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PubMed1991

Pathogenesis and treatment of delayed post-traumatic syringomyelia.

The role of trauma in the pathogenesis of syringomyelia has been known for a long time. Repeated microtraumata have been considered to play a role in triggering the classical syringomyelia on the basis of a congenital predisposition. It has been proven that post-traumatic arachnoiditis can cause cavitation in the spinal cord and probably syringomyelia stricto sensu as well. The delayed appearance of syringomyelia after a severe single spinal trauma resulting in contusion of the spinal cord without the complication of arachnoiditis is a more recent issue, but is now well-known. Delayed syringomyelia after a minor single spinal trauma, with at first complete recovery, is extremely rare, and is probably often disregarded as a diagnosis. Having studied such a case, with complete neuroradiological work-up, we want to draw attention to the pathogenetic aspects and the possibilities for treatment, of which the syringoperitoneal shunt seems to be the most efficient one.

Adult↗
PubMed1979

Terminal ventriculostomy in syringomyelia.

The authors report their experiences with two patients suffering from syringomyelia on whom terminal ventriculostomies were carried out. The good results which can be obtained if the patient is in a relatively good neurological condition are pointed out. The principle of terminal ventriculostomy is based on the assumption that syringomyelia is hydromyelia that has become symptomatic; with this in mind, it seems reasonable to relieve the patient's symptoms by draining the distended central canal at the lowest possible level. The father of the operation is Dr. Gardner, who has thoroughly studied syringomyelia, and proved that syringomyelia is not only an active hydromyelia but, that a "non-communicating" syringomyelia is a non-existent entity (1976, 1977).

Adult↗
PubMed2001

Postural tachycardia syndrome in syringomyelia: response to fludrocortisone and beta-blockers.

Orthostatic intolerance is occasionally reported by patients with syringomyelia and is usually attributed to vestibular symptoms or neurogenic orthostatic hypotension. Postural tachycardia syndrome has not been previously described in syringomyelia. A patient with long-standing syringomyelia and a Chiari type I anomaly developed disabling "panic-like" attacks associated to orthostatic intolerance five years after posterior fossa decompression and shunting of the syrinx. A head-up tilt test showed an early phase of postural orthostatic tachycardia followed by progressive arterial hypotension and bradycardia as seen in neurally mediated syncope. A magnetic resonance imaging scan showed a collapsed syrinx from the 3rd cervical to the 12th thoracic vertebra without syringobulbia. Fludrocortisone and beta-blockers led to resolution of symptoms. Partial sympathetic denervation of the legs in syringomyelia might explain the occasional occurrence of postural tachycardia syndrome. Postural tachycardia syndrome may be included as a possible cause of orthostatic symptoms in syringomyelia patients.

Adrenergic beta-Antagonists↗
PubMed1993

Familial syringomyelia: case report and review of the literature.

We report a case of familial syringomyelia consisting of two sisters, one of whom had syringomyelia and Chiari type I malformation and the other, who had died 5 years earlier, had syringomyelia and Klippel-Feil syndrome. Although syringomyelia is uncommon and familial cases rare, they continue to be reported from time to time. Review of the literature certainly suggests that genetic factors play a part in the etiopathogenesis of syringomyelia related to abnormalities of the hindbrain structures, although the number of cases is too small for the exact nature of transmission be established.

Adult↗
PubMed2005

Hindbrain-hernia-related syringomyelia without syringobulbia, complicated by permanent nocturnal central hypoventilation requiring non-invasive ventilation.

INTRODUCTION: In the absence of syringobulbia, hindbrain hernia does not commonly cause permanent respiratory complications. We present two patients who developed permanent central nocturnal hypoventilation following acute deterioration of hindbrain-hernia-related syringomyelia despite successful surgery. PATIENTS: Two children (one boy and one girl, aged 7 and 13 years, respectively) presented with acute neurological deterioration. The boy presented with a 6-week history of progressive tiredness and weakness and a short history of respiratory depression. The girl presented with a 2-year history of left hemiparesis and a short history of left hemiplegia and respiratory depression. On magnetic resonance scan, both had hindbrain hernia, hydrocephalus and cervical syringomyelia. Following cranio-vertebral decompression and, later, a ventriculoperitoneal shunt, the syringomyelia remained well controlled in both patients. In contrast, their respiratory depression improved minimally. Repeated multichannel respiratory monitoring revealed a persistently slow sleeping respiratory rate, with nocturnal hypercapnia and hypoxemia, managed successfully by night-time non-invasive ventilation. Neither the boy nor the girl, after 7 and 4 years, respectively, has developed any complications of nocturnal hypoventilation. When awake, both breathe normally. RESULT: Both children appear to have suffered a permanent impairment of respiratory control, resulting in nocturnal hypoventilation. This is usually seen with brain stem lesions and is unexpected when syringomyelia does not extend above C2, in the absence of syringobulbia. DISCUSSION: Central nocturnal hypoventilation is postulated to be due to permanent damage of central brain stem pathways controlling the respiratory muscles, due to local pressure at the cranio-cervical junction associated with acute deterioration of the hindbrain herniation. CONCLUSION: Sudden deterioration of hindbrain hernia and cervical syringomyelia can rarely impair the central mechanisms of respiratory control, with long-term implications on quality of life.

