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Joseph R. Linzey, Christopher S. Graffeo, Justin Z. Wang, Ali S. Haider and Naif M. Alotaibi

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Christopher S. Graffeo, Avital Perry, Ross C. Puffer, Lucas P. Carlstrom, Wendy Chang, Grant W. Mallory and Michelle J. Clarke

OBJECTIVE

Type II odontoid fracture is a common injury among elderly patients, particularly given their predisposition toward low-energy falls. Previous studies have demonstrated a survival advantage following early surgery among patients older than 65 years, yet octogenarians represent a medically distinct and rapidly growing population. The authors compared operative and nonoperative management in patients older than 79 years.

METHODS

A single-center prospectively maintained trauma database was reviewed using ICD-9 codes to identify octogenarians with C-2 cervical fractures between 1998 and 2014. Cervical CT images were independently reviewed by blinded neurosurgeons to confirm a Type II fracture pattern. Prospectively recorded outcomes included Glasgow Coma Scale (GCS) score, Abbreviated Injury Scale (AIS) score, Injury Severity Score (ISS), additional cervical fracture, and cord injury. Primary end points were mortality at 30 days and at 1 year. Statistical tests included the Student t-test, chi-square test, Fisher's exact test, Kaplan-Meier test, and Cox proportional hazard.

RESULTS

A total of 111 patients met inclusion criteria (94 nonoperative and 17 operative [15 posterior and 2 anterior]). Mortality data were available for 100% of patients. The mean age was 87 years (range 80–104 years). Additional cervical fracture, spinal cord injury, GCS score, AIS score, and ISS were not associated with either management strategy at the time of presentation. The mean time to death or last follow-up was 22 months (range 0–129 months) and was nonsignificant between operative and nonoperative groups (p = 0.3). Overall mortality was 13% in-hospital, 26% at 30 days, and 41% at 1 year. Nonoperative and operative mortality rates were not significant at any time point (12% vs 18%, p = 0.5 [in-hospital]; 27% vs 24%, p = 0.8 [30-day]; and 41% vs 41%, p = 1.0 [1-year]). Kaplan-Meier analysis did not demonstrate a survival advantage for either management strategy. Spinal cord injury, GCS score, AIS score, and ISS were significantly associated with 30-day and 1-year mortality; however, Cox modeling was not significant for any variable. Additional cervical fracture was not associated with increased mortality. The rate of nonhome disposition was not significant between the groups.

CONCLUSIONS

Type II odontoid fracture is associated with high morbidity among octogenarians, with 41% 1-year mortality independent of intervention—a dramatic decrease from actuarial survival rates for all 80-, 90-, and 100-year-old Americans. Poor outcome is associated with spinal cord injury, GCS score, AIS score, and ISS.

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Maria Peris-Celda, Avital Perry, Lucas P. Carlstrom, Christopher S. Graffeo, Colin L. W. Driscoll and Michael J. Link

OBJECTIVE

Middle fossa surgery is challenging, and reliable surgical landmarks are essential to perform accurate and safe surgery. Although many descriptions of the middle fossa components have been published, a clinically practical description of this very complex anatomical region is lacking. Small structure arrangements in this area are often not well visualized or accurately demarcated with neuronavigation systems. The objective is to describe a “roadmap” of key surgical reference points and landmarks during middle fossa surgery to help the surgeon predict where critical structures will be located.

METHODS

The authors studied 40 dry skulls (80 sides) obtained from the anatomical board at their institution. Measurements of anatomical structures in the middle fossa were made with a digital caliper and a protractor, taking as reference the middle point of the external auditory canal (MEAC). The results were statistically analyzed.

RESULTS

The petrous part of the temporal bone was found at a mean of 16 mm anterior and 24 mm posterior to the MEAC. In 87% and 99% of the sides, the foramen ovale and foramen spinosum, respectively, were encountered deep to the zygomatic root. The posterior aspect of the greater superficial petrosal nerve (GSPN) groove was a mean of 6 mm anterior and 25 mm medial to the MEAC, nearly parallel to the petrous ridge. The main axis of the IAC projected to the root of the zygoma in all cases. The internal auditory canal (IAC) porus was found 5.5 mm lateral and 4.5 mm deep to the lateral aspect of the trigeminal impression along the petrous ridge (mean measurement values). A projection from this point to the middle aspect of the root of the zygoma, being posterior to the GSPN groove, could estimate the orientation of the IAC.

CONCLUSIONS

In middle fossa approaches, the external acoustic canal is a reliable reference before skin incision, whereas the zygomatic root becomes important after the skin incision. Deep structures can be related to these 2 anatomical structures. An easy method to predict the location of the IAC in surgery is described. Careful study of the preoperative imaging is essential to adapt this knowledge to the individual anatomy of the patient.

