Adenoid cystic carcinoma of the lacrimal gland is frequently characterized by MYB rearrangement.

PurposeAdenoid cystic carcinoma (ACC) represents ~10-15% of salivary neoplasms and almost universally exhibits a lethal clinical course. ACC is also known to occur in the lacrimal gland. ACC is characterized by its heterogeneous morphology and may demonstrate tubular, cribriform, and/or solid architectural patterns. Unfortunately, these histopathological features are not specific to ACC and can be seen in other salivary gland-type neoplasms, introducing a diagnostic dilemma. The discovery of fusion transcripts has revolutionized the diagnosis, surveillance, and treatment of epithelial malignancies. In several anatomic subsites ACC is frequently characterized by a fusion transcript involving genes MYB and NFIB; more specifically, t(6;9)(q22-23;p23-24). This study explores the incidence of MYB rearrangement in cases of lacrimal gland ACC using fluorescent in situ hybridization.Materials and methodsRetrospective clinical and histopathological review of 12 cases of lacrimal gland ACC seen at Mayo Clinic over a 25-year period (1990-2015) was performed. Demographic and clinical data were obtained from medical records. Surgical pathology archival material including H&E slides and immunostains was re-examined. Formalin-fixed paraffin-embedded material was further evaluated using immunohistochemistry when appropriate. Fluorescent in situ hybridization (FISH) using a MYB break-apart probe was applied to all histologically confirmed cases of ACC and benign salivary gland parenchyma.ResultsThe median patient age was 53.6 years (range 12-64) and distributed equally by gender (six male and six female). Rearrangement of MYB was identified using FISH in seven cases (58%). Twenty-five sections of benign salivary gland parenchyma showed no evidence of MYB rearrangement. Primary surgical resection was most common treatment, and 78% of the patient received adjuvant radiation therapy. Median overall survival (OS) was 11 years. Rearrangement of MYB did not affect OS.ConclusionsIn summary, our results indicate that the MYB rearrangement defines a significant subset of lacrimal gland ACCs. Importantly, FISH for MYB rearrangement may be used as a diagnostic tool during pathological examination of lacrimal gland neoplasms. Our results showed no relationship between rearrangement status and clinical outcome. Lastly, the presence of t(6;9) in ACC may provide a platform for molecular-targeting strategies in the future.

Immunoglobulin G4-related disease of the orbit: imaging features in 27 patients.

BACKGROUND AND PURPOSE: Immunoglobulin G4-related disease is a systemic fibroinflammatory process of unknown etiology, characterized by tissue infiltration by immunoglobulin G4 plasma cells. The purpose of this study was to retrospectively identify the spectrum of imaging features seen in immunoglobulin G4-related disease of the orbit. MATERIALS AND METHODS: This study included 27 patients with biopsy-proved immunoglobulin G4-related disease of the orbit and either a CT or MR imaging of the orbits. These CT or MR imaging examinations were evaluated for the following: extraocular muscle size, extraocular muscle tendon enlargement, lacrimal gland enlargement, infiltrative process in the orbital fat (increased attenuation on CT or abnormal signal on MR imaging), infraorbital nerve enlargement, mucosal thickening in the paranasal sinuses, and extension of orbital findings intracranially. RESULTS: Extraocular muscles were enlarged in 24 of 27 (89%) patients, 21 (88%) bilaterally. In 32 of 45 (71%) affected orbits, the lateral rectus was the most enlarged muscle. In 26 (96%) patients, the tendons of the extraocular muscles were spared. Nineteen (70%) patients had lacrimal gland enlargement. Twelve (44%) patients had an infiltrative process within the orbital fat. Infraorbital nerve enlargement was seen in 8 (30%) patients. Twenty-four (89%) patients had sinus disease. Cavernous sinus or Meckel cave extension was seen in 3 (11%) patients. CONCLUSIONS: In patients with extraocular muscle enlargement, particularly when the tendons are spared and the lateral rectus is the most enlarged, and even more so when other noted findings are present, immunoglobulin G4-related disease should be a leading differential consideration, even over more commonly known etiologies of extraocular muscle enlargement.

