Syringomyelia associated with foramen magnum arachnoiditis

2002 ◽  
Vol 97 (3) ◽  
pp. 317-322 ◽  
Author(s):  
Jörg Klekamp ◽  
Giorgio Iaconetta ◽  
Ulrich Batzdorf ◽  
Madjid Samii
Keyword(s):  
Clinical Course ◽  
Diagnostic Procedures ◽  
Foramen Magnum ◽  
Content Type ◽  
Number Of Patients ◽  
Dural Graft ◽  
Sensory Disturbances ◽  
Chiari I ◽  
Gait Disturbances ◽  
Fine Print

Object. Syringomyelia is often linked to pathological lesions of the foramen magnum. The most common cause is hindbrain herniation, usually referred to as Chiari I or II malformation. Foramen magnum arachnoiditis without either Chiari I or II malformation is a rare cause of syringomyelia. The authors undertook a retrospective analysis of 21 patients with foramen magnum arachnoiditis (FMA) and syringomyelia treated between 1978 and 2000 to determine clinical course and optimum management. Methods. In the review of records, 21 patients with FMA and syringomyelia were documented. A stable clinical course was demonstrated in three patients in whom surgery was not performed, and one patient refused surgical intervention. Seventeen patients underwent 23 operations to treat progressive neurological disease. Of these 23 operations, 18 involved opening of the foramen magnum, arachnoid dissection, and placement of a large dural graft. One patient underwent insertion of a ventriculoperitoneal shunt for treatment of accompanying hydrocephalus, one patient received a cystoperitoneal shunt for an accompanying arachnoid cyst; two syringoperitoneal and one syringosubarachnoid shunts were also inserted. Hospital and outpatient files, neuroimaging studies, and intraoperative photographic and video material were analyzed. Additional follow-up information was obtained by telephone interview and questionnaires. Standard and cardiac-gated magnetic resonance imaging studies are the diagnostic procedures of choice in these patients. Sensory disturbances, dysesthesias, and pain were the only symptoms likely to improve after foramen magnum surgery. Motor weakness and gait disturbances, which were severe in a considerable number of patients, and swallowing disorders tended to remain unchanged. As a consequence of the rather severe arachnoid lesions in most patients, clinical recurrences were observed in 57% over a 5-year period. Conclusions. Surgery for FMA and syringomyelia has to provide clear cerebrospinal fluid pathways between the cerebellopontine cisterns, spinal canal, and fourth ventricle. If this can be achieved successfully, the syrinx decreases in size and the clinical course of the patient may even improve. In patients with severe and widespread areas of arachnoiditis, however, multiple operations may be required at least to stabilize the clinical course.

Elucidating the pathophysiology of syringomyelia

1999 ◽  
Vol 91 (4) ◽  
pp. 553-562 ◽  
Author(s):  
John D. Heiss ◽  
Nicholas Patronas ◽  
Hetty L. DeVroom ◽  
Thomas Shawker ◽  
Robert Ennis ◽  
...  
Keyword(s):  
Mr Imaging ◽  
Subarachnoid Space ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Content Type ◽  
Cine Mr ◽  
Spinal Subarachnoid Space ◽  
Chiari I ◽  
Cerebellar Tonsils ◽  
Fine Print

Object. 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.Methods. 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 pressure 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.Conclusions. 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.

Inclination of the odontoid process in the pediatric Chiari I malformation

2003 ◽  
Vol 98 (1) ◽  
pp. 43-49 ◽  
Author(s):  
R. Shane Tubbs ◽  
John C. Wellons ◽  
Jeffrey P. Blount ◽  
Paul A. Grabb ◽  
W. Jerry Oakes
Keyword(s):  
Odontoid Process ◽  
Postoperative Outcome ◽  
Craniocervical Junction ◽  
Foramen Magnum ◽  
Chiari I Malformation ◽  
Magnetic Resonance Images ◽  
Posterior Aspect ◽  
Content Type ◽  
Chiari I ◽  
Fine Print

