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PubMed2003

Hyperkyphosis as an indicator of syringomyelia in idiopathic scoliosis: a case report.

STUDY DESIGN: A case of thoracic hyperkyphosis is reported as an atypical presentation of otherwise typical adolescent idiopathic scoliosis that showed a large syringomyelia on magnetic resonance imaging. OBJECTIVE To review atypical curve patterns that indicate the need for more intensive preoperative workup including a magnetic resonance imaging scan. SUMMARY OF BACKGROUND DATA: The literature on atypical curve patterns of adolescent "non-idiopathic" scoliosis is reviewed. No report in the literature describes patients with an idiopathic coronal plane and thoracic hyperkyphosis as an atypical feature found to have a large syringomyelia and Chiari I malformation on magnetic resonance imaging examination. METHODS: The clinical and radiographic features associated with thoracic hyperkyphosis and juvenile and adolescent scoliosis with syringomyelia are presented. RESULTS: The neurosurgical treatment of the syringomyelia subsequently led to a potentially safer spinal fusion. The patient tolerated the procedures well and at this writing has remained asymptomatic during 3 years of follow-up evaluation. CONCLUSIONS: The reported case adds to the hyperkyphosis literature category of unusual spinal deformities seen in scoliosis associated with syringomyelia and should further help to categorize adolescent idiopathic scoliosis as a diagnosis of exclusion.

Acute Disease↗
PubMed2004

Clinical and neuroimaging features of "idiopathic" syringomyelia.

In some adult patients with cervical syringomyelia, MRI studies do not identify primary disease within the foramen magnum or spinal canal. To identify the etiology of this idiopathic type of syringomyelia, clinical features and posterior fossa (PF) measurements from 17 of these patients, 17 patients with Chiari I-type syringomyelia, and 32 control subjects were compared. Idiopathic syringomyelia and Chiari I-type syringomyelia manifested central cervical myelopathy and a small PF with narrow CSF spaces, suggesting that they develop by the same mechanism.

Adult↗
PubMed1994

Posttraumatic syringomyelia: its characteristic magnetic resonance imaging findings and surgical management.

Posttraumatic syringomyelia should be considered in any patient showing a delayed neurological deterioration after spinal cord injury. The purpose of this article is to assess the posttraumatic syringomyelia on magnetic resonance images and to evaluate the results of its surgical treatment. Fourteen patients with posttraumatic syringomyelia were studied. There were 10 men and 4 women ranging in age from 25 to 67 years. Eleven patients with syringomyelia were symptomatic, and the three others were asymptomatic. The periods from spinal cord injury to onset of symptoms due to syringomyelia ranged from 3 to 33 years with a mean of 14 years. On magnetic resonance images, the mean length of the syrinx was 14 vertebral levels and ranged from 3 to 20 levels. At the rostral part of the injured cord, the syrinx was located off center. In contrast, at the caudal part of the injury, the syrinx was markedly large in size and its location was central. In 6 of 13 patients, the syringes extended into the medulla oblongata. Eleven symptomatic patients underwent surgical treatments: a syringosubarachnoid shunt was done in six patients, a syringoperitoneal shunt was done in four, and a ventriculoperitoneal shunt was carried out in one. Shunt malfunction was encountered in three of the four syringoperitoneal shunts and in the one ventriculoperitoneal shunt. Final neurological outcomes were satisfactory in all 11 patients who underwent surgery. Motor function improved in eight of nine patients, sensory disturbance improved in five of five patients, and relief of local pain or numbness was obtained in four of four patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed2003

Acute syringomyelia: case report.

OBJECTIVE AND IMPORTANCE: Syringomyelia is generally regarded as a chronic, slowly progressive disorder. We describe a case of acute dilation of the central canal of the spinal cord that presented with rapidly progressive segmental signs. CLINICAL PRESENTATION: A 29-year-old female patient who had previously undergone surgical treatment for a Chiari I malformation, syringomyelia, and hydrocephalus presented with an 8-day history of headaches, progressive paraparesis, and urinary retention. Magnetic resonance imaging scans demonstrated panventricular hydrocephalus in association with a holocord syrinx that extended to the obex. Magnetic resonance imaging scans that had been coincidentally obtained just 3 days before the onset of symptoms had revealed no evidence of hydrocephalus or syringomyelia. INTERVENTION: The patient underwent emergency revision of a failed ventriculoperitoneal shunt. Postoperatively, there was prompt resolution of the syringomyelia, hydrocephalus, and associated neurological deficits. CONCLUSION: Among patients with communicating syringomyelia, the central canal of the spinal cord participates as a "fifth ventricle" and can undergo rapid dilation in association with acute hydrocephalus. Appropriate treatment in such cases involves placement of a ventriculoperitoneal shunt.