Clinical features and long-term prognosis of trochlear headaches.

BACKGROUND AND PURPOSE: Trochlear headaches are a recently recognized cause of headache, of which both primary and inflammatory subtypes are recognized. The clinical features, long-term prognosis and optimal treatment strategy have not been well defined. METHODS: A cohort of 25 patients with trochlear headache seen at the Mayo Clinic between 10 July 2007 and 28 June 2012 were identified. RESULTS: The diagnosis of trochlear headache was not recognized by the referring neurologist or ophthalmologist in any case. Patients most often presented with a new daily from onset headache (n = 22, 88%). The most characteristic headache syndrome was reported as continuous, achy, periorbital pain associated with photophobia and aggravation by eye movement, especially reading. Individuals with a prior history of migraine were likely to have associated nausea and experience trochlear migraine. Amongst individuals with trochleitis, 5/12 (41.6%) had an identified secondary mechanism. Treatment responses were generally, but not invariably, favorable to dexamethasone/lidocaine injections near the trochlea. At a median follow-up of 34 months (range 0-68), 10/25 (40%) of the cohort had experienced complete remission. CONCLUSIONS: Trochlear headaches are poorly recognized, have characteristic clinical features, and often require serial injections to optimize the treatment outcome. The identification of trochleitis should prompt neuroimaging to look for a secondary cause.

Risk factors for ischemic optic neuropathy after cardiopulmonary bypass: a matched case/control study.

UNLABELLED: Visual loss (acuity or field) secondary to ischemic optic neuropathy (ION) is a rare but devastating complication of cardiac surgery involving cardiopulmonary bypass (CPB). We determined clinical features and risk factors for ION by a retrospective time-matched, case-control study. ION was identified in 17 (0.06%) patients out of 27,915 patients who underwent CPB between January 1, 1976, and December 31, 1994. For each ION patient, two patients who underwent CPB exactly 2 wk before the ION patient were selected as controls. Data were analyzed by using conditional logistic regression with the 1:2 matched-set feature of 17 cases and 34 controls. Two-tailed P values < or =0.05 were considered significant. From bivariate analysis, smaller minimum postoperative hemoglobin concentration (odds ratio [OR] = 1.9, P = 0.047) and the presence of atherosclerotic vascular disease (OR = 7.0, P = 0.026) were found to be independently associated with ION after CPB, as were smaller minimum postoperative hemoglobin concentration (OR = 2.2, P = 0.027) and preoperative angiogram within 48 h of surgery (OR = 7.2, P = 0.042). In ION patients, 13 (76.5%) of 17 experienced a minimum postoperative hemoglobin value of < 8.5 g/dL, whereas only 14 (41.2%) of 34 control patients experienced values < 8.5 g/dL. IMPLICATIONS: Patients with clinically significant vascular disease history or preoperative angiogram may be at increased risk for ischemic optic neuropathy after cardiac surgery, especially if the hemoglobin remains low in the postoperative period.

The frequency of perioperative vision loss.

UNLABELLED: The frequency of perioperative vision loss, especially for spinal surgery, has been increasing recently. We undertook a retrospective study to determine the frequency of this outcome in a large surgical population receiving general or central neuraxis regional anesthesia for noncardiac procedures from 1986 to 1998. Specific criteria were used to separate cases in which the surgical procedure likely directly contributed to the vision loss. Vision loss was present if any part of the visual field was affected. Initial database screening found 405 cases of new-onset vision loss or visual changes in 410,189 patients who underwent 501,342 anesthetics and who survived at least 30 days after their final procedures. Two hundred sixteen of these patients regained full vision or acuity within 30 days. Of the 189 patients who developed vision deficits for longer than 30 days, 185 underwent ophthalmologic or neurologic procedures in which ocular or cerebral tissues were surgically damaged or resected. The remaining 4 patients (1 per 125,234 overall; 0.0008%) developed prolonged vision loss without direct surgical trauma to optic or cerebral tissues. In this large study population of noncardiac surgical patients, including those who underwent spinal surgical procedures, the frequency of perioperative vision loss persisting for longer than 30 days was very small. IMPLICATIONS: Vision loss and blindness after surgery and anesthesia is a very rare event. In this study, only one per 125,234 patients undergoing noncardiac surgery developed vision loss persisting for longer than 30 days.

