Roger E. Turbin

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.

Effects of weight loss on the course of idiopathic intracranial hypertension in women

Objective: To determine the role of weight loss in the treatment of idiopathic intracranial hypertension (IIH) in obese women. Methods: Chart review of 250 patients with suspected IIH revealed 58 women who met our criteria, did not undergo early surgical intervention, and had adequate documentation of visual status, papilledema, and weight at the baseline evaluation and at 6 months or longer. Patients were divided into two groups based on whether weight loss ≥2.5 kg occurred during any 3-month interval. Papilledema grade, visual acuity, and visual field grade at 6 months or longer and the time to improve each were recorded. Results: Mean time in months to improve one grade for papilledema and visual field in one eye was 4.0 versus 6.7 (p = 0.013) and 4.6 versus 12.2(p = 0.032), respectively, for the 38 patients with weight loss compared with the 20 patients with no weight loss. Papilledema resolved in 28/38 with weight loss (mean, 7.6 months) and 8/20 without weight loss (mean, 10.2 months; p = 0.352). There were no differences in final visual acuity or visual field between the two groups, but the papilledema grade was slightly better in the worst eye in each patient at baseline in the weight loss group (p = 0.03). Conclusions: Weight reduction is associated with more rapid recovery of both papilledema and visual field dysfunction in patients with IIH compared with those who do not lose weight.

Diagnosis and treatment of orbital optic nerve sheath meningioma

BACKGROUND Primary and secondary optic nerve sheath meningiomas (ONSMs) are neoplasms that account for a large proportion of optic nerve and orbital tumors. The diagnosis is not always straightforward and is based on the appropriate clinical findings and neuroimaging. Biopsy or surgical intervention may occasionally be necessary but is associated with significant morbidity. METHODS Issues related to clinical signs and symptoms, diagnosis, natural history, and treatment strategies are reviewed based on a review of published literature. RESULTS Diagnosis is usually based on radiographic and clinical findings. Biopsies are not obtained in most cases, thus adding further to the bias of possible misdiagnosis in all reported case series that do not have the benefit of histopathologic confirmation. Natural history typically shows inexorable progression in most cases, although long periods of stability are occasionally reported. Treatment options include observation, radiation alone, surgery alone, and combined radiation and surgery. The optimum timing of interventional therapy and radiation are evolving. CONCLUSIONS After serial examination documents new decline in acuity and/or visual field, fractionated radiotherapy appears most likely to preserve visual function and is a valid treatment approach for primary orbital ONSM. Tumor enlargement, as determined by serial imaging, may also provide an indication to begin radiotherapy.

Adalimumab-associated optic neuritis

We present, to our knowledge, the first published cases of optic neuritis associated with adalimumab, a medication in the class of anti-tumor necrosis factor-alpha (TNF-alpha) antagonists. Approved in recent years by the FDA, adalimumab (Humira, Abbott Laboratories; Abbott Park, IL) is a recombinant monoclonal antibody that targets and blocks the physiologic effects of TNF. Other TNF antagonists have had associations with optic neuritis and demyelinating events.

Sarcoidosis of the anterior visual pathway: 24 new cases.

Objectives To describe the clinical spectrum and a rational approach to the diagnosis of anterior visual pathway sarcoidosis. Methods Retrospective chart review of all patients examined in neuro-ophthalmic consultation by 1 author from 1989 to 1998 with a diagnosis of sarcoidosis. Results There were 24 patients (17 female, 7 male, mean age 40 years) with anterior visual pathway sarcoidosis, 17 (71%) of whom were not previously known to have sarcoidosis. Visual acuity ranged from 20/20 to NLP. Normal fundi were observed in 15%. Among the 85% who had fundus abnormalities, pallor was present in 55%, disc edema in 26%, periphlebitis/sheathing in 14%, and optic disc granuloma in 10%. Ten patients (42%) had uveitis, active in only 3 (13%). An elevated angiotensin-converting enzyme (ACE) was present in 16 (76%) of 21 patients tested; evidence of sarcoidosis on chest radiograph was present in 13 (72%) of 18; gallium scanning was abnormal in 13 (93%) of 14; neuroimaging abnormalities of the optic nerves, chiasm, or tract were present in 16 (70%) of 23; lymphocytic pleocytosis or elevated cerebrospinal fluid protein was identified in 14 (88%) of 16 patients, with both values elevated in 7 (44%) patients. Histologic confirmation was obtained in 13 (81%) of 16 who underwent biopsy; in the remaining patients, diagnosis was based entirely on clinical and laboratory evidence. Conclusions Anterior visual pathway disease may be underrecognized as a presentation of sarcoidosis. Classic fundus findings of periphlebitis and optic granuloma are typically absent. An aggressive diagnostic evaluation may help establish the diagnosis early in its course.

