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Brenton Nash, Matthew L. Carlson and Jamie J. Van Gompel

OBJECTIVE

The objective of this study was to examine operative outcomes in cases of microvascular decompression (MVD) of cranial nerve (CN) VIII for tinnitus through a critical review of the literature.

METHODS

Forty-three English-language articles were gathered from PubMed and analyzed. In this review, two different case types were distinguished: 1) tinnitus-only symptomatology, which was defined as a patient with tinnitus with or without sensorineural hearing loss; and 2) mixed symptomatology, which was defined as tinnitus with symptoms of other CN dysfunction. This review reports outcomes of those with tinnitus-only symptoms.

RESULTS

Forty-three tinnitus-only cases were found in the literature with a 60% positive outcome rate following MVD. Analysis revealed a 5-year cutoff of preoperative symptom duration before which a good outcome can be predicted with 78.6% sensitivity, and after which a poor outcome can be predicted with 80% specificity.

CONCLUSIONS

As the 60% success rate is more promising than several other therapeutic options open to the chronic tinnitus sufferer, future research into this field is warranted.

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Kathryn M. Van Abel, Matthew L. Carlson, Colin L. Driscoll, Brian A. Neff and Michael J. Link

Object

The authors' objective was 2-fold: 1) to compare outcomes of microsurgical resection for vestibular schwannoma (VS) between patients aged 70 years or older and patients younger than 70 years and 2) to test the hypothesis that symptomatic tumors in elderly patients represent a more aggressive variant of VS than those in younger adults, resulting in increased morbidity and a higher rate of recurrence after subtotal resection (STR).

Methods

A retrospective matched cohort study was conducted. Patients aged 70 years or older who had undergone microsurgical resection of VS were matched to adult patients younger than 70 years; the matching was performed on the basis of surgical approach, completeness of resection, and tumor size. Associations between clinical, radiographic, and surgical data and postoperative outcome were analyzed using conditional logistic regression.

Results

Twenty patients aged 70 years or older (mean age ± SD 75.9 ± 5.3, range 70–86 years) were identified and matched to a cohort of younger adult patients (mean age ± SD 55.7 ± 13.8, range 25–69 years). The mean tumor size in both groups was approximately 3 cm. Overall, the elderly patients had a poorer preoperative American Society of Anesthesiology physical status score (p = 0.038), were more likely to report imbalance (OR 9.61, p = 0.016), and more commonly exhibited compromised balance and coordination (OR 9.61, p = 0.016) than patients in the younger cohort.

There were no differences between the 2 cohorts in perioperative complications (p = 0.26) or facial nerve function (p > 0.5) at any time. The elderly patients were 13 times more likely to have long-term postoperative imbalance (OR 13.00, p = 0.013) than the younger patients. Overall, 9 tumors recurred among 32 patients undergoing STR; 6 of these patients underwent additional interventions (stereotactic radiosurgery in 5 patients and microsurgery in 1) and showed no evidence of tumor progression at the last follow-up. The median growth rate of the recurrent tumor in the 6 elderly patients was 4.8 mm/year (range 2.1–14.9 mm/year) and, in the 3 control patients, 2.2 mm/year (range 1.9–4.0 mm/year). Overall, the mortality data showed a trend toward statistical significance (p = 0.068) with a higher risk of death in the elderly.

Conclusions

As the number of elderly patients with VS increases, microsurgical resection will remain an important management option for these patients. Despite a poorer preoperative physical status in elderly patients, their morbidity profiles are similar to those in adult patients younger than 70 years. However, elderly patients may require longer convalescence due to prolonged postoperative imbalance. Not surprisingly, overall diminished functional reserve and advanced comorbidities may increase the mortality risk associated with surgical intervention in the elderly patients. Finally, there was a high risk of further tumor growth following STR in the elderly patients (6 [37.5%] of 16), underscoring the need for close postoperative radiological surveillance and consideration of early stereotactic radiosurgery for the tumor remnant following the STR.