Acute Disease↗
PubMed1998

Clinical and neuroimaging features of Chiari type I malformations with and without associated syringomyelia.

The clinical and neuroimaging characteristics of 22 consecutive patients with Chiari type I malformations were evaluated to investigate the pathogenesis of syrinx formation. All patients underwent magnetic resonance imaging and x-ray tomography before surgery. The electric manometric Queckenstedt test was performed on 16 patients pre- and postoperatively. Syringomyelia was present in 17 patients and absent in five patients. All patients without syringomyelia suffered from foramen magnum compression syndrome, with a wider basal angle, more acute clivo-axial angle, shorter clivus, and more prominent tonsillar ectopia than patients with syringomyelia. Low brain stem position, basilar impression, and beaking of the cervicomedullary junction were also more prominent in patients without syringomyelia. Marked to complete block with the neck in flexed position by Queckenstedt test was present in all patients except one. Patients with Chiari malformation not associated with syringomyelia have more pronounced compression of the brain stem at the foramen magnum. Therefore, despite a block of the cerebrospinal fluid pathway at the foramen magnum, syrinx formation may be prevented by severe compression.

Adolescent↗
PubMed2000

A genetic hypothesis for Chiari I malformation with or without syringomyelia.

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.

Arnold-Chiari Malformation↗
PubMed2000

Toward a rational treatment of Chiari I malformation and syringomyelia.

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 subarachnoid 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.

Adolescent↗
PubMed1982

Favorable results with syringosubarachnoid shunts for treatment of syringomyelia.

From 1969 to 1979, 20 patients with syringomyelia were treated with a syringosubarachnoid shunt. The principal indications for this procedure were: significant progressive neurological deterioration and absent or minimal tonsillar ectopia. There were 15 patients with idiopathic syringomyelia, four with posttraumatic syringomyelia, and one with syringomyelia secondary to spinal arachnoiditis. The operations were performed with an operating microscope, and attention was directed to preserving thearachnoid membrane to ensure proper placement of the distal end of the shunt in an intact subarachnoid space. In all cases, a silicone rubber ventricular catheter was inserted into the syrinx through a posterior midline myelotomy. The average follow-up period was 5 years. A favorable result was obtained in 15 of the 20 patients (75%), including an excellent result with improvement of neurological deficit in 11 patients and a good result with cessation of progression in four patients. In the remaining five patients the result was poor with further progression of neurological deficit. A short duration of preoperative symptoms was usually a favorable prognostic feature. Four patients with a history of less than 6 months all had excellent results. Thirteen patients had a syringosubarachnoid shunt only, and all had good or excellent results. Seven patients had other surgical procedures, before, accompanying, or after shunt placement, and two had favorable results. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for many patients with syringomyelia, particularly if there is little or no tonsillar herniation.

Adult↗
PubMed1994

Pathophysiology of syringomyelia associated with Chiari I malformation of the cerebellar tonsils. Implications for diagnosis and treatment.

The mechanisms previously proposed for the progression of syringomyelia associated with Chiari I malformation of the cerebellar tonsils are controversial, leave many clinical observations unexplained, and underlie the prevalence of different operations currently used as initial treatment. To explore the mechanism of syringomyelia progression in this setting, the authors used anatomical and dynamic (phase-contrast and phase-contrast cine) magnetic resonance (MR) imaging, and intraoperative ultrasonography to examine the anatomy and dynamics of movement of the cerebellar tonsils, the wall of the spinal cord surrounding the syrinx, and the movement of cerebrospinal fluid (CSF) and syrinx fluid at rest, during the respiratory and cardiac cycles, and during Valsalva maneuver in seven affected patients. In all patients the cerebellar tonsils occluded the subarachnoid space at the level of the foramen magnum. Syringomyelia extended from the cervical to the lower thoracic segment of the spinal cord. No patient had evidence of a patent communication between the fourth ventricle and the syrinx on anatomical MR images, dynamic MR images, or intraoperative ultrasound studies. Dynamic MR images of three patients revealed abrupt downward movement of the spinal CSF and the syrinx fluid during systole and upward movement during diastole, but limited movement of CSF across the foramen magnum during the cardiac cycle. Intraoperative ultrasound studies demonstrated abrupt downward movement of the cerebellar tonsils during systole that was synchronous with sudden constriction of the spinal cord and syrinx. Decompression of the foramen magnum was achieved via suboccipital craniectomy, laminectomy of C-1 and C-2, and dural grafting, leaving the arachnoid intact. Immediately after surgery, the pulsatile downward thrust of the tonsils and constriction of the spinal cord and syrinx disappeared. Syringomyelia resolved within 1 to 6 months after surgery in all patients. Observations by the authors suggest the following previously unrecognized mechanism for progression of syringomyelia associated with occlusion of the subarachnoid space at the foramen magnum. The brain expands as it fills with blood during systole, imparting a systolic pressure wave to the intracranial CSF that is accommodated in normal subjects by sudden movement of CSF from the basal cisterns to the upper portion of the spinal canal. With obstruction to rapid movement of CSF at the foramen magnum, the cerebellar tonsils, which plug the subarachnoid space posteriorly, move downward with each systolic pulse, acting as a piston on the partially isolated spinal CSF and producing a systolic pressure wave in the spinal CSF that acts on the surface of the spinal cord.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗
PubMed1998

