✓ Tonsillar descent of the cerebellum in Chiari I malformations is often considered a congenital defect. A patient is presented in whom magnetic resonance (MR) imaging revealed normally positioned cerebellar tonsils; however, 1 year later MR imaging was repeated for evaluation of gait abnormalities and showed descent of the cerebellar tonsils. This case illustrates worsening symptoms with progressive descent of the cerebellar tonsils and suggests that Chiari I malformations can evolve postnatally.
Paul P. Huang and Shlomo Constantini
Omar Tanweer, Akwasi Boah and Paul P. Huang
Patients undergoing placement of an external ventricular drain (EVD) are at increased risk for development of venous thromboembolisms (VTEs). Early chemical prophylaxis has been shown to decrease rates of embolism formation, but the risks for bleeding and the optimal time to initiate prophylaxis have not been clearly defined for this patient population. The authors evaluated the safety and risks for bleeding when chemical prophylaxis for VTEs was started within 24 hours of EVD placement.
To compare rates of hemorrhage among patients who received prophylaxis within 24 hours and those who received it later than 24 hours after admission, the authors conducted an institutional review board–approved retrospective review. Patients were those who had had an EVD placed and postprocedural imaging conducted at Bellevue Hospital, New York, from January 2009 through April 2012. Data collected included demographics, diagnosis, coagulation panel results, time to VTE prophylaxis and imaging, and occurrence of VTEs. The EVD-associated hemorrhages were classified as Grade 0, no hemorrhage; Grade 1, petechial hyperdensity near the drain; Grade 2, hematoma of 1–15 ml; Grade 3, epidural or subdural hematoma greater than 15 ml; or Grade 4, intraventricular hemorrhage or hematoma requiring surgical intervention.
Among 99 patients, 111 EVDs had been placed. Low-dose unfractionated heparin had been given within 24 hours of admission (early prophylaxis) to 56 patients and later than 24 hours after admission (delayed prophylaxis) to 55 patients. There were no statistical differences across all grades (0–4) among those who received early prophylaxis (n = 45, 5, 5, 1, and 0, respectively) and those who received delayed prophylaxis (n = 46, 4, 1, 1, and 3, respectively) (p = 0.731). In the early prophylaxis group, 3 VTEs were discovered among 32 of 56 patients screened for clinically suspected VTEs. In the delayed prophylaxis group, 5 VTEs were discovered among 33 of 55 patients screened for clinically suspected VTEs (p = 0.71).
Hemorrhagic complications did not increase when chemical prophylaxis was started within 24 hours of admission. Also, the incidence of VTEs did not differ between patients in the early and delayed prophylaxis groups. Larger randomized controlled trials are probably needed to assess decreases in VTEs with earlier prophylaxis.
Shaun D. Rodgers, Bryan J. Marascalchi, Russell G. Strom and Paul P. Huang
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection and lacrimation, suggestive of SUNCT syndrome. After failed medical treatment an MRI sequence was obtained in this patient, demonstrating an epidermoid tumor in the left cerebellopontine angle. The patient's symptoms completely resolved after a gross-total resection of the tumor. This case demonstrates the effectiveness of resection as definitive treatment for SUNCT syndrome associated with tumoral compression of the trigeminal nerve. Early MRI studies should be considered in all patients with SUNCT, especially those with atypical signs and symptoms.
Omar Tanweer, Taylor A. Wilson, Stephen P. Kalhorn, John G. Golfinos, Paul P. Huang and Douglas Kondziolka
Physicians are often solicited by patients or colleagues for clinical recommendations they would make for themselves if faced by a clinical situation. The act of making a recommendation can alter the clinical course being taken. The authors sought to understand this dynamic across different neurosurgical scenarios by examining how neurosurgeons value the procedures that they offer.
The authors conducted an online survey using the Congress of Neurological Surgeons listserv in May 2013. Respondents were randomized to answer either as the surgeon or as the patient. Questions encompassed an array of distinct neurosurgical scenarios. Data on practice parameters and experience levels were also collected.
