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Joanna Y. Wang, Amir H. Dorafshar, Ann Liu, Mari L. Groves and Edward S. Ahn

OBJECTIVE

Because the metopic suture normally fuses during infancy, there are varying degrees of severity in head shape abnormalities associated with premature fusion. A method for the objective and reproducible assessment of metopic synostosis is needed to guide management, as current methods are limited by their reliance on aesthetic markers. The object of this study was to describe the metopic index (MI), a simple anthropometric cranial measurement. The measurements can be obtained from CT scans and, more importantly, from palpable cranial landmarks, and the index provides a rapid tool for evaluating patients in both pre- and postoperative settings.

METHODS

High-resolution head CT scans obtained in 69 patients (age range 0–24 months) diagnosed with metopic craniosynostosis were retrospectively reviewed. Preoperative 3D reconstructions were available in 15 cases, and these were compared with 3D reconstructions of 324 CT scans obtained in a control group of 316 infants (age range 0–24 months) who did not have any condition that might affect head size or shape and also in a subset of this group, comprising 112 patients precisely matched to the craniosynostosis patients with respect to age and sex. Postoperative scans were available and reviewed in 9 of the craniosynostosis patients at a mean time of 7.1 months after surgical repair. 3D reconstructions of these scans were matched with controls based upon age and sex.

RESULTS

The mean preoperative MI for patients with trigonocephaly was 0.48 (SD 0.05), significantly lower than the mean values of 0.57 (SD 0.04) calculated on the basis of all 324 scans obtained in controls (p < 0.001) and 0.58 (SD 0.04) for the subset of 112 age- and sex-matched controls (p < 0.001). For 7 patients with both pre- and postoperative CT scans available for evaluation, the mean postoperative MI was 0.55 (SD 0.03), significantly greater than their preoperative MIs (mean 0.48 [SD 0.04], p = 0.001) and comparable to the mean MI of the controls (p = 0.30). In 4 patients, clinically obtained postoperative MIs by caliper measurement were comparable to measurements derived from CT (p = 0.141).

CONCLUSIONS

The MI is a useful measurement of the severity of trigonocephaly in patients with metopic synostosis. This simple quantitative assessment can potentially be used in the clinical setting to guide preoperative evaluation, surgical repair, and postoperative degree of correction.

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Ann Liu, Elizabeth N. Kuhn, John T. Lucas Jr., Adrian W. Laxton, Stephen B. Tatter and Michael D. Chan

OBJECT

Neurofibromatosis Type 2 (NF2) is a rare autosomal dominant disorder predisposing patients to meningiomatosis. The role of stereotactic radiosurgery (SRS) is poorly defined in NF2, and although the procedure has excellent control rates in the non-NF2 population, its utility has been questioned because radiation has been hypothesized to predispose patients to malignant transformation of benign tumors. To the authors' knowledge, this is the first study to examine the use of SRS specifically for meningiomas in patients with NF2.

METHODS

The authors searched a tumor registry for all patients with NF2 who had undergone Gamma Knife radiosurgery (GKRS) for meningioma in the period from January 1, 1999, to September 19, 2013, at a single tertiary care cancer center. Medical records were retrospectively reviewed for patient and tumor characteristics and outcomes.

Results

Among the 12 patients who met the search criteria, 125 meningiomas were identified, 87 (70%) of which were symptomatic or progressive and thus treated with GKRS. The median age at the first GKRS was 31 years (interquartile range [IQR] 27–37 years). Five patients (42%) had multiple treatments with a median of 27 months (IQR 14–50 months) until the subsequent GKRS. The median follow-up in surviving patients was 43 months (IQR 34–110 months). The 5-year local tumor control and distant treatment failure rates were 92% and 77%, respectively. Toxicities occurred in 25% of the GKRS treatments, although the majority were Grade 1 or 2. At the last follow-up, 4 patients (33%) had died a neurological death at a median age of 39 years (IQR 37–46 years), and their cases accounted for 45% of all tumors, 55% of all treated tumors, and 58% of all GKRSs. Univariate analysis revealed several predictive variables for distant failure, including male sex (HR 0.28, 95% CI 0.086–0.92, p = 0.036), age at distant failure (HR 0.92, 95% CI 0.90–0.95, p < 0.0001), and prior number of GKRS treatments (HR 1.2, 95% CI 1.1–1.4, p = 0.0049). Local failure, maximum size of the treated tumor, delivered tumor margin dose, and WHO grade were not significant. On multivariate analysis, age at distant failure (HR 0.91, 95% CI 0.88–0.95, p < 0.0001) and prior number of GKRSs (HR 1.3, 95% CI 1.1–1.5, p = 0.004) remained significant. No malignant transformation events among treated tumors were observed.