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Avital Perry, Christopher S. Graffeo, Waleed Brinjikji, William R. Copeland III, Alejandro A. Rabinstein and Michael J. Link

Spontaneous intracranial hypotension (SIH) is an uncommon headache etiology, typically attributable to an unprovoked occult spinal CSF leak. Although frequently benign, serious complications may occur, including cerebral venous thrombosis (CVT). The objective of this study was to examine a highly complicated case of CVT attributable to SIH as a lens for understanding the heterogeneous literature on this rare complication, and to provide useful, evidence-based, preliminary clinical recommendations. A 43-year-old man presented with 1 week of headache, dizziness, and nausea, which precipitously evolved to hemiplegia. CT venography confirmed CVT, and therapeutic heparin was initiated. He suffered a generalized seizure due to left parietal hemorrhage, which subsequently expanded. He developed signs of mass effect and herniation, heparin was discontinued, and he was taken to the operating room for clot evacuation and external ventricular drain placement. Intraoperatively, the dura was deflated, suggesting underlying SIH. Ventral T-1 CSF leak was identified, which failed multiple epidural blood patches and required primary repair. The patient ultimately made a complete recovery. Systematic review identified 29 publications describing 36 cases of SIH-associated CVT. Among 31 patients for whom long-term neurological outcome was reported, 25 (81%) recovered completely. Underlying coagulopathy/risk factors were identified in 11 patients (31%). CVT is a rare and potentially lethal sequela occurring in 2% of SIH cases. Awareness of the condition is poor, risking morbid complications. Evaluation and treatment should be directed toward identification and treatment of occult CSF leaks. Encouragingly, good neurological outcomes can be achieved through vigilant multidisciplinary neurosurgical and neurocritical care.

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Maria Peris-Celda, Soliman Oushy, Avital Perry, Christopher S. Graffeo, Lucas P. Carlstrom, Richard S. Zimmerman, Fredric B. Meyer, Bruce E. Pollock and Michael J. Link

OBJECTIVE

Geniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN).

METHODS

A retrospective review of a prospective neurosurgical database at our institution was performed, 2000–2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem.

RESULTS

Eleven patients were surgically treated for GN—9 primary, 2 reoperations. The median age was 48, 7 patients were female, and the median follow-up was 11 months (range 3–143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)—all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients.

CONCLUSIONS

GN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.

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Avital Perry, Christopher S. Graffeo, Lucas P. Carlstrom, William J. Anding, Michael J. Link and Leonardo Rangel-Castilla

OBJECTIVE

Sylvian fissure dissection following subarachnoid hemorrhage (SAH) is a challenging but fundamental skill in microneurosurgery, and one that has become increasingly difficult to develop during residency, given the overarching management trends. The authors describe a novel rodent model for simulation of sylvian fissure dissection and cerebrovascular bypass under SAH conditions.

METHODS

A standardized microvascular anastomosis model comprising rat femoral arteries and veins was used for the experimental framework. In the experimental protocol, following exposure and skeletonization of the vessels, extensive, superficial (1- to 2-mm) soft-tissue debridement was conducted and followed by wound closure and delayed reexploration at intervals of 7, 14, and 28 days. Two residents dissected 1 rat each per time point (n = 6 rats), completing vessel skeletonization followed by end-to-end artery/vein anastomoses. Videos were reviewed postprocedure to assess scar score and relative difficulty of dissection by blinded raters using 4-point Likert scales.

RESULTS

At all time points, vessels were markedly invested in friable scar, and exposure was subjectively assessed as a reasonable surrogate for sylvian fissure dissection under SAH conditions. Scar score and relative difficulty of dissection both indicated 14 days as the most challenging time point.

CONCLUSIONS

The authors’ experimental model of femoral vessel skeletonization, circumferential superficial soft-tissue injury, and delayed reexploration provides a novel approximation of sylvian fissure dissection and cerebrovascular bypass under SAH conditions. The optimal reexploration interval appears to be 7–14 days. To the authors’ knowledge, this is the first model of SAH simulation for microsurgical training, particularly in a live animal system.

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Christopher S. Graffeo, Avital Perry, Lucas P. Carlstrom, Fredric B. Meyer, John L. D. Atkinson, Dana Erickson, Todd B. Nippoldt, William F. Young Jr., Bruce E. Pollock and Jamie J. Van Gompel

OBJECTIVE

Nelson-Salassa syndrome (NSS) is a rare consequence of bilateral adrenalectomy (ADX) for refractory hypercortisolism due to Cushing disease (CD). Although classically defined by rapid growth of a large, invasive, adrenocorticotropin hormone (ACTH)–secreting pituitary tumor after bilateral ADX that causes cutaneous hyperpigmentation, visual disturbance, and high levels of ACTH, clinical experience suggests more variability.

