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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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Bermans J. Iskandar, Mark Quigley and Victor M. Haughton

surgery, which is another fact that raises questions about the adequacy of surgical treatment. These controversies regarding the pathophysiology of the Chiari I malformation and syringomyelia have not been resolved by experimental studies. Williams 22, 23 has shown experimentally that, in patients with a Chiari I malformation, a pressure differential existed between the cranial and spinal compartments, indicating a relative obstruction of CSF flow at the foramen magnum. Although such experimental designs were considered revolutionary when Williams published his

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Bermans J. Iskandar and Victor Haughton

, Jolivet O , Hurth M , et al : CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol 21 : 1785 – 1792 , 2000 Brugieres P, Idy-Peretti I, Iffenecker C, Parker F, Jolivet O, Hurth M, et al: CSF flow measurement in syringomyelia. AJNR Am J Neuroradiol 21: 1785–1792, 2000 4. Dolar MT , Haughton VM , Iskandar BJ , Quigley M : Effect of craniocervical decompression on peak CSF velocities in symptomatic patients with Chiari I malformation. AJNR Am J Neuroradiol 25 : 142 – 145 , 2004

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Bermans J. Iskandar, Jerry Oakes, Colleen McLaughlin, Alan K. Osumi and Robert D. Tien

in patients with anal agenesis. J Pediatr Surg 26: 1001–1005, 1991 5. Gates PC , Fox AJ , Barnett HJM : CT metrizamide myelography in syringomyelia. Sensitivity and specificity. Neurology 36 : 1245 – 1248 , 1986 Gates PC, Fox AJ, Barnett HJM: CT metrizamide myelography in syringomyelia. Sensitivity and specificity. Neurology 36: 1245–1248, 1986 6. Gupta RK , Sharma A , Jena A , et al : Magnetic resonance evaluation of spinal dysraphism in children. Childs Nerv Syst 6 : 161

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R. Shane Tubbs, Bermans J. Iskandar, Alfred A. Bartolucci and W. Jerry Oakes

, and a history of atlantooccipital fusion. The angulation of the odontoid process 20 ranged from 65 to 100° (mean 84.4 ± 9.3°[standard deviation]) ( Fig. 1 upper ). The distance of the obex beneath the foramen magnum ranged from 9 to 20 mm (mean 14.4 ± 3.96 mm) ( Fig. 1 center ). The anteroposterior width of the medulla oblongata (< 15 mm in all patients) resulted in a flattened appearance in the midsagittal region. 7 In 11 patients (50%) syringomyelia was demonstrated: six with a holocord syrinx, two with a cervical syrinx, one with a thoracic syrinx, and two

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Bermans J. Iskandar, Gary L. Hedlund, Paul A. Grabb and W. Jerry Oakes

35 : 214 – 224 , 1994 Armonda RA, Citrin CM, Foley KT, et al: Quantitative cinemode magnetic resonance imaging of Chiari I malformations: an analysis of cerebrospinal fluid dynamics. Neurosurgery 35: 214–224, 1994 3. Ball MJ , Dayan AD : Pathogenesis of syringomyelia. Lancet 2 : 799 – 801 , 1972 Ball MJ, Dayan AD: Pathogenesis of syringomyelia. Lancet 2: 799–801, 1972 4. Bhadelia RA , Bogdan AR , Wolpert SM , et al : Cerebrospinal fluid flow waveforms: analysis in patients with

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Harold L. Rekate

canulation). J Neurosurg Sci 30: 47–53, 1986 3. Lapras C , Poirier N , Deruty R , Bret P , Jyeux O : [Catheterization of the sylvian aqueduct. Its present role in the surgical treatment of sylvian aqueduct stenosis of PCF tumors, and of syringomyelia.] Neurochirurgie 21 : 101 – 109 , 1975 (Fr) Lapras C, Poirier N, Deruty R, Bret P, Jyeux O: [Catheterization of the sylvian aqueduct. Its present role in the surgical treatment of sylvian aqueduct stenosis of PCF tumors, and of syringomyelia.] Neurochirurgie 21

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R. Shane Tubbs, John C. Wellons III, Bermans J. Iskandar and W. Jerry Oakes

of associated varieties of OSD with isolated flat capillary hemangiomas of the lumbosacral spine in this series No. of Cases meningocele manqué 1 SCM w/ meningocele manqué 2 dermal sinus tract w/ posterior spinal lipoma 2 terminal syrinx 5 syringomyelia 3 fatty terminal filum 5 lipomyelomeningocele 3 Fig. 1. Four examples of isolated lumbosacral flat capillary hemangiomas in our series. Fig. 2. Sagittal T 2 -weighted MR image of the thoracolumbosacral

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Sharad Rajpal, R. Shane Tubbs, Timothy George, W. Jerry Oakes, Herbert E. Fuchs, Mark N. Hadley and Bermans J. Iskandar

was made. Surgical Treatment In all cases the surgery was performed by one of five neurosurgeons (H.F., T.G., W.J.O., M.H., or B.I.). Surgery consisted of TCR in which the tethering lesion was excised when appropriate. The spinal defects repaired included lipomyelomeningoceles (25 patients), tight terminal fila (22 patients), SCMs (15 patients), syringomyelia (seven patients), dermoid cysts (two patients), and a meningocele (one patient) ( Fig. 1 ). In 12 patients (20%) we documented more than one anomaly. Seven patients had scoliosis and one patient had a