Isolated Progressive Visual Loss after Coiling of Paraclinoid Aneurysms

BACKGROUND AND PURPOSE: The proximity of the paraclinoid segment of the internal carotid artery to the visual pathways may result in visual deficits when patients present with aneurysms in this segment. Although surgical clip ligation of these aneurysms has been the standard of care for decades, the advent of coil embolization has permitted endovascular therapy in those aneurysms with favorable dome-to-neck ratios. Although immediate nonprogressive visual loss after coil embolization of paraclinoid aneurysms has been well described, isolated progressive visual loss immediately or shortly following coil embolization, to our knowledge, has not. We have identified 8 patients who experienced progressive loss of vision, unassociated with any other neurologic deficits, developing immediately or shortly after apparently uncomplicated coil embolization of a paraclinoid aneurysm. MATERIALS AND METHODS: This study is a retrospective case series of 8 patients seen at 4 separate academic institutions. Inpatient and outpatient records were examined to determine patient demographics, previous ocular and medical history, and ophthalmic status before endovascular embolization. In addition, details of the primary endovascular therapy and subsequent surgical and nonsurgical interventions were recorded. Follow-up data, including most recent best-corrected visual acuity, postoperative course, and duration of follow-up were documented. RESULTS: Eight patients developed progressive visual loss in 1 or both eyes immediately or shortly after apparently uncomplicated coiling of a paraclinoid aneurysm. MR imaging findings suggested that the visual loss was most likely caused by perianeurysmal inflammation related to the coils used to embolize the aneurysm, enlargement or persistence of the aneurysm despite coiling, or a combination of these mechanisms. Most patients experienced improvement in vision, 2 apparently related to treatment with systemic corticosteroids. CONCLUSION: Patients in whom endovascular treatment of a paraclinoid aneurysm is contemplated should be warned about the potential for both isolated nonprogressive and progressive visual loss in 1 or both eyes. Patients in whom progressive visual loss occurs may benefit from treatment with systemic corticosteroids.

Isolated sixth nerve palsy: an uncommon presenting sign of multiple sclerosis.

Abstract We describe three patients in whom an isolated sixth nerve palsy was the only clinical symptom or sign of multiple sclerosis (MS). Data were collected prospectively over 6 years on these three patients, who showe no other signs of brainstem dysfunction or prior symptoms; in addition. Retrospective analysis of all patients with MS and all patients with sixth nerve palsy referred to a neuro-ophthalmology service between 1982 and 1998 showed isolated sixth nerve palsy to be the presenting sign of MS in only 0.5% of these patients. MS was the cause of isolated sixth nerve palsy in 0.8% of all patients and in 1.6% of those aged 18–50 years. Although it has been previously suggested that sixth nerve palsy is an ot uncommon presenting sign of MS, our results suggest it is rare.

In the eyes of the law: malpractice litigation in oculoplastic surgery.

Purpose: To assess characteristics associated with various outcomes of malpractice litigation, resulting from injuries sustained during oculoplastic procedures. Methods: The Westlaw legal database (Thomson Reuters, New York, NY, U.S.A.) was used to obtain jury verdicts and settlements. Pertinent data were extracted from 69 malpractice cases litigated from 1988 to 2012 involving oculoplastic procedures, including alleged cause of malpractice, outcome, and defendant specialty. Results: The most commonly litigated surgical procedures were blepharoplasty (63.8% of total) and brow lift surgery (11.6%). The most commonly alleged complications included excessive scarring (24.6%), lagophthalmos (24.6%), visual defects (23.2%), and exposure keratitis (21.7%). Plastic surgeons were the most commonly named defendants (46.4%), followed by both comprehensive ophthalmologists and fellowship-trained ophthalmic plastic surgeons (17.3% each). A defense verdict was held in 60.9% of cases, a plaintiff verdict in 31.9% of cases, and a settlement was reached in 7.2% of cases. Blindness, cranial nerve injury, and the allegation of a permanent deficit increased the likelihood of a case being resolved with payment to the plaintiff (Fisher exact tests, p < 0.05). Conclusions: Most litigated oculoplastic malpractice cases were resolved in favor of the defendant, while settlements and plaintiff decisions averaged

Efficacy of corticosteroids and external beam radiation in the management of moderate to severe thyroid eye disease.

455,703. Blepharoplasty constituted two-thirds of cases, with the most frequently cited associated complications being unsightly scarring, lagophthalmos, and visual deficits. An alleged lack of informed consent (30.4%) or the need for additional surgery (39.1%) was present in a considerable proportion of cases, emphasizing the importance of a detailed informed consent and clear communication preoperatively regarding patient expectations.

Effects of Prone and Reverse Trendelenburg Positioning on Ocular Parameters

Thyroid Eye Disease (TED, Graves ophthalmopathy, thyroid ophthalmopathy) is the most common cause of orbital inflammation and proptosis in adults. There is no agreement on its management although corticosteroids and external beam orbital radiation (XRT) have traditionally been believed to provide benefit in active inflammation. Our review of the published literature in English disclosed an overall corticosteroid-mediated treatment response of 66.9% in a total of 834 treated patients who had moderate or severe TED. Intravenous corticosteroids used in repeated weekly pulses were more effective (overall favorable response = 74.6%, n = 177) and had fewer side effects than daily oral corticosteroids (overall favorable response = 55.5%, n = 265). A combination of corticosteroid and radiation therapy seemed to be more effective than corticosteroids alone. Our conclusions are tempered by a notable lack of standardization within and between study designs, treatment protocols, and outcome measures. Accordingly, the North American Neuro-Ophthalmology Society (NANOS), American Society of Ophthalmic Plastic and Reconstructive Surgery (ASOPRS) and the Orbital Society, in conjunction with Neuro-Ophthalmology Research and Development Consortium (NORDIC), will investigate the design and funding of a multi-center controlled trial.