Radiation-induced meningiomas involving the orbit.

PURPOSE: To review the clinical features and outcomes of patients with radiation-induced meningiomas involving the orbit. DESIGN: Retrospective case series. PARTICIPANTS: Eight patients with radiation-induced meningiomas affecting the orbit. METHODS: Clinical and pathologic data of the patients were reviewed. MAIN OUTCOME MEASURES: Age at diagnosis, mean interval between radiation therapy and meningioma diagnosis, tumor recurrence, histologic atypia, and mean follow-up time after initial diagnosis. RESULTS: The mean age at diagnosis was 42 years (range, 21 years to 70 years). The mean interval between radiation therapy and meningioma diagnosis was 26 years (range, 3 years to 54 years). All patients underwent gross total resection or subtotal resection of the meningioma. Five tumors (62.5%) recurred, based on clinical findings and CT imaging. The mean interval between resection of the meningioma and recurrence was 3 years (range, 9 months to 9 years). Three patients (37.5%) had atypical meningiomas. One patient (12.5%) had multiple tumors. The mean follow-up interval was 7 years after initial diagnosis of the meningioma (range, 15 months to 19 years). CONCLUSIONS: This series of radiation-induced meningiomas, the first in the ophthalmic literature, illustrates the aggressive nature of this tumor.

A prospective, randomized, double-blind, placebo-controlled study of orbital radiotherapy for Graves' ophthalmopathy.

CONTEXT: Although widely used for more than 85 years, the efficacy of radiotherapy for Graves' ophthalmopathy (GO) has not been established convincingly. OBJECTIVE: To evaluate the efficacy of radiotherapy for GO. DESIGN: Prospective, randomized, internally controlled, double-blind clinical trial in a tertiary care academic medical center. PARTICIPANTS: The patients were ethnically diverse males and females over age 30 seen in a referral practice. The patients had moderate, symptomatic Graves' ophthalmopathy (mean clinical activity score, 6.2) but no optic neuropathy, diabetes, recent steroid treatment, previous decompression, or muscle surgery. Forty-two of 53 consecutive patients were enrolled after giving informed consent and fulfilling study entry criteria. Eleven eligible patients declined to participate because of inconvenience, desire for alternative therapy, or concern about radiation. INTERVENTION: One randomly selected orbit was treated with 20 Gy of external beam therapy; sham therapy was given to the other side. Six months later, the therapies were reversed. MAIN OUTCOME MEASURES: Every 3 months for 1 year, we measured the volume of extraocular muscle and fat, proptosis, range of extraocular muscle motion, area of diplopia fields, and lid fissure width. Effective treatment for GO will modify one or more of these parameters. RESULTS: No clinically or statistically significant difference between the treated and untreated orbit was observed in any of the main outcome measures at 6 months. At 12 months, muscle volume and proptosis improved slightly more in the orbit that was treated first. CONCLUSIONS: In this group of patients, representative of those for whom radiotherapy is frequently recommended, we were unable to demonstrate any beneficial therapeutic effect. The slight improvement noted in both orbits at 12 months may be the result of natural remission or of radiotherapy, but the changes are of marginal clinical significance.

Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging.

