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Matthew M. Peterson, Liviu Craciun and John D. Heiss

S yringomyelia is a progressive disease, which if left untreated often leads to debilitating central myelopathy with permanent sensory and motor deficits. Syringomyelia was identified in the late 19th century 1 and its resistance to permanent treatment spurred neurosurgeons in the early and mid-20th century to develop innovative therapeutic approaches. 3 , 5 , 11 , 18 , 19 , 23 , 24 , 26 , 27 , 33 , 34 One technique that was devised to treat syringomyelia but is no longer practiced was reported by Dr. Dean H. Echols ( Fig. 1 ). His technique to treat

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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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John D. Heiss, Nicholas Patronas, Hetty L. DeVroom, Thomas Shawker, Robert Ennis, William Kammerer, Alec Eidsath, Thomas Talbot, Jonathan Morris, Eric Eskioglu and Edward H. Oldfield

W ith a prevalence of 8.4 cases per 100,000 population, approximately 21,000 Americans have syringomyelia, a disorder in which a cyst forms within the spinal cord, resulting in myelopathy. 6 Symptoms of paralysis, sensory loss, and chronic pain commonly develop during the second through fifth decades of life. The natural history of syringomyelia is typically one of gradual, stepwise neurological deterioration extending over many years. 5 Syringomyelia is usually associated with an inferior position of the cerebellar tonsils through the foramen magnum: the

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Russell R. Lonser, John D. Heiss and Edward H. Oldfield

Syringomyelia can be associated with Chiari malformation, spinal neoplasms, trauma, arachnoiditis, and other structural spinal lesions. 1 Our patient had two potential underlying causes for a cervicothoracic syrinx: acquired Chiari I malformation and a spinal neoplasm. This 18-year-old woman with von Hippel—Lindau (VHL) disease suffered from increasingly intense occipital headaches. Magnetic resonance (MR) imaging revealed two contrast-enhancing lesions in the right cerebellar hemisphere ( Fig. 1A ), herniation of the cerebellar tonsils to C-1 ( Fig. 1B ), and

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John D. Heiss, Kendall Snyder, Matthew M. Peterson, Nicholas J. Patronas, John A. Butman, René K. Smith, Hetty L. DeVroom, Charles A. Sansur, Eric Eskioglu, William A. Kammerer and Edward H. Oldfield

S yringomyelia associated with spinal pathology is known as primary spinal syringomyelia (PSS). 3 , 4 The natural history of this and other forms of syringomyelia is one of gradual, stepwise deterioration over many years. 4 , 73 Primary spinal syringomyelia, which accounts for about one-sixth of all cases of syringomyelia, may be a consequence of traumatic injury, inflammatory conditions, or compressive lesions that compromise the subarachnoid space. 71 Posttraumatic syringomyelia manifests as progressive neurological deficit above the level of previous

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John D. Heiss, Giancarlo Suffredini, René Smith, Hetty L. DeVroom, Nicholas J. Patronas, John A. Butman, Francine Thomas and Edward H. Oldfield

S yringomyelia is more often associated with CM-I than any other etiology. 17 , 18 , 37 The objective of surgical treatment of syringomyelia associated with the CM-I is to decompress the cerebellar tonsils, eliminate the syrinx, and prevent the progression of myelopathy. A reduced syrinx size, which is associated with disease stabilization, occurs after craniocervical decompression or syrinx fluid shunting. Unlike craniocervical decompression, syrinx shunting does not address the underlying cause of the syringomyelia, requires invasion of the spinal cord

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John D. Heiss, Nicholas Patronas, Hetty L. DeVroom, Thomas Shawker, Robert Ennis, William Kammerer, Alec Eidsath, Thomas Talbot, Jonathan Morris, Eric Eskioglu and Edward H. Oldfield


Syringomyelia causes progressive myelopathy. Most patients with syringomyelia have a Chiari I malformation of the cerebellar tonsils. Determination of the pathophysiological mechanisms underlying the progression of syringomyelia associated with the Chiari I malformation should improve strategies to halt progression of myelopathy.


