The authors report the case of a 30-year-old woman who was a long-term intranasal cocaine abuser and who presented with transient ischemic attacks and multiple cerebral infarctions that were associated with moyamoya syndrome. The authors suggest that, because of its sympathomimetic effects, chronic cocaine use may promote intracranial arterial stenosis, distal ischemia, and subsequent formation of moyamoya-like vessels. The patient has remained clinically stable with no new episodes of stroke 6 years after undergoing “pial synangiosis” (modified encephaloduroarteriosynangiosis) to revascularize both hemispheres. Cocaine abuse may lead to moyamoya syndrome and may represent a chronic effect on the cerebral vasculature.
Marc S. Schwartz and R. Michael Scott
Marc S. Schwartz and Gregory P. Lekovic
The CO2 laser has been used on an intermittent basis in the microsurgical resection of brain tumors for decades. These lasers were typically cumbersome to use due to the need for a large, bulky design since infrared light cannot be transmitted via fiber-optic cables. Development of the OmniGuide cable, which is hollow and lined with an omnidirectional dielectric mirror, has facilitated the reintroduction of the CO2 laser in surgical use in a number of fields. This device allows for handheld use of the CO2 laser in a much more ergonomically favorable configuration, holding promise for microneurosurgical applications. This device was introduced into the authors’ practice for use in the microsurgical resection of skull base tumors, including vestibular schwannomas.
The authors reviewed the initial 41 vestibular schwannomas that were treated using the OmniGuide CO2 laser during an 8-month period from March 2010 to October 2010. The laser was used for all large tumors, and select medium-sized tumors were treated via both the translabyrinthine and retrosigmoid approaches. The estimated time of tumor resection and estimated blood loss were obtained from operating room records. Data regarding complications, facial nerve and hearing outcomes, and further treatment were collected from hospital and clinic records, MRI reports, and direct review of MR images. Time of resection and blood loss were compared to a control group (n = 18) who underwent surgery just prior to use of the laser.
A total of 41 patients with vestibular schwannomas were surgically treated. The median estimated time of tumor resection was 150 minutes, and the median estimated blood loss was 300 ml. The only operative complication was 1 CSF leak. Thirty-eight patients had normal facial nerve function at late follow-up. The median MRI follow-up was 52 months, and, during that time, only 1 patient required further treatment for regrowth of a residual tumor.
The OmniGuide CO2 laser is a useful adjunct in the resection of large vestibular schwannomas. This device was used primarily as a cutting tool rather than for tumor vaporization, and it was found to be of most use for very large and/or firm tumors. There were no laser-associated complications, and the results compared favorably to earlier reports of vestibular schwannoma resection.
Gregory P. Lekovic, Marc S. Schwartz and John L. Go
In this report the authors report on a patient with a very indolent course of granulocytic sarcoma, characterized by steroid-induced remission of spinal and cranial tumors and recurrence over a period of several years. This 24-year-old man with history of leukemia presented with rapid-onset quadriparesis secondary to multiple extraaxial masses of the cervicothoracic spine, from C-5 to T-3, and lumbosacral spine, from L-5 to the coccyx. Although the imaging features were highly suggestive of neurofibromatosis Type 2, the patient's history and clinical course were consistent with granulocytic sarcoma; repeat imaging and, later, needle biopsy definitively established the diagnosis of granulocytic sarcoma. Laminectomy and surgical decompression of the spine were not required and, arguably, could have posed unnecessary risk to the patient. This case illustrates that the successful management of a patient presenting with profound neurological deficits due to intradural spinal cord tumors may sometimes be nonsurgical.
Michael Hoa, Doniel Drazin, George Hanna, Marc S. Schwartz and Gregory P. Lekovic
With the increasing prevalence and decreasing cost of MRI scans, incidental discovery of vestibular schwannoma (VS) has become more common. Scarce literature exists regarding management of the tumors in those patients with incidentally discovered VSs, and clear guidelines for management do not exist. In this review, the authors examine the available literature for insights into management of incidentally diagnosed VS and provide an algorithm for their management.
Michael A. Horgan, Jennifer C. Kernan, Johnny B. Delashaw, Marc S. Schwartz and Todd Kuether
Wouter I. Schievink, Marc S. Schwartz, M. Marcel Maya, Franklin G. Moser and Todd D. Rozen
Spontaneous intracranial hypotension is an important cause of headaches and an underlying spinal CSF leak can be demonstrated in most patients. Whether CSF leaks at the level of the skull base can cause spontaneous intracranial hypotension remains a matter of controversy. The authors' aim was to examine the frequency of skull base CSF leaks as the cause of spontaneous intracranial hypotension.
Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period.
Among 273 patients who met the diagnostic criteria for spontaneous intracranial hypotension and 42 who did not, not a single instance of CSF leak at the skull base was encountered. Clear nasal drainage was reported by 41 patients, but a diagnosis of CSF rhinorrhea could not be established. Four patients underwent exploratory surgery for presumed CSF rhinorrhea. In addition, the authors treated 3 patients who had a postoperative CSF leak at the skull base following the resection of a cerebellopontine angle tumor and developed orthostatic headaches; spinal imaging, however, demonstrated the presence of a spinal source of CSF leakage in all 3 patients.