OBJECTIVE: To demonstrate spontaneous regression of large, clinically symptomatic optic pathway gliomas in patients with and without neurofibromatosis type 1 (NF-1). METHODS: Patient cases were collected through surveys at 2 consecutive annual meetings of the North American Neuro-Ophthalmology Society (NANOS) and through requests on the NANOSNET Internet listserv. Serial documentation of tumor signal and size, using magnetic resonance imaging in 11 patients and computed tomography in 2 patients, was used to evaluate clinically symptomatic optic pathway gliomas. All tumors met radiologic criteria for the diagnosis of glioma and 4 patients had biopsy confirmation of their tumors. In 3 patients, some attempt at therapy had been made many years before regression occurred. In one of these, radiation treatment had been given 19 years before tumor regression, while in another, chemotherapy had been administered 5 years before signal changes in the tumor. In the third patient, minimal surgical debulking was performed 1 year before the tumor began to shrink. RESULTS: Spontaneous tumor shrinkage was noted in 12 patients. Eight patients did not have NF-1. In an additional patient without NF-1, a signal change within the tumor without associated shrinkage was detected. Tumor regression was associated with improvement in visual function in 10 of 13 patients, stability of function in 1, and deterioration in 2. CONCLUSIONS: Large, clinically symptomatic optic gliomas may undergo spontaneous regression. Regression was seen in patients with and without NF-1. Regression may manifest either as an overall shrinkage in tumor size, or as a signal change on magnetic resonance imaging. A variable degree of improvement in visual function may accompany regression. The possibility of spontaneous regression of an optic glioma should be considered in the planning of treatment of patients with these tumors.

Orbital lymphoma: radiotherapy outcome and complications.

BACKGROUND AND PURPOSE: Orbital non-Hodgkin's lymphomas (NHL) have traditionally been treated with radiation. Forty-eight patients presenting with orbital NHL were treated with radiation and were evaluated for local control, overall survival, cause-specific survival, and complications. MATERIALS AND METHODS: Forty-five patients had low-grade and 3 patients had intermediate-grade histologic findings. Orbit-only disease occurred in 22 patients, the conjunctiva in 16, both in five, and lacrimal gland only in five. Patient age ranged from 35 to 94 years (median, 68). Ann Arbor stages were cIEA (34), cIIEA (six), cIIIEA (two), and cIVEA (six). Radiation doses ranged between 15 and 53.8 Gy (median, 27.5 Gy). RESULTS: Follow-up ranged from 0.14 to 18.23 years (median, 5.35). Median overall survival and cause-specific survival were 6.5 and 15.5 years, respectively. Patients with clinical stage I or II disease had significantly better overall and cause-specific survival than patients with stage III or IV disease. Ten-year relapse-free survival in 41 patients with stage I or II disease was 66%. However, there was continued downward pressure on relapse-free survival out to 18 years. One local failure occurred. Twenty-five patients sustained acute complications. There were 17 minor and four major late complications. All major late complications occurred with doses more than 35 Gy. CONCLUSIONS: Excellent local control with radiation doses ranging from 15 to 30 Gy is achieved. Patients with stage I or II disease have better overall and cause-specific survival than patients with stage III or IV disease. Late relapse occurs in sites other than the treated orbit, even in patients with early-stage disease. Doses 35 Gy or higher result in significant late complications and are therefore not indicated for patients with low-grade tumors.

Refractory idiopathic intracranial hypertension treated with stereotactically planned ventriculoperitoneal shunt placement.

OBJECT: Ventriculoperitoneal (VP) shunts have not been widely used for idiopathic intracranial hypertension (IIH) because of the difficulty of placing a shunt into normal or small-sized ventricles. The authors report their experience with stereotactic placement of VP shunts for IIH. METHODS: The authors reviewed the clinical records of all patients in whom stereotaxis was used to guide the placement of a VP shunt for IIH at their institution. All shunts were placed using stereotactic guidance to target the frontal horn of the lateral ventricle. Patients were contacted at a mean postoperative interval of 15.1 months. No patients were lost to follow up. The authors identified 13 patients who underwent placement of a stereotactically guided VP shunt for IIH over a 6-year period. A trial of either acetazolamide or steroid therapy had failed in all patients. Prior surgical treatments included optic nerve sheath fenestrations in seven patients and cerebrospinal fluid diversionary procedures, other than stereotactic VP shunt procedures, in nine patients. Twelve patients reported excellent or good durable symptomatic relief at the time of follow up. No patient suffered progression of visual deficits. Four patients experienced persistent headaches following the procedure. Three patients required a revision of the VP shunt for technical failure. CONCLUSIONS: Stereotactically guided VP shunt placement is an effective and durable treatment option in many cases of IIH that are refractory to more traditional medical and surgical approaches.

