Study and Surgical Treatment of Syringomyelia
Summary
The goal of this study is to establish the mechanism(s) of progression of primarily spinal syringomyelia (PSS). Our preliminary study of syringomyelia emphasized syringomyelia associated with craniocervical junction abnormalities (CCJAS), such as the Chiari I malformation. This new protocol will expand the scope of our investigation to include primarily spinal syringomyelia (PSS), which is defined as syringomyelia not associated with craniocervical junction abnormalities (CCJAS). Etiologies of primarily spinal syringomyelia include 1) intradural scarring which is post-traumatic, post-inflammatory, or post-operative, 2) intradural-extramedullary masses such as arachnoid cysts or meningiomas, and 3) extramedullary-extradural spinal lesions such as cervical spondylosis or spinal deformity.
Our hypothesis is the following: Primarily spinal syringomyelia (PSS), results from obstruction of cerebrospinal fluid (CSF) flow within the spinal subarachnoid space; this obstruction affects spinal CSF dynamics because the spinal subarachnoid space accepts the fluid that is displaced from the intracranial subarachnoid space as the brain expands during cardiac systole; in the case of primarily spinal syringomyelia (PSS), a subarachnoid block effectively shortens the spinal subarachnoid space, reducing CSF compliance and the capacity of the spinal theca to dampen the subarachnoid CSF pressure waves produced by the brain expansion during cardiac systole; the exaggerated spinal subarachnoid pressure waves occur with every heartbeat and act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. Presyringomyelia, a recently described state of spinal cord edema associated with progressive myelopathy and obstruction in CSF flow, is a precursor stage to syringomyelia that is consistent with this hypothesis. Because of the importance of this condition to the pathophysiology of syringomyelia, we will also study patients with presyringomyelia in this protocol. After a syrinx is formed, the enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression.
Many neurosurgeons at prominent academic centers routinely use syrinx shunts to treat primarily spinal syringomyelia. This study should provide data that a surgical procedure that opens the spinal subarachnoid space corrects the underlying pathophysiology and resolves the syrinx and that invasion of the spinal cord is unnecessary.
Description
Objective: The goal of this study is to establish the mechanism(s) of progression of primarily spinal syringomyelia (PSS), the type of syringomyelia that is associated with pathology in the spinal column and not at the craniocervical junction. Our hypothesis is that when a lesion obstructs the spinal subarachnoid space, it shortens the segment of spinal canal that dampens the CSF pressure waves that are produced with each heartbeat and creates enlarged spinal subarachnoid pressure waves that act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. After a syrinx is formed, enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression.
Study Population: Subjects will have primary spinal syringomyelia associated with 1) intradural scarring which is post-traumatic, post-inflammatory, or post-operative, 2) intradural-extramedullary masses such as arachnoid cysts or meningiomas, and 3) extramedullary-extradural spinal lesions such as cervical spondylosis or spinal deformity, or 4) an intramedullary tumor.
Design: Subjects will have testing before and after standard surgical therapy of syringomyelia. Testing includes measurement of CSF pressure, neurologic examination, CT-myelography, and MR scanning. Results of CSF pressure measurements before surgery will be compared to measurements from normal controls that were previously studied. The effect of surgery on CSF pressure, neurologic examination, CT-myelography, and MRI scans will be evaluated.
Outcome Measures: The primary outcome measure is cervical CSF pulse pressure, which is the amplitude of the CSF pressure wave, compared to normal values. Secondary outcomes measures include change in CSF pulse pressure, neurologic examination, CT-myelography, and MRI scans between before and after surgery.
Study Design
N/A
Conditions
Syringomyelia
Location
National Institutes of Health Clinical Center, 9000 Rockville Pike
Bethesda
Maryland
United States
20892
Status
Active, not recruiting
Source
National Institutes of Health Clinical Center (CC)
Results (where available)
Links
- Source: http://clinicaltrials.gov/show/NCT00011245
- Information obtained from ClinicalTrials.gov on July 15, 2010
Medical and Biotech [MESH] Definitions
Syringomyelia
Longitudinal cavities in the spinal cord, most often in the cervical region, which may extend for multiple spinal levels. The cavities are lined by dense, gliogenous tissue and may be associated with SPINAL CORD NEOPLASMS; spinal cord traumatic injuries; and vascular malformations. Syringomyelia is marked clinically by pain and PARESTHESIA, muscular atrophy of the hands, and analgesia with thermoanesthesia of the hands and arms, but with the tactile sense preserved (sensory dissociation). Lower extremity spasticity and incontinence may also develop. (From Adams et al., Principles of Neurology, 6th ed, p1269)
Spina Bifida Cystica
A form of spinal dysraphism associated with a protruding cyst made up of either meninges (i.e., a MENINGOCELE) or meninges in combination with spinal cord tissue (i.e., a MENINGOMYELOCELE). These lesions are frequently associated with spinal cord dysfunction, HYDROCEPHALUS, and SYRINGOMYELIA. (From Davis et al., Textbook of Neuropathology, 2nd ed, pp224-5)
Spinal Cord Neoplasms
Benign and malignant neoplasms which occur within the substance of the spinal cord (intramedullary neoplasms) or in the space between the dura and spinal cord (intradural extramedullary neoplasms). The majority of intramedullary spinal tumors are primary CNS neoplasms including ASTROCYTOMA; EPENDYMOMA; and LIPOMA. Intramedullary neoplasms are often associated with SYRINGOMYELIA. The most frequent histologic types of intradural-extramedullary tumors are MENINGIOMA and NEUROFIBROMA.
Clinical Trials
Establishing the Physiology of Syringomyelia
The brain and spinal cord are surrounded by fluid called cerebrospinal fluid (CSF). The CSF flows through channels in the brain and around the spinal cord. Occasionally, people are born...
A Prospective Natural History Study of Patients With Syringomyelia
Background: - Syringomyelia is a disorder in which a cyst (syrinx) forms within the spinal cord and causes spinal cord injury, with symptoms worsening over many years, including...
Genetic Analysis of the Chiari I Malformation
The purpose of this study is to better understand the genetic factors related to the Chiari I malformation. In people with this abnormality, the lower part of the skull is smaller than no...
The Genetics of Chiari Type I Malformation
The Duke Center for Human Genetics, is investigating the hereditary basis of Chiari type I malformations with or without syringomyelia (CM1/S). Our research is aimed at learning if CM1/S i...
PubMed Articles
Noncommunicating syringomyelia may complicate repeated untethering procedures in patients with spinal lipomas. Surgical intervention is indicated when syringomyelia is symptomatic. However, the prefer...
Abstract Although it is easier to accurately diagnose syringomyelia with the advent of magnetic resonance (MR) imaging, syringomyelia still poses challenges to clinicians because of its complex sympto...
Charcot shoulder caused by Chiari type I malformation with syringomyelia with six-year follow-up.
We are presenting a case report of neuropathic arthropathy of the shoulder secondary to a syringomyelia and Chiari type I malformation, with detailed clinical and radiological findings. Neuropathic jo...
ABSTRACT: BACKGROUND: With increasing use of magnetic resonance imaging (MRI), underlying syringomyelia is increasingly found in patients with presumed idiopathic scoliosis. To determine the indicatio...
HISTORY: A 20-year-old male presented with neck pain and motor impairment of the upper extremities because of recurrent syringomyelia caused by ventriculoperitoneal shunt malfunction. EXAMINATION AND...