There is no evidence for an association between spontaneous intracranial hypotension and CSF leaks at the level of the skull base. Moreover, the authors' study suggests that a spinal source for CSF leakage should even be suspected in patients with orthostatic headaches who have a documented skull base CSF leak.
Marc S. Schwartz, Gregory P. Lekovic, Derald E. Brackmann and Courtney C. J. Voelker
We present video of gross-total resection of a large cerebellopontine angle tumor consisting of both vestibular and facial schwannoma components via the translabyrinthine route in a patient with neurofibromatosis type 2. The facial nerve is reconstructed using a greater auricular nerve graft, and an auditory brainstem implant is placed. Prior to surgery the patient had no facial nerve function on the operative side and had lost useful hearing. He also had usable vision only on the ipsilateral side and had contralateral vocal cord paralysis.
The video can be found here: http://youtu.be/IOkEND-0vhI.
Richard B. Schwartz, B. Leonard Holman, Basem M. Garada, Paulo A. Carvalho, Rebecca Folkerth, Marc S. Schwartz, Jay S. Loeffler, Dennis C. Shrieve, Joseph F. Polak, Peter McL. Black and Eben Alexander III
This study was conducted to determine the sensitivity of dual-isotope single-photon emission computerized tomography (SPECT) in predicting tumor recurrence and survival in patients treated with high-dose radiotherapy for malignant gliomas.
Studies using SPECT with thallium-201 (Tl-201) and technetium-99m (Tc-99m) hexamethypropyleneamine oxime (HMPAO) in 50 consecutive patients with malignant astrocytomas treated by surgery and high-dose radiotherapy were performed 1 day before reoperation. Maximum uptake of Tl-201 in the lesion was expressed as a ratio to that of the contralateral scalp, and uptake of Tc-99m HMPAO was expressed as a ratio to that of the cerebellar cortex. Patients were stratified into groups based on maximum radioisotope uptake values in their tumor beds. Differences in tumor histopathology at reoperation and 1-year survival between SPECT groups were determined by using chi-square analysis.
The majority of patients in Group IA (Tl-201 ratio less than 2 or Tc-99m HMPAO ratio less than 0.5) showed radiation changes in their biopsy specimens (85.7%); they had an 85.7% 1-year survival rate. Group II (Tl-201 ratio between 2 and 3.5) had predominantly infiltrating tumor (78.6%); they had a 42% 1-year survival rate. Almost all of the patients in Group III (Tl-201 ratio greater than 3.5) had solid tumor (93.3%) and they had a 6.7% 1-year survival. Survival and pathological data differed significantly among groups (p less than 0.009).
Dual-isotope SPECT data correlates with histological findings on reoperation and postoperative survival in patients with malignant gliomas.
Richard B. Schwartz, B. Leonard Holman, Joseph F. Polak, Basem M. Garada, Marc S. Schwartz, Rebecca Folkerth, Paulo A. Carvalho, Jay S. Loeffler, Dennis C. Shrieve, Peter McL. Black and Eben Alexander III
Object. The study was conducted to determine the association between dual-isotope single-photon emission computerized tomography (SPECT) scanning and histopathological findings of tumor recurrence and survival in patients treated with high-dose radiotherapy for glioblastoma multiforme.
Methods. Studies in which SPECT with 201Tl and 99mTc-hexamethypropyleneamine oxime (HMPAO) were used were performed 1 day before reoperation in 47 patients with glioblastoma multiforme who had previously been treated by surgery and high-dose radiotherapy. Maximum uptake of 201Tl in the lesion was expressed as a ratio to that in the contralateral scalp, and uptake of 99mTc-HMPAO was expressed as a ratio to that in the cerebellar cortex. Patients were stratified into groups based on the maximum radioisotope uptake values in their tumor beds. The significance of differences in patient gender, histological characteristics of tissue at reoperation, and SPECT uptake group with respect to 1-year survival was elucidated by using the chi-square statistic. Comparisons of patient ages and time to tumor recurrence as functions of 1-year survival were made using the t-test.
Survival data at 1 year were presented according to the Kaplan—Meier method, and the significance of potential differences was evaluated using the log-rank method. The effects of different variables (tumor type, time to recurrence, and SPECT grouping) on long-term survival were evaluated using Cox proportional models that controlled for age and gender.
All patients in Group I (201Tl ratio < 2 and 99mTc-HMPAO ratio < 0.5) showed radiation changes in their biopsy specimens: they had an 83.3% 1-year survival rate. Group II patients (201T1 ratio < 2 and 99mTc-HMPAO ratio of ≥ 0.5 or 201Tl ratio between 2 and 3.5 regardless of 99mTc-HMPAO ratio) had predominantly infiltrating tumor (66.6%); they had a 29.2% 1-year survival rate. Almost all of the patients in Group III (201Tl ratio > 3.5 and 99mTc-HMPAO ratio ≥ 0.5) had solid tumor (88.2%) and they had a 6.7% 1-year survival rate. Histological data were associated with 1-year survival (p < 0.01); however, SPECT grouping was more closely associated with 1-year survival (p < 0.001) and was the only variable significantly associated with long-term survival (p < 0.005).
Conclusions. Dual-isotope SPECT data correlate with histopathological findings made at reoperation and with survival in patients with malignant gliomas after surgical and high-dose radiation therapy.