The role of chief White House physician has traditionally been held by an individual with a background in a broad medical field, such as emergency medicine, family medicine, or internal medicine. Dr. Daniel Ruge, who served as the director of the Spinal Cord Injury Service for the Veterans Administration and was appointed during President Ronald Reagan’s first term, was the first neurosurgeon to become the chief White House physician. Aside from being the first neurosurgeon to serve in this capacity, Dr. Ruge also stands apart from others who have held this esteemed position because of how he handled Reagan’s care after an attempt was made on the then-president’s life. Instead of calling upon leading medical authorities of the time to care for the president, Dr. Ruge instead decided that Reagan should be treated as any trauma patient would be treated. Dr. Ruge’s actions after the assassination attempt on President Reagan resulted in the rapid, smooth recovery of the then-president. Daniel Ruge’s background, his high-profile roles and heavy responsibilities, and his critical decision-making are characteristics that make his role in the history of medicine and of neurosurgery unique.
A. Karim Ahmed, Eduardo Martinez-del-Campo and Nicholas Theodore
Eduardo Martinez-del-Campo, Leonardo Rangel-Castilla, Hector Soriano-Baron and Nicholas Theodore
Performance of MR imaging in patients with gunshot wounds at or near the lumbar spinal canal is controversial. The authors reviewed the literature on the use of MR imaging in gunshot wounds to the spine. They discuss the results from in vitro and clinical studies, analyze the physical properties of common projectiles, and evaluate the safety and indications for MR imaging when metallic fragments are located near the spinal canal.
A review of the English-language literature was performed. Data from 25 articles were analyzed, including 5 in vitro studies of the interaction between 95 projectiles and the MR system's magnetic fields, and the clinical outcomes in 22 patients with metallic fragments at or near the spinal canal who underwent MR imaging.
Properties of 95 civilian and military projectiles were analyzed at a magnet strength of 1, 1.5, 3, and 7 T. The most common projectiles were bullets with a core of lead, either with a copper jacket or unjacketed (73 [76.8%] of 95). Steel-containing (core or jacket) projectiles comprised 14.7%. No field interaction was evident in 78 (96.3%) of the 81 nonsteel projectiles. All steel projectiles showed at least positive deflection forces, longitudinal migration, or rotation. Heating of the projectiles was clinically insignificant. Image artifact was significant in all 9 steel bullets tested, but was not significant in 39 (88.6%) of the 44 nonsteel bullets tested. Overall, 22 patients with complete (82%) and incomplete (14%) spinal cord injury secondary to a projectile lodged inside the spinal canal underwent MR imaging. Discomfort and further physical or neurological deficits were not reported by any patient. Two patients with spinal cord injuries underwent MR imaging studies before surgical decompression and had subsequent, significant neurological improvement.
Metallic implants near or at the spinal canal are a relative contraindication for MR imaging. However, safe MR imaging might be feasible when a projectile's properties and a patient's individualized clinical presentation are considered.
Eduardo Martinez-del-Campo, Jay D. Turner, Leonardo Rangel-Castilla, Hector Soriano-Baron, Samuel Kalb and Nicholas Theodore
If left untreated, occipitocervical (OC) instability may lead to serious neurological injury or death. Open internal fixation is often necessary to protect the neurovascular elements. This study reviews the etiologies for pediatric OC instability, analyzes the radiographic criteria for surgical intervention, discusses surgical fixation techniques, and evaluates long-term postoperative outcomes based on a single surgeon's experience.
The charts of all patients < 18 years old who underwent internal OC fixation conducted by the senior author were retrospectively reviewed. Forty consecutive patients were identified for analysis. Patient demographic data, OC junction pathology, radiological diagnostic tools, surgical indications, and outcomes are reported.
The study population consisted of 20 boys and 20 girls, with a mean age of 7.3 years. Trauma (45% [n = 18]) was the most common cause of instability, followed by congenital etiologies (37.5% [n = 15]). The condyle-C1 interval had a diagnostic sensitivity of 100% for atlantooccipital dislocation. The median number of fixated segments was 5 (occiput–C4). Structural bone grafts were used in all patients. Postsurgical neurological improvement was seen in 88.2% (15/17) of patients with chronic myelopathy and in 25% (1/4) of patients with acute myelopathy. Preoperatively, 42.5% (17/40) of patients were neurologically intact and remained unchanged at last follow-up, 42.5% (17/40) had neurological improvement, 12.5% (5/40) remained unchanged, and 2.5% (1/40) deteriorated. All patients had successful fusion at 1-year follow-up. The complication rate was 7.5% (3/40), including 1 case of vertebral artery injury.
