History of vestibular schwannoma surgery.

Attempts of surgical removal of vestibular schwannomas started 150 years ago with major limitations in terms of diagnosis and understanding of the disease but also in respect of surgical technique and instrumentation. Then came Cushing followed by Dandy, two pioneers and legendary neurosurgeons who understood the natural history of the disease and set the landmarks of the current surgery of the cerebellopontine angle. In this century of medicine, results and expectations shifted from a life-threatening affection to the actual standard of cranial nerve preservation and conservation of quality of life. In this overview, it is shown how the standard of the current surgery came from two distinct medical cultures, otologists and neurosurgeons, respectively. Now and in the near future, these competencies will be gathered in multidisciplinary teams who will display the whole panel of the treatment options in order to offer the best individual solutions for the patients.

Translabyrinthine approach for vestibular schwannomas: operative technique.

For large vestibular schwannomas (VSs) for which removal is the primary therapy, the goals are complete tumor resection and maintenance of normal neurological function. The authors analyzed their results about facial nerve preservation, extent of resection and complications following resection of large VSs via a widened translabyrinthine approach. Between 1991 and 2001, 110 patients with a unilateral large VS (Koos stage IV) were operated on using the same technique in the same institution. The main steps of the operative technique were detailed and the clinical outcomes analyzed and compared with the results that were extracted from matched series in the literature. The main postoperative complications were cerebrospinal fluid leakage through the scalp wound in 4%, cerebrospinal fluid rhinorrhea in 4% requiring surgical revision in 3%. One percent of meningitis, 1% of posterior fossa hematoma and 4% of transient lower cranial nerve palsies were observed. There was no death related to the surgery. Total tumor removal was achieved in 85% of cases, near-total in 11% and subtotal in 4% of cases. Sixty-two percent of patients obtained normal to near-normal facial function (House-Brackmann grades 1 and 2). The authors suggest that the translabyrinthine approach is a suitable route for the safe removal of large VSs.

Recurrence of vestibular schwannomas after surgery.

The issue of recurrence of vestibular schwannomas is poorly studied by the surgical literature and is probably underestimated. Our own long-term retrospective analysis after translabyrinthine approach has indicated a 9.2% recurrence rate. This long-term event is mainly due to regrowth of microfragments that have been left in the operative field along the course of the facial nerve or at the surface of the pons. Management of recurrence depends on the tumor size and patient's condition. Our current policy is to propose a Gamma Knife radiosurgical treatment in eligible cases. Prospective long-term follow-up studies using serial MR imaging after radical removal should bring reliable data about the incidence of vestibular schwannoma recurrence.

Hearing preservation in patients with unilateral vestibular schwannoma after gamma knife surgery.

INTRODUCTION: The majority of patients still lose the functionality of their hearing in spite of the technical advances in microsurgery. Our aim was to evaluate the hearing preservation potential of Gamma Knife Surgery. We have reviewed our experience and the literature in order to evaluate the probability to obtain such functional preservation and the factors influencing it. METHODS: Since July 1992, 2,053 patients have been operated on by Gamma Knife Radiosurgery in Timone University Hospital. This population included 184 unilateral schwannoma patients with functional preoperative hearing (Gardner-Robertson 1 or 2) treated by first intention radiosurgery with a marginal dose lower than 13 Gy. The population included 74 patients with subnormal hearing (class 1). All have been studied with a follow-up longer than 3 years. Univariate and multivariate analyses have been carried out. RESULTS: Numerous parameters greatly influence the probability of functional hearing preservation at 3 years, which is globally 60%. The main preoperative parameters of predictability are limited hearing loss that is Gardner-Robertson stage 1 (vs. 2), presence of tinnitus, young age of the patient and small size of the lesion. The functional hearing preservation at 3 years is 77.8% when the patient is initially in stage 1, 80% in patients with tinnitus as a first symptom and 95% when the patient has both. In these patients, the probability of functional preservation at 5 years is 84%. Comparison of these results with the main series of the literature confirms the reproducibility of our results. Additionally, we have demonstrated a higher chance of hearing preservation when the dose to the cochlea is lower than 4 Gy. CONCLUSION: We report a large population of patients treated by radiosurgery with functional preoperative hearing. These results demonstrate the possibility to preserve functional hearing in a high percentage of selected patients. Radiosurgery offers them a higher chance of functional hearing preservation than microsurgery or simple follow-up.