Object. The quantitative analysis of odontoid process angulation has had scant attention in the Chiari I malformation population. In this study the authors sought to elucidate the correlation between posterior angulation of the odontoid process and patients with Chiari I malformation. Methods. Magnetic resonance images of the craniocervical junction obtained in 100 children with Chiari I malformation and in 50 children with normal intracranial anatomy (controls) were analyzed. Specific attention was focused on measuring the degree of angulation of the odontoid process and assigning a score to the various degrees. Postoperative outcome following posterior cranial fossa decompression was then correlated to grades of angulation. Other measurements included midsagittal lengths of the foramen magnum and basiocciput, the authors' institutions' previously documented pB—C2 line (a line drawn perpendicular to one drawn between the basion and the posterior aspect of the C-2 body), level of the obex from a midpoint of the McRae line, and the extent of tonsillar herniation. Higher grades of odontoid angulation (retroflexion) were found to be more frequently associated with syringomyelia and particularly holocord syringes. Higher grades of angulation were more common in female patients and were often found to have obices that were caudally displaced greater than three standard deviations below normal. Conclusions. These results not only confirm prior reports of an increased incidence of a retroflexed odontoid process in Chiari I malformation but quantitatively define grades of inclination. Grades of angulation were not found to correlate with postoperative outcome. It is the authors' hopes that these data add to our current limited understanding of the mechanisms involved in hindbrain herniation.

Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases

1998 ◽  
Vol 88 (2) ◽  
pp. 237-242 ◽  
Author(s):  
John L. D. Atkinson ◽  
Brian G. Weinshenker ◽  
Gary M. Miller ◽  
David G. Piepgras ◽  
Bahram Mokri
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Chiari I Malformation ◽  
Csf Leak ◽  
Cerebrospinal Fluid Leakage ◽  
Content Type ◽  
Intracranial Hypotension Syndrome ◽  
Csf Leakage ◽  
Chiari I ◽  
Fine Print

Object. Spontaneous spinal cerebrospinal fluid (CSF) leakage with development of the intracranial hypotension syndrome and acquired Chiari I malformation due to lumbar spinal CSF diversion procedures have both been well described. However, concomitant presentation of both syndromes has rarely been reported. The object of this paper is to present data in seven cases in which both syndromes were present. Three illustrative cases are reported in detail. Methods. The authors describe seven symptomatic cases of spontaneous spinal CSF leakage with chronic intracranial hypotension syndrome in which magnetic resonance (MR) images depicted dural enhancement, brain sagging, loss of CSF cisterns, and acquired Chiari I malformation. Conclusions. This subtype of intracranial hypotension syndrome probably results from chronic spinal drainage of CSF or high-flow CSF shunting and subsequent loss of brain buoyancy that results in brain settling and herniation of hindbrain structures through the foramen magnum. Of 35 cases of spontaneous spinal CSF leakage identified in the authors' practice over the last decade, MR imaging evidence of acquired Chiari I malformation has been shown in seven. Not to be confused with idiopathic Chiari I malformation, ideal therapy requires recognition of the syndrome and treatment directed to the site of the spinal CSF leak.

Terminal ventriculostomy for syringomyelia

1977 ◽  
Vol 46 (5) ◽  
pp. 609-617 ◽  
Author(s):  
W. James Gardner ◽  
Herbert S. Bell ◽  
Pete N. Poolos ◽  
Donald F. Dohn ◽  
Marta Steinberg
Keyword(s):  
Clinical Course ◽  
Adult Life ◽  
Central Canal ◽  
Conus Medullaris ◽  
Fetal Life ◽  
Filum Terminale ◽  
Content Type ◽  
Fine Print

✓ The clinical course of 12 patients who underwent terminal ventriculostomy for syringomyelia is presented. Opening the central canal at the tip of the conus medullaris is a relatively benign procedure that improves the symptoms of syringomyelia and syringobulbia. This canal normally terminates at the tip of the conus, but in each of the 12 surgical specimens it continued into the filum terminale for distances up to 8 cm. In most cases the tip of the conus was located more caudally than normal, indicating some degree of tethering in fetal life. This belief is supported by the fact that the newborn, whose conus is tethered to a lipoma at the sacral level, may develop syringomyelia in adult life.

Angioplasty for basilar artery atherosclerosis

1992 ◽  
Vol 77 (6) ◽  
pp. 941-944 ◽  
Author(s):  
Arvind Ahuja ◽  
Lee R. Guterman ◽  
Leo N. Hopkins
Keyword(s):  
Basilar Artery ◽  
Balloon Angioplasty ◽  
Medical Management ◽  
Clinical Course ◽  
Anticoagulation Therapy ◽  
Transient Ischemic Attacks ◽  
Content Type ◽  
Severe Atherosclerosis ◽  
Percutaneous Transluminal ◽  
Fine Print

✓ A case is presented of severe atherosclerosis of the basilar artery, successfully treated with percutaneous transluminal balloon angioplasty. Crescendo daily transient ischemic attacks consisted of alternating hemiplegia and were refractory to medical management, including anticoagulation therapy. The clinical course, endovascular treatment, and results are described. Prior published experiences with this condition are reviewed.

