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Sumit Thakar, Narayanam Anantha Sai Kiran and Alangar S. Hegde

change in the patient's clinical status at discharge 1 week later. At a follow-up visit 6 months later, his motor and sensory deficits had resolved, whereas he had persistent sphincteric dysfunction, with a few related episodes of bladder infection. Follow-up MR imaging showed complete resolution of the syringomyelia ( Fig. 2 ) in the absence of the sacral AC ( Fig. 3 ). F ig . 2. Postoperative lumbosacral spine T2-weighted MR images, sagittal sections, showing no recurrence of the AC and disappearance of the syrinx (A) , with normal-looking thoracic spine (B

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Martin M. Mortazavi, R. Shane Tubbs, Maja Andrea Brockerhoff, Marios Loukas and W. Jerry Oakes

herniation occurs as an imprecise continuum ranging from mild tonsillar herniation through the foramen magnum to severe cerebellar and brainstem herniation into a posterior encephalocele leading to syringomyelia, medullary compression, and ischemia; 3) despite their common anatomical location, the 4 anomalies have distinct clinicoanatomical features; and 4) although the large majority of cases are congenital, cases of acquired Chiari I malformation have been reported. Other Early Reports of Hindbrain Herniation Chiari was not the first to observe and report on Type

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James S. Walkden, Richard A. Cowie and John A. Thorne

T he bony anomalies of the craniocervical junction (CCJ) and their underlying embryology are well described in the surgical literature. 6 We noted only one published case report of occipitocondylar hyperplasia that was associated with myelopathy and previous spinal cord injury. 9 We describe a previously unreported presentation involving facial pain and chest dysesthesia secondary to extensive syringomyelia associated with this rare craniovertebral junction abnormality. We describe the presentation, investigation, and long-term management of this unusual

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Rohan Ramakrishna, Jeffrey C. Mai, Tanya Filardi, Samuel R. Browd and Richard G. Ellenbogen

C hiari malformations constitute a group of entities with very different etiologies, clinical features, and pathophysiologies. 2 , 5 , 8 , 11 , 14–16 Little in the literature, however, has focused on patients who develop an acquired Chiari-like syndrome with its attendant syringomyelia as a complication of previous posterior fossa pathological entities. Likewise, while hydrocephalus and its various treatments have been widely discussed in the neurosurgical tome, nonneoplastic brainstem hypertrophy as a consequence of sustained elevated intracranial

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Coleman P. Riordan and R. Michael Scott

I n patients with syringomyelia and type I Chiari malformation (CM-I) who have required reoperation because of persistent, recurrent, or enlarging syrinx, the senior author (R.M.S.) has placed a stent from the fourth ventricle to the cervical subarachnoid space if the second operative exploration demonstrated evidence of obstructed circulation of CSF from the foramen of Magendie. 3 This study was undertaken to determine the long-term success of this operative stratagem in eliminating the syrinx, as well as to document the complications that occurred following

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

C hiari malformation Type I is a disorder in which the cerebellar tonsils protrude through the foramen magnum and into the spinal canal. This anomaly is the leading cause of syringomyelia and occurs with or without associated osseous abnormalities at the craniovertebral junction. 3–5 , 7–10 , 12–14 In contrast to other types of Chiari malformations, which usually present in infancy, CM-I tends to present during or after the second decade of life. Overcrowding of the hindbrain by an underdeveloped posterior fossa commonly causes tonsillar ectopia in CM

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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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Brandon G. Rocque, Timothy M. George, John Kestle and Bermans J. Iskandar

C hiari malformation Type I is commonly associated with syringomyelia. The clinical presentation of CM-I with a syrinx varies from the incidentally diagnosed asymptomatic patient to the patient with severe neurological deficits. Three pediatric neurosurgical surveys have been previously conducted to determine if there is consensus regarding indications for operative treatment and choice of surgical procedure. 1 , 3 , 4 In this article, we report the results of a survey administered at the 2006 meeting of the ASPN to all members present. The survey was

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Ben J. Bixenmann, Beth M. Kline-Fath, Karin S. Bierbrauer and Danesh Bansal

T he estimated prevalence of syringomyelia in the general population is 1.94 to 8.4 per 100,000 people. 3 , 5 , 13 The pathogenesis of the formation of a spinal cord syrinx has been debated for some time, with theories originally describing disorderly embryonic cellular development and migration. 10 Recently, the pathophysiology of a syrinx has been believed to originate from disorderly CSF flow. 2 , 4–6 , 9 , 14 , 15 The population of patients with open spinal neural tube defects (NTDs) has long been known to have a much higher rate of syrinx formation

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Brian J. Dlouhy, Jeffrey D. Dawson and Arnold H. Menezes

C hiari malformation Type I (CM-I) 2 , 6–8 is a condition characterized by inferior displacement of the cerebellar tonsils through the foramen magnum, is associated with syringomyelia, and has been well described in the literature, with various presentations and a constellation of signs and symptoms. 23 , 24 , 38 There are extensive case series of both pediatric 38 and adult patients in which presentation, nonoperative or operative management, surgical procedure, and outcome analysis are described, with widely varying results. 24 A limitation to better