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Hussam Metwali, Katja Kniese, Babak Kardavani, Venelin Gerganov and Madjid Samii

OBJECTIVE

The purpose of this prospective study was to evaluate the dysfunction of the nervus intermedius (NI) after vestibular schwannoma (VS) surgery. The authors present a clinically feasible method for this purpose.

METHODS

In this prospective study, the authors included 30 patients who underwent surgery at the International Neuroscience Institute between May 2014 and February 2017 for resection of VS. The patients’ taste sensation was examined using taste strips. Lacrimation was tested using the Schirmer I test. The clinical evaluation was performed before surgery and repeated at 2 weeks and at 6 months after surgery as well as during the follow-up, which extended up to 2 years. The authors tested the correlation between the NI dysfunctions and the House-Brackmann grade of facial nerve palsy.

RESULTS

The taste sensation was lost on the side of surgery in 2 patients (6.6%) and decreased in 4 patients (13.3%). The disturbance of taste sensation was not statistically correlated with dysfunctions of the motor portion of the facial nerve. The taste impairment resolved in 4 patients within 6 months, but 2 patients suffered from persistent loss of the taste sensation on the side of surgery during the follow-up. In 23 patients (76.6%), the baseline lacrimation was lower on the side of surgery, and it was significantly correlated with outcome for dysfunctions of the motor portion of the facial nerve. During the follow-up, baseline lacrimation improved in correlation with the improvement in the dysfunctions of the motor portion of the facial nerve. None of the patients reported change in salivation or nasal secretion.

CONCLUSIONS

The NI can be affected after VS surgery. The disturbance of baseline lacrimal secretion was correlated with dysfunctions of the motor portion of the facial nerve. However, the disturbance of the taste sensation was not correlated with the grade of facial nerve palsy. Dysfunctions of the NI should be evaluated and separately reported while analyzing facial nerve outcome after VS surgery.

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Michael J. Link, Douglas Kondziolka and Madjid Samii

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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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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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Mario Giordano, Amir Samii, Anna C. Lawson McLean, Helmut Bertalanffy, Rudolf Fahlbusch, Madjid Samii and Concezio Di Rocco

OBJECTIVE

The use of high-field intraoperative MRI has been largely studied for the treatment of intracranial tumors in adult patients. In this study, the authors investigated the safety, advantages, and limitations of high-field iMRI for cranial neurosurgical procedures in pediatric patients, with particular attention to craniopharyngiomas and gliomas.

METHODS

The authors performed 82 surgical procedures in patients under 16 years of age (range 0.8–15 years) over an 8-year period (2007–2014) using iMRI. The population was divided into 3 groups based on the condition treated: sellar region tumors (Group 1), gliomas (Group 2), and other pathological entities (Group 3). The patients' pre- and postoperative neurological status, the presence of residual tumor, the number of intraoperative scans, and complications were evaluated.

RESULTS

In Group 1, gross-total resection (GTR) was performed in 22 (88%) of the procedures and subtotal resection (STR) in 3 (12%). In Group 2, GTR, STR, and partial resection (PR) were performed, respectively, in 15 (56%), 7 (26%), and 5 (18%) of the procedures. In Group 3, GTR was performed in 28 (93%) and STR in 2 (7%) of the procedures. In cases of craniopharyngioma (Group 1) and glioma (Group 2) in which a complete removal was planned, iMRI allowed localization of residual lesions and attainment of the surgical goal through further resection, respectively, in 18% and 27% of the procedures. Moreover, in gliomas the resection could be extended from partial to subtotal in 50% of the cases. In 17% of the patients in Group 3, iMRI enabled the identification and further removal of tumor remnants. There was no intra- or postoperative complication related to the use of iMRI despite special technical difficulties in smaller children.

CONCLUSIONS

In this study, the use of iMRI in children proved to be safe. It was most effective in increasing the extent of tumor resection, especially in patients with low-grade gliomas and craniopharyngiomas. The most prominent disadvantage of high-field iMRI was the limitation with respect to operative positioning due to the configuration of the surgical table.

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Madjid Samii, Hussam Metwali and Venelin Gerganov

OBJECTIVE

Microsurgical treatment of recurrent vestibular schwannoma (VS) is difficult and poses specific challenges. The authors report their experience with 53 cases of surgically treated recurrent VS. Outcome of these tumors was compared to that of primarily operated on VS. Special attention was given to the facial nerve functional outcome.

METHODS

A retrospective analysis was performed of the patients who underwent surgery for recurrent VS at one institution from 2000 to 2013. The preoperative data, intraoperative findings, and outcome in terms of facial nerve function and improvement of the preoperative symptoms were analyzed and compared with those in a control group of 30 randomly selected patients with primarily operated on VS. A multivariate regression analysis was performed to test the factors that could affect the facial nerve outcome in each group.

RESULTS

Fifty-three consecutive patients underwent surgery for recurrent VS. Seventeen patients were previously operated on and received postoperative radiosurgery (Group A). Thirty-six patients were previously operated on but did not receive postoperative radiosurgery (Group B). The overall postoperative facial nerve function was significantly worse in Groups A and B in comparison with the control group (Group C). Interestingly, there was no significant difference in the facial nerve outcome among the 3 groups in patients who had good preoperative facial nerve function. The tumor size and the preoperative facial nerve function are variables that significantly affect the facial nerve outcome. Most of the patients showed improvement of the preoperative symptoms, such as trigeminal hypesthesia, gait disturbance, and headache.

CONCLUSIONS

Complete microsurgical tumor removal is the optimal management for patients with recurrent or regrowing VS. The procedure is safe, associated with favorable facial nerve outcome, and may also improve existing neurological symptoms.

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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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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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Maysam Alimohamadi, Christian Hartmann, Vincenzo Paterno and Madjid Samii

Erdheim-Chester disease (ECD) is non-Langerhans histiocytosis that can affect multiple organ systems. It usually affects middle-aged patients, and only a few reports of ECD in children appear in the literature. Central nervous system involvement is a common feature that usually occurs as infiltration of the hypothalamus-pituitary axis, cerebellum, and/or brainstem. Meningeal involvement occurs less commonly. In this article, the authors discuss a rare pediatric case of ECD presenting as an infiltrative mass of the trigeminal nerve and resembling the clinical and imaging features of a trigeminal schwannoma.

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Maysam Alimohamadi and Madjid Samii