Dose—response tolerance of the visual pathways and cranial nerves of the cavernous sinus to stereotactic radiosurgery

1998 ◽  
Vol 88 (1) ◽  
pp. 43-50 ◽  
Author(s):  
Klaus A. Leber ◽  
Jutta Berglöff ◽  
Gerhard Pendl
Keyword(s):  
Stereotactic Radiosurgery ◽  
Cavernous Sinus ◽  
Dose Response ◽  
Cranial Nerves ◽  
Visual Pathways ◽  
Content Type ◽  
Number Of Patients ◽  
Response Tolerance ◽  
Fine Print

As the number of patients treated with stereotactic radiosurgery increases, it becomes particularly important to define with precision adverse effects on distinct structures of the nervous system. Object. This study was designed to assess the dose—response tolerance of the visual pathways and cranial nerves after exposure of the cavernous sinus to radiation. Methods. A total of 66 sites in the visual system and 210 cranial nerves of the middle cranial fossa were investigated in 50 patients who had undergone gamma knife treatment for benign skull base tumors. The mean follow-up period was 40 months (range 24–60 months). Follow-up examinations consisted of neurological, neuroradiological, and neuroophthalmological evaluations. The actuarial incidence of optic neuropathy was zero for patients who received a radiation dose of less than 10 Gy, 26.7% for patients receiving a dose in the range of 10 to less than 15 Gy, and 77.8% for those who received doses of 15 Gy or more (p < 0.0001). Previously impaired vision improved in 25.8% and was unchanged in 51.5% of patients. No sign of neuropathy was seen in patients whose cranial nerves of the cavernous sinus received radiation doses of between 5 and 30 Gy. Because tumor control appeared to have been achieved in 98% of the patients, the deterioration in visual function cannot be attributed to tumor progression. Conclusions. The structures of the visual pathways (the optic nerve, chiasm, and tract) exhibit a much higher sensitivity to single-fraction radiation than other cranial nerves, and their particular dose—response characteristics can be defined. In contrast, the oculomotor and trigeminal nerves have a much higher dose tolerance.

Syrinx shunt to posterior fossa cisterns (syringocisternostomy) for bypassing obstructions of upper cervical theca

1992 ◽  
Vol 77 (6) ◽  
pp. 871-874 ◽  
Author(s):  
Thomas H. Milhorat ◽  
Walter D. Johnson ◽  
John I. Miller
Keyword(s):  
Posterior Fossa ◽  
Symptomatic Improvement ◽  
Foramen Magnum ◽  
Content Type ◽  
Results Of Treatment ◽  
Spinal Subarachnoid Space ◽  
Upper Cervical ◽  
Spinal Arachnoiditis ◽  
Chiari I

✓ Syrinx shunts to the spinal subarachnoid space are likely to fail if the cerebrospinal fluid pathways rostral to the syrinx are blocked. To bypass obstructions at or below the level of the foramen magnum, a technique was developed for shunting the syrinx to the posterior fossa cisterns, termed “syringocisternostomy.” Syrinxes were shunted to the cisterna magna in two patients with spinal arachnoiditis and to the cerebellopontine angle cistern in four patients with Chiari I malformations. There was symptomatic improvement and collapse of the syrinx in each case, with no complications or recurrences over a follow-up interval of 14 to 27 months (average 20.3 months). The surgical technique and results of treatment are described.