METHODS

The authors conducted a retrospective chart review of all patients 18 years and older with a history of bilateral ADX for CD, adequate pituitary MRI, and at least 2 years of clinical follow-up. Statistical tests included Student's t-test, chi-square test, Fisher's exact test, multivariate analysis, and derived receiver operating characteristic curves.

RESULTS

Between 1956 and 2015, 302 patients underwent bilateral ADX for the treatment of hypercortisolism caused by CD; 88 had requisite imaging and follow-up (mean 16 years). Forty-seven patients (53%) had radiographic progression of pituitary disease and were diagnosed with NSS. Compared with patients who did not experience progression, those who developed NSS were significantly younger at the time of CD diagnosis (33 vs 44 years, p = 0.007) and at the time of bilateral ADX (35 vs 49 years, p = 0.007), had larger tumors at the time of CD diagnosis (6 mm vs 1 mm, p = 0.03), and were more likely to have undergone external-beam radiation therapy (EBRT, 43% vs 12%, p = 0.005). Among NSS patients, the mean tumor growth was 7 mm/yr (SE 6 mm/yr); the median tumor growth was 3 mm/yr. Prevalence of pathognomonic symptoms was low; the classic triad occurred in 9%, while hyperpigmentation without visual field deficit was observed in 23%, and 68% remained asymptomatic despite radiographic disease progression. NSS required treatment in 14 patients (30%).

CONCLUSIONS

NSS is a prevalent sequela of CD after bilateral ADX and affects more than 50% of patients. However, although radiological evidence of NSS is common, it is most often clinically indolent, with only a small minority of patients developing the more aggressive disease phenotype characterized by clinically meaningful symptoms and indications for treatment. Young age at the time of CD diagnosis or treatment with bilateral ADX, large tumor size at CD diagnosis, and EBRT are associated with progression to NSS and may be markers of aggressiveness.

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Christopher S. Graffeo, Kathryn M. Van Abel, Jonathan M. Morris, Matthew L. Carlson, Jamie J. Van Gompel, Eric J. Moore, Daniel L. Price, Jan L. Kasperbauer, Jeffrey R. Janus, Kerry D. Olsen and Michael J. Link

OBJECTIVE

Vagus nerve and sympathetic chain cervical schwannomas (VNCSs and SCCSs) are benign nerve sheath tumors that arise in the head and neck. Despite similar presentations that make accurate preoperative diagnosis more difficult, the potential for morbidity following resection is significantly higher for patients with VNCS. Therefore, the authors analyzed a retrospective case series and performed a comparative analysis of the literature to establish diagnostic criteria to facilitate more accurate preoperative diagnoses.

METHODS

The authors conducted a blinded review of imaging studies from retrospectively collected, operatively confirmed cases of VNCS and SCCS. They also performed a systematic review of published series that reported patient-specific preoperative imaging findings in VNCS or SCCS.

RESULTS

Nine patients with VNCS and 11 with SCCS were identified. In the study cohort, splaying of the internal carotid artery (ICA) and internal jugular vein (IJV) did not significantly predict the nerve of origin (p = 0.06); however, medial and lateral ICA displacement were significantly associated with VNCS and SCCS, respectively (p = 0.01 and p = 0.003, respectively). Multivariate analysis demonstrated that ICA and IJV splaying with medial ICA displacement carried an 86% probability of VNCS (p = 0.001), while the absence of splaying with lateral ICA displacement carried a 91% probability of SCCS (p = 0.006). The presence of vocal cord symptoms or peripheral enhancement significantly augmented the predictive probability of VNCS, as did Horner's syndrome or homogeneous enhancement for SCCS.

A review of the literature produced 25 publications that incorporated a total of 106 patients, including the present series. Splaying of the ICA and IJV was significantly, but not uniquely, associated with VNCS (p < 0.0001); multivariate analysis demonstrated that ICA and IJV splaying with medial ICA displacement carries a 75% probability of VNCS (p < 0.0001), while the absence of such splaying with lateral ICA displacement carries an 87% probability of SCCS (p = 0.0003).

CONCLUSIONS

ICA and IJV splaying frequently predicts VNCS; however, this finding is also commonly observed in SCCS and, among the 9 cases in the present study, was observed more often than previously reported. When congruent with splaying, medial or lateral ICA displacement significantly enhances the reliability of preoperative predictions, empowering more accurate prognostication.

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Hirokazu Takami, Christoph M. Prummer, Christopher S. Graffeo, Maria Peris-Celda, Caterina Giannini, Colin L. Driscoll and Michael J. Link

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.