Management of invasive juvenile nasopharyngeal angiofibromas: the role of a multimodality approach.

OBJECTIVE: Juvenile nasopharyngeal angiofibromas involving the cranial base and intracranial compartment are challenging tumors. We reviewed our experience of these tumors and analyzed the efficacy of a multimodality management. METHODS: Between 1981 and 2000, 15 extensive juvenile nasopharyngeal angiofibromas (Fisch Grade III or IV) were treated at our institution. The mean age of the patients was 14.5 years, and the mean interval between the first symptom and diagnosis was 12.9 months. Initial management included preoperative embolization of the external carotid artery feeders, followed by tumor removal. A maxillofacial procedure was performed in eight cases, a combination of maxillofacial and neurosurgical approach was performed in four cases, and a neurosurgical cranial base approach was performed in three cases. RESULTS: Total removal after the initial procedure was obtained in eight patients. Subtotal removal justified additional surgery in one case, gamma knife radiosurgery in two cases, and fractionated irradiation in four cases. True recurrences were observed in four cases at a mean interval of 37 months (range, 24-46 mo) and required tailored multimodality management. No cases of perioperative death were observed. One patient underwent hemiparesis after embolization in the early period of our experience. Permanent facial numbness was reported in four cases, moderate cosmetic problems were reported in three cases, and hyposmia was reported in three cases. Except for one patient who was lost to follow-up at 18 months, 12 patients were free of disease and two patients were free of tumor progression. All patients had normal or near-normal daily life at the last check-up, with a median follow-up period of 108 months (mean, 117 mo; range, 91-252 mo). CONCLUSION: Extensive juvenile nasopharyngeal angiofibromas are efficiently managed with a multimodal protocol in which preoperative embolization is followed by optimal surgical removal using various transcranial or transfacial approaches. Adjunctive gamma knife radiosurgery is a valuable option for intracavernous residual tumor. Our protocol offers long-term cure with acceptable morbidity.

Modern management of vestibular schwannomas.

Within the last 3 decades, microsurgery and stereotactic radiosurgery (SRS) have become well-established management options for vestibular schwannomas (VSs). Advancement in the management of VSs can be separated into three periods: the microsurgical pioneer period, the demonstration of SRS as a first-line therapy for small and medium-sized VSs, and currently, a period of SRS maturity based on a large worldwide patient accrual. The Marseille SRS experience includes 1,500 patients, with 1,000 patients having follow-up longer than 3 years. A long-term tumor control rate of 97%, transient facial palsy lower than 1%, and a probability of functional hearing preservation between 50 and 95% was achieved in this large series of patients treated with state-of-the-art SRS.

Long-term outcome in intensive care unit survivors after mechanical ventilation for intracerebral hemorrhage.