Of the 534 survey responses, 279 responded as the “neurosurgeon” and 255 as the “patient.” For both vestibular schwannoma and arteriovenous malformation management, more respondents chose resection for their patient but radiosurgery for themselves (p = 0.002 and p = 0.001, respectively). Aneurysm coiling was chosen more often than clipping, but those whose practice was ≥ 30% open cerebrovascular neurosurgery were less likely to choose coiling. Overall, neurosurgeons who focus predominantly on tumors were more aggressive in managing the glioma, vestibular schwannoma, arteriovenous malformation, and trauma. Neurosurgeons more than 10 years out of residency were less likely to recommend surgery for management of spinal pain, aneurysm, arteriovenous malformation, and trauma scenarios.
In the majority of cases, altering the role of the surgeon did not change the decision to pursue treatment. In certain clinical scenarios, however, neurosurgeons chose treatment options for themselves that were different from what they would have chosen for (or recommended to) their patients. For the management of vestibular schwannomas, arteriovenous malformations, intracranial aneurysms, and hypertensive hemorrhages, responses favored less invasive interventions when the surgeon was the patient. These findings are likely a result of cognitive biases, previous training, experience, areas of expertise, and personal values.
Conor Grady, Omar Tanweer, David Zagzag, Jafar J. Jafar, Paul P. Huang and Douglas Kondziolka
Stereotactic radiosurgery is widely used to treat cerebral arteriovenous malformations (AVMs), with the goal of complete angiographic obliteration. A number of case series have challenged the assumption that absence of residual AVM on follow-up angiograms is consistent with elimination of the risk of hemorrhage. The authors describe 3 cases in which patients who had angiographic evidence of AVM occlusion presented with late hemorrhage in the area of their prior lesions. They compare the radiographic, angiographic, and histological features of these patients with those previously described in the literature.
Delayed hemorrhage from the tissue of occluded AVMs has been reported as early as 4 and as late as 11 years after initial stereotactic radiosurgery. In all cases for which data are available, hemorrhage occurred in the area of persistent imaging findings despite negative findings on conventional angiography. The hemorrhagic lesions that were resected demonstrated a number of distinct histological findings.
While rare, delayed hemorrhage from the tissue of occluded AVMs may occur from a number of distinct, angiographically occult postirradiation changes. The hemorrhages in the authors' 3 cases were symptomatic and localized. The correlation of histological and imaging findings in delayed hemorrhage from occluded AVMs is an area requiring further investigation.
Marian Wisniewski, Cyril Toker, Paul J. Anderson, Yun P. Huang and Leonard I. Malis
✓ A rare case of benign chondroblastoma of the cervical spine is described, and the differential diagnosis of benign lesions in the spine discussed.
Radek Kolecki, Vikalpa Dammavalam, Abdullah Bin Zahid, Molly Hubbard, Osamah Choudhry, Marleen Reyes, ByoungJun Han, Tom Wang, Paraskevi Vivian Papas, Aylin Adem, Emily North, David T. Gilbertson, Douglas Kondziolka, Jason H. Huang, Paul P. Huang and Uzma Samadani
The precise threshold differentiating normal and elevated intracranial pressure (ICP) is variable among individuals. In the context of several pathophysiological conditions, elevated ICP leads to abnormalities in global cerebral functioning and impacts the function of cranial nerves (CNs), either or both of which may contribute to ocular dysmotility. The purpose of this study was to assess the impact of elevated ICP on eye-tracking performed while patients were watching a short film clip.
Awake patients requiring placement of an ICP monitor for clinical purposes underwent eye tracking while watching a 220-second continuously playing video moving around the perimeter of a viewing monitor. Pupil position was recorded at 500 Hz and metrics associated with each eye individually and both eyes together were calculated. Linear regression with generalized estimating equations was performed to test the association of eye-tracking metrics with changes in ICP.
Eye tracking was performed at ICP levels ranging from −3 to 30 mm Hg in 23 patients (12 women, 11 men, mean age 46.8 years) on 55 separate occasions. Eye-tracking measures correlating with CN function linearly decreased with increasing ICP (p < 0.001). Measures for CN VI were most prominently affected. The area under the curve (AUC) for eye-tracking metrics to discriminate between ICP < 12 and ≥ 12 mm Hg was 0.798. To discriminate an ICP < 15 from ≥ 15 mm Hg the AUC was 0.833, and to discriminate ICP < 20 from ≥ 20 mm Hg the AUC was 0.889.
Increasingly elevated ICP was associated with increasingly abnormal eye tracking detected while patients were watching a short film clip. These results suggest that eye tracking may be used as a noninvasive, automatable means to quantitate the physiological impact of elevated ICP, which has clinical application for assessment of shunt malfunction, pseudotumor cerebri, concussion, and prevention of second-impact syndrome.
