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PubMed1991

Foramen magnum decompression for syringomyelia associated with basilar impression and Chiari I malformation--report of three cases.

Anterior or posterior decompression of the foramen magnum was performed in three patients with syringomyelia associated with basilar impression and Chiari I malformation. The operative results were evaluated using the pre- and postoperative magnetic resonance (MR) images. Two patients with combined anterior and posterior cervicomedullary compression due to basilar impression and tonsillar descent received suboccipital craniectomy, upper cervical laminectomy, and dural plasty without any intradural manipulations via the posterior approach. One patient with prominent anterior cervicomedullary compression due to basilar impression and a sharp clivoaxial angle was operated on by the transoral anterior approach. Postoperatively, all patients showed a sustained shrinkage of the syrinx and rounding of the flattened cerebellar tonsils. Two patients showed upward movement of the herniated tonsils. All patients had improved symptoms during 2-4 years follow-up. Treatment of syringomyelia associated with basilar impression and Chiari I malformation requires more efficient decompressive procedures at the foramen magnum based on neurological and MR findings.

Adult↗
PubMed2005

Craniovertebral junction realignment for the treatment of basilar invagination with syringomyelia: preliminary report of 12 cases.

Twelve selected patients, eight males and four females aged 14 to 50 years, with syringomyelia associated with congenital craniovertebral bony anomalies including basilar invagination and fixed atlantoaxial dislocation, and associated Chiari I malformation in eight, were treated by atlantoaxial joint manipulation and restoration of the craniovertebral region alignment between October 2002 and March 2004. Three patients had a history of trauma prior to the onset of symptoms. Spastic quadriparesis and ataxia were the most prominent symptoms. The mean duration of symptoms was 11 months. The atlantoaxial dislocation and basilar invagination were reduced by manual distraction of the facets of the atlas and axis, stabilization by placement of bone graft and metal spacers within the joint, and direct atlantoaxial fixation using an inter-articular plate and screw method technique. Following surgery all patients showed symptomatic improvement and restoration of craniovertebral alignment during follow up from 3 to 20 months (mean 7 months). Radiological improvement of the syrinx could not be evaluated as stainless steel metal plates, screws, and spacers were used for fixation. Manipulation of the atlantoaxial joints and restoring the anatomical craniovertebral alignments in selected cases of syringomyelia leads to remarkable and sustained clinical recovery, and is probably the optimum surgical treatment.

Adolescent↗
PubMed1988

Syringomyelia as a consequence of compressive extramedullary lesions: postoperative clinical and radiological manifestations.

MR imaging was performed to determine the cause of the onset of new neurologic symptoms in five patients who had previously undergone surgical excision of extramedullary masses. Syringomyelia and the absence of recurrent or residual lesions were documented in all cases. Three patients showed long cysts (multiseptated in two and smooth in one) with low signal intensity on both T1- and T2-weighted images. The flow-void phenomenon related to fluid motion in these three cysts, which were enlarging clinically, was responsible for the hypointensity on the T2-weighted images. In two patients the fluid within the lesions behaved similarly to normal nonpulsatile CSF and may have represented syrinx cavities in a state of "arrested growth." Three patients had surgical decompression under real-time intraoperative sonographic control, which showed the presence of intramedullary cyst-fluid pulsations in two cases and the absence of cyst-fluid pulsations in one case. These sonographic observations correlated with the MR findings. We postulate that these syrinx cavities form as a result both of the effect that the original extramedullary lesion had upon the underlying spinal cord and the subsequent postoperative alterations in the CSF dynamics at the level of prior surgery. Syringomyelia should be considered in patients with recurrent or new symptoms who previously had surgery for extramedullary lesions.

Adult↗
PubMed2005

Hindbrain decompression in a dog with scoliosis associated with syringomyelia.

A 6-month-old female Border Collie was examined because of a 1-month history of progressive curvature of the cervical portion of the vertebral column. Radiography revealed severe cervical and thoracic scoliosis. Cervical syringomyelia and hydrocephalus were observed by means of magnetic resonance imaging. Suboccipital craniotomy and laminectomy of the first cervical vertebra were performed, and substantial improvement in the scoliosis and syringomyelia was observed 3 months after surgery. No recurrences were seen during the first year after surgery.

Animals↗
PubMed1987

Syringomyelia of the thoracic spinal cord associated with spinal meningiomas.

Syringomyelia is known to occur secondary to compression of the spinal cord. We report the case of a female patient who underwent removal of a spinal meningioma. She re-presented 30 years later with multiple meningiomas causing cord compression. After a 4 year interval she was found to have developed a syrinx proximal to the site of compression. Comparison with previous case reports suggest that the causation of syringomyelia is multi-factorial.

Adult↗
PubMed1979

A late neurologic complication of scoliosis surgery in connection with syringomyelia.

