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Arnold-Chiari deformity

Michael Faloon, Nikhil Sahai, Todd P Pierce, Conor J Dunn, Kumar Sinha, Ki Soo Hwang, Arash Emami
BACKGROUND: Several studies have sought to address the role of routine preoperative MRI in patients with adolescent idiopathic scoliosis (AIS) undergoing deformity correction. Despite similar results regarding the prevalence of neuraxial anomalies detected on MRI, published conclusions conflict and give opposing recommendations. Lack of consensus has led to important variations in use of MRI before spinal surgery for patients with AIS. QUESTIONS/PURPOSES: This systematic review and meta-analysis of studies about patients with AIS evaluated (1) the overall proportion of neuraxial abnormalities; (2) the patient factors and curve characteristics that may be associated with abnormalities; and (3) the proportion of patients who underwent neurosurgical intervention before scoliosis surgery and the kinds of neuraxial lesions that were identified...
July 2018: Clinical Orthopaedics and related Research
Johan L Heemskerk, Moyo C Kruyt, Dino Colo, René M Castelein, Diederik H R Kempen
BACKGROUND: There is ongoing controversy about the routine use of magnetic resonance imaging (MRI) preoperatively in patients with presumed idiopathic scoliosis (IS). Routine MRI can help identify possible causes for the deformity and detect anomalies that could complicate deformity surgery. However, routine MRI increases health-care costs significantly and may reveal mild variations from normal findings without clinical relevance, which can still lead to anxiety and influence decision-making...
July 2018: Spine Journal: Official Journal of the North American Spine Society
Shifu Sha, Yang Li, Yong Qiu, Zhen Liu, Xu Sun, Weiguo Zhu, Zhenhua Feng, Tao Wu, Jian Jiang, Zezhang Zhu
OBJECTIVE: To investigate whether posterior fossa decompression (PFD) could improve denervation of the paraspinal muscles in patients with Chiari I malformation (CMI). BACKGROUND: Paraspinal muscle denervation is one of the essential elements in the pathophysiology of CMI/syringomyelia-related scoliosis. Although PFD has been widely used for managing CMI, whether denervation of the paraspinal muscles may benefit from this neurosurgical procedure remains ambiguous...
May 2017: Journal of Neurology, Neurosurgery, and Psychiatry
Alexander W Aleem, Earl D Thuet, Anne M Padberg, Michael Wallendorf, Scott J Luhmann
STUDY DESIGN: Retrospective. OBJECTIVES: The purpose of this study is to review the efficacy of monitoring data and outcomes in pediatric patients with spinal cord pathology. SUMMARY OF BACKGROUND DATA: The incidence of spinal cord pathology in pediatric patients with scoliosis has been reported between 3% and 20%. Previous studies demonstrated that intraoperative spinal cord monitoring (IOM) during scoliosis surgery can be reliable despite underlying pathology...
January 2015: Spine Deformity
Jeffrey A Fearon, Vanessa Dimas, Kanlaya Ditthakasem
BACKGROUND: A relationship between lambdoid craniosynostosis and Chiari deformations has been suggested, but the true extent of this association remains uncertain. The authors reviewed a single center's experience treating lambdoid synostosis to further elucidate this relationship, examine surgical outcomes, and identify associations that might impact future treatments. METHODS: A retrospective chart review was performed of all patients treated for lambdoid craniosynostosis, excluding the syndromic craniosynostoses...
March 2016: Plastic and Reconstructive Surgery
Michael P Kelly, Tenner J Guillaume, Lawrence G Lenke
Despite the frequency of Chiari-associated spinal deformities, this disease process remains poorly understood. Syringomyelia is often present; however, this is not necessary and scoliosis has been described in the absence of a syrinx. Decompression of the hindbrain is often recommended. In young patients (<10 years old) and/or those with small coronal Cobb measurements (<40°), decompression of the hindbrain may lead to resolution of the spinal deformity. Spinal fusion is reserved for those curves that progress to deformities greater than 50°...
October 2015: Neurosurgery Clinics of North America
Akbar Fakhri, Manish N Shah, Manu S Goyal
Type I Chiari malformations are congenital deformities involving cerebellar tonsillar herniation downward through the foramen magnum. Structurally, greater than 5 mm of tonsillar descent in adults and more than 6 mm in children is consistent with type I Chiari malformations. However, the radiographic severity of the tonsillar descent does not always correlate well with the clinical symptomatology. Advanced imaging can help clinically correlate imaging to symptoms. Specifically, cerebrospinal fluid (CSF) flow abnormalities are seen in patients with type I Chiari malformation...
