferior homonymous wedge defect consistent with superior compression, without any detectable radiological progression. It was decided to resect her tumor. INTERVENTION The patient underwent a fronto-orbital approach for tumoral excision. Intraoperatively, a groove by the anterior cerebral artery complex was found along the superior surface of the chiasm. Postoperatively, the patient’s visual deficit resolved. CONCLUSION This case illustrates an unusual visual field deficit associated with a suprasellar meningioma. It also emphasizes the importance of frequent and careful visual field monitoring, which can precede radiological and symptomatic progression.

ORBITAL DISEASE, OPTIC NERVE AND CHIASM

The diagnosis and management of diseases of the orbit, optic nerve and chiasm continue to evolve. Advances in neuroradiology are remarkable and continue to expand diagnosis and management. This review will highlight the clinical assessment of disease, orbital and neuroimaging, medical and surgical management.