A case of scoliosis in connection with syringomyelia is described. Theories are proposed to explain the progression of the neurological symptoms after surgical correction and fusion of the deformity. Special points are emphasized that will aid in the recognition of syringomyelia in scoliosis patients. i) Abnormal neurology, in particular a dissociated disturbance of pain and temperature in the upper extremity. ii) Abnormal localization of a scoliosis curve. iii) Rapid progression of the scoliosis. iv) Bony anomalies of the upper cervical spine. v) Increased diameter of the cervical spinal canal.

Adolescent↗
PubMed2000

Classification of syringomyelia.

Syringomyelia poses special challenges for the clinician because of its complex symptomatology, uncertain pathogenesis, and multiple options of treatment. The purpose of this study was to classify intramedullary cavities according to their most salient pathological and clinical features. Pathological findings obtained in 175 individuals with tubular cavitations of the spinal cord were correlated with clinical and magnetic resonance (MR) imaging findings in a database of 927 patients. A classification system was developed in which the morbid anatomy, cause, and pathogenesis of these lesions are emphasized. The use of a disease-based classification of syringomyelia facilitates diagnosis and the interpretation of MR imaging findings and provides a guide to treatment.

Adolescent↗
PubMed2000

Pain and syringomyelia: a review.

The pathophysiological basis of chronic pain syndromes remains poorly defined. Central and dysesthetic pain are probably the most disabling of sensory disturbances associated with syringomyelia, and, unfortunately, effective treatment remains elusive. In this paper, the authors review their institutional experience with both clinical and laboratory studies of patients with syringomyelia, and they review the relevant literature. To date, there is no consensus as to the best treatment for central cord pain syndromes, although there are many promising areas of current research involving the use of neurochemicals in the spinal cord.

Animals↗
PubMed2000

Primary spinal syringomyelia: a personal perspective.

In this paper the author summarizes currently available surgical approaches to spinal syringomyelia that is unrelated to Chiari I malformation or hindbrain descent. Primary spinal syringomyelia is most commonly associated with spinal trauma but is also encountered as a sequela to intradural inflammatory processes (infections or chemical), as a delayed response to surgical procedures, and in association with intra- and extradural neoplasms as well as disc protrusions. The advantages of placing a shunt are its technical simplicity and immediate reduction of syrinx size; its major disadvantages are the high rate of failure observed in long-term follow up and the difficulty in applying this technique in septated cysts. Expansion of the subarachnoid space with resection of scars has better long-term results. Patients in whom a syrinx cavity has caused a kyphotic spinal deformity may need to undergo a procedure in which the kyphotic deformity is corrected to expand the subarachnoid space. Cyst obliteration is an experimental approach that cannot be evaluated at the present time.

Cerebrospinal Fluid Shunts↗
PubMed2005

Arachnoid cyst resulting in tonsillar herniation and syringomyelia in a patient with achondroplasia. Case report.

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.

Achondroplasia↗
PubMed1976

Combined isotope ventriculography and lumbar myelocisternography in the diagnosis of communicating syringomyelia. Case report.

Several investigative procedures are currently used to diagnose communicating syringomyelia. The combination of isotope ventriculography and lumbar myelocisternography has never proposed nor carried out to verify this condition. We feel that this is a safe and valuable procedure to identify a patent central canal communicating with the fourth ventricle and is worth considering when diagnosing communicating syringomyelia.

Adult↗
PubMed1977

Syringomyelia following lumboureteral shunting for communicating hydrocephalus. Report of three cases.

Three patients are described in whom syringomyelia was identified long after the treatment of communicating hydrocephalus by a lumboureteral shunt. The reason for syrinx formation in these cases could not be determined. In two there was either autopsy-proven or presumed evidence for arachnoiditis, and in the third patient the symptoms of syringomyelia were acutely aggravated by temporary obstruction of shunt. The development of a pressure drop from the intracranial compartment to the spinal compartment with crowding at the foramen magnum is also a suggested mechanism.

Adolescent↗
PubMed1980

Experimental syringomyelia: the relationship between intraventricular and intrasyrinx pressures.

The influence of ventricular pressure changes on syrinx pressure was investigated in this study of experimental canine kaolin-induced syringomyelia. The pressures of the ventricles, syrinx, and cervical subarachnoid space were measured. A complete ventriculosubarachnoid block occurred in the animals with syringomyelia. The baseline syringeal pressures exceeded those of both the ventricles and the subarachnoid space. Raising ventricular pressure elevated the syringeal pressure, but aspiration of ventricular fluid did not acutely lower the pressure. These findings suggest a ventriculosyrinx valve effect that may inflate the syrinx during transient rises of intracranial pressure. A respiratory pressure pattern was found in the syrinx similar to that of the ventricles and subarachnoid space. This wave was reduced but not abolished by ligating the subarachnoid space distal to the syrinx. The arterial pulse was much diminished within the syrinx at rest. These findings indirectly support the possibility that transmission of thoracic pressures to the spinal subarachnoid space with compression of the syrinx is a principal force that enlarges the syrinx.

