the tonsils. 10 Compression of the spinal cord, whether caused by CM-I, by a disc herniation, or by a mass lesion, has been associated with syringomyelia. The mechanism of syringomyelia formation is poorly understood, and several hypotheses have been proposed. 6 , 8 , 9 , 11 Some have postulated that the drainage of CSF produced by the ependymal cells of the central canal is blocked, leading to a caudal accumulation of fluid. Others have argued that, in the case of CM-I, occlusion of the foramen magnum causes the intracranial CSF pulsations to be driven into the
Jean-Valery Coumans, Brian P. Walcott, William E. Butler, Brian V. Nahed and Kristopher T. Kahle
Ash Singhal, Tim Bowen-Roberts, Paul Steinbok, Doug Cochrane, Angela T. Byrne and John M. Kerr
T he natural history of syringomyelia in nonoperatively treated patients remains unclear. With its pathogenesis not well defined, 13 ambiguity exists with regard to managing this condition, especially when syringomyelia is identified incidentally on MR imaging. Most neurosurgeons would choose to operate when a syrinx is progressing in size or deemed symptomatic. 7 There is no accepted strategy, however, for the management of syringes found on MR images when symptoms are subtle or not thought to be related to the syrinx. Although some other studies have
Anil K. Roy, Nicholas P. Slimack and Aruna Ganju
A syrinx is defined as a fluid-filled cavity that anatomically lies within the spinal cord parenchyma or the central canal. 10 Although syringomyelia is clinically associated with a centromedullary syndrome with predominantly sensory symptoms such as pain and temperature insensitivity, 34 in many cases it is an incidental finding. This entity is most frequently associated with a CM-I, 30 although other known causes include spinal cord tumor, trauma, and posttraumatic or infectious adhesive arachnoiditis. 18 , 22 , 36 With the increasing use of MR
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.
Langston T. Holly and Ulrich Batzdorf
Intradural arachnoid cysts are relatively uncommon pouches of cerebrospinal fluid (CSF) found within the subarachnoid space. The authors present a series of eight symptomatic patients in whom syrinx cavities were associated with arachnoid cysts, and they discuss treatment strategies for this entity.
The population comprised eight men whose mean age was 50 years (range 35–81 years). All patients experienced gait difficulty, and it was the chief complaint in seven; arm pain was the primary complaint in one. No patient had a history of spinal trauma, meningitis, or previous spinal surgery at the level of the syrinx cavity or arachnoid cyst. In each patient imaging revealed a syrinx cavity affecting two to 10 vertebral levels. Posterior thoracic arachnoid cysts were found in proximity to the syrinx cavity in each case. There was no evidence of cavity enhancement, Chiari malformation, tethered cord, or hydrocephalus.
All patients underwent thoracic laminectomy and resection of the arachnoid cyst wall, and postoperative neurological improvement was documented in each case. The mean follow-up duration was 19 months (range 4–37 months). Follow-up magnetic resonance imaging demonstrated the disappearance of the arachnoid cyst and a significant decrease in syrinx cavity size in each patient.
Spinal arachnoid cysts can be associated with syringomyelia, likely due to alterations in normal CSF dynamics. In symptomatic patients these cysts should be resected and the normal CSF flow restored. The results of the present series indicate that neurological improvement and reduction in syrinx cavity size can be achieved in patients with syringomyelia associated with intradural arachnoid cysts.
Kazutoshi Hida and Yoshinobu Iwasaki
The authors describe the surgical procedures for placing syringosubarachnoid shunts and the results of surgery, as well as the prevention of shunt malfunction.
The series consisted of 59 patients with syringomyelia associated with Chiari I malformation in whom syringosubarachnoid shunts were placed. Their ages ranged from 4 to 62 years (median 28 years). The follow-up period ranged from 13 to 219 months. The authors principally implanted the shunts in patients with large-sized syringes. Neurological improvement was satisfactory, and postoperative magnetic resonance imaging demonstrated that the syringes had resolved or decreased in size in all patients. Reoperation was necessary in 10 patients who were treated before 1993.
To prevent shunt malfunction, both dorsal root entry zone myelotomy and placement of the syringo-subarachnoid shunt tube into the ventral subarachnoid space are useful.
Marcy C. Speer, Timothy M. George, David S. Enterline, Amy Franklin, Chantelle M. Wolpert and Thomas H. Milhorat
In several reports the authors have suggested occasional familial aggregation of syringomyelia and/or Chiari 1 malformation (CM1). Familial aggregation is one characteristic of traits that have an underlying genetic basis. The authors provide evidence for familial aggregation of CM1 and syringomyelia (CM1/S) in a large series of families, establishing that there may be a genetic component to CM1/S in at least a subset of families. The authors observed no cases of isolated familial syringomyelia in their family studies, suggesting that familial syringomyelia is more accurately classified as familial CM1 with associated syringomyelia.
These data, together with the cosegregation of the trait with known genetic syndromes, support the authors' hypothesis of a genetic basis for some CM1/S cases.
Richard G. Ellenbogen, Rocco A. Armonda, Dennis W. W. Shaw and H. Richard Winn
In patients with Chiari I malformation with and without associated syringomyelia, aberrant cerebrospinal fluid (CSF) dynamics and a spectrum of posterior fossa pathological findings are demonstrated. In this study, the authors test the validity of using prospective cardiac-gated phase-contrast cine-mode magnetic resonance (MR) imaging to define the malformation, delineate its pathophysiology, and assist in implementing a rational treatment plan.