October 2015: Neurosurgery Clinics of North America
Jonathan Pindrik, James M Johnston
Chiari I malformation and syringomyelia may be associated with a wide spectrum of symptoms and signs in children. Clinical presentations vary based on patient age and relative frequency; some diagnoses represent incidental radiographic findings. Occipitocervical pain, propagated or intensified by Valsalva maneuvers (or generalized irritability in younger patients unable to communicate verbally), and syringomyelia with or without scoliosis are the most common clinical presentations. Cranial nerve or brainstem dysfunction also may be observed in younger patients, and is associated with more complex deformity that includes ventral compression secondary to basilar invagination, retroflexion of the dens, and/or craniocervical instability...
October 2015: Neurosurgery Clinics of North America
Ken R Winston, Nicholas V Stence, Arianne J Boylan, Kathryn M Beauchamp
Cerebellar tonsils moved significantly upward in 3 patients with Chiari type I who underwent supratentorial cranial vault expansion to alleviate intracranial pressure related to multisutural craniosynostosis. The Chiari type I deformities in these patients were the biomechanical consequence of posterior fossa-cerebellar disproportion caused by supratentorial craniocerebral disproportion secondary to multisutural craniosynostosis. The authors postulate that all cases of Chiari type I deformity share the sine qua non feature of posterior fossa-cerebellar disproportion...
2015: Pediatric Neurosurgery
Zezhang Zhu, Shifu Sha, Winnie C C Chu, Huang Yan, Dingding Xie, Zhen Liu, Xu Sun, Weiguo Zhu, Jack C Y Cheng, Yong Qiu
PURPOSE: Although the more readily available MR imaging has brought about more incidental findings of idiopathic syringomyelia (IS), no published study has specifically addressed the clinical and imaging features of IS-associated scoliosis. Since IS and Chiari I malformation (CMI)-type syringomyelia are hypothesized to share a common underlying developmental pathomechanism, this study aimed to investigate the scoliosis curve patterns and MRI syrinx cord characteristics of patients with IS comparing with those seen in CMI...
February 2016: European Spine Journal
J Godzik, A Dardas, M P Kelly, T F Holekamp, L G Lenke, M D Smyth, T S Park, J R Leonard, D D Limbrick
PURPOSE: To describe curve patterns in patients with Chiari malformation I (CIM) without syringomyelia, and compare to patients with Chiari malformation with syringomyelia (CIM + SM). METHODS: Review of medical records from 2000 to 2013 at a single institution was performed to identify CIM patients with scoliosis. Patients with CIM were matched (1:1) by age and gender to CIM + SM. Radiographic curve patterns, MRI-based craniovertebral junction parameters, and associated neurological signs were compared between the two cohorts...
February 2016: European Spine Journal
Jakub Godzik, Terrence F Holekamp, David D Limbrick, Lawrence G Lenke, T S Park, Wilson Z Ray, Keith H Bridwell, Michael P Kelly
BACKGROUND CONTEXT: Chiari malformation, Type 1, with syringomyelia (CIM+SM) is often associated with spinal deformity. The safety of scoliosis surgery this population is controversial and has never been directly compared with adolescent idiopathic scoliosis (AIS). PURPOSE: The purpose of this study was to compare the safety and subjective outcomes of spinal deformity surgery between patients with Chiari malformation Type 1-associated scoliosis and a matched AIS cohort...
September 1, 2015: Spine Journal: Official Journal of the North American Spine Society
Melinda A Costa, Laurie L Ackerman, Sunil S Tholpady, S Travis Greathouse, Youssef Tahiri, Roberto L Flores
Patients with multisutural craniosynostosis can develop anomalous venous connections between the intracranial sinuses and cutaneous venous system through enlarged emissary veins. Cranial vault remodeling in this subset of patients carries the risk of massive intraoperative blood loss and/or occlusion of collateral draining veins leading to intracranial venous hypertension and raised intracranial pressure, increasing the morbidity of cranial expansion. The authors report the use of spring-mediated expansion as a technique for cranial reconstruction in which the collateral intracranial venous drainage system can be preserved...