CONCLUSIONS

Radiosurgery represents a feasible modality with minimal toxicity for NF2-associated meningiomas. Increasing patient age was associated with a decreased rate of distant failure, whereas an increasing number of prior GKRS treatments predicted distant failure. Further studies are necessary to determine the long-term patterns of treatment failure in these patients.

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Ann Liu, Eric W. Sankey, C. Rory Goodwin, Thomas A. Kosztowski, Benjamin D. Elder, Ali Bydon, Timothy F. Witham, Jean-Paul Wolinsky, Ziya L. Gokaslan and Daniel M. Sciubba

OBJECT

Spinal metastases from gynecological cancers are rare, with few cases reported in the literature. In this study, the authors examine a series of patients with spinal metastases from gynecological cancer and review the literature.

METHODS

The cases of 6 consecutive patients who underwent spine surgery for metastatic gynecological cancer between 2007 and 2012 at a single institution were retrospectively reviewed. The recorded demographic, operative, and postoperative factors were reviewed, and the functional outcomes were determined by change in Karnofsky Performance Scale and the American Spine Injury Association (ASIA) score during follow-up. A systematic review of the literature was also performed to evaluate outcomes for patients with similar gynecological metastases to the spine.

RESULTS

In this series, details regarding metastatic gynecological cancers to the spine are as follows: 2 patients with cervical cancer (both presented at age 46 years, mean postoperative survival of 32 months), 2 patients with endometrial cancer (mean age of 40 years, mean postoperative survival of 26 months), and 2 patients with leiomyosarcoma (mean age of 44 years, mean postoperative survival of 20 months). All patients presented with pain, and no complications were noted following surgery. All patients with known follow-up had stable or improved neurological outcomes, performance status, and improved pain, without local recurrence of tumor. Overall median survival after diagnosis of metastatic spine lesions for all cases in the literature as well as those treated by the authors was 15 months. When categorized by type, median survival of patients with cervical cancer (n = 2), endometrial cancer (n = 26), and leiomyosarcoma (n = 16) was 32, 10, and 22.5 months, respectively.

CONCLUSIONS

Gynecological cancers metastasizing to the spine are rare. In this series, overall survival following diagnosis of spinal metastasis and surgery was 27 months, with cervical cancer, endometrial cancer, and leiomyosarcoma survival being 32, 26, and 20 months, respectively. Combined with literature cases, survival differs depending on primary histology, with decreasing survival from cervical cancer (32 months) to leiomyosarcoma (22.5 months) to endometrial cancer (10 months). Integrating such information with other patient factors may more accurately guide decision making regarding management of such spinal lesions.

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David S. Hersh, Rajiv R. Iyer, Tomas Garzon-Muvdi, Ann Liu, George I. Jallo and Mari L. Groves

OBJECTIVE

Spinal deformity has become a well-recognized complication of intramedullary spinal cord tumor (IMSCT) resection. In particular, laminectomy can result in biomechanical instability caused by loss of the posterior tension band. Therefore, laminoplasty has been proposed as an alternative to laminectomy. Here, the authors describe the largest current series of pediatric patients who have undergone laminoplasty for IMSCT resection and investigate the need for surgical fusion after both laminectomy and laminoplasty.

METHODS

The medical records of pediatric patients who underwent resection of an IMSCT at a single institution between November 2003 and May 2014 were reviewed retrospectively. Demographic, clinical, radiological, surgical, histopathological, and follow-up data were collected.

RESULTS

Sixty-six consecutive patients underwent resection of an IMSCT during the study period. Forty-three (65%) patients were male. The patients had a median age of 12.9 years (interquartile range [IQR] 7.2–16.5 years) at the time of surgery. Patients typically presented with a tumor that involved the cervical and/or thoracic spine. Nineteen (29%) patients underwent laminectomy, and 47 (71%) patients underwent laminoplasty. Patients in each cohort had a similar rate of postoperative deformity. Overall, 10 (15%) patients required instrumented spinal fusion for spinal deformity. Four patients required revision of the primary fusion.