Intraoperative anatomical studies in patients with aneurysms of the anterior communicating artery complex

1992 ◽  
Vol 76 (4) ◽  
pp. 629-634 ◽  
Author(s):  
Edgar Nathal ◽  
Nobuyuki Yasui ◽  
Takeshi Sampei ◽  
Akifumi Suzuki
Keyword(s):  
Surgical Approach ◽  
Radical Surgery ◽  
Anatomical Variations ◽  
Surgical Approaches ◽  
Anterior Communicating Artery ◽  
Anatomical Studies ◽  
Content Type ◽  
Number Of Patients ◽  
Vascular Elements ◽  
Fine Print

✓ The intraoperative anatomical findings of the anterior communicating artery (ACoA) complex in 46 patients with anatomical variations were compared to those in an equal number of patients without variations in order to determine the visualization of the elements of the vascular complex. All patients underwent radical surgery for an ACoA aneurysm by one of three different surgical approaches: transsylvian, anterior interhemispheric, or basal interhemispheric. Visualization of the vascular elements was similar in patients with or without anatomical variations. The differences observed were dependent on the surgical approach selected and on the projection of the aneurysm. It was found that, even when the intraoperative anatomical field and the number of vascular elements visualized are different from those obtained in autopsy studies, the vascular microanatomical characteristics can be confirmed with each surgical approach to the extent necessary to ensure safe clipping of aneurysms in patients both with and without anatomical variations.

Traumatic atlanto-occipital dislocation

1986 ◽  
Vol 65 (6) ◽  
pp. 863-870 ◽  
Author(s):  
Vincent C. Traynelis ◽  
Gary D. Marano ◽  
Ralph O. Dunker ◽  
Howard H. Kaufman
Keyword(s):  
Neurological Outcome ◽  
New Classification ◽  
Content Type ◽  
Long Term Stability ◽  
Transport Services ◽  
Number Of Patients ◽  
Emergency Transport ◽  
Serious Injury ◽  
Fine Print

✓ Traumatic atlanto-occipital dislocation is a serious injury that is usually fatal. The number of patients surviving this injury, however, appears to be increasing, and most of these survivors are children. This may reflect an improvement in emergency transport services. Seventeen previously reported cases of patients surviving atlanto-occipital dislocation for more than 48 hours are reviewed and an additional case is presented. Many of these patients had an excellent neurological outcome. The radiographic criteria necessary for the diagnosis of atlanto-occipital dislocation are discussed. Cervical computerized tomography may confirm the diagnosis when necessary. It is suggested that there are three types of atlanto-occipital dislocation; utilizing this new classification, a rationale for treatment is described. Fusion is favored for long-term stability.

Tonsillar herniation: the rule rather than the exception after lumboperitoneal shunting in the pediatric population

1993 ◽  
Vol 78 (4) ◽  
pp. 568-573 ◽  
Author(s):  
Paul D. Chumas ◽  
Derek C. Armstrong ◽  
James M. Drake ◽  
Abhaya V. Kulkarni ◽  
Harold J. Hoffman ◽  
...  
Keyword(s):  
Morphological Changes ◽  
Pediatric Population ◽  
Pseudotumor Cerebri ◽  
Foramen Magnum ◽  
Tonsillar Herniation ◽  
Content Type ◽  
Asymptomatic Patients ◽  
The Mean ◽  
Sick Children ◽  
Fine Print

✓ Although the development of tonsillar herniation (acquired Chiari malformation) in association with lumboperitoneal (LP) shunting is well recognized, it has previously been considered rare. In order to ascertain the incidence of this complication after LP shunting, the authors undertook a retrospective study of all patients in whom this form of shunt had been inserted between 1974 and 1991 at The Hospital for Sick Children, Toronto. In the 143 patients, the mean age at insertion was 3.3 years and the indications for shunt placement were hydrocephalus (81%), pseudotumor cerebri (7%), cerebrospinal fluid fistula (6%), and posterior fossa pseudomeningocele (6%). The mean follow-up period was 5.7 years, during which time there was one shunt-related death due to unsuspected tonsillar herniation. Five other patients developed symptomatic tonsillar herniation treated by suboccipital decompression. Review of all computerized tomography (CT) scans not degraded by artifact showed evidence of excess soft tissue at the level of the foramen magnum in 38 (70%) of 54 patients so studied. In order to confirm that this CT finding represented hindbrain herniation, sagittal and axial magnetic resonance (MR) images were obtained for 17 asymptomatic patients and revealed tonsillar herniation (range 2 to 21 mm) in 12 (70.6%). In addition, some of these asymptomatic patients had evidence of uncal herniation and mesencephalic distortion. Similarities and distinctions are drawn between the morphological changes occurring after LP shunting and those seen in association with the Chiari I and II malformations. Although less than 5% of this study population required treatment for tonsillar herniation, the incidence of this complication was high in asymptomatic patients; MR imaging surveillance for patients with LP shunts is therefore recommended.

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