Eighty-five cases were prospectively analyzed using cine MR imaging. Sixty-five patients, adults and children, with symptomatic Chiari malformation, with and without syringomyelia, were surgically treated from 1990 to 1999. All patients underwent pre- and postoperative cine MR evaluation. Ten patients were treated after a previous surgical procedure had failed. To establish CSF flow characteristics and normative CSF profiles, 20 healthy volunteers were examined.
Compared with normal volunteers, in Chiari I malformation patients with and without syringomyelia, uniformly abnormal craniocervical junction CSF flow profiles were revealed. After intradural exploration, nearly all patients with Chiari I malformation experienced clinical improvement and CSF flow profiles, paralleling those of normal volunteers, were shown. In all patients in whom treatment had failed, abnormal preoperative CSF flow profiles, which correlated with suspected physiological abnormalities and the pathological findings noted at reoperation, were demonstrated.
Symptomatic Chiari I malformation is a dynamic process characterized by the impaction of the hindbrain in an abnormal posterior fossa. This compression obstructs the normal venting of CSF in and out of the craniocervical sub-arachnoid space, throughout the cardiac cycle. Therefore, decompression or enlargement of the posterior fossa to establish normal CSF pathways should be the primary goal of surgical intervention. Aberrant CSF flow appears to be only one aspect of the pathological condition found in patients with Chiari I malformation. Arachnoid scarring in the posterior fossa and selective vulnerability of the spinal cord may also be factors in the pathogenesis and maintenance of associated syringomyelia. Phase-contrast cine MR imaging is a useful tool in defining physiological and anatomical problems in patients with Chiari I and syringomyelia, and it can help guide an appropriate primary or salvage surgical therapy.
Andrew M. Bauer, Diane M. Mueller and John J. Oró
Achondroplasia has been associated with varying degrees of cervicomedullary and spinal compression, although usually in the pediatric population. Large arachnoid cysts have also been found to result in tonsillar herniation and syringomyelia. The authors present the case of a patient with achondroplasia who presented with symptoms of foramen magnum compression and syringomyelia, and who was subsequently found to have a large posterior fossa arachnoid cyst.
This 38-year-old woman with achondroplasia presented with an 8-month history of headache and numbness of the hands and fingers. Admission magnetic resonance (MR) imaging of the head and spine revealed a large arachnoid cyst in the posterior cranial fossa, a 6-mm tonsillar herniation consistent with an acquired Chiari malformation, and a large cervicothoracic syrinx. The patient was treated using suboccipital craniectomy, C-1 laminectomy, fenestration of the arachnoid cyst, and decompression of the acquired Chiari malformation with duraplasty.
Surgical decompression resulted in improvement of the presenting symptoms, adequate decompression of crowding at the foramen magnum, and resolution of the syrinx. Although there was only partial reduction in the retrocerebellar cisternal space on follow-up MR imaging, no residual symptoms were related to this.
Juan C. Alzate, Karl F. Kothbauer, George I. Jallo and Fred J. Epstein
The authors describe the results of performing a standard posterior craniovertebral decompression and placement, if indicated, of a syringosubarachnoid shunt for the treatment of patients with Chiari I malformation with and without syringomyelia.
This is a retrospectively analyzed consecutive series of 66 patients (mean patient age 15 years, range 1–53 years). The uniform posterior craniovertebral decompression consisted of a small suboccipital craniectomy, a C-1 laminectomy, microsurgical reduction of the cerebellar tonsils, and dural closure with a synthetic dural graft to increase the cerebrospinal fluid space at the craniocervical junction. The presence of a large syrinx, with significant thinning of the spinal cord tissue and obliteration of the spinal subarachnoid space, particularly when combined with syrinx-related symptoms, was an indication for the placement of a syringosubarachnoid shunt.
In 32 patients Chiari I malformation alone was present, and 34 in patients it was present in combination with syringomyelia. Clinical findings included pain, neurological deficits, and spinal deformity. The presence of syringomyelia was significantly associated with the presence of scoliosis (odds ratio 74.4 [95% confidence interval 8.894–622.4]).
All patients underwent a posterior craniovertebral decompression procedure. In 22 of the 34 patients with syringomyelia a syringosubarachnoid shunt was also placed. The mean follow-up period was 24 months (range 3–95 months). Excellent outcome was achieved in 54 patients (82%) and good outcome in 12 (18%). In no patient were symptoms unchanged or worse at follow-up examination, including four patients who initially required a second operation for persistent syringomyelia. Pain was more likely to resolve than sensory and motor deficits after decompressive surgery.
Radiological examination revealed normalization of tonsillar position in all patients. The syrinx had disappeared in 15 cases, was decreased in size in 17, and remained unchanged in two.
Posterior craniovertebral decompression and selective placement of a syringosubarachnoid shunt in patients with Chiari I malformation and syringomyelia is an effective and safe treatment. Primary placement of a shunt in the presence of a sufficiently large syrinx appears to be beneficial. The question of if and when to place a shunt, however, requires further, preferably prospective, investigation.