CONCLUSIONS

These findings show that among pediatric patients with an IMSCT, postoperative surgical fusion rates remain high, even after laminoplasty. Known risk factors, such as the age of the patient, location of the tumor, and the number of involved levels, might play a larger role than replacement of the laminae in determining the rate of surgical fusion after IMSCT resection.

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Analiz Rodriguez, Matthew T. Neal, Ann Liu, Aravind Somasundaram, Wesley Hsu and Charles L. Branch Jr

Object

Symptomatic adjacent-segment lumbar disease (ASLD) after lumbar fusion often requires subsequent surgical intervention. The authors report utilizing cortical bone trajectory (CBT) pedicle screw fixation with intraoperative CT (O-arm) image-guided navigation to stabilize spinal levels in patients with symptomatic ASLD. This unique technique results in the placement of 2 screws in the same pedicle (1 traditional pedicle trajectory and 1 CBT) and obviates the need to remove preexisting instrumentation.

Methods

The records of 5 consecutive patients who underwent lumbar spinal fusion with CBT and posterior interbody grafting for ASLD were retrospectively reviewed. All patients underwent screw trajectory planning with the O-arm in conjunction with the StealthStation navigation system. Basic demographics, operative details, and radiographic and clinical outcomes were obtained.

Results

The average patient age was 69.4 years (range 58–82 years). Four of the 5 surgeries were performed with the Minimal Access Spinal Technologies (MAST) Midline Lumbar Fusion (MIDLF) system. The average operative duration was 218 minutes (range 175–315 minutes). In the entire cohort, 5.5-mm cortical screws were placed in previously instrumented pedicles. The average hospital stay was 2.8 days (range 2–3 days) and there were no surgical complications. All patients had more than 6 months of radiographic and clinical follow-up (range 10–15 months). At last follow-up, all patients reported improved symptoms from their preoperative state. Radiographic follow-up showed Lenke fusion grades of A or B.

Conclusions

The authors present a novel fusion technique that uses CBT pedicle screw fixation in a previously instrumented pedicle with intraoperative O-arm guided navigation. This method obviates the need for hardware removal. This cohort of patients experienced good clinical results. Computed tomography navigation was critical for accurate CBT screw placement at levels where previous traditional pedicle screws were already placed for symptomatic ASLD.

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Eric W. Sankey, Benjamin D. Elder, Ann Liu, Kathryn A. Carson, C. Rory Goodwin, Ignacio Jusué-Torres and Daniele Rigamonti

OBJECTIVE

Factors associated with emergency department admission and/or shunt revision for idiopathic intracranial hypertension (IIH) are unclear. In this study, the associations of several factors with emergency department admission and shunt revision for IIH were explored.

METHODS

The authors performed a retrospective review of 31 patients (169 total emergency department visits) who presented to the emergency department for IIH-related symptoms between 2003 and 2015. Demographics, comorbidities, symptoms, IIH diagnosis and treatment history, ophthalmological examination, diagnostic lumbar puncture (LP), imaging findings, and data regarding admission and management decisions were collected. Multivariable general linear models regression analysis was performed to assess the predictive factors associated with admission and shunt revision.

RESULTS

Thirty-one adult patients with a history of shunt placement for IIH visited the emergency department a total of 169 times for IIH-related symptoms, with a median of 3 visits (interquartile range 2–7 visits) per patient. Five patients had more than 10 emergency department visits. Baseline factors associated with admission included male sex (OR 10.47, 95% CI 2.13–51.56; p = 0.004) and performance of an LP (OR 3.10, 95% CI 1.31–7.31; p = 0.01). Contrastingly, older age at presentation (OR 0.94, 95% CI 0.90–0.99; p = 0.01), and a greater number of prior emergency department visits (OR 0.94, 95% CI 0.89–0.99; p = 0.02) were slightly protective against admission. The presence of papilledema (OR 11.62, 95% CI 3.20–42.16; p < 0.001), Caucasian race (OR 40.53, 95% CI 2.49–660.09 p = 0.009), and systemic hypertension (OR 7.73, 95% CI 1.11–53.62; p = 0.03) were independent risk factors for shunt revision. In addition, a greater number of prior emergency department visits (OR 0.86, 95% CI 0.77–0.96; p = 0.009) and older age at presentation (OR 0.93, 95% CI 0.87–0.99; p = 0.02) were slightly protective against shunt revision, while there was suggestive evidence that presence of a programmable shunt (OR 0.23, 95% CI 0.05–1.14; p = 0.07) was a protective factor against shunt revision. Of note, location of the proximal catheter in the ventricle or lumbar subarachnoid space was not significantly associated with admission or shunt revision in the multivariable analyses.