Robert M. Starke, Dale Ding, Hideyuki Kano, David Mathieu, Paul P. Huang, Caleb Feliciano, Rafael Rodriguez-Mercado, Luis Almodovar, Inga S. Grills, Danilo Silva, Mahmoud Abbassy, Symeon Missios, Douglas Kondziolka, Gene H. Barnett, L. Dade Lunsford and Jason P. Sheehan
Pediatric patients (age < 18 years) harboring brain arteriovenous malformations (AVMs) are burdened with a considerably higher cumulative lifetime risk of hemorrhage than adults. Additionally, the pediatric population was excluded from recent prospective comparisons of intervention versus conservative management for unruptured AVMs. The aims of this multicenter, retrospective cohort study are to analyze the outcomes after stereotactic radiosurgery for unruptured and ruptured pediatric AVMs.
We analyzed and pooled AVM radiosurgery data from 7 participating in the International Gamma Knife Research Foundation. Patients younger than 18 years of age who had at least 12 months of follow-up were included in the study cohort. Favorable outcome was defined as AVM obliteration, no post-radiosurgical hemorrhage, and no permanently symptomatic radiation-induced changes (RIC). The post-radiosurgery outcomes of unruptured versus ruptured pediatric AVMs were compared, and statistical analyses were performed to identify predictive factors.
The overall pediatric AVM cohort comprised 357 patients with a mean age of 12.6 years (range 2.8–17.9 years). AVMs were previously treated with embolization, resection, and fractionated external beam radiation therapy in 22%, 6%, and 13% of patients, respectively. The mean nidus volume was 3.5 cm3, 77% of AVMs were located in eloquent brain areas, and the Spetzler-Martin grade was III or higher in 59%. The mean radiosurgical margin dose was 21 Gy (range 5–35 Gy), and the mean follow-up was 92 months (range 12–266 months). AVM obliteration was achieved in 63%. During a cumulative latency period of 2748 years, the annual post-radiosurgery hemorrhage rate was 1.4%. Symptomatic and permanent radiation-induced changes occurred in 8% and 3%, respectively. Favorable outcome was achieved in 59%. In the multivariate logistic regression analysis, the absence of prior AVM embolization (p = 0.001) and higher margin dose (p < 0.001) were found to be independent predictors of a favorable outcome. The rates of favorable outcome for patients treated with a margin dose ≥ 22 Gy vs < 22 Gy were 78% (110/141 patients) and 47% (101/216 patients), respectively. A margin dose ≥ 22 Gy yielded a significantly higher probability of a favorable outcome (p < 0.001). The unruptured and ruptured pediatric AVM cohorts included 112 and 245 patients, respectively. Ruptured AVMs had significantly higher rates of obliteration (68% vs 53%, p = 0.005) and favorable outcome (63% vs 51%, p = 0.033), with a trend toward a higher incidence of post-radiosurgery hemorrhage (10% vs 4%, p = 0.07). The annual post-radiosurgery hemorrhage rates were 0.8% for unruptured and 1.6% for ruptured AVMs.
Radiosurgery is a reasonable treatment option for pediatric AVMs. Obliteration and favorable outcomes are achieved in the majority of patients. The annual rate of latency period hemorrhage after radiosurgery for both ruptured and unruptured pediatric AVM patients conveys a significant risk until the nidus is obliterated.
Dale Ding, Robert M. Starke, Hideyuki Kano, David Mathieu, Paul P. Huang, Caleb Feliciano, Rafael Rodriguez-Mercado, Luis Almodovar, Inga S. Grills, Danilo Silva, Mahmoud Abbassy, Symeon Missios, Douglas Kondziolka, Gene H. Barnett, L. Dade Lunsford and Jason P. Sheehan
Brain arteriovenous malformations (AVMs) are the most common cause of spontaneous intracranial hemorrhage in pediatric patients (age < 18 years). Since the cumulative lifetime risk of AVM hemorrhage is considerable in children, an improved understanding of the risk factors influencing hemorrhagic presentation may aid in the management of pediatric AVMs. The aims of this first of a 2-part multicenter, retrospective cohort study are to evaluate the incidence and determine the predictors of hemorrhagic presentation in pediatric AVM patients.
