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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 and David D. Limbrick

C hiari malformation Type I (CM-I) is a developmental abnormality of the craniovertebral junction (CVJ), often associated with spinal cord abnormalities such as syringomyelia and scoliosis. 10 , 33 The rate of scoliosis in pediatric patients with CM-I has been reported to be as high as 80% in those with concurrent syringomyelia. 6 , 10 , 16 , 20 , 31 Some authors have speculated that the etiology of scoliosis in such patients is related to the effect of the expanding spinal cord syrinx on the function of medially located motor neurons; the resulting

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R. Shane Tubbs, Matthew D. Smyth, John C. Wellons III and W. Jerry Oakes

cavity confirmed. In some reports the authors have noted intradural “membranes, veils, pouches, and webs” interfering with CSF flow into the craniocervical subarachnoid space. 1–3, 6, 11, 12, 14 To date, few writers have discussed these anomalies in relation to the Chiari I malformation and associated syringomyelia. Clinical Material and Methods We retrospectively reviewed the surgical experience of the senior author (W.J.O.) in the treatment of pediatric Chiari I malformation at two institutions. The first 30 patients in this group underwent surgery at Duke

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Chester K. Yarbrough, Alexander K. Powers, Tae Sung Park, Jeffrey R. Leonard, David D. Limbrick and Matthew D. Smyth

cord changes and CM-I on initial MR imaging. Five patients had syringomyelia, and 1 had T2 hyperintensity at the cervicomedullary junction. One patient showed T2 changes in the brainstem ( Fig. 1G ). Three patients presented after a clear history of minor trauma (1 with paraparesis, 2 with sensory deficits). One patient reported a mild blow to the head during a flag football game, another reported a fall from standing immediately preceding onset of symptoms, and the third patient performed a flip on a trampoline, landing on her feet prior to the onset of symptoms

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Spiros l. Blackburn and Matthew D. Smyth

syringomyelia ( Fig. 2 ). Intravenous dexamethasone was administered for several days but failed to improve the patient's neurological condition. While undergoing inpatient rehabilitation, the girl had an increase in her weakness and bulbar symptoms. An additional MR imaging study performed on postoperative Day 15 demonstrated a significantly enlarged extraaxial mass collection causing cervicomedullary compression ( Fig. 3 ). F ig . 2. Postoperative T 1 -weighted contrast-enhanced (left) and T 2 -weighted (right) MR images obtained 2 days after the initial surgery

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Jeffrey H. Miller, David D. Limbrick Jr., Matthew Callen and Matthew D. Smyth

spontaneous resolution of syringomyelia with associated CM-I has been previously reported in several studies, 1 , 3 , 5 , 15 and spontaneous resolution of isolated tonsillar ectopia in CM-I has been described only twice before. 4 This is, however, the first report of spontaneous resolution in monozygotic twins. Despite being one of the most commonly evaluated pathological entities in pediatric neurosurgery, the natural history of CM-I is not fully understood. Unlike CM-II or CM-III, there is no relation to neural tube closure defects. 16 Chiari malformation Type I may

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R. Shane Tubbs, Daniel Webb, Matthew D. Smyth and W. Jerry Oakes

pediatric Chiari I malformations. J Neurosurg 99 : 291 – 296 , 2003 Tubbs RS, McGirt MJ, Oakes WJ: Surgical experience with 130 pediatric Chiari I malformations. J Neurosurg 99: 291–296, 2003 13. Vanaclocha V , Saiz-Sapena N , Garcia-Casasola MC : Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation. Acta Neurochir 139 : 529 – 540 , 1997 Vanaclocha V, Saiz-Sapena N, Garcia-Casasola MC: Surgical technique for cranio-cervical decompression in

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Suresh N. Magge, Matthew D. Smyth, Lance S. Governale, Liliana Goumnerova, Joseph Madsen, Becca Munro, Stephen V. Nalbach, Mark R. Proctor, R. Michael Scott and Edward R. Smith

present the combined data of 2 major pediatric neurosurgical centers to describe our experience with this condition. Methods This was an institutional review board–approved retrospective study of all pediatric patients (age < 19 years) in whom idiopathic syrinx had been diagnosed during the period available for review (October 2006–March 2009), as documented on MR imaging. This study was done at Children's Hospital Boston and St. Louis Children's Hospital. Departmental databases were searched for patients with diagnosis codes including “syringomyelia,” “cyst

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Chester K. Yarbrough, Jacob K. Greenberg, Matthew D. Smyth, Jeffrey R. Leonard, Tae Sung Park and David D. Limbrick Jr.

“unchanged” if the following features were present: some or all major symptoms failed to improve to a significant degree after surgery; new CM-I–related symptoms developed after surgery that caused significant impairment; or a patient required repeat surgery for CM-I–related symptoms or syringomyelia, regardless of final outcome. A patient was considered “worse” if overall quality of life decreased as a result of increased severity of major preoperative symptoms, or if new CM-I–related symptoms developed postoperatively. When analyzing outcomes for headache symptoms, only

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Michael Anthony Ciliberto, David Limbrick, Alexander Powers, Jeffrey B. Titus, Rebecca Munro and Matthew D. Smyth

Health, National Institute of Neurological Disorders and Stroke (Grant Nos. R01 NS066932 and R01 NS052478), the Pediatric Surgical Sciences Institute of St. Louis Children's Hospital, and the Park/Reeves Center for Syringomyelia. He also receives industry support for a project entitled Randomized, Blinded Placebo Controlled Trial of Epidural, Sustained-relief Morphine For Acute Post-operative Analgesia Following Selective Dorsal Rhizotomy in Children. Dr. Smyth receives monetary support from the CURE special grant program (CURE Proposal No. 08149006), private

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Jacob K. Greenberg, Eric Milner, Chester K. Yarbrough, Kim Lipsey, Jay F. Piccirillo, Matthew D. Smyth, Tae Sung Park and David D. Limbrick Jr.

: Greenberg, Milner, Yarbrough, Piccirillo, Smyth, Park, Limbrick. Drafting the article: Greenberg, Lipsey. Critically revising the article: all authors. Reviewed submitted version of manuscript: all authors. Approved the final version of the manuscript on behalf of all authors: Greenberg. Statistical analysis: Greenberg. Administrative/technical/material support: Limbrick. Study supervision: Limbrick. References 1 Alfieri A , Pinna G : Long-term results after posterior fossa decompression in syringomyelia with adult Chiari Type I malformation. Clinical article