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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.

Open access

Avital Perry, Thomas J. Sorenson, Christopher S. Graffeo, Colin L. Driscoll and Michael J. Link

Cavernous malformations (CMs) are low-pressure, focal, vascular lesions that may occur within the brainstem and require treatment, which can be a substantial challenge. Herein, we demonstrate the surgical resection of a hemorrhaged brainstem CM through a posterior petrosectomy approach. After dissection of the overlying vascular and meningeal structures, a safe entry zone into the brainstem is identified based on local anatomy and intraoperative neuronavigation. Small ultrasound probes can also be useful for obtaining real-time intraoperative feedback. The CM is internally debulked and resected in a piecemeal fashion through an opening smaller than the CM itself. As brainstem CMs are challenging lesions, knowledge of several surgical nuances and adoption of careful microsurgical techniques are requisite for success.

The video can be found here: https://youtu.be/szB6YpzkuCo.

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Jenna Meyer, Avital Perry, Soliman Oushy, Christopher S. Graffeo, Lucas P. Carlstrom and Fredric B. Meyer

Pediatric pituitary adenomas (PPAs) are rare neoplasms with a propensity for unusual presentations and an aggressive clinical course. Here, the authors describe 6 highly atypical PPAs to highlight this tendency and discuss unexpected management challenges.

A 14-year-old girl presented with acute hemiparesis and aphasia. MRI revealed a pituitary macroadenoma causing internal carotid artery invasion/obliteration without acute apoplexy, which was treated via emergent transsphenoidal resection (TSR). Another 14-year-old girl developed precocious galactorrhea due to macroprolactinoma, which was medically managed. Several years later, she re-presented with acute, severe, bitemporal hemianopia during her third trimester of pregnancy, requiring emergent induction of labor followed by TSR. A 13-year-old boy was incidentally diagnosed with a prolactinoma after routine orthodontic radiographs captured a subtly abnormal sella. An 18-year-old male self-diagnosed pituitary gigantism through a school report on pituitary disease. A 17-year-old boy was diagnosed with Cushing disease by his basketball coach, a former endocrinologist. A 12-year-old girl with growth arrest and weight gain was diagnosed with Cushing disease, which was initially treated via TSR but subsequently recurred and ultimately required 12 operations, 5 radiation treatments involving 3 modalities, bilateral adrenalectomy, and chemotherapy. Despite these efforts, she ultimately died from pituitary carcinoma.

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Hirokazu Takami, Christopher S. Graffeo, Avital Perry, Caterina Giannini and David J. Daniels

OBJECTIVE

Intracranial germ cell tumors (iGCTs) often arise at the neurohypophysis, their second most common origination, following the pineal region. Neurohypophyseal iGCTs present with stereotypical symptoms, including pituitary dysfunction and visual field deficit, due to their suprasellar location. The goal of this study was to present a large, longitudinal single-institution experience with neurohypophyseal iGCTs to better understand their natural history and identify opportunities for further improvement in treatment outcomes.

METHODS

This is a retrospective, single-institution cohort study of neurohypophyseal iGCTs treated between 1988 and 2017, with a focus on the epidemiology, presentation, natural history, and treatment.

RESULTS

Thirty-five neurosurgically managed patients met inclusion criteria; the median age was 18 years (3 months to 49 years), and 74% of patients were male (n = 26). Thirty-one tumors were germinomas, and 4 were nongerminomatous iGCTs. Presenting symptoms included pituitary insufficiency in 76% (n = 25), visual deficit in 45% (n = 15), and diabetes insipidus (DI) in 61% (n = 20) of patients. Index symptoms included isolated DI in 10 (36%), isolated hormone deficiency in 14 (50%), and concomitant DI and hormone deficiency symptoms in 4 (14%). Radiographic diagnostic latency was common, occurring at a median of 363 days (range 9–2626 days) after onset of the first symptoms and was significantly associated with both DI and hormone deficiency as the index symptoms (no DI vs DI: 360 vs 1083 days, p = 0.009; no hormone deficiency vs hormone deficiency: 245 vs 953 days, p = 0.004). Biochemical abnormalities were heterogeneous; each pituitary axis was dysfunctional in at least 1 patient, with most patients demonstrating at least 2 abnormalities, and pretreatment dysfunction demonstrating a nonsignificant trend toward association with long-term posttreatment hormone supplementation. Among germinomas, whole-brain or whole-ventricle radiotherapy demonstrated significantly improved progression-free and overall survival compared with local therapy (p = 0.009 and p = 0.004, respectively).

