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Efficacy of microsurgical tumor removal for treatment of patients with intracanalicular vestibular schwannoma presenting with disabling vestibular symptoms

Madjid Samii, Hussam Metwali, and Venelin Gerganov

OBJECTIVE

The aim of this study was to analyze the efficacy and risks of microsurgery via the hearing-preserving retrosigmoid approach in patients with intracanalicular vestibular schwannoma (VS) suffering from disabling vestibular symptoms, with special attention to vertigo.

METHODS

This is a retrospective analysis of 19 patients with intracanalicular VS and disabling vestibular dysfunction as the main or only symptom (Group A). All of the patients reported having had disabling vertigo attacks. Subjective evaluation of the impairment of patients was performed before surgery, 3 weeks after surgery, 3 months after surgery, and 1 year after surgery, using the Dizziness Handicap Inventory (DHI). The main outcome measures were improvement in quality of life as measured using the DHI, and general and functional outcomes, in particular facial function and hearing. Patient age, preoperative tumor size, preoperative DHI score, and preservation of the nontumorous vestibular nerve were tested using a multivariate regression analysis to determine factors affecting the postoperative DHI score. The Mann-Whitney U-test was used to compare the postoperative DHI score at 3 weeks, 3 months, and 1 year after surgery with a control group of 19 randomly selected patients with intracanalicular VSs, who presented without vestibular symptoms (Group B). The occurrence of early postoperative discrete vertigo attacks was also compared between groups.

RESULTS

The preoperative DHI score was ≥ 54 in all patients. All patients reported having had disabling rotational vertigo before surgery. The only significant factor to affect the DHI outcome 3 weeks and 3 months after surgery was the preoperative DHI score. The DHI outcome after 1 year was not affected by the preoperative DHI score. Compared with the control group, the DHI score at 3 weeks and 3 months after surgery was significantly worse. There was no significant difference between the groups after 1 year. Vertigo was improved in all patients and completely resolved after 1 year in 17 patients.

CONCLUSIONS

Disabling vestibular dysfunction that affects quality of life should be considered an indication for surgery, even in otherwise asymptomatic patients with intracanalicular VS. Surgical removal of the tumor is safe and very effective in regard to symptom relief. All patients had excellent facial nerve function within 1 year after surgery, with a very good chance of hearing preservation.

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Endoscope-assisted retrosigmoid infralabyrinthine approach to jugular foramen tumors

Madjid Samii, Maysam Alimohamadi, and Venelin Gerganov

OBJECT

Removal of jugular foramen (JF) tumors usually requires extensive skull base approaches and is frequently associated with postoperative morbidities such as lower cranial nerve injury. The endoscope-assisted retrosigmoid infralabyrinthine approach is a relatively new approach to tumors extending into the bony canal of the JF. The authors present their experience with this approach.

METHODS

The endoscope-assisted retrosigmoid infralabyrinthine approach was used in 7 patients, including 5 with schwannomas and 2 with paragangliomas. The access to the tumor, extent of its removal, postoperative neurological outcome, and approach-related morbidities were evaluated.

RESULTS

Two patients had a history of previous partial tumor removal, and 1 was treated by embolization followed by two courses of Gamma Knife radiosurgery. In this latter patient near-total resection was achieved. Gross-total resection was possible in the remaining 6 patients. Five patients benefited from endoscopic assistance: in 2 patients it showed a tumor remnant after microscopic tumor removal, while in 3 patients it allowed safe removal of the intraforaminal tumor by visualizing the surrounding structures. No permanent neurological deficit was observed after the operation. Two patients presenting with swallowing disturbance had temporary postoperative worsening that improved later. One patient developed CSF leakage that was managed with a lumbar drain.

CONCLUSIONS

This study shows that the judicious application of the endoscope-assisted retrosigmoid infralabyrinthine approach is safe and effective for removal of the schwannomas extending into the JF and selected paragangliomas without significant luminal invasion of the sigmoid-jugular system.

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A preliminary study of the clinical application of optic pathway diffusion tensor tractography in suprasellar tumor surgery: preoperative, intraoperative, and postoperative assessment

Mohamadreza Hajiabadi, Madjid Samii, and Rudolf Fahlbusch

OBJECT

Visual impairments are the most common objective manifestations of suprasellar lesions. Diffusion tensor imaging (DTI) is a noninvasive MRI modality that depicts the subcortical white matter tracts in vivo. In this study the authors tested the value of visual pathway tractography in comparison with visual field and visual acuity analyses.

METHODS

This prospective study consisted of 25 patients with progressive visual impairment due to suprasellar mass lesions and 6 control patients with normal vision without such lesions. Visual acuity, visual field, and the optic fundus were examined preoperatively and repeated 1 week and 3 months after surgery. Visual pathway DTI tractography was performed preoperatively, intraoperatively immediately after tumor resection, and 1 week and 3 months after surgery.