Chiari Type I malformation and syringomyelia in unrelated patients with blepharophimosis. Report of two cases.

Syringomyelia is a rare, mainly sporadic disease of the spinal cord, which is associated with 80% of cases in which a Chiari Type I malformation is also present. A mendelian transmission of syringomyelia (autosomal dominant or recessive) has been proposed in approximately 2% of reported cases. The association of syringomyelia with hereditary diseases (Noonan's syndrome, phacomatoses) has been mentioned frequently in the literature. The authors report the presence of a Chiari Type I malformation accompanied by syringomyelia in two unrelated patients affected by a familial Type II blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). The first patient was a 35-year-old woman who presented with a right C-8 root paresia. The second case involved a 20-year-old man who complained of cervical radicular pain. Both belong to families in which BPES was segregated in an autosomal dominant modality, but other family members had no known neurological symptoms. To the authors' knowledge, such a combination has never been described. Perhaps the possible involvement of a genetic component in some cases of Chiari Type I-associated syringomyelia will someday be debated.

Adult↗
PubMed2006

Spontaneous resolution of syringomyelia and Chiari malformation Type I in a patient with cerebrospinal fluid otorrhea. Case report.

The spontaneous resolution of syringomyelia in the setting of a Chiari malformation Type I (CM-I) has been reported infrequently. Several theories about the pathogenesis and spontaneous resolution of syringomyelia associated with CM-I have been proposed. The authors present the case of a patient with spontaneous resolution of a CM-I and syringomyelia coinciding with the development of cerebrospinal fluid (CSF) otorrhea. Although cases of spontaneous resolution of syringomyelia have been reported, this is the first reported case of spontaneous resolution of syringomyelia and a CM-I associated with the simultaneous development of CSF otorrhea.

Adult↗
PubMed2004

Syringomyelia complicating myelomeningocele: review of the evidence.

Syringomyelia is frequently found in association with myelomeningocele. Although often asymptomatic, it can, in exceptional cases, cause severe morbidity. The author performed a structured literature review to address the following aspects of this clinical problem. What is the natural history? There are data concerning the imaging prevalence, the autopsy prevalence, and the prevalence of clinically active syringomyelia among patients with myelomeningocele, but literature provides no description of the course of this condition over time. What is the clinical significance? That correlations among symptoms, signs, treatments, and imaging findings are poorly described is no surprise in view of the large fraction of patients with syringomyelia who are asymptomatic. There is, however, an impressive mass of anecdotal evidence associating progressive syringomyelia with untreated (or inadequately treated) hydrocephalus. How to make the diagnosis? There is no disagreement that magnetic resonance imaging is the diagnostic modality of choice, but the literature provides very little guidance about who should undergo this investigation. There is no analysis of the costs and benefits of screening or periodic surveillance. What is the best treatment? Many surgical procedures have been reported to be useful as components of complex algorithms relating clinical factors and imaging data to treatment recommendations. There have been no controlled studies. There have been no prospective studies. There have been no multiinstitutional studies. No studies in which outcomes have been evaluated using objective, validated instruments. No studies in which the robustness of treatment effects over time have been documented. The complexity of treatment selection in contemporary practice makes syringomyelia unsuitable for randomized controlled trials of different therapies. Prospective cohort studies are feasible, however, and hold the potential to address many important questions about natural history and patient outcomes.

Adolescent↗
PubMed2002

Syringomyelia associated with foramen magnum arachnoiditis.

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.

Adult↗
PubMedDate not supplied

Gardner's hydrodynamic theory of syringomyelia revisited.