July 2015: Journal of Neurosurgery. Pediatrics
Daniel Felbaum, Steven Spitz, Faheem A Sandhu
A subset of patients with Chiari Type I malformation may develop neurological dysfunction secondary to an abnormally obtuse clivoaxial angle (CXA) and clivoaxial deformity causing deformative stress injury to the neural axis. Clivoaxial deformity can occur after initial standard suboccipital craniectomy, duraplasty, and C-1 laminectomy for brainstem compression, or severe clivoaxial deformity may be present in conjunction with a Chiari malformation. Clivoaxial deformity and abnormal CXA can be treated with an occipitocervical fusion (OCF)...
July 2015: Journal of Neurosurgery. Spine
Dingding Xie, Yong Qiu, Shifu Sha, Zhen Liu, Long Jiang, Huang Yan, Ling Chen, Benlong Shi, Zezhang Zhu
PURPOSE: Chiari malformation type I (CMI) is characterized by deformed hindbrain. This study aimed to quantitatively evaluate the alterations in position of hindbrain after Posterior fossa decompression (PFD), and to identify the factors associated with syrinx resolution in pediatric patients with CMI. METHODS: Eighty-seven patients, aged from 5 to 18 years, who underwent PFD for CMI between September 2006 and September 2012 were retrospectively reviewed. On mid-sagittal MR images, the position of medulla oblongata and cerebellum was quantitatively evaluated preoperatively and at follow-up...
January 2015: European Spine Journal
Masato Tanaka, Yoshihisa Sugimoto, Shinya Arataki, Tomoyuki Takigawa, Toshifumi Ozaki
Spinal deformity is an important clinical manifestation of Chiari I malformation (CM-I) and syringomyelia. Here we report the result of an 8-year follow-up of a 13-year-old girl with severe scoliosis associated with Chiari malformation and a large syringomyelia. The patient presented at our hospital at the age of 13 with a 68° scoliosis. Magnetic resonance imaging showed Chiari malformation and a large syringomyelia. Neurosurgical treatment involved foramen magnum decompression and partial C1 laminectomy, but the scoliosis still progressed...
2014: Acta Medica Okayama
T Kozłowski, M Cybulska, B Błaszczyk, M Krajewska, C Jeśman
of morphological and tomographic (CT) studies of the skull that was found in the crypt of the Silesian Piasts in the St. Jadwiga church in Brzeg (Silesia, Poland) are presented and discussed here. The established date of burial of probably a 20-30 years old male was 16th-17th century. The analyzed skull showed premature obliteration of the major skull sutures. It resulted in the braincase deformation, similar to the forms found in oxycephaly and microcephaly. Tomographic analysis revealed gross pathology. Signs of increased intracranial pressure, basilar invagination and hypoplasia of the occipital bone were observed...
October 2014: Homo: Internationale Zeitschrift Für die Vergleichende Forschung Am Menschen
Krzysztof Zapałowicz, Maciej Radek, Maciej Wojdyn, Piotr Pietkiewicz, Jurek Olszewski
A 48-year-old man was admitted for the management of congenital anomalies: Arnold-Chiari type I malformation combined with odontoid upward migration. He also had degenerative stenosis of the spinal canal by spurs at C2/C3 and C3/C4 levels. Osseous deformities caused ischaemic changes of the brainstem as well as spinal cord compression. Authors used the Biocage - interbody cage covered by bioresorbable layer to fill the surgically created gap after removal of the right part of C3 vertebral body. Twenty-seven months after implantation, the implant was extruded through posterior pharyngeal wall...
2014: Neurologia i Neurochirurgia Polska
Jakub Godzik, Michael P Kelly, Alireza Radmanesh, David Kim, Terrence F Holekamp, Matthew D Smyth, Lawrence G Lenke, Joshua S Shimony, Tae Sung Park, Jeffrey Leonard, David D Limbrick
OBJECT: Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I-associated syringomyelia...
April 2014: Journal of Neurosurgery. Pediatrics
Jason Pui-Yin Cheung, Dino Samartzis, Kenneth Man-Chee Cheung
BACKGROUND CONTEXT: Acute correction of severe spinal deformities significantly increases the risk of neurologic complications. Previously used methods to safely correct these deformities include halo-traction devices and internal distraction rods. PURPOSE: This report introduces a novel method for gradual correction of severe spinal deformity by using the magnetically controlled growing rod (MCGR). STUDY DESIGN: This is a case report. METHODS: A 12-year-old girl with severe kyphoscoliosis and concurrent syringomyelia and Arnold-Chiari Type I malformation underwent implantation of the MCGR...
July 1, 2014: Spine Journal: Official Journal of the North American Spine Society
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