The authors analyzed pooled AVM radiosurgery data from 7 institutions participating in the International Gamma Knife Research Foundation (IGKRF). Patients younger than 18 years at the time of radiosurgery and who had at least 12 months of follow-up were included in the study cohort. Patient and AVM characteristics were compared between unruptured and ruptured pediatric AVMs.
A total of 357 pediatric patients were eligible for analysis, including 112 patients in the unruptured and 245 patients in the ruptured AVM cohorts (69% incidence of hemorrhagic presentation). The annual hemorrhage rate prior to radiosurgery was 6.3%. Hemorrhagic presentation was significantly more common in deep locations (basal ganglia, thalamus, and brainstem) than in cortical locations (frontal, temporal, parietal, and occipital lobes) (76% vs 62%, p = 0.006). Among the factors found to be significantly associated with hemorrhagic presentation in the multivariate logistic regression analysis, deep venous drainage (OR 3.2, p < 0.001) was the strongest independent predictor, followed by female sex (OR 1.7, p = 0.042) and smaller AVM volume (OR 1.1, p < 0.001).
Unruptured and ruptured pediatric AVMs have significantly different patient and nidal features. Pediatric AVM patients who possess 1 or more of these high-risk features may be candidates for relatively more aggressive management strategies.
Uzma Samadani, Sameer Farooq, Robert Ritlop, Floyd Warren, Marleen Reyes, Elizabeth Lamm, Anastasia Alex, Elena Nehrbass, Radek Kolecki, Michael Jureller, Julia Schneider, Agnes Chen, Chen Shi, Neil Mendhiratta, Jason H. Huang, Meng Qian, Roy Kwak, Artem Mikheev, Henry Rusinek, Ajax George, Robert Fergus, Douglas Kondziolka, Paul P. Huang and R. Theodore Smith
Automated eye movement tracking may provide clues to nervous system function at many levels. Spatial calibration of the eye tracking device requires the subject to have relatively intact ocular motility that implies function of cranial nerves (CNs) III (oculomotor), IV (trochlear), and VI (abducent) and their associated nuclei, along with the multiple regions of the brain imparting cognition and volition. The authors have developed a technique for eye tracking that uses temporal rather than spatial calibration, enabling detection of impaired ability to move the pupil relative to normal (neurologically healthy) control volunteers. This work was performed to demonstrate that this technique may detect CN palsies related to brain compression and to provide insight into how the technique may be of value for evaluating neuropathological conditions associated with CN palsy, such as hydrocephalus or acute mass effect.
The authors recorded subjects' eye movements by using an Eyelink 1000 eye tracker sampling at 500 Hz over 200 seconds while the subject viewed a music video playing inside an aperture on a computer monitor. The aperture moved in a rectangular pattern over a fixed time period. This technique was used to assess ocular motility in 157 neurologically healthy control subjects and 12 patients with either clinical CN III or VI palsy confirmed by neuro-ophthalmological examination, or surgically treatable pathological conditions potentially impacting these nerves. The authors compared the ratio of vertical to horizontal eye movement (height/width defined as aspect ratio) in normal and test subjects.
In 157 normal controls, the aspect ratio (height/width) for the left eye had a mean value ± SD of 1.0117 ± 0.0706. For the right eye, the aspect ratio had a mean of 1.0077 ± 0.0679 in these 157 subjects. There was no difference between sexes or ages. A patient with known CN VI palsy had a significantly increased aspect ratio (1.39), whereas 2 patients with known CN III palsy had significantly decreased ratios of 0.19 and 0.06, respectively. Three patients with surgically treatable pathological conditions impacting CN VI, such as infratentorial mass effect or hydrocephalus, had significantly increased ratios (1.84, 1.44, and 1.34, respectively) relative to normal controls, and 6 patients with supratentorial mass effect had significantly decreased ratios (0.27, 0.53, 0.62, 0.45, 0.49, and 0.41, respectively). These alterations in eye tracking all reverted to normal ranges after surgical treatment of underlying pathological conditions in these 9 neurosurgical cases.
This proof of concept series of cases suggests that the use of eye tracking to detect CN palsy while the patient watches television or its equivalent represents a new capacity for this technology. It may provide a new tool for the assessment of multiple CNS functions that can potentially be useful in the assessment of awake patients with elevated intracranial pressure from hydrocephalus or trauma.