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Jonathan P. S. Knisely, Rohan Ramakrishna and Theodore H. Schwartz

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Matthew L. Carlson, Jeffrey T. Jacob, Bruce E. Pollock, Brian A. Neff, Nicole M. Tombers, Colin L. W. Driscoll and Michael J. Link

Object

The goals of this retrospective cohort study were as follows: 1) to describe the long-term prevalence and timing of hearing deterioration following low-dose (12- to 13-Gy marginal dose) stereotactic radiosurgery (SRS) for vestibular schwannoma (VS); and 2) to identify clinical variables associated with long-term preservation of useful hearing following treatment.

Methods

Patients with serviceable hearing who underwent SRS for VS between 1997 and 2002 were studied. Data including radiosurgery treatment plans, tumor characteristics, pre- and posttreatment pure tone average, speech discrimination scores, and American Academy of Otolaryngology–Head and Neck Surgery hearing class were collected. Time to nonserviceable hearing was estimated using the Kaplan-Meier method. Univariate and multivariate associations with time to nonserviceable hearing were evaluated using Cox proportional hazards regression models.

Results

Forty-four patients met the study criteria and were included. The median duration of audiometric follow-up was 9.3 years. Thirty-six patients developed nonserviceable hearing at a mean of 4.2 years following SRS. The Kaplan-Meier estimated rates of serviceable hearing at 1, 3, 5, 7, and 10 years following SRS were 80%, 55%, 48%, 38%, and 23%, respectively. Multivariate analysis revealed that pretreatment ipsilateral pure tone average (p < 0.001) and tumor size (p = 0.009) were statistically significantly associated with time to nonserviceable hearing.

Conclusions

Durable hearing preservation a decade after low-dose SRS for VS occurs in less than one-fourth of patients. Variables including preoperative hearing capacity and tumor size may be used to predict hearing outcomes following treatment. These findings may assist in pretreatment risk disclosure. Furthermore, these data demonstrate the importance of long-term follow-up when reporting audiometric outcomes following SRS for VS.

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Michelle A. Holman, William R. Schmitt, Matthew L. Carlson, Colin L. W. Driscoll, Charles W. Beatty and Michael J. Link

Object

The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors.

Methods

The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed.

Results

Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9–18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon.

Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening.

Conclusions

Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.

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Brian A. Neff, Matthew L. Carlson, Megan M. O'Byrne, Jamie J. Van Gompel, Colin L. W. Driscoll and Michael J. Link

OBJECTIVE

The aim of this study was to evaluate the incidence, presentation, and treatment outcomes of trigeminal nerve–mediated symptoms secondary to large vestibular schwannomas (VSs) with trigeminal nerve contact. Specifically, the symptomatic results of pain, paresthesias, and numbness after microsurgical resection or stereotactic radiosurgery (SRS) were examined.

METHODS

The authors conducted a retrospective review of a database for concomitant diagnosis of trigeminal neuralgia (TN) or trigeminal neuropathy and VS between 1994 and 2014 at a tertiary academic center. All patients with VS with TN or neuropathy were included, with the exception of those patients with neurofibromatosis Type 2 and patients who elected observation. Patient demographic data, symptom evolution, and treatment outcomes were collected. Population data were summarized, and outcome comparisons between microsurgery and SRS were analyzed at last follow-up.

RESULTS

Sixty (2.2%) of 2771 total patients who had large VSs and either TN or neuropathy symptoms met inclusion criteria. The average age of trigeminal symptom onset was 53.6 years (range 24–79 years), the average age at VS diagnosis was 54.4 years (range 25–79 years), and the average follow-up for the microsurgery and SRS groups was 30 and 59 months, respectively (range 3–132 months). Of these patients, 50 (83%) had facial numbness, 16 (27%) had TN pain, and 13 (22%) had paresthesias (i.e., burning or tingling). Subsequently, 50 (83%) patients underwent resection and 10 (17%) patients received SRS.

Treatment of VS with SRS did not improve trigeminal symptoms in any patient. This included 2 subjects with unimproved facial numbness and 4 patients with worsened numbness. Similarly, SRS worsened TN pain and paresthesias in 5 patients and failed to improve pain in 2 additional patients. The Barrow Neurological Institute neuralgia and hypesthesia scale scores were significantly worse for patients undergoing SRS compared with microsurgery.