CONCLUSIONS

The decision to admit a shunt-treated patient from the emergency department for symptoms related to IIH is challenging. Knowledge of factors associated with the need for admission and/or shunt revision is required. In this study, factors such as male sex, younger age at presentation, lower number of prior emergency department visits, and performance of a diagnostic LP were independent predictors of admission. In addition, papilledema was strongly predictive of the need for shunt revision, highlighting the importance of an ophthalmological examination for shunt-treated adults with IIH who present to the emergency department.

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David S. Hersh, Nir Shimony, Mari L. Groves, Gerald F. Tuite, George I. Jallo, Ann Liu, Tomas Garzon-Muvdi, Thierry A. G. M. Huisman, Ryan J. Felling, Joseph A. Kufera and Edward S. Ahn

OBJECTIVE

Pediatric cerebral venous sinus thrombosis has been previously described in the setting of blunt head trauma; however, the population demographics, risk factors for thrombosis, and the risks and benefits of detection and treatment in this patient population are poorly defined. Furthermore, few reports differentiate between different forms of sinus pathology. A series of pediatric patients with skull fractures who underwent venous imaging and were diagnosed with intrinsic cerebral venous sinus thrombosis or extrinsic sinus compression is presented.

METHODS

The medical records of patients at 2 pediatric trauma centers were retrospectively reviewed. Patients who were evaluated for blunt head trauma from January 2003 to December 2013, diagnosed with a skull fracture, and underwent venous imaging were included.

RESULTS

Of 2224 pediatric patients with skull fractures following blunt trauma, 41 patients (2%) underwent venous imaging. Of these, 8 patients (20%) had intrinsic sinus thrombosis and 14 patients (34%) displayed extrinsic compression of a venous sinus. Three patients with intrinsic sinus thrombosis developed venous infarcts, and 2 of these patients were treated with anticoagulation. One patient with extrinsic sinus compression by a depressed skull fracture underwent surgical elevation of the fracture. All patients with sinus pathology were discharged to home or inpatient rehabilitation. Among patients who underwent follow-up imaging, the sinus pathology had resolved by 6 months postinjury in 80% of patients with intrinsic thrombosis as well as 80% of patients with extrinsic compression. All patients with intrinsic thrombosis or extrinsic compression had a Glasgow Outcome Scale score of 4 or 5 at their last follow-up.

CONCLUSIONS

In this series of pediatric trauma patients who underwent venous imaging for suspected thrombosis, the yield of detecting intrinsic thrombosis and/or extrinsic compression of a venous sinus was high. However, few patients developed venous hypertension or infarction and were subsequently treated with anticoagulation or surgical decompression of the sinus. Most had spontaneous resolution and good neurological outcomes without treatment. Therefore, in the setting of pediatric skull fractures after blunt injury, venous imaging is recommended when venous hypertension or infarction is suspected and anticoagulation is being considered. However, there is little indication for pervasive venous imaging after pediatric skull fractures, especially in light of the potential risks of CT venography or MR venography in the pediatric population and the unclear benefits of anticoagulation.

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C. Rory Goodwin, Eric W. Sankey, Ann Liu, Benjamin D. Elder, Thomas Kosztowski, Sheng-Fu L. Lo, Charles G. Fisher, Michelle J. Clarke, Ziya L. Gokaslan and Daniel M. Sciubba

OBJECT

Surgical procedures and/or adjuvant therapies are effective modalities for the treatment of symptomatic spinal metastases. However, clinical results specific to the skin cancer spinal metastasis cohort are generally lacking. The purpose of this study was to systematically review the literature for treatments, clinical outcomes, and survival following the diagnosis of a skin cancer spinal metastasis and evaluate prognostic factors in the context of spinal skin cancer metastases stratified by tumor subtype.