RESULTS

In the control group, pre- and postoperative visual status were normal and visual pathway tractography revealed fibers crossing the optic chiasm without any alteration. In patients with suprasellar lesions, vision improved in 24 of 25. The mean distance between optic tracts in tractography decreased after tumor resection and detectable fibers crossing the optic chiasm increased from 12% preoperatively to 72% postoperatively 3 months after tumor resection, and undetectable fibers crossing the optic chiasm decreased from 88% preoperatively to 27% postoperatively 3 months after tumor resection. Visual improvement after tumor removal 1 week and 3 months after surgery was significantly correlated with the distance between optic tracts in intraoperative tractography (p < 0.01).

CONCLUSIONS

Visual pathway DTI tractography appears to be a promising adjunct to the standard clinical and paraclinical visual examinations in patients with suprasellar mass lesions. The intraoperative findings, in particular the distance between optic tract fibers, can predict visual outcome after tumor resection. Furthermore, postoperative application of this technique may be useful in following anterior optic pathway recovery.

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Surgery or Gamma Knife

Jeremy C. Ganz

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Syringomyelia associated with foramen magnum arachnoiditis

Jörg Klekamp, Giorgio Iaconetta, Ulrich Batzdorf, and Madjid Samii

Object. Syringomyelia is often linked to pathological lesions of the foramen magnum. The most common cause is hindbrain herniation, usually referred to as Chiari I or II malformation. Foramen magnum arachnoiditis without either Chiari I or II malformation is a rare cause of syringomyelia. The authors undertook a retrospective analysis of 21 patients with foramen magnum arachnoiditis (FMA) and syringomyelia treated between 1978 and 2000 to determine clinical course and optimum management.

Methods. In the review of records, 21 patients with FMA and syringomyelia were documented. A stable clinical course was demonstrated in three patients in whom surgery was not performed, and one patient refused surgical intervention. Seventeen patients underwent 23 operations to treat progressive neurological disease. Of these 23 operations, 18 involved opening of the foramen magnum, arachnoid dissection, and placement of a large dural graft. One patient underwent insertion of a ventriculoperitoneal shunt for treatment of accompanying hydrocephalus, one patient received a cystoperitoneal shunt for an accompanying arachnoid cyst; two syringoperitoneal and one syringosubarachnoid shunts were also inserted. Hospital and outpatient files, neuroimaging studies, and intraoperative photographic and video material were analyzed. Additional follow-up information was obtained by telephone interview and questionnaires.

Standard and cardiac-gated magnetic resonance imaging studies are the diagnostic procedures of choice in these patients. Sensory disturbances, dysesthesias, and pain were the only symptoms likely to improve after foramen magnum surgery. Motor weakness and gait disturbances, which were severe in a considerable number of patients, and swallowing disorders tended to remain unchanged. As a consequence of the rather severe arachnoid lesions in most patients, clinical recurrences were observed in 57% over a 5-year period.

Conclusions. Surgery for FMA and syringomyelia has to provide clear cerebrospinal fluid pathways between the cerebellopontine cisterns, spinal canal, and fourth ventricle. If this can be achieved successfully, the syrinx decreases in size and the clinical course of the patient may even improve. In patients with severe and widespread areas of arachnoiditis, however, multiple operations may be required at least to stabilize the clinical course.

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Surgical management of cerebellopontine angle arachnoid cysts associated with hearing deficit in pediatric patients

Mario Giordano, Massimo Gallieni, Amir Samii, Concezio Di Rocco, and Madjid Samii

OBJECTIVE

Few cases of cerebellopontine angle (CPA) arachnoid cysts in pediatric patients have been described in the literature, and in only 2 of these cases were the patients described as suffering from hearing deficit. In this article, the authors report on 3 pediatric patients with CPA arachnoid cysts (2 with hearing loss and 1 with recurrent headaches) who underwent neurosurgical treatment at the authors’ institution.

METHODS

Four pediatric patients were diagnosed with CPA arachnoid cysts at the International Neuroscience Institute during the period from October 2004 through August 2012, and 3 of these patients underwent surgical treatment. The authors describe the patients’ clinical symptoms, the surgical approach, and the results on long-term follow-up.

RESULTS

One patient (age 14 years) who presented with headache (without hearing deficit) became asymptomatic after surgical treatment. The other 2 patients who underwent surgical treatment both had hearing loss. One of these children (age 9 years) had recent-onset hypacusia and experienced complete recovery immediately after the surgery. The other (age 6 years) had a longer history (2 years) of progressive hearing loss and showed an interruption of the deficit progression and only mild improvement at the follow-up visit.

CONCLUSIONS

CPA arachnoid cysts are uncommon in pediatric patients. The indication and timing of the surgical treatment are fundamental, especially when a hearing deficit is present.

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Editorial: Acoustics and hydrocephalus

Donald P. Becker

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Diffusion tensor imaging–based fiber tracking for prediction of the position of the facial nerve in relation to large vestibular schwannomas

Clinical article

Venelin M. Gerganov, Mario Giordano, Madjid Samii, and Amir Samii

Object

The reliable preoperative visualization of facial nerve location in relation to vestibular schwannoma (VS) would allow surgeons to plan tumor removal accordingly and may increase the safety of surgery. In this prospective study, the authors attempted to validate the reliability of facial nerve diffusion tensor (DT) imaging–based fiber tracking in a series of patients with large VSs. Furthermore, the authors evaluated the potential of this visualization technique to predict the morphological shape of the facial nerve (tumor compression–related flattening of the nerve).

