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Nicholas M. Wetjen, Jonathan A. Friedman and Fredric B. Meyer

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Nicholas M. Wetjen, P. Charles Garell, Nicholas V. Stence and Christopher M. Loftus

There have been few investigations of moyamoya disease in the United States and no systematic description of the management practices or outcome from this population. The authors reviewed their experience with this disease to gain a better understanding and improve the treatment of patients with moyamoya disease in the United States. Over a 25-year period 30 patients with moyamoya disease have been treated at the University of Iowa. The cases were divided into patients who had classic, probable, and akin moyamoya disease.

Results indicated that there was a bimodal age distribution and a female predominance of cases. In estimating the referral pattern of our institution, the authors determined that there were greater numbers of epidemiological characteristics than previously anticipated. Patients were treated either surgically or nonsurgically, and different management strategies were utilized in each of the major groups: superficial temporal artery to middle cerebral artery anastomosis and encephalodurosynangiosis in the surgical group; or antiplatelet, anticoagulation, or nonpharmacological intervention in the nonsurgical group.

The authors conclude that there is a higher prevalence and incidence of moyamoya disease in the United States than previously reported and that there are some clinical characteristics of this disease that differ from the cases reported in southeast Asia. These differences may be due to genetic or environmental factors but can also be partly explained by the lower index of suspicion for this disease and, thus, a delay in or complete absence of the correct diagnosis.

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Jonathan A. Friedman, Fredric B. Meyer, Nicholas M. Wetjen and Douglas A. Nichols

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Cormac O. Maher, Nicholas M. Wetjen, Jonathan A. Friedman and Fredric B. Meyer

Object. Many surgeons inject a local anesthetic agent into the carotid sinus before carotid endarterectomy in an attempt to ameliorate perioperative hemodynamic instability. The purpose of this study is to analyze the effect of carotid sinus injection with lidocaine on perioperative hemodynamics and complications.

Methods. The authors prospectively studied 92 patients in whom 100 consecutive carotid endarterectomies were performed by a single surgeon (eight procedures were bilateral). Patients were randomly assigned to one of two groups, in which either 0.5 ml of 1% lidocaine was injected into the carotid sinus nerve or no injection of lidocaine was administered before the arteriotomy. All patients were treated postoperatively according to a standard endarterectomy protocol. There were no significant differences between the two groups in the incidence of hypertension, hypotension, or the use of vasoactive medications in the operating room following restoration of carotid artery (CA) blood flow, in the recovery room, or in the intensive care unit.

Conclusions. Injection of lidocaine into the carotid sinus at the time of endarterectomy is not associated with a significant improvement in any hemodynamic factor, from the time of restoration of CA blood flow to postoperative Day 1.

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Scott C. Robertson, Nicholas M. Wetjen, Bradley J. Beer and Christopher M. Loftus

✓ The authors studied the effects of pre- and postischemic administration of dizocilpine maleate (MK-801) on collateral and regional cerebral blood flow (CBF). The ischemic penumbra appears to benefit most from the neuroprotective effects of MK-801. The precise mechanism by which MK-801 provides this neuroprotection remains controversial. Alterations in CBF have been demonstrated with MK-801 administration, but whether the response is an increase or decrease in flow has remained unclear.

A left-sided craniectomy was performed in 20 dogs. A branch of the middle cerebral artery (MCA) was cannulated and collateral blood supply—dependent tissue (CDT) was identified using the “shadow flow” technique. Regional CBF was measured using radiolabeled microspheres. Six dogs received MK-801 (1 mg/kg administered intravenously) before they underwent MCA branch occlusion; the remaining 14 dogs received MK-801 after they underwent MCA occlusion. Cerebral blood flow and vascular pressures were measured 30 and 60 minutes after MK-801 administration. In animals that received MK-801 before MCA occlusion, CBF did not change significantly from baseline values before or after occlusion. In contrast, in animals that received MK-801 after MCA occlusion, CBF was significantly reduced in all regions of the brain, including the CDT. Collateral blood supply—dependent tissue showed a 51.7% reduction in flow, whereas normal CBF was reduced by 29.7%. The MK-801 induced cerebral vasoconstriction in both groups. The neuroprotective effects of MK-801 do not appear to be caused by the augmentation of collateral or global cerebral circulation and, in fact, may block the glutamate-mediated vasodilation that occurs during ischemia.

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Michelle J. Clarke, Andrew B. Foy, Nicholas Wetjen and Corey Raffel

Object

Subependymal giant cell astrocytomas (SEGAs) are a common manifestation of tuberous sclerosis (TS). These evolving tumors have a propensity to cause obstructive hydrocephalus, usually due to obstruction at the level of the foramen of Monro. Differentiating SEGAs from subependymal nodules (SENs) before obstruction occurs may improve the morbidity associated with these tumors. In this study the authors' aim was to determine imaging characteristics of proven tumors in a single-center pediatric population.

Methods

The authors retrospectively reviewed all records and images obtained in patients with TS in whom results of biopsy sampling had proven that their tumors were SEGAs. Time to presentation, signs and symptoms at presentation, and imaging characteristics of the evolving tumors were noted. Twelve patients with 14 SEGAs proven by the results of biopsy sampling were reviewed. Resection was recommended for symptomatic and neuroimaging evidence of hydrocephalus (41%), tumor growth without evidence of hydrocephalus (33%), and for poorly controlled seizures (25%). The mean diameter of the tumors at the time of resection was 1.9 cm (range 0.3–4 cm), and no tumor recurred. Because of the pathological and radiographic continuum of SENs and SEGAs, it remains difficult to predict whether and when a given lesion will progress. Tumor growth and contrast enhancement are the most common signs of progression on neuroimages, and may be seen prior to the development of obstructive hydrocephalus.