Eye care for patients receiving neuromuscular blocking agents or propofol during mechanical ventilation.

BACKGROUND: The presence of a corneal reflex and the ability to maintain eye closure are instrumental in protecting the cornea. Use of neuromuscular blocking agents or propofol can result in impaired eyelid closure and loss of corneal reflex, leading to corneal exposure. The cornea is then at risk for drying, infection, and scarring, which may lead to permanent visual loss. OBJECTIVES: To determine whether applying artificial tear ointment to the eyes of paralyzed or heavily sedated patients receiving mechanical ventilation decreases the prevalence of exposure keratitis more than does passive closure of the eyelid. METHODS: A prospective, randomized control trial was done. The sample was 50 patients in the intensive care unit receiving either neuromuscular blocking agents or propofol during mechanical ventilation. In each patient, artificial tear ointment was applied to one eye; passive closure of the eyelid was used for the other eye (control eye). RESULTS: Nine patients had evidence of exposure keratitis in the untreated eye, and 2 had corneal abrasions in both the treated and the control eyes. The remaining 39 patients did not have corneal abrasions in either eye. Use of the artificial tear ointment was more effective in preventing corneal exposure than was passive eyelid closure (P = .004). CONCLUSIONS: Eye care with a lubricating ointment on a regular, set schedule can effectively reduce the prevalence of corneal abrasions in patients who are either paralyzed or heavily sedated and thus can help prevent serious complications such as corneal ulceration, infection, and visual loss.

Traumatic carotid artery dissection causing blindness.

A case of delayed postoperative visual loss due to bilateral traumatic carotid artery dissection is presented. In patients with a major craniofacial injury due to a high-speed motor vehicle accident, we suggest that carotid artery duplex ultrasonography be used in the initial evaluation for possible carotid artery dissection. Magnetic resonance imaging of the head and neck with magnetic resonance angiography should be performed subsequently if indicated. Early diagnosis and initiation of therapy can minimize complications.

Stereotactic radiosurgery and particulate embolization for cavernous sinus dural arteriovenous fistulae.

OBJECTIVE: To evaluate the safety and efficacy of stereotactic radiosurgery, either with or without transarterial embolization, in the treatment of patients with dural arteriovenous fistulae (DAVFs) of the cavernous sinus. METHODS: We reviewed the findings, from a prospectively established database, for 20 patients with cavernous sinus DAVFs who were treated with either radiosurgery alone (n = 7) or radiosurgery and transarterial embolization (n = 13) in a 7-year period. The median follow-up period after radiosurgery was 36 months (range, 4-59 mo). RESULTS: Nineteen of 20 patients (95%) experienced improvement of their clinical symptoms. Fourteen of 15 patients (93%) experienced either total (n = 13) or nearly total (n = 1) obliteration of their DAVFs, as documented by angiography performed a median of 12 months after radiosurgery. No patient experienced a recurrence of symptoms after angiography showed DAVF obliteration. Two patients developed new neurological deficits after embolization procedures. One patient exhibited temporary aphasia secondary to a venous infarction; another patient exhibited permanent VIth cranial nerve weakness related to acute cavernous sinus thrombosis. Two patients experienced recurrent symptoms and underwent repeat transarterial embolization at 7 and 12 months; both patients achieved clinical and angiographic cures (5 and 10 mo later, respectively). One patient experienced recurrent visual symptoms and underwent transvenous embolization 4 months after radiosurgery. CONCLUSION: Staged radiosurgery and transarterial embolization provided both rapid symptom relief and long-term cures for patients with cavernous sinus DAVFs. Radiosurgery alone was effective for patients with DAVFs whose arterial supply was not accessible via a transarterial approach, although the time course of symptom improvement was longer, compared with patients who also underwent embolization.