CONCLUSIONS

Neurohypophyseal iGCTs are insidious tumors that may pose a diagnostic dilemma, as evidenced by the prolonged latency before radiographic confirmation. Serial imaging and close endocrine follow-up are recommended in patients with a characteristic clinical syndrome and negative imaging, due to the propensity for radiographic latency. Pretreatment biochemical abnormalities may indicate higher risk of posttreatment pituitary insufficiency, and all patients should receive careful endocrine follow-up. Local radiotherapy is prone to treatment failure, while whole-ventricle treatment is associated with improved survival in germinomas.

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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.

Free access

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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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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Satoshi Kiyofuji, Amanda M. Casabella, Christopher S. Graffeo, Avital Perry, James A. Garrity and Michael J. Link

OBJECTIVE

Sphenoorbital meningioma (SOM) is a unique skull base tumor, characterized by infiltrative involvement and hyperostosis primarily of the lesser wing of sphenoid bone, with frequent involvement of the orbital compartment. SOM often manifests with proptosis and visual impairment. Surgical technique and outcome are highly variable among studies reported in the literature. The authors present a single-surgeon experience with SOM.

METHODS

A retrospective review of a prospectively maintained institutional database was performed. A blinded imaging review by 2 study team members was completed to confirm SOM, after which chart review was carried out to capture demographics and outcomes. All statistical testing was completed using JMP Pro version 14.1.0, with significance defined as p < 0.05.

RESULTS

Forty-seven patients who underwent surgery between 2000 and 2017 were included. The median age at surgery was 47 years (range 36–70 years), 81% of patients were female, and the median follow-up was 43 months (range 0–175 months). All operations were performed via a frontotemporal craniotomy, orbitooptic osteotomy, and anterior clinoidectomy, with extensive resection of all involved bone and soft tissue. Preoperatively, proptosis was noted in 44 patients, 98% of whom improved. Twenty-eight patients (60%) had visual deficits before surgery, 21 (75%) of whom improved during follow-up. Visual field defect other than a central scotoma was the only prognostic factor for improvement in vision on multivariate analysis (p = 0.0062). Nine patients (19%) had recurrence or progression during follow-up.

CONCLUSIONS

SOM is a unique skull base tumor that needs careful planning to optimize outcome. Aggressive removal of involved bone and periorbita is crucial, and proptosis and visual field defect other than a central scotoma can improve after surgery.

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Soliman Oushy, Avital Perry, Christopher S. Graffeo, Aditya Raghunathan, Lucas P. Carlstrom and David J. Daniels

OBJECTIVE

Ganglioglioma is a low-grade central nervous system neoplasm with a pediatric predominance, accounting for 10% of all brain tumors in children. Gangliogliomas of the cervicomedullary junction (GGCMJs) and brainstem (GGBSs) present a host of management challenges, including a significant risk of surgical morbidity. At present, understanding of the prognostic factors—including BRAF V600E status—is incomplete. Here, the authors report a single-institution GGCMJ and GGBS experience and review the pertinent literature.

METHODS

A prospectively maintained neurosurgical database at a large tertiary care academic referral center was retrospectively queried for cases of GGCMJ pathologically confirmed in the period from 1995 to 2015; appropriate cases were defined by diagnosis codes and keywords. Secondary supplemental chart review was conducted to confirm or capture relevant data. The primary study outcome was treatment failure as defined by evidence of radiographic recurrence or progression and/or clinical or functional decline. A review of the literature was conducted as well.

RESULTS

Five neurosurgically managed GGBS patients were identified, and the neoplasms in 4 were classified as GGCMJ. All 5 patients were younger than 18 years old (median 15 years, range 4–16 years) and 3 (60%) were female. One patient underwent gross-total resection, 2 underwent aggressive subtotal resection (STR), and 2 underwent stereotactic biopsy only. All patients who had undergone STR or biopsy required repeat resection for tumor control or progression. Progressive disease was treated with radiotherapy in 2 patients, chemotherapy in 2, and chemoradiotherapy alone in 1. Immunostaining for BRAF V600E was positive in 3 patients (60%). All 5 patients experienced at least one major complication, including wound infection, foot drop, hemiparesis, quadriparesis, cranial neuropathy, C2–3 subluxation, syringomyelia, hydrocephalus, aspiration, and coma. Overall mortality was 20%, with 1 death observed over 11 years of follow-up.

CONCLUSIONS

GGBS and GGCMJ are rare, benign posterior fossa tumors that carry significant perioperative morbidity. Contemporary management strategies are heterogeneous and include combinations of resection, radiotherapy, and chemotherapy. The BRAF V600E mutation is frequently observed in GGBS and GGCMJ and appears to have both prognostic and therapeutic significance with targeted biological agents.