Occipitocervical fixation is safe in children and provides immediate immobilization, with excellent survival and arthrodesis rates. Of the radiographic tools evaluated, the condyle-C1 interval was the most predictive of atlantooccipital dislocation.
Eduardo Martinez-del-Campo, Jay D. Turner, Hector Soriano-Baron, Anna G. U. S. Newcomb, Samuel Kalb and Nicholas Theodore
The authors assessed the rate of vertebral growth, curvature, and alignment for multilevel constructs in the cervical spine after occipitocervical fixation (OCF) in pediatric patients and compared these results with those in published reports of growth in normal children.
The authors assessed cervical spine radiographs and CT images of 18 patients who underwent occipitocervical arthrodesis. Measurements were made using postoperative and follow-up images available for 16 patients to determine cervical alignment (cervical spine alignment [CSA], C1–7 sagittal vertical axis [SVA], and C2–7 SVA) and curvature (cervical spine curvature [CSC] and C2–7 lordosis angle). Seventeen patients had postoperative and follow-up images available with which to measure vertebral body height (VBH), vertebral body width (VBW), and vertical growth percentage (VG%—that is, percentage change from postoperative to follow-up). Results for cervical spine growth were compared with normal parameters of 456 patients previously reported on in 2 studies.
Ten patients were girls and 8 were boys; their mean age was 6.7 ± 3.2 years. Constructs spanned occiput (Oc)–C2 (n = 2), Oc–C3 (n = 7), and Oc–C4 (n = 9). The mean duration of follow-up was 44.4 months (range 24–101 months). Comparison of postoperative to follow-up measures showed that the mean CSA increased by 1.8 ± 2.9 mm (p < 0.01); the mean C2–7 SVA and C1–7 SVA increased by 2.3 mm and 2.7 mm, respectively (p = 0.3); the mean CSC changed by −8.7° (p < 0.01) and the mean C2–7 lordosis angle changed by 2.6° (p = 0.5); and the cumulative mean VG% of the instrumented levels (C2–4) provided 51.5% of the total cervical growth (C2–7). The annual vertical growth rate was 4.4 mm/year. The VBW growth from C2–4 ranged from 13.9% to 16.6% (p < 0.001). The VBW of C-2 in instrumented patients appeared to be of a smaller diameter than that of normal patients, especially among those aged 5 to < 10 years and 10–15 years, with an increased diameter at the immediately inferior vertebral bodies compensating for the decreased width. No cervical deformation, malalignment, or detrimental clinical status was evident in any patient.
The craniovertebral junction and the upper cervical spine continue to present normal growth, curvature, and alignment parameters in children with OCF constructs spanning a distance as long as Oc–C4.
Leonardo Rangel-Castilla, Jonathan J. Russin, Eduardo Martinez-del-Campo, Hector Soriano-Baron, Robert F. Spetzler and Peter Nakaji
Arteriovenous malformations (AVMs) are classically described as congenital static lesions. However, in addition to rupturing, AVMs can undergo growth, remodeling, and regression. These phenomena are directly related to cellular, molecular, and physiological processes. Understanding these relationships is essential to direct future diagnostic and therapeutic strategies. The authors performed a search of the contemporary literature to review current information regarding the molecular and cellular biology of AVMs and how this biology will impact their potential future management.
A PubMed search was performed using the key words “genetic,” “molecular,” “brain,” “cerebral,” “arteriovenous,” “malformation,” “rupture,” “management,” “embolization,” and “radiosurgery.” Only English-language papers were considered. The reference lists of all papers selected for full-text assessment were reviewed.
Current concepts in genetic polymorphisms, growth factors, angiopoietins, apoptosis, endothelial cells, pathophysiology, clinical syndromes, medical treatment (including tetracycline and microRNA-18a), radiation therapy, endovascular embolization, and surgical treatment as they apply to AVMs are discussed.