Methods

Diffusion tensor imaging and anatomical images (constructive interference in steady state) were acquired in a series of 22 consecutive patients with large VSs and postprocessed with navigational software to obtain facial nerve fiber tracking. The location of the cerebellopontine angle (CPA) part of the nerve in relation to the tumor was recorded during surgery by the surgeon, who was blinded to the results of the fiber tracking. A correlative analysis was performed of the imaging-based location of the nerve compared with its in situ position in relation to the VS.

Results

Fibers corresponding to the anatomical location and course of the facial nerve from the brainstem to the internal auditory meatus were identified with the DT imaging–based fiber tracking technique in all 22 cases. The location of the CPA segment of the facial nerve in relation to the VS determined during surgery corresponded to the location of the fibers, predicted by the DT imaging–based fiber tracking, in 20 (90.9%) of the 22 patients. No DT imaging–based fiber tracking correlates were found with the 2 morphological types of the nerve (compact or flat).

Conclusions

The current study of patients with large VSs has shown that the position of the facial nerve in relation to the tumor can be predicted reliably (in 91%) using DT imaging–based fiber tracking. These are preliminary results that need further verification in a larger series.

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Brachial plexus injury: factors affecting functional outcome in spinal accessory nerve transfer for the restoration of elbow flexion

Amir Samii, Gustavo Adolpho Carvalho, and Madjid Samii

Object. Between 1994 and 1998, 44 nerve transfers were performed using a graft between a branch of the accessory nerve and musculocutaneous nerve to restore the flexion of the arm in patients with traumatic brachial plexus injuries. A retrospective study was conducted, including statistical evaluation of the following pre- and intraoperative parameters in 39 patients: 1) time interval between injury and surgery; and 2) length of the nerve graft used to connect the accessory and musculocutaneous nerves.

Methods. The postoperative follow-up interval ranged from 23 to 84 months, with a mean ± standard deviation of 36 ± 13 months. Reinnervation of the biceps muscle was achieved in 72% of the patients. Reinnervation of the musculocutaneous nerve was demonstrated in 86% of the patients who had undergone surgery within the first 6 months after injury, in 65% of the patients who had undergone surgery between 7 and 12 months after injury, and in only 50% of the patients who had undergone surgery 12 months after injury. A statistical comparison of the different preoperative time intervals (0–6 months compared with 7–12 months) showed a significantly better outcome in patients treated with early surgery (p < 0.05). An analysis of the impact of the length of the interposed nerve grafts revealed a statistically significant better outcome in patients with grafts 12 cm or shorter compared with that in patients with grafts longer than 12 cm (p < 0.005).

Conclusions. Together, these results demonstrated that outcome in patients who undergo accessory to musculocutaneous nerve neurotization for restoration of elbow flexion following brachial plexus injury is greatly dependent on the time interval between trauma and surgery and on the length of the nerve graft used.

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Surgical treatment of patients with vestibular schwannomas after failed previous radiosurgery

Clinical article

Venelin M. Gerganov, Mario Giordano, Amir Samii, and Madjid Samii

Object

An increasing number of patients with vestibular schwannomas (VSs) are being treated with radiosurgery. Treatment failure or secondary regrowth after radiosurgery, however, has been observed in 2%–9% of patients. In large tumors that compress the brainstem and in patients who experience rapid neurological deterioration, surgical removal is the only reasonable management option.

Methods

The authors evaluated the relevance of previous radiosurgery for the outcome of surgery in a series of 28 patients with VS. The cohort was further subdivided into Group A (radiosurgery prior to surgery) and Group B (partial tumor removal followed by radiosurgery prior to current surgery). The functional and general outcomes in these 2 groups were compared with those in a control group (no previous treatment, matched characteristics).

Results

There were 15 patients in Group A, 13 in Group B, and 30 in the control group. The indications for surgery were sustained tumor enlargement and progression of neurological symptoms in 12 patients, sustained tumor enlargement in 15 patients, and worsening of neurological symptoms without evidence of tumor growth in 1 patient. Total tumor removal was achieved in all patients in Groups A and B and in 96.7% of those in the control group. There were no deaths in any group. Although no significant differences in the neurological morbidity or complication rates after surgery were noted, the risk of new cranial nerve deficits and CSF leakage was highest in patients in Group B. Patients who underwent previous radiosurgical treatment (Groups A and B) tended to be at higher risk of developing postoperative hematomas in the tumor bed or cerebellum. The rate of facial nerve anatomical preservation was highest in those patients who were not treated previously (93.3%) and decreased to 86.7% in the patients in Group A and to 61.5% in those in Group B. Facial nerve function at follow-up was found to correlate to the previous treatment; excellent or good function was seen in 87% of the patients from the control group, 78% of those in Group A, and 68% of those in Group B.

Conclusions

Complete microsurgical removal of VSs after failed radiosurgery is possible with an acceptable morbidity rate. The functional outcome, however, tends to be worse than in nontreated patients. Surgery after previous partial tumor removal and radiosurgery is most challenging and related to worse outcome.