The authors prospectively studied 20 adult patients with both Chiari I malformation and symptomatic syringomyelia. Testing before surgery included the following: clinical examination; evaluation of anatomy by using T1-weighted magnetic resonance (MR) imaging; evaluation of the syrinx and cerebrospinal fluid (CSF) velocity and flow by using phase-contrast cine MR imaging; and evaluation of lumbar and cervical subarachnoid pressure at rest, during the Valsalva maneuver, during jugular compression, and following removal of CSF (CSF compliance measurement). During surgery, cardiac-gated ultrasonography and pressure measurements were obtained from the intracranial, cervical subarachnoid, and lumbar intrathecal spaces and syrinx. Six months after surgery, clinical examinations, MR imaging studies, and CSF pressure recordings were repeated. Clinical examinations and MR imaging studies were repeated annually. For comparison, 18 healthy volunteers underwent T1-weighted MR imaging, cine MR imaging, and cervical and lumbar subarachnoid pressure testing.

Compared with healthy volunteers, before surgery, the patients had decreased anteroposterior diameters of the ventral and dorsal CSF spaces at the foramen magnum. In patients, CSF velocity at the foramen magnum was increased, but CSF flow was reduced. Transmission of intracranial pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was partially obstructed. Spinal CSF compliance was reduced, whereas cervical subarachnoid pressure and pulse pressure were increased. Syrinx fluid flowed inferiorly during systole and superiorly during diastole on cine MR imaging. At surgery, the cerebellar tonsils abruptly descended during systole and ascended during diastole, and the upper pole of the syrinx contracted in a manner synchronous with tonsillar descent and with the peak systolic cervical subarachnoid pressure wave. Following surgery, the diameter of the CSF passages at the foramen magnum increased compared with preoperative values, and the maximum flow rate of CSF across the foramen magnum during systole increased. Transmission of pressure across the foramen magnum to the spinal subarachnoid space in response to jugular compression was normal and cervical subarachnoid mean pressuree and pulse pressure decreased to normal. The maximum syrinx diameter decreased on MR imaging in all patients. Cine MR imaging documented reduced velocity and flow of the syrinx fluid. Clinical symptoms and signs improved or remained stable in all patients, and the tonsils resumed a normal shape.


The progression of syringomyelia associated with Chiari I malformation is produced by the action of the cerebellar tonsils, which partially occlude the subarachnoid space at the foramen magnum and act as a piston on the partially enclosed spinal subarachnoid space. This creates enlarged cervical subarachnoid pressure waves that compress the spinal cord from without, not from within, and propagate syrinx fluid caudally with each heartbeat, which leads to syrinx progression. The disappearance of the abnormal shape and position of the tonsils after simple decompressive extraarachnoidal surgery suggests that the Chiari I malformation of the cerebellar tonsils is acquired, not congenital. Surgery limited to suboccipital craniectomy, C-1 laminectomy, and duraplasty eliminates this mechanism and eliminates syringomyelia and its progression without the risk of more invasive procedures.

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Neurosurgical Forum: Letters to the Editor To The Editor Lucio Palma , M.D. Siena, Italy 1071 1073 I read with great interest and pleasure the article by Heiss, et al. (Heiss JD, Patronas N, De-Vroom H, et al: Elucidating the pathophysiology of syringomyelia. J Neurosurg 91: 553–562, October, 1999). I wholeheartedly agree with the authors and congratulate their excellent study. However I feel it is very important to stress that a simple osteodural craniocerebral (CCD) procedure for treating syringomyelia has already been used with success by myself

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Han Soo Chang, Tsukasa Tsuchiya and Toru Matsui

T o the E ditor : We read with great interest the recent article by Heiss and colleagues (Heiss JD, Suffredini G, Smith R, et al: Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery. Clinical article. J Neurosurg Spine 13: 729–742, December, 2010). 5 The authors conducted a prospective study in 16 patients with Chiari malformation Type I (CM-I) who had persistent syringomyelia despite previous craniocervical decompression. The authors found partial blockage of CSF pathways at the craniovertebral junction with abnormally

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Elad I. Levy, John D. Heiss, Michael S. Kent, Charles J. Riedel and Edward H. Oldfield

I n several different theories there is an attempt to explain the pathophysiology of syringomyelia associated with the Chiari I malformation. In some of them, mechanisms in which the syrinx expands progressively from an initially small locus of syringomyelia, a “drop” of fluid, to a cavity that is large enough to distend the spinal cord are proposed. This is the mechanism proposed for the theories advocated by Gardner and Angel, 4, 5 and Williams 18–20 in which the persistence of a communication between the fourth ventricle and the central canal at the obex