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

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Tyler S. Cole and Robert F. Spetzler

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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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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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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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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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Enrico Giordan, Christopher S. Graffeo, Alejandro A. Rabinstein, Robert D. Brown Jr., Walter A. Rocca, Alanna M. Chamberlain, and Giuseppe Lanzino

OBJECTIVE

Recent population-based and hospital cohort studies have reported a decreasing incidence of aneurysmal subarachnoid hemorrhage (aSAH) and declining aSAH-associated case-fatality rates. Principal drivers of these trends are debated, but improvements in smoking cessation and hypertension control may be critical factors.

METHODS

The population-based medical records linkage system of the Rochester Epidemiological Project was used to document aSAH incidence and 30-day case fatality rates during a 20-year study period (1996–2016) in Olmsted County, Minnesota. Incidence rates in the study period were compared with data from a previous Olmsted County study concerning aSAH incidence from 1965 to 1995 and with regional trends in tobacco use.

RESULTS

One hundred nineteen incident cases of aSAH were included. The median age at hemorrhage was 59 years (range 16–94 years), and 74 patients were female (62.2%). The overall average annual aSAH incidence rate was 4.2/100,000 person-years (P-Y). The aSAH incidence rate decreased from 5.7/100,000 in 1996 to 3.5/100,000 P-Y in 2011–2016. The overall aSAH-associated 30-day case-fatality rate was 21.9% and declined by approximately 0.5% annually. An accelerated decline in the fatality rate (0.9%/year) was observed from 2006–2016. Smoking among adult Olmsted County residents decreased from 20.4% in 2000 to 9.1% in 2018.

CONCLUSIONS

A decline in the incidence of aSAH and 30-day case-fatality rate from 1996 to 2016 was observed, as well as an accelerated decline of the fatality rate from 2006 to 2016. These findings confirm and extend the trends reported by prior studies in the same population. The decrease in aSAH in the years studied paralleled a noticeable reduction in the population smoking rates.

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Scott L. Zuckerman, Natalie Limoges, Aaron M. Yengo-Kahn, Christopher S. Graffeo, Lola B. Chambless, Rohan Chitale, J Mocco, and Susan Durham

OBJECTIVE

Residency interviews are integral to the recruitment process yet imperfect. Through surveys of neurosurgery residency applicants, the authors describe interview content and the perceived utility and stress of topics from the applicant’s perspective.

METHODS

All 2018–2019 neurosurgery resident applicants applying to three particular programs were surveyed. Across 10 interview topics, survey questions assessed topic frequency and the applicant’s opinion of the utility and stress of each topic (Likert scale 1–5). Analyses included descriptive statistics, Spearman’s rank correlation, and logistic regression.

RESULTS

One hundred thirty-three of 265 surveyed US residency applicants (50%) responded. Extracurricular activities, research, future career, non-medicine interests, and small talk were discussed in all interviews. The least frequent topics included neurosurgical knowledge assessment (79%) and manual dexterity tests (45%). The most useful topics according to respondents were future career objectives (4.78 ± 0.49) and prior research (4.76 ± 0.50); the least useful were neurosurgical knowledge assessment (2.67 ± 1.09) and manual dexterity tests (2.95 ± 1.05). The most stressful topics were neurosurgical knowledge assessment (3.66 ± 1.23) and ethical/behavioral scenarios (2.94 ± 1.28). The utility and stress of manual dexterity tests and neurosurgical knowledge assessments were inversely correlated (r = −0.40, p < 0.01; r = −0.36, p < 0.01), whereas no such correlation existed for ethical/behavioral questions (r = −0.12, p = 0.18), indicating that ethical/behavioral questions may have been stressful but were potentially useful topics. Respondents who attended ≥ 15 interviews were more likely to be asked about the three most stressful topics (each p < 0.05). Respondents with children were less likely to be asked about ethical/behavioral scenarios (OR 0.13, 95% CI 0.03–0.52, p < 0.01).

CONCLUSIONS

Applicants found several of the most frequently discussed topics to be less useful, indicating a potential disconnect between applicant opinion and the faculty’s preferred questions. Ethical/behavioral scenarios were rated as stressful but still useful, representing a potentially worthwhile type of question. These data provide several avenues for potential standardization and improvement of the interview process.

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Juliana Rotter, Victor M. Lu, Christopher S. Graffeo, Avital Perry, Colin L. W. Driscoll, Bruce E. Pollock, and Michael J. Link

OBJECTIVE

Intracranial facial nerve schwannomas (FNS) requiring treatment are frequently recommended for surgery or stereotactic radiosurgery (SRS). The objective of this study was to compare facial nerve function outcomes between these two interventions for FNS via a systematic review and meta-analysis.

METHODS

A search of the Ovid EMBASE, PubMed, SCOPUS, and Cochrane databases from inception to July 2019 was conducted following PRISMA guidelines. Articles were screened against prespecified criteria. Facial nerve outcomes were classified as improved, stabilized, or worsened by last follow-up. Incidence was pooled by random-effects meta-analysis of proportions.

RESULTS

Thirty-three articles with a pooled cohort of 519 patients with FNS satisfied all criteria. Twenty-five articles described operative outcomes in 407 (78%) patients; 10 articles reported SRS outcomes in 112 (22%). In the surgical cohort, facial nerve function improved in 23% (95% CI 15%–32%), stabilized in 41% (95% CI 32%–50%), and worsened in 30% (95% CI 21%–40%). In the SRS cohort, facial nerve function was improved in 20% (95% CI 9%–34%), stable in 66% (95% CI 54%–78%), and worsened in 9% (95% CI 3%–16%). Compared with SRS, microsurgery was associated with a significantly lower incidence of stable facial nerve function (p < 0.01) and a significantly higher incidence of worsened facial nerve function (p < 0.01). Tumor progression and complication rates were comparable. Outcome certainty assessments were very low to moderate for all parameters.

CONCLUSIONS

Unfavorable facial nerve function outcomes are associated with surgical treatment of intracranial FNS, whereas stable facial nerve function outcomes are associated with SRS. Therefore, SRS should be recommended to patients with FNS who require treatment, and surgery should be reserved for patients with another indication, such as decompression of the brainstem. Further study is required to definitively optimize and validate management strategies for these rare skull base tumors.

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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.