OBJECTIVE: To evaluate long-term survival and functional outcome in intensive care unit survivors after mechanical ventilation for intracerebral hemorrhage. DESIGN: Retrospective chart review and prospective follow-up study. SETTING: Outpatient follow-up. PATIENTS: Between 1997 and 2000, 120 patients were mechanically ventilated for an intracerebral hemorrhage at our intensive care unit. Sixty-two patients were discharged from hospital (in-hospital mortality = 48%). Sixty patients were evaluated for survival and functional outcome (two were lost to follow-up). Time between discharge and follow-up was > or =1 yr and was a mean of 27 +/- 14 months (range, 12-56). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients' physicians were first asked about survival, and patients or proxies were interviewed by phone. Barthel Index and modified Rankin Scale scores were collected, and demographic information and general data were reviewed. The estimated life-table survival curve after discharge was 64.6% at 1 yr and 57% at 3 yrs. In the 24 patients who died, the mean time between discharge and death was 5 +/- 6 months. Probability of death after discharge significantly increased if age at admission was >65 yrs (p <.01; odds ratio, 3.5; 95% confidence interval, 1.4-9.1) and if Glasgow Coma Scale score at discharge was <15 (p <.01; odds ratio, 3.9; 95% confidence interval, 1.6-9.5). In the 36 long-term survivors, Barthel Index was 67.5 +/- 15 (median +/- median absolute dispersion) and modified Rankin Scale score was 2.6 +/- 0.5. Fifteen patients (42%) had a slight or no disability (Barthel Index > or =90 and modified Rankin Scale score < or =2), whereas 21 patients (58%) had moderate or severe disability (Barthel Index < or =85 and modified Rankin Scale score >2). CONCLUSIONS: Probability of survival at 3 yrs after mechanical ventilation for an intracerebral hemorrhage was >50%. Age was an important determinant of long-term survival. Forty-two percent of long-term survivors were independent for activities of daily living. Only a few long-term survivors had a very high degree of disability.

Functional outcome after gamma knife surgery or microsurgery for vestibular schwannomas.

OBJECT: Microsurgical excision is an established treatment for vestibular schwannoma (VS). In 1992 the authors used a patient questionnaire to evaluate the functional outcome and quality of life in a series of 224 consecutive patients. In addition, starting with gamma knife surgery (GKS) in 1992, the authors decided to use the same methodology to evaluate prospectively the results of this modality to compare the two alternatives. METHODS: Among the 500 patients who were included prospectively, the authors only evaluated patients in whom GKS was the primary treatment for unilateral VS. Four years of follow up was available for the first 104 consecutive patients. Statistical analysis of the GKS and microsurgery populations has shown that only a comparison of Stage II and III (according to the Koos classification) was meaningful in terms of group size and preoperative risk factor distribution. Objective results and questionnaire answers from the first 97 consecutive patients were compared with the 110 patients in the microsurgery group who fulfilled the inclusion criteria. Questionnaire answers indicated that 100% of patients who underwent GKS compared with 63% of patients who underwent microsurgery had no new facial motor disturbance. Forty-nine percent of patients who underwent GKS (17% in the microsurgery study) had no ocular symptoms, and 91% of patients treated with GKS (61% in the microsurgery study) had no functional deterioration after treatment. The mean hospitalization stay was 3 days after GKS and 23 days after microsurgery. All the patients who underwent GKS who had been employed, except one, had kept the same professional activity (56% in the microsurgery study). The mean time away from work was 7 days for GKS (130 days in the microsurgery study). Among patients whose preoperative hearing level was Class 1 according to the Gardner and Robertson scale, 70% preserved functional hearing after GKS (Class 1 or 2) compared with only 37.5% in the microsurgery group. CONCLUSIONS: Functional side effects happen during the first 2 years after radiosurgery. Findings after 4 years of follow up indicated that GKS provided better functional outcomes than microsurgery in this patient series.

Results of the MXM Digisonic auditory brainstem implant clinical trials in Europe.

OBJECTIVE: To assess the potential benefit of the MXM auditory brainstem implant for patients with neurofibromatosis type 2. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral centers. PATIENTS: Fourteen patients with neurofibromatosis type 2 and bilateral acoustic neuromas underwent implantation with the MXM auditory brainstem implant during surgery to remove the second-side tumor. RESULTS: There were no complications related to the auditory brainstem implantation. Auditory sensations were present for 12 of 14 patients (86%). Global results indicated an improved quality of life for the patients receiving auditory sensations, in part because of their auditory orientation within the environment. Eighty-nine percent of patients tested with an open-set sentence test demonstrated enhancement of speech understanding as a result of lip-reading improvement when auditory brainstem implant sound was combined with lip-reading. A few patients (36%) had some speech understanding in sound-only mode. One patient was able to have limited phone conversations. CONCLUSION: These results indicate that significant auditory benefit can be derived from the MXM auditory brainstem implant.