Conclusions

Patients with SENs and SEGAs should undergo follow-up neuroimaging at yearly intervals, and if lesions show signs of progression (contrast enhancement or growth), these intervals should be shortened and consideration given to early resection.

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Nicholas M. Wetjen, John D. Heiss and Edward H. Oldfield

Object

To better understand syrinx pathophysiology, the authors performed a prospective study in which they used findings from serial clinical and magnetic resonance (MR) imaging examinations performed before and after craniocervical decompression to establish the time course of syrinx narrowing.

Methods

Serial clinical examinations and cervical MR imaging were performed in 29 consecutive patients with Chiari malformation Type I (CM-I) and syringomyelia before surgery, 1 week, and 3–6 months after surgery, and then annually. Time to narrowing of the syrinx (> 50% decrease in maximal anteroposterior diameter) following surgery was calculated using the Kaplan–Meier method.

Results

All syringes decreased in diameter and length (number of segments) on MR images at 3–6 months, 1 year, and 2 years or later. The syrinx diameter decreased from 6.9 ± 2.1 mm (mean ± standard deviation) preoperatively to < 1.5 mm at last evaluation (p < 0.0001). The median time to syrinx narrowing was 3.6 months following CM-I decompression (95% confidence interval 3.0–6.5 months). After surgery 94% of patients had improved symptoms, but symptoms resolved incompletely in 68% of patients; 52 and 59% of patients had residual dysesthesias and sensory loss, respectively. Clinical improvement occurred before partial or complete disappearance of the syrinx on MR images. Patient age, duration of symptoms, sex, preoperative syrinx diameter, and length of syrinx were unrelated to time to syrinx narrowing.

Conclusions

Most patients improve after decompression for CM-I, but many have residual symptoms. Syringes may continue to diminish for months to years after surgical decompression. A collapsed syrinx (absence of distention of the spinal cord) indicates that the pathophysiology has been reversed by treatment regardless of the completeness of elimination of the cavity on MR images.

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Ravi Kumar, Jeffrey T. Jacob, Kirk M. Welker, Fred M. Cutrer, Michael J. Link, John L. D. Atkinson and Nicholas M. Wetjen

This report reviews a series of 3 patients who developed superficial siderosis following posterior fossa operations in which dural closure was incomplete. In all 3 patients, revision surgery and complete duraplasty was performed to halt the progression of superficial siderosis. Following surgery, 2 patients experienced resolution of their CSF xanthochromia while 1 patient had reduced CSF xanthochromia. In this paper the authors also review the etiology, pathophysiology, diagnosis, and treatment of this condition. The authors suggest that posterior fossa dural patency and pseudomeningocele are risk factors for the latent development of superficial siderosis and recommend that revision duraplasty be performed in patients with posterior fossa pseudomeningoceles and superficial siderosis to prevent progression of the disease.

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Patrick R. Maloney, Meghan E. Murphy, Molly J. Sullan, Kathryn M. Van Abel, Shelagh A. Cofer, John C. Cheville and Nicholas M. Wetjen

Split cord malformation (SCM) is a rare abnormality of notochord development. The majority of cases occur in the thoracolumbar region, with more than 30 cases of cervical SCM reported. The clinical impact of SCMs involving the cervical cord is therefore largely unknown. In addition, the concomitant finding of brainstem involvement is presumably incompatible with life in the majority of patients, resulting in a paucity of data regarding this clinical scenario. In this paper the authors present the first case, to their knowledge, of an incomplete cervical SCM involving the brainstem and discuss its clinical impact, diagnosis, and management.

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David J. Daniels, T. David Luo, Ross Puffer, Amy L. McIntosh, A. Noelle Larson, Nicholas M. Wetjen and Michelle J. Clarke

OBJECT

Motocross racing is a popular sport; however, its impact on the growing/developing pediatric spine is unknown. Using a retrospective cohort model, the authors compared the degree of advanced degenerative findings in young motocross racers with findings in age-matched controls.

METHODS

Patients who had been treated for motocross-related injury at the authors' institution between 2000 and 2007 and had been under 18 years of age at the time of injury and had undergone plain radiographic or CT examination of any spinal region were eligible for inclusion. Imaging was reviewed in a blinded fashion by 3 physicians for degenerative findings, including endplate abnormalities, loss of vertebral body height, wedging, and malalignment. Acute pathological segments were excluded. Spine radiographs from age-matched controls were similarly reviewed and the findings were compared.

RESULTS

The motocross cohort consisted of 29 riders (mean age 14.7 years; 82% male); the control cohort consisted of 45 adolescents (mean age 14.3 years; 71% male). In the cervical spine, the motocross cohort had 55 abnormalities in 203 segments (average 1.90 abnormalities/patient) compared with 20 abnormalities in 213 segments in the controls (average 0.65/patient) (p = 0.006, Student t-test). In the thoracic spine, the motocross riders had 51 abnormalities in 292 segments (average 2.04 abnormalities/patient) compared with 25 abnormalities in 299 segments in the controls (average 1.00/patient) (p = 0.045). In the lumbar spine, the motocross cohort had 11 abnormalities in 123 segments (average 0.44 abnormalities/patient) compared with 15 abnormalities in 150 segments in the controls (average 0.50/patient) (p = 0.197).

CONCLUSIONS

Increased degenerative changes in the cervical and thoracic spine were identified in adolescent motocross racers compared with age-matched controls. The long-term consequences of these changes are unknown; however, athletes and parents should be counseled accordingly about participation in motocross activities.