Lacrimal bypass surgery in patients with sarcoidosis.

PURPOSE: To examine the results of lacrimal bypass surgery in patients with sarcoidosis. METHODS: Patients with sarcoidosis who underwent dacryocystorhinostomy (DCR) or conjunctivodacryocystorhinostomy (CDCR) in two practice settings from 1986 through 1995 were identified and their medical records reviewed. RESULTS: Twelve patients, of whom eight were women, underwent bilateral DCR or CDCR to treat nasolacrimal duct obstruction associated with sarcoidosis. The initial diagnosis of sarcoidosis was established in four patients from a biopsy specimen obtained during DCR. The ages of the patients at diagnosis of sarcoidosis ranged from 39 to 64 years (mean, 49.6 years; median, 45.5 years), whereas their ages at the time of surgery ranged from 42 to 72 years (mean and median, 55 years). The average duration of postoperative follow-up evaluation was 44 months (median, 38.5 months; range, 10 to 82 months). All patients received local corticosteroids postoperatively, and nine patients (75%) were treated with prednisone. Of the 24 lacrimal procedures, 23 (95.8%) were patent to irrigation at the last follow-up examination, and all patients were asymptomatic. CONCLUSION: Lacrimal drainage obstruction may be the initial manifestation of sarcoidosis, and tissue obtained during DCR may help to establish the diagnosis. A successful surgical outcome may require intensive and occasionally long-term therapy with local and systemic corticosteroids.

Visual impairment due to macular disciform scars in a 20-year-old man with Smith-Magenis syndrome: another ophthalmologic complication.

We describe a 20-year-old man with Smith-Magenis syndrome and a 46,XY,del(17)(p11.2p11.2) karyotype. The interstitial deletion was confirmed by metaphase analysis using the fluorescent in situ hybridization probe (D17S29) for the Smith-Magenis region. The patient had hypertelorism, exotropia, and high myopia. Examination under anesthesia showed a lacquer crack near the right macula and a disciform scar of the left macula. Six months later, the patient presented with subacute visual loss. Examination demonstrated end-stage macula degeneration with bilateral disciform scars. There was no evidence of retinal detachment. Prior reports of Smith-Magenis syndrome mention telecanthus, ptosis, strabismus, iris anomalies, cataract, microcornea, optic nerve hypoplasia, myopia, retinal detachment, and lattice retinal degeneration. Bilateral macular degeneration has not been reported previously, and it may be an additional ophthalmologic manifestation of Smith-Magenis syndrome, either as a primary manifestation or as a direct consequence of high myopia.

Anterior visual pathway meningiomas primarily resected between 1978 and 1988: the Mayo Clinic Rochester experience.

The relapse rate, overall survival, and factors associated with a decreased recurrence-free survival rate in patients with anterior visual pathway (AVP) meningioma were compared with these features in patients who had meningiomas at other sites. Management of these patients is discussed. A review of the records of 581 consecutive patients who had primary resection of meningiomas between 1978 and 1988 identified 43 patients with AVP meningioma. Multiple clinical, surgical, and pathologic factors at the initial examination were analyzed to assess their association with recurrence, and the patients who had AVP meningioma were compared with patients who had non-AVP meningiomas to determine the factors that may influence recurrence. Recurrence-free and overall survival rates were determined. The AVP tumors were associated with a higher rate of recurrence. Subtotal resection was more common in the AVP tumors, but it alone was not associated with the decrease in recurrence-free or overall survival rates. Several factors that may explain the higher recurrence rate in patients with AVP meningioma were identified. Anterior visual pathway meningioma is associated with a higher rate of recurrence than are meningiomas at other sites. Operation remains the mainstay of treatment for symptomatic nonseeing eyes. Radiation therapy seems to be effective for managing recurrent tumor.

The role of second-intention healing in periocular reconstruction.

