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Mark R. Proctor, R. Michael Scott, W. Jerry Oakes and Karin M. Muraszko

different surgical approaches based on presentation, but there is little in the literature to guide this decision tree. In this issue of Neurosurgical Focus, we try to clarify and provide evidence addressing some of the questions surrounding Chiari malformation and syringomyelia. Authors address topics such as familial Chiari malformation, its association with craniosynostosis and other associated disorders, and the use of newer imaging modalities that can help us establish the successful treatment of Chiari and syringomyelia. We hope that these papers will help to

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D. Andrew Wilkinson, Kyle Johnson, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

at any point either before or after the CM surgery, including ventricular shunt placement (CPT 62220, 62223), cervical fusion (CPT 22590, 22595, 22548), and spinal cord untethering (CPT 63200). The ICD-9 diagnosis codes from any claim were used to identify the comorbidities of syringomyelia (336.0), hydrocephalus (742.3, 331.x), and scoliosis (737.3x, 754.2). Results were stratified by age group, sex, year of operation, and US census division ( Table 1 ). The average longest duration of consecutive insurance coverage was calculated for all beneficiaries of the plan

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Wajd N. Al-Holou, Karin M. Muraszko, Hugh J. Garton, Steven R. Buchman and Cormac O. Maher

deformity in a patient with a known tethered cord. Prior to surgical untethering, shunt failure, syringomyelia, pseudomeningocele, and Chiari malformation Type II were ruled out as a cause of the presenting complaints. The presence of syringomyelia was not by itself an indication for surgery in any of the cases. All patients were confirmed to have a tethered cord intraoperatively. Monitoring with somatosensory evoked potentials, anal sphincter electromyography, and anal pressure sensors are regularly used intraoperatively. Results Patient Population Eighty

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Siri Sahib S. Khalsa, Ndi Geh, Bryn A. Martin, Philip A. Allen, Jennifer Strahle, Francis Loth, Desale Habtzghi, Aintzane Urbizu Serrano, Daniel McQuaide, Hugh J. L. Garton, Karin M. Muraszko and Cormac O. Maher

position < 5 mm below the foramen magnum may present with typical CM-I symptoms or syringomyelia. 1 , 17 , 34 Conversely, many asymptomatic or minimally symptomatic individuals have low tonsil position. 7 , 24 , 28 , 29 , 35 , 37 , 43 Because the number of cranial and cervical imaging studies have been increasing for decades, the incidental discovery of individuals with low tonsil position has become more common. 14 , 24 , 25 , 42 Using the tonsil position criterion, current estimates of CM-I prevalence among patients undergoing MRI range from 0.8% to 3.7% in children

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Jennifer Strahle, Karin M. Muraszko, Hugh J. L. Garton, Brandon W. Smith, Jordan Starr, Joseph R. Kapurch II, and Cormac O. Maher

pediatric neurosurgeons continue to recommend surgery for otherwise asymptomatic patients with CM-I and syrinx. 22 Because both CM-I and syringomyelia are not uncommon incidental findings on imaging, and decompression is frequently recommended for CM-I patients with syrinx found on MRI, it is important to address whether all such syringes are likely related to the CM-I, or whether in some cases other forces are at work that may not necessarily respond to posterior fossa decompression. Our goal was to analyze syrinx size and location of syringes associated with CM-I and

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Jennifer Strahle, Karin M. Muraszko, Joseph Kapurch, J. Rajiv Bapuraj, Hugh J. L. Garton and Cormac O. Maher

device. Electronic records were reviewed using the Electronic Medical Record Search Engine (EMERSE), 14 a search engine that queries all free-text documents within the electronic medical records of a specified patient population. In this study, we used EMERSE to identify a population in whom the terms “tonsillar ectopia,” “tonsillar herniation,” “tonsillar descent,” “syrinx,” “syringomyelia,” “hydromyelia,” and “Chiari” were used in any part of the medical record. We then reviewed the records of all patients identified by EMERSE to select those who met the criteria

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Jennifer Strahle, Brandon W. Smith, Melaine Martinez, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

CM-I, syrinx, and/or scoliosis by searching for the key words “tonsillar ectopia,” “tonsillar herniation,” “tonsillar descent,” “tonsil,” “syrinx,” “syringomyelia,” “hydromyelia,” “Chiari,” or “scoliosis” in any part of the electronic medical record. All imaging records and all other medical records for patients selected in this way were reviewed to confirm the diagnoses. Each patient was assigned to a category on the basis of his or her syrinx status, CM-I status, and scoliosis status. For the purpose of this analysis, CM-I was defined on imaging as a cerebellar

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Jennifer Strahle, Karin M. Muraszko, Joseph Kapurch, J. Rajiv Bapuraj, Hugh J. L. Garton and Cormac O. Maher

, 27 , 38 , 42 Studies describing groups of patients that have been surgically treated for CM-I tend to have a bias for including symptomatic cases and excluding those that are more minimally affected. Syringomyelia is found in as many as 75% of patients undergoing surgical treatment for CM-I. 9 , 26 , 27 , 29 , 42 , 43 Since surgeons are more likely to recommend surgery if a spinal syrinx is present, the true prevalence of a syrinx in patients with CM-I is lower. 17 , 41 The radiographic characteristics of CM-I and syrinx have not been described in a large

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Brandon W. Smith, Jennifer Strahle, Erick Kazarian, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

and even syringomyelia caused by crowding at the foramen magnum. 11 Nevertheless, to make our analysis clear and consistent with other analyses in the literature, we followed the usual custom of considering those with tonsils that are ≥ 5 mm below the foramen magnum as a separate group. Conclusions For most patients, cerebellar tonsil position is not influenced by BMI. Acknowledgment We thank Holly Wagner for providing editorial assistance. Author Contributions Conception and design: Maher. Acquisition of data: Smith, Strahle, Kazarian. Analysis

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Brandon W. Smith, Jennifer Strahle, J. Rajiv Bapuraj, Karin M. Muraszko, Hugh J. L. Garton and Cormac O. Maher

radiology report of only 39 (53%) of 74 patients. Several groups have shown that patients with CM have a reduced posterior fossa volume resulting in craniocerebral disproportion, or crowding, in the posterior fossa. 6 , 15 , 17 , 26 , 30–32 , 37 Although cerebellar tonsil measurements are a convenient marker for crowding at the foramen magnum, the correlation is far from exact. In some cases, patients with tonsils less than 5 mm below the basion-opisthion line can have clinical presentation of Chiari syndrome and even syringomyelia due to crowding at the foramen magnum