Resection alleviated facial numbness in 22 (50%) patients, paresthesias in 5 (42%) patients, and TN in 7 (70%) patients. In several patients, surgery was not successful in relieving facial numbness, which failed to improve in 17 (39%) cases and became worse in 5 (11%) cases. Also, surgery did not change the intensity of facial paresthesias or neuralgia in 6 (50%) and 3 (25%) patients, respectively. Microsurgery exacerbated facial paresthesias in 1 (8%) patient but, notably, did not aggravate TN in any patient.

CONCLUSIONS

Overall, resection of large VSs provided improved outcomes for patients with concomitant TN, facial paresthesia, and numbness compared with SRS. However, caution should be used when counseling surgical candidates because a number of patients did not experience improvement. This was especially true in patients with preoperative facial numbness and paresthesias, who frequently reported that these symptoms were unchanged following surgery.

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Matthew L. Carlson, Kathryn M. Van Abel, William R. Schmitt, Colin L. W. Driscoll, Brian A. Neff, John I. Lane and Michael J. Link

Object

The authors describe the unique occurrence of nodular enhancement within the fundus of the internal auditory canal (IAC) lateral to the preoperative radiological tumor margin following gross-total vestibular schwannoma (VS) resection.

Methods

The nature of the study was a retrospective chart review of records. The authors reviewed the cases of all patients who underwent microsurgical resection of a VS between January 2000 and January 2010 at a single tertiary referral center. Patients with incomplete resection, neurofibromatosis Type 2, and those with fewer than 2 postoperative MR images available for review were excluded.

Postsurgical patients with IAC enhancement located lateral to the preoperative imaging–delineated tumor margin were identified. Lesion morphology was characterized on serial MR imaging studies. Clinical follow-up and outcomes were recorded.

Results

Over the past decade, 350 patients underwent microsurgical VS resection. Of these, 16 patients met study criteria and were found to have postsurgical enhancement in the distal aspect of the IAC lateral to the imaging limits of the preoperative tumor margin on the first postoperative MR imaging study (37.5% women, median age 45 years). Initial MR imaging was performed at a mean of 3.1 months following surgery, and the mean radiological follow-up duration was 39.8 months (range 16.4–101.9 months). None of the 16 patients developed recurrence during the follow-up course.

Conclusions

In contrast to previous publications that have reported a high rate of recurrence in cases involving nodular enhancement within the original tumor bed, postoperative enhancement in the IAC lateral to the original tumor margin appears to carry much less risk for tumor recurrence. These findings may be helpful when counseling patients on the recommended frequency of postoperative follow-up imaging.

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Alexander P. Marston, Jeffrey T. Jacob, Matthew L. Carlson, Bruce E. Pollock, Colin L. W. Driscoll and Michael J. Link

OBJECTIVE

Over the last 30 years, stereotactic radiosurgery (SRS) has become an established noninvasive treatment alternative for small- to medium-sized vestibular schwannoma (VS). This study aims to further define long-term SRS tumor control in patients with documented pretreatment tumor growth for whom conservative observation failed.

METHODS

A prospective clinical database was queried, and patients with sporadic VS who elected initial observation and subsequently underwent SRS after documented tumor growth between 2004 and 2014 were identified. Posttreatment tumor growth or shrinkage was determined by a ≥ 2-mm increase or decrease in maximum linear dimension, respectively.

RESULTS

Sixty-eight patients met study inclusion criteria. The median pre- and posttreatment observation periods were 16 and 43.5 months, respectively. The median dose to the tumor margin was 13 Gy (range 12–14 Gy), and the median maximum dose was 26 Gy (range 24–28 Gy). At the time of treatment, 59 tumors exhibited extracanalicular (EC) extension, and 9 were intracanalicular (IC). Of the 59 EC VSs, 50 (85%) remained stable or decreased in size following treatment, and 9 (15%) enlarged by > 2 mm. Among EC tumors, the median pretreatment tumor growth rate was 2.08 mm/year for tumors that decreased or were stable, compared with 3.26 mm/year for tumors that grew following SRS (p = 0.009). Patients who demonstrated a pretreatment growth rate of < 2.5 mm/year exhibited a 97% tumor control rate, compared with 69% for those demonstrating ≥ 2.5 mm/year of growth prior to SRS (p = 0.007). No other analyzed variables were found to predict tumor growth following SRS.

