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  • Author or Editor: Kaith K. Almefty x
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Kaith K. Almefty, Svetlana Pravdenkova, Jeffrey R. Sawyer and Ossama Al-Mefty

Object

Cytogenetic studies of chordomas are scarce and show multiple changes involving different chromosomes. These abnormalities are implicated in the pathogenesis of chordoma, but the clinical significance of these changes is yet to be determined. In this study, the authors discuss the cytogenetic changes in a large series of skull base chordomas with long-term follow-up and focus on the impact of these changes on the prognosis, progression, and management of the disease.

Methods

The karyotypes of chordomas in 64 patients (36 men and 28 women) were studied in relation to survival and recurrence or progression over a mean follow-up period of 48 ± 37.5 months. The standard G-banding technique was used for karyotype analysis. Statistical analysis was performed with the Fisher exact test and ORs, and Kaplan-Meier curves were generated for survival and recurrence/progression of disease.

Results

Seventy-four percent of de novo chordomas had normal karyotypes and a 3% recurrence rate; there was a 45% recurrence rate in de novo tumors with abnormal karyotypes (p < 0.01). Recurrent tumors were associated with a high incidence of abnormal karyotype (75%). The OR for recurrence in lesions with an abnormal versus a normal karyotype was 12. Aberrations in chromosomes 3, 4, 12, 13, and 14 were associated with frequent recurrence and decreased survival time. Ninety-five percent of cases with progression involved chromosome 3 and/or 13. The median survival time was 4 months when both of these chromosomes had aberrations (p = 0.02).

Conclusions

Chordomas with normal karyotypes were associated with a low rate of recurrence and a long patient survival, and recurrent chordomas were associated with an abnormal karyotype, disease progression, and poor survival. De novo chordomas with normal karyotypes may be amenable to radical resection and adjunctive proton beam therapy. Recurrent and de novo chordomas with abnormal karyotypes were associated with complex cytogenetic abnormalities and a poor prognosis, particularly in the presence of aberrations underlying tumor progression in chromosomes 3, 4, 12, 13, and 14.

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Mohammad Abolfotoh, Wenya Linda Bi, Chang-Ki Hong, Kaith K. Almefty, Abraham Boskovitz, Ian F. Dunn and Ossama Al-Mefty

OBJECT

The combined microscopic and endoscopic technique has shown significant advantages in the management of various lesions through different approaches. Endoscopic-assisted techniques have frequently been applied to cerebellopontine angle (CPA) surgery in the context of minimally invasive craniotomies. In this paper the authors report on the use of the endoscope in the CPA as a tool to increase the extent of resection, minimize complications, and preserve the function of the delicate CPA structures. They also describe a technique of the simultaneous use of the microscope and endoscope in the CPA and dissection of CPA tumors under tandem endoscopic and microscopic vision to overcome the shortcomings of introducing the endoscope alone in the CPA. The reliability of using the microscope alone in dissecting CPA tumors is evaluated, as is the effectiveness of the combined technique in increasing the resectability of various types of CPA tumors.

METHODS

The authors conducted a retrospective analysis of 50 patients who underwent combined microscopic-endoscopic resection of CPA tumors by the senior author over a period of 3 years (February 2011 to February 2014) at Brigham and Women's Hospital, Harvard Medical School. The reliability of the extent of microscopic removal was evaluated with endoscopic exploration. Additional resection was performed with both microscopic and the combined microscopic-endoscopic technique. Endoscopically verified total resection was validated by intraoperative or postoperative MRI. The function of the cranial nerves was evaluated to assess the impact of the combined technique on their function.

RESULTS

A tumor remnant was endoscopically identified in 69% of the 26 patients who were believed to have microscopic total resection. The utilization of the endoscopic visualization and dissection increased endoscopically verified total removal to 38 patients, and 82% of these patients had no sign of residual tumor on postoperative imaging. The technique was most effective with epidermoid tumors. There were a total of 17 new cranial nerve deficits in 10 patients. Preoperative fifth cranial nerve deficits improved in 52% and hearing improved in 29% of patients after surgery.

CONCLUSIONS

This method provides simultaneous microscopic and endoscopic visualization and dissection techniques through skull-base approaches to CPA tumors. It overcomes some of the shortcomings of endoscopic-assisted surgery, further extends the surgical field, and increases the radicality of tumor resection with good functional outcomes.

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Kaith K. Almefty, Andrew F. Ducruet, R. Webster Crowley, Ruth Bristol, Sean D. Lavine and Felipe C. Albuquerque

The authors report the presentation and management of a 13-year-old girl with Schimmelpenning syndrome, a rare neurocutaneous syndrome; this patient suffered hemorrhage of a spinal arteriovenous malformation. This is the first case of a spinal arteriovenous malformation reported in association with Schimmelpenning syndrome. Neurosurgeons should be aware of this rare phacomatosis as well as of the various neurological disorders associated with this diagnosis. The threshold for imaging the neuraxis in these patients should be low.