Second-intention healing is a time-honored method of wound management. Its role in periocular reconstruction, however, is currently controversial. The objectives of this thesis are threefold: to evaluate carefully the outcomes in a cohort of patients treated by this technique, to provide a comprehensive review of published reports, and to formulate recommendations and guidelines for appropriate application in selected patients. Eyelid and periorbital defects after excision of periocular tumors were allowed to heal by second intention in 59 patients. The locations of the wounds were the medial canthus (n = 32), lower eyelid (n = 20), upper eyelid, (n = 4), glabella (n = 2), and nasojugal fold (n = 1). Five excised areas involved the eyelid margin, and in three patients the defect included the canalicular system. The size of the defects ranged from 3 x 3 mm to 22 x 27 mm. The average duration of follow-up was 19 months (range, 6 months to 8 years). The functional and cosmetic results were satisfactory in 49 patients (83%). Complications occurred in 10 patients and included ectropion, medial canthal webbing, trichiasis, eyelid notching, and hypertrophic scarring. Only two patients, however, required secondary repair. Healing by second intention is a safe, effective, and inexpensive alternative to surgical reconstruction after tumor excision in selected patients.

Sarcoid masquerading as optic nerve sheath meningioma.

Sarcoid manifesting as an optic nerve tumor without evidence of systemic disease is uncommon. Throughout a 2-year period, a 22-year-old white woman had progressive monocular loss of vision to the level of no light perception. Optic atrophy but no uveitis was noted in the affected eye. Magnetic resonance imaging revealed thickening and enhancement of the apical optic nerve, with "tram-tracking." The presumptive diagnosis was optic nerve sheath meningioma; however, a biopsy specimen from the optic nerve revealed sarcoid. Extensive postoperative investigations revealed no systemic sarcoidosis. To our knowledge, 17 cases similar to ours, with the diagnosis proved by optic nerve biopsy, have been previously reported in the English-language literature. Most of these were mistaken preoperatively for optic nerve sheath meningioma. None of the patients had evidence of systemic sarcoidosis on initial postoperative testing. Neuroimaging, serum level of angiotensin-converting enzyme, and clinical characteristics such as age, race, sex, and optochoroidal collaterals do not distinguish optic nerve sheath meningioma from sarcoid of the optic nerve. In the absence of uveitis or systemic involvement, optic nerve sarcoid manifesting as an orbital tumor is virtually impossible to diagnose without results of biopsy.

Long-term follow-up of Graves ophthalmopathy in an incidence cohort.

PURPOSE: To provide long-term follow-up data on patients with Graves ophthalmopathy in an incidence cohort of 120 patients. METHODS: Data were obtained from a comprehensive review of each patient's community medical record, a follow-up survey, or both. RESULTS: The median interval between the initial ophthalmic examination and most recent follow-up was 9.8 years (range, 64 days to 17.4 years). Follow-up of more than 5 years was available for 96 patients (80.0 percent), whereas follow-up exceeding 10 years was achieved for 59 patients (49.2 percent). Persistent visual loss from optic neuropathy occurred in two eyes, with final visual acuities of 20/30 and 20/60, respectively. None of the patients reported deterioration of vision attributable to Graves ophthalmopathy in the interval since their last ophthalmic examination at the authors' institution. Two patients (2.2 percent) had constant diplopia, but it was correctable with spectacles (prisms) in each case. Nearly one third of respondents had had ocular discomfort during the preceding 4 weeks; the most frequent cause in 72 percent of patients was dry eyes. Among the respondents to the survey, 60.5 percent believed that the appearance of their eyes had not returned to what it had been before the development of thyroid disease, 51.6 percent thought that their eyes appeared abnormal, and 37.9 percent were dissatisfied with the appearance of their eyes. CONCLUSIONS: Although with treatment few patients have long-term functional impairment from Graves ophthalmopathy, more than one third of patients are dissatisfied with their ultimate appearance. The psychologic, aesthetic, economic, and social sequelae of the disorder require further definition by formal outcomes studies.