CONCLUSIONS

Overall, SRS administered using a marginal dose between 12–14 Gy is highly effective in treating VSs in which initial observation fails. Tumor control is achieved in 97% of VSs that exhibit slow (< 2.5 mm/year) pretreatment growth; however, SRS is less successful in treating tumors exhibiting rapid growth (≥ 2.5 mm/year).

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Jonathan A. Forbes, Matthew L. Carlson, Saniya S. Godil, Marc L. Bennett, George B. Wanna and Kyle D. Weaver

In this publication, video format is utilized to review the operative technique of retrosigmoid craniotomy for resection of acoustic neuroma with attempted hearing preservation. Steps of the operative procedure are reviewed and salient principles and technical nuances useful in minimizing complications and maximizing efficacy are discussed.

The video can be found here: http://youtu.be/PBE5rQ7B0Ls.

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Matthew L. Carlson, Jeffrey T. Jacob, Elizabeth B. Habermann, Amy E. Glasgow, Aditya Raghunathan and Michael J. Link

OBJECTIVE

Malignant peripheral nerve sheath tumors (MPNSTs) of the eighth cranial nerve (CN) are exceedingly rare. To date the literature has focused on MPNSTs occurring after radiation therapy for presumed benign vestibular schwannomas (VSs), while MPNSTs arising without prior irradiation have received little attention. The objectives of the current study are to characterize the epidemiology, clinical presentation, disease course, and outcome using a large national cancer registry database and a systematic review of the English literature. Additionally, a previously unreported case is presented.

METHODS

The authors conducted an analysis of the Surveillance, Epidemiology, and End Results (SEER) database, a systematic review of the literature, and present a case report. Data from all patients identified in the SEER database with a diagnosis of MPNST involving the eighth CN, without a history of prior radiation, were analyzed. Additionally, all cases reported in the English literature between January 1980 and March 2015 were reviewed. Finally, 1 previously unreported case is presented.

RESULTS

The SEER registries identified 30 cases between 1992 and 2012. The average incidence was 0.017 per 1 million persons per year (range 0.000–0.0687 per year). The median age at diagnosis was 55 years, and 16 (53%) were women. Thirteen cases were diagnosed upon autopsy. Of the 17 cases diagnosed while alive, the median follow-up was 118 days, with 3 deaths (18%) observed. When compared with the incidence of benign VS, 1041 VSs present for every 1 MPNST arising from the eighth CN. Including a previously unreported case from the authors' center, a systematic review of the English literature yielded 24 reports. The median age at diagnosis was 44 years, 50% were women, and the median tumor size at diagnosis was 3 cm. Eleven patients (46%) reported isolated audiovestibular complaints typical for VS while 13 (54%) exhibited facial paresis or other signs of a more aggressive process. Treatment included microsurgery alone, microsurgery with adjuvant radiation, or microsurgery with chemoradiation. Sixty-one percent of patients receiving treatment experienced recurrence, 22% of which were diagnosed with drop metastases to the spine. Ultimately, 13 patients (54%) died of progressive disease at a median of 3 months following diagnosis. The ability to achieve gross-total resection was the only feature that was associated with improved disease-specific survival.

CONCLUSIONS

MPNSTs of the eighth CN are extremely rare and portend a poor prognosis. Nearly half of patients initially present with findings consistent with a benign VS, often making an early diagnosis challenging. In light of these data, early radiological and clinical follow-up should be considered in those who elect nonoperative treatment, particularly in patients with a short duration of symptoms or atypical presentation. These data also provide a baseline rate of malignancy that should be considered when estimating the risk of malignant transformation following stereotactic radiosurgery for VS.