Understanding the complex cellular biology, physiology, hemodynamics, and flow-related phenomena of AVMs is critical for defining and predicting their behavior, developing novel drug treatments, and improving endovascular and surgical therapies.
Eduardo Martinez-del-Campo, Samuel Kalb, Hector Soriano-Baron, Jay D. Turner, Matthew T. Neal, Timothy Uschold and Nicholas Theodore
Atlantooccipital dislocation (AOD) in adults cannot be diagnosed with adequate specificity and sensitivity using only CT or plain radiography, and the spine literature offers no guidelines. In children, the most sensitive and specific radiographic measurement for the diagnosis of AOD is the CT-based occipital condyle–C1 interval (CCI). The goal of the current study was to identify the normal CCI in healthy adults and compare it with the CCI in adults with AOD to establish a highly sensitive and specific cutoff value for the neuroimaging diagnosis of AOD.
A total of 81 patients, 59 without AOD and 22 with AOD, were included in this study. Measurements obtained from thin-slice CT scans of the craniovertebral joint to assess atlantooccipital dislocation included the CCI, condylar sum, the Wholey and Harris intervals, Powers and Sun ratios, Wackenheim line, and Lee X-lines.
The group of patients without AOD included 30 men (50.8%) and 29 women (49.2%) with a mean age of 42.4 ± 16 years (range 19–87 years). The group of patients with AOD included 10 men (45.5%) and 12 women (54.5%) with a mean age of 38.2 ± 9.7 years (range 20–56 years). Interrater reliabilities within a 95% CI were all greater than 0.98 for CCI measurements. A total of 1296 measurements of the CCI were made in 81 patients. The mean CCI for non-AOD patients was 0.89 ± 0.12 mm, the single largest CCI measurement was 1.4 mm, and the largest mean for either right or left CCI was 1.2 mm. The mean condylar sum was 1.8 ± 0.2 mm, and the largest condylar sum value was 2.2 mm. Linear regression with age predicted an increase in CCI of 0.001 mm/year (p < 0.05). The mean CCI in AOD patients was 3.35 ± 0.18 mm (range 1.5 mm–6.4 mm). The shortest single CCI measurements in the AOD patients were 1.1 mm and 1.2 mm. The mean condylar sum for all 22 AOD patients was 6.7 ± 2.7 mm and the shortest condylar sums were 3.0 mm. Cutoff values for AOD were set at 1.5 mm for the CCI and 3.0 mm for the condylar sum, both with a sensitivity of 1 and false-negative rate of 0. Sensitivity for the Powers, Wholey, Harris, Sun, Wackenheim, and Lee criteria were determined to be 0.55, 0.46, 0.27, 0.23, 0.41, and 0.41, respectively.
The CCI is shorter in adult patients as opposed to the pediatric population. The revised CCI (1.5 mm) and condylar sum (3.0 mm) cutoff values have the highest sensitivity and specificity for the diagnosis of AOD in the adult population.
Tyler S. Cole, Kaith K. Almefty, Jakub Godzik, Amy H. Muma, Randall J. Hlubek, Eduardo Martinez-del-Campo, Nicholas Theodore, U. Kumar Kakarla and Jay D. Turner
Cervical spondylotic myelopathy (CSM) is the primary cause of adult spinal cord dysfunction. Diminished hand strength and reduced dexterity associated with CSM contribute to disability. Here, the authors investigated the impact of CSM severity on hand function using quantitative testing and evaluated the response to surgical intervention.
Thirty-three patients undergoing surgical treatment of CSM were prospectively enrolled in the study. An occupational therapist conducted 3 functional hand tests: 1) palmar dynamometry to measure grip strength, 2) hydraulic pinch gauge test to measure pinch strength, and 3) 9-hole peg test (9-HPT) to evaluate upper extremity dexterity. Tests were performed preoperatively and 6–8 weeks postoperatively. Test results were expressed as 1) a percentile relative to age- and sex-stratified norms and 2) achievement of a minimum clinically important (MCI) difference. Patients were stratified into groups (mild, moderate, and severe myelopathy) based on their modified Japanese Orthopaedic Association (mJOA) score. The severity of stenosis on preoperative MRI was graded by three independent physicians using the Kang classification.