Animals↗
PubMed1982

Considerations in the diagnosis and treatment of syringomyelia and the Chiari malformation.

A series of patients with abnormalities of the craniocervical junction with and without syringomyelia is reviewed. The impact of computerized tomography scanning on current radiological evaluation is discussed. Air myelography may no longer be necessary for accurate diagnosis. While the results of surgery for the Chiari malformation were good, at least one-half of the patients with syringomyelia showed continued progression of symptoms after posterior fossa surgery. More effective surgical therapy requires a better understanding of the pathogenesis of the syrinx.

Adolescent↗
PubMed1990

Acute presentations of syringomyelia. Report of three cases.

Three unusual cases are reported in which communicating syringomyelia presented acutely. The first patient presented with paraplegia, the second with acute respiratory distress secondary to bilateral vocal cord paralysis, and the third with symptoms of acute brain-stem ischemia. Each patient had a communicating spinal cord syrinx associated with a posterior fossa and foramen magnum region anomaly (a huge posterior fossa arachnoid cyst in one and Chiari malformations in two). The mechanisms of craniospinal pressure dissociation and hindbrain herniation are discussed, along with other reported emergency presentations of syringomyelia.

Acute Disease↗
PubMed1993

Noncommunicating syringomyelia following occlusion of central canal in rats. Experimental model and histological findings.

This report describes a new and reliable technique for producing experimental noncommunicating syringomyelia. In 30 rats, 1.2 to 1.6 microliters of kaolin was microinjected into the dorsal columns and central gray matter of the spinal cord at C-6. The inoculations caused transient neurological deficits in four animals and no deficits in 26 animals. Within 24 hours, kaolin and polymorphonuclear leukocytes entered the central canal and drained rostrally. The clearance of inflammatory products induced a proliferation of ependymal cells and periependymal fibrous astrocytes, which formed synechiae and obstructed the canal at the level of injection and at one or more levels up to C-1. In 22 animals followed for 48 hours or longer, the upper end of the central canal became acutely dilated and formed an ependyma-lined syrinx that enlarged to massive dimensions within 6 weeks. The rostral syrinxes did not communicate with the fourth ventricle and were not associated with hydrocephalus. The histological findings in acute noncommunicating syringomyelia were characterized by progressive stretching and thinning of the ependyma, elongation of intracanalicular septae, and the formation of periependymal edema. After 3 weeks, there was progressive compression of the periependymal tissues associated with stretching of axons, fragmentation of myelin sheaths, and the formation of myelin droplets. These findings and the sequence in which they evolved were identical in most respects to those occurring in acute and subacute noncommunicating hydrocephalus.

Animals↗
PubMed1993

Spontaneous drainage of syringomyelia. Report of two cases.

Two cases are reported of Arnold-Chiari type I malformation associated with syringomyelia, in which magnetic resonance (MR) imaging revealed spontaneous decompression of the syrinx. In one case axial MR imaging sections showed a communication between the syrinx and the spinal subarachnoid space, which supports the hypothesis that fissuring of the cord parenchyma is instrumental in the spontaneous resolution of syringomyelia. The MR imaging changes were not accompanied by variations in the patients' clinical course.

Adult↗
PubMed1994

Experimental model of posttraumatic syringomyelia: the role of adhesive arachnoiditis in syrinx formation.

An experimental model was devised to elucidate the role of spinal blockade in posttraumatic syringomyelia. Thirty-eight Japanese White rabbits, each weighing about 3 kg, were used in this study. The animals were divided into four groups: in Group 1, eight animals received traumatic injury only; in Group 2, 12 animals received traumatic injury following injection of 100 mg kaolin suspended in 1 cc normal saline solution into the subarachnoid space at the site of trauma; in Group 3, nine animals received traumatic injury following injection of 200 mg kaolin in 1 cc normal saline solution into the subarachnoid space at the site of trauma; and in Group 4, nine animals without traumatic injury received an injection of 200 mg kaolin in 1 cc normal saline solution into the subarachnoid space. The subjective criteria for syrinx formation were the presence of a definite round cyst having a smooth margin and an upper or lower extension of more than 2 cm from the injured site. Syrinx formation was seen in 12.5% (one of eight rabbits) in Group 1, 41.7% (five of 12 animals) in Group 2, 55.5% (five of nine rabbits) in Group 3 and 0% (none of nine animals) in Group 4 (p < 0.05). There was a tendency for the combined trauma/kaolin injection groups to be more prone to develop a syrinx. In the kaolin injection only group (Group 4), no animal showed a definite cyst or an extending cavity during the experimental period. The results suggest that kaolin enhances the extension of multiple small cavities that have already formed at the time of initial injury. The difference between the frequency of syrinx formation and the time of survival was statistically significant well beyond the 0.05% level. The overall difference, relating to the frequency of syrinx development, group, and duration of survival, was also statistically significant. In summary, subarachnoid block secondary to adhesive arachnoiditis is important in initiating the extension of the syringomyelia cavity.

Animals↗