METHODS

The authors performed a literature review using PubMed, Embase, CINAHL, and Web of Science to identify articles since 1950 that reported survival, clinical outcomes, and/or prognostic factors for the skin cancer patient population with spinal metastases. The methodological quality of reviews was assessed using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) tool.

RESULTS

Sixty-five studies met the preset criteria and were included in the analysis. Of these studies, a total of 25, 40, 25, and 12 studies included patients who underwent some form of surgery, radiotherapy, chemotherapy, or observation alone, respectively. Sixty-three of the 65 included studies were retrospective in nature (Class of Evidence [CoE] IV), and the 2 prospective studies were CoE II. Based on the studies analyzed, the median overall survival for a patient with a spinal metastasis from a primary skin malignancy is 4.0 months; survival by tumor subtype is 12.5 months for patients with basal cell carcinoma (BCC), 4.0 months for those with melanoma, 4.0 months for those with squamous cell carcinoma, 3.0 months for those with pilomatrix carcinoma, and 1.5 months for those with Merkel cell carcinoma (p < 0.0001). The overall percentage of known continued disease progression after spine metastasis diagnosis was 40.1% (n = 244/608, range 25.0%–88.9%), the rate of known recurrence of the primary skin cancer lesion was 3.5% (n = 21/608, range 0.2%–100.0%), and the rate of known spine metastasis recurrence despite treatment for all skin malignancies was 2.8% (n = 17/608, range 0.0%–33.3%). Age greater than 65 years, sacral spinal involvement, presence of a neurological deficit, and nonambulatory status were associated with decreased survival in patients diagnosed with a primary skin cancer spinal metastasis. All other clinical or prognostic parameters were of low or insufficient strength.

CONCLUSIONS

Patients diagnosed with a primary skin cancer metastasis to the spine have poor overall survival with the exception of those with BCC. The median duration of survival for patients who received surgical intervention alone, medical management (chemotherapy and/or radiation) alone, or the combination of therapies was similar across interventions. Age, spinal region, and neurological status may be associated with poor survival following surgery.

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Jaclyn J. Renfrow, Analiz Rodriguez, Ann Liu, Julie G. Pilitsis, Uzma Samadani, Aruna Ganju, Isabelle M. Germano, Deborah L. Benzil and Stacey Quintero Wolfe

OBJECT

Women compose a minority of neurosurgery residents, averaging just over 10% of matched applicants per year during this decade. A recent review by Lynch et al. raises the concern that women may be at a higher risk than men for attrition, based on analysis of a cohort matched between 1990 and 1999. This manuscript aims to characterize the trends in enrollment, attrition, and postattrition careers for women who matched in neurosurgery between 2000 and 2009.

METHODS

Databases from the American Association of Neurological Surgeons (AANS) and the American Board of Neurological Surgery (ABNS) were analyzed for all residents who matched into neurosurgery during the years 2000–2009. Residents were sorted by female gender, matched against graduation records, and if graduation was not reported from neurosurgery residency programs, an Internet search was used to determine the residents’ alternative path. The primary outcome was to determine the number of women residents who did not complete neurosurgery training programs during 2000–2009. Secondary outcomes included the total number of women who matched into neurosurgery per year, year in training in which attrition occurred, and alternative career paths that these women chose to pursue.

RESULTS

Women comprised 240 of 1992 (12%) matched neurosurgery residents during 2000–2009. Among female residents there was a 17% attrition rate, compared with a 5.3% male attrition rate, with an overall attrition rate of 6.7%. The majority who left the field did so within the first 3 years of neurosurgical training and stayed in medicine—pursuing anesthesia, neurology, and radiology.

CONCLUSIONS

Although the percentage of women entering neurosurgical residency has continued to increase, this number is still disproportionate to the overall number of women in medicine. The female attrition rate in neurosurgery in the 2000–2009 cohort is comparable to that of the other surgical specialties, but for neurosurgery, there is disparity between the male and female attrition rates. Women who left the field tended to stay within medicine and usually pursued a neuroscience-related career. Given the need for talented women to pursue neurosurgery and the increasing numbers of women matching annually, the recruitment and retention of women in neurosurgery should be benchmarked and assessed.