Several theories have been put forth to explain the pathogenesis of syringomyelia, the formation of longitudinal, fluid-filled cavities within the spinal cord. Chief among them is Gardner's hydrodynamic theory, widely accepted for more than two decades. Gardner attributed the genesis of syringomyelia to craniospinal pressure differentials in the setting of fourth ventricular outlet obstruction; these differentials favor cerebrospinal fluid shifts from the fourth ventricle of the brain through the central canal of the spinal cord. Gardner's theory has been questioned, and several alternative theories of syringomyelia have been proposed. Physiological data and new information from magnetic resonance imaging support many of Gardner's concepts; however, a more comprehensive elucidation of the pathophysiologic mechanisms of syringomyelia requires incorporating facets of the other theories. We propose a unified theory of the pathogenesis of syringomyelia based on recent experience with magnetic resonance imaging, and on elements of other current theories.

Cerebrospinal Fluid Pressure↗
PubMed1999

[Treatment of syringomyelia].

Treatment of syringomyelia utilizes two operative approaches: posterior fossa decompression and syrinx shunting (including subarachnoid, pleural and peritoneal shunting). MRI study is nowadays the indispensable tool for the evaluation of patients with syringomyelia and allows to choose the best therapeutic option. Posterior fossa decompression is regarded as the procedure of choice for syringomyelia with Chiari. After intradural exploration, additional steps may be necessary as excision of the cerebellar tonsils. Other therapeutic alternatives are associated with higher complication rates. Patients with persistent focal syrinxes after PFD respond best to syringoperitoneal shunts. For the management of post-traumatic syringomyelia, a large decompressive laminectomy at the fracture site is recommended; the use of a drain does not offer any long-term therapeutic advantage. Syringoperitoneal shunting is the treatment of post-infection syringomyelia but good long-term result is rare in this type of syrinx.

Budd-Chiari Syndrome↗
PubMed2000

[Posttraumatic syringomyelia in 2 patients with thoracic spinal cord lesions].

Two patients, men aged 42 and 40 years, developed new neurological symptoms 3 months and 22 years, respectively, after a traumatic high thoracic spinal cord injury. The MRI scan showed a cavity in the central part of the spinal cord, on which the diagnosis of 'posttraumatic syringomyelia' could be based. In one of the patients a syringo-subarachnoidal shunt was created, the other was treated conservatively because of a severe concomitant thoracic kyphosis. Posttraumatic syringomyelia is a potentially life-threathening late complication of spinal cord injury and is characterized by development of new neurological symptoms after a variable time interval. The most typical symptom of non-traumatic syringomyelia, viz. diminution of vital sensitivity without loss of gnostic sensitivity, is not necessarily present in posttraumatic syringomyelia. Surgical treatment of posttraumatic syringomyelia is advocated if there is progressive neurological deterioration, and consists of drainage of the syrinx.

Adult↗
PubMed2001

[The role of magnetic resonance tomography in diagnosis of syringomyelia].

The paper presents the analysis of the results of the examination of 90 patients with syringomyelia using magnetic-resonance "Bruker" tomograph with the intensity of the magnetic field 0.23 T. The observation was carried out according to the authors' classification. Symptom complex of syringomyelia corresponding to the anatomic disorders of the spinal cord was described. The authors made a conclusion, that the magnetic-resonance tomography, as the most informative method, was a method of choice in syringomyelia. In 21% of the patients there was idiopathic syringomyelia; in 47.3% a process spread to the caudal regions of medulla oblongata. In 79% cases syringomyelia manifested as a syndrome; in 50% of the total cases it was found in Arnold--Chiari anomaly.

Arnold-Chiari Malformation↗
PubMed1992

[The pattern of neurological deterioration and the mechanism of neurological deficit in syringomyelia].

Sixty-five cases of syringomyelia were evaluated. The cases were classified into two groups: group 1 (56 cases) was patients who presented with hind-brain related syringomyelia and group 2 (9 cases) was patients who presented with primary spinal syringomyelia. Group 1 was further divided into two subgroups, group 1a and group 1b: group 1a (46 cases) consisted of patients with hind-brain related syringomyelia without basal arachnoiditis and group 1b (10 cases) consisted of patients with hind-brain related syringomyelia with basal arachnoiditis. The most common initial symptom of group 1a patients was abnormal motor function of an upper limb (14 cases), followed by pain in an upper limb (12 cases), and dissociated sensory loss (10 cases). In group 1b, motor symptoms of an upper limb were also the most common initial symptom, again followed by pain in an upper limb. Paraplegia was the most common initial symptom in group 2. About 80% of patients in groups 1a and 1b had both sensory and motor deficits at the time of examination and the majority of group 1b patients also had brain stem signs and/or pain. Brain stem signs were not commonly seen in group 1a patients, however. The neurologic deficits of group 1b patients were generally more severe than those of group 1a patients. Most group 2 patients also had sensory and motor deficits of both lower limbs. The progression of neurological deficits in groups 1a and 1b was classified into four stages.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