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Evgenii Belykh, Ting Lei, Sam Safavi-Abbasi, Kaan Yagmurlu, Rami O. Almefty, Hai Sun, Kaith K. Almefty, Olga Belykh, Vadim A. Byvaltsev, Robert F. Spetzler, Peter Nakaji and Mark C. Preul

OBJECTIVE

Microvascular anastomosis is a basic neurosurgical technique that should be mastered in the laboratory. Human and bovine placentas have been proposed as convenient surgical practice models; however, the histologic characteristics of these tissues have not been compared with human cerebral vessels, and the models have not been validated as simulation training models. In this study, the authors assessed the construct, face, and content validities of microvascular bypass simulation models that used human and bovine placental vessels.

METHODS

The characteristics of vessel segments from 30 human and 10 bovine placentas were assessed anatomically and histologically. Microvascular bypasses were performed on the placenta models according to a delineated training module by “trained” participants (10 practicing neurosurgeons and 7 residents with microsurgical experience) and “untrained” participants (10 medical students and 3 residents without experience). Anastomosis performance and impressions of the model were assessed using the Northwestern Objective Microanastomosis Assessment Tool (NOMAT) scale and a posttraining survey.

RESULTS

Human placental arteries were found to approximate the M2–M4 cerebral and superficial temporal arteries, and bovine placental veins were found to approximate the internal carotid and radial arteries. The mean NOMAT performance score was 37.2 ± 7.0 in the untrained group versus 62.7 ± 6.1 in the trained group (p < 0.01; construct validity). A 50% probability of allocation to either group corresponded to 50 NOMAT points. In the posttraining survey, 16 of 17 of the trained participants (94%) scored the model's replication of real bypass surgery as high, and 16 of 17 (94%) scored the difficulty as “the same” (face validity). All participants, 30 of 30 (100%), answered positively to questions regarding the ability of the model to improve microsurgical technique (content validity).

CONCLUSIONS

Human placental arteries and bovine placental veins are convenient, anatomically relevant, and beneficial models for microneurosurgical training. Microanastomosis simulation using these models has high face, content, and construct validities. A NOMAT score of more than 50 indicated successful performance of the microanastomosis tasks.

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Ali M. Elhadi, Joseph M. Zabramski, Kaith K. Almefty, George A. C. Mendes, Peter Nakaji, Cameron G. McDougall, Felipe C. Albuquerque, Mark C. Preul and Robert F. Spetzler

OBJECT

Hemorrhagic origin is unidentifiable in 10%–20% of patients presenting with spontaneous subarachnoid hemorrhage (SAH). While the patients in such cases do well clinically, there is a lack of long-term angiographic followup. The authors of the present study evaluated the long-term clinical and angiographic follow-up of a patient cohort with SAH of unknown origin that had been enrolled in the Barrow Ruptured Aneurysm Trial (BRAT).

METHODS

The BRAT database was searched for patients with SAH of unknown origin despite having undergone two or more angiographic studies as well as MRI of the brain and cervical spine. Follow-up was available at 6 months and 1 and 3 years after treatment. Analysis included demographic details, clinical outcome (Glasgow Outcome Scale, modified Rankin Scale [mRS]), and repeat vascular imaging.

RESULTS

Subarachnoid hemorrhage of unknown etiology was identified in 57 (11.9%) of the 472 patients enrolled in the BRAT study between March 2003 and January 2007. The mean age for this group was 51 years, and 40 members (70%) of the group were female. Sixteen of 56 patients (28.6%) required placement of an external ventricular drain for hydrocephalus, and 4 of these subsequently required a ventriculoperitoneal shunt. Delayed cerebral ischemia occurred in 4 patients (7%), leading to stroke in one of them. There were no rebleeding events. Eleven patients were lost to followup, and one patient died of unrelated causes. At the 3-year follow-up, 4 (9.1%) of 44 patients had a poor outcome (mRS > 2), and neurovascular imaging, which was available in 33 patients, was negative.

CONCLUSIONS

Hydrocephalus and delayed cerebral ischemia, while infrequent, do occur in SAH of unknown origin. Long-term neurological outcomes are generally good. A thorough evaluation to rule out an etiology of hemorrhage is necessary; however, imaging beyond 6 weeks from ictus has little utility, and rebleeding is unexpected.

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Jay D. Turner, Richard Williamson, Kaith K. Almefty, Peter Nakaji, Randall Porter, Victor Tse and M. Yashar S. Kalani

MicroRNAs (miRNAs) are now recognized as the primary RNAs involved in the purposeful silencing of the cell's own message. In addition to the established role of miRNAs as developmental regulators of normal cellular function, they have recently been shown to be important players in pathological states such as cancer. The authors review the literature on the role of miRNAs in the formation and propagation of gliomas and medulloblastomas, highlighting the potential of these molecules and their inhibitors as therapeutics.