The primary presenting symptoms were neck pain (33%), numbness (21%), imbalance (12%), and upper extremity weakness (12%). Among the 33 patients, 61% (20) underwent anterior approach decompression, with a mean (SD) of 2.9 (1.5) levels treated. At baseline, patients with moderate and low mJOA scores (indicating more severe myelopathy) had lower preoperative pinch (p < 0.001) and grip (p = 0.01) strength than those with high mJOA scores/mild myelopathy. Postoperative improvement was observed in all hand function domains except pinch strength in the nondominant hand, with MCI differences at 6 weeks ranging from 33% of patients in dominant-hand strength tests to 73% of patients in nondominant-hand dexterity tests. Patients with moderate baseline mJOA scores were more likely to have MCI improvement in dominant grip strength (58.3%) than those with low mJOA scores/severe myelopathy (30%) and high mJOA scores/mild myelopathy (9%, p = 0.04). Dexterity in the dominant hand as measured by the 9-HPT ranged from < 1 in patients with cord signal change to 15.9 in patients with subarachnoid effacement only (p = 0.03).
Patients with CSM achieved significant improvement in strength and dexterity postoperatively. Baseline strength measures correlated best with the preoperative mJOA score; baseline dexterity correlated best with the severity of stenosis on MRI. The majority of patients experienced MCI improvements in dexterity. Baseline pinch strength correlated with postoperative mJOA MCI improvement, and patients with moderate baseline mJOA scores were the most likely to have improvement in dominant grip strength postoperatively.
Leonardo Rangel-Castilla, Jonathan J. Russin, Hasan A. Zaidi, Eduardo Martinez-del-Campo, Min S. Park, Felipe C. Albuquerque, Cameron G. McDougall, Peter Nakaji and Robert F. Spetzler
Spinal arteriovenous fistulas (AVFs) and arteriovenous malformations (AVMs) are rare, complex spinal vascular lesions that are challenging to manage. Recently, understanding of these lesions has increased thanks to neuroimaging technology. Published reports of surgical results and clinical outcome are limited to small series. The authors present a large contemporary series of patients with spinal AVFs and AVMs who were treated at Barrow Neurological Institute in Phoenix, Arizona.
Retrospective detailed review of a prospective vascular database was performed for all patients with spinal AVFs and AVMs treated between 2000 and 2013. Patient demographic data, AVF and AVM characteristics, surgical results, clinical outcomes, complications, and long-term follow-up were reviewed.
Between 2000 and 2013, 110 patients (57 male and 53 female) underwent obliteration of spinal AVFs and AVMs. The mean age at presentation was 42.3 years (range 18 months–81 years). There were 44 patients with AVFs and 66 with AVMs. The AVM group included 27 intramedullary, 21 conus medullaris, 12 metameric, and 6 extradural. The most common location was thoracic spine (61%), followed by cervical (22.7%), lumbar (14.5%), and sacral (1.8%). The most common presenting signs and symptoms included paresis/paralysis (75.5%), paresthesias (60%), pain (51.8%), bowel/bladder dysfunction (41.8%), and myelopathy (36.4%). Evidence of rupture was seen in 26.4% of patients. Perioperative embolization was performed in 42% of patients. Resection was performed in 95 patients (86.4%). Embolization alone was the only treatment in 14 patients (12.7%). One patient was treated with radiosurgery alone. Angiographically verified AVF and AVM obliteration was achieved in 92 patients (83.6%). At a mean follow-up duration of 30.5 months (range 1–205 months), 43 patients (97.7%) with AVFs and 57 (86.4%) with AVMs remained functionally independent (McCormick Scale scores ≤ 2). Perioperative complications were seen in 8 patients (7%). No deaths occurred. Temporary neurological deficits were observed in 27 patients (24.5%). These temporary deficits recovered 6–8 weeks after treatment. Recurrence was identified in 6 patients (13.6%) with AVFs and 10 (15.2%) with AVMs.
Spinal AVFs and AVMs are complex lesions that should be considered for surgical obliteration. Over the last several decades the authors have changed surgical strategies and management to achieve better clinical outcomes. Transient neurological deficit postoperatively is a risk associated with intervention; however, clinical outcomes appear to exceed the natural history based on patients’ ability to recover during the follow-up period. Due to the recurrence rate associated with these lesions, long-term follow-up is required.