Adolescent↗
PubMed1997

Surgical treatment of syringomyelia associated with spinal dysraphism.

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.

Child↗
PubMed2002

Fluid flow in an animal model of post-traumatic syringomyelia.

More than a quarter of patients with spinal cord injury develop syringomyelia, often with progressive neurological deficit. Treatment options remain limited and long-term failure rates are high. The current poor understanding is impeding development of improved therapies. The source and route of fluid flow into syringes has been investigated using cerebrospinal fluid (CSF) tracers. Previous work using a model of canalicular syringomyelia has shown that fluid enters the dilated central canal from perivascular spaces. The aim of this study was to determine the source and route of fluid flow in an animal model of extracanalicular (post-traumatic) syringomyelia. A model of post-traumatic syringomyelia was established in 25 Sprague-Dawley rats with intraparenchymal injections of quisqualic acid and kaolin-induced arachnoiditis. Rats survived for 6 weeks before injection of the CSF tracer horseradish peroxidase into the cisterna magna. Examination of the spatial distribution of horseradish peroxidase at 0, 3, 5, 10, or 20 min after injection was used to determine the route of fluid flow. Horseradish peroxidase rapidly spread to the ventromedian fissure, perivascular spaces, central canal, and extracanalicular syrinx. Flow occurred into the syrinx prior to significant perivascular flow in the rostral spinal cord. Preferential flow into the syrinx occurred from the perivascular spaces of the central penetrating branches of the anterior spinal artery in the grey matter. Transparenchymal flow into the syrinx was less prominent than perivascular flow. This is the first report of fluid flow within the spinal cord in a model of post-traumatic syringomyelia. Fluid from perivascular spaces moves preferentially into extracanalicular syringes and the surrounding parenchyma. Obstruction to CSF flow and loss of compliance from traumatic arachnoiditis might potentiate fluid flow in the perivascular space.

Animals↗
PubMed2004

Syringomyelia with Chiari malformation; 3 unusual cases with implications for pathogenesis.

Syringomyelia is an important cause of neurological deficit. Most cases of non-traumatic syringomyelia occur in association with a Chiari malformation. We present three unusual examples of syringomyelia with such an association. The first case is that of syringomyelia in a young woman with Marfan's syndrome, a spontaneous CSF leak and intractable intracranial hypotension. The second is a woman with long-standing lumbo-peritoneal shunt for pseudotumour cerebri who developed an acquired Chiari malformation. A young woman with a Dandy-Walker cyst that herniated into the upper cervical canal is the third case. These cases provide a basis for discussion of the pathogenesis and management of syringomyelia and the Chiari malformation in such cases.

Adult↗
PubMed1995

Familial syringomyelia: case report and review of the literature.

BACKGROUND: Syringomyelia is an uncommon disease of the spinal cord, occurring sporadically. However, rare familial cases with autosomal dominant or recessive inheritance patterns are reported and their incidence quoted as approximately 2%. Only one previous report originated from the United States. METHODS: We present a brother and sister with syringomyelia and associated Chiari type I malformation; both patients responded to surgical treatment. We review the world literature and briefly discuss pathogenetic theories of syringomyelia as well as the relevance of the histocompatibility leukocyte antigen profile. RESULTS: Both genetic and environmental factors appear to be involved in familial syringomyelia. CONCLUSION: We recommend that close relatives of patients affected with familial syringomyelia undergo routine neurologic and radiologic surveys.

Adult↗
PubMedDate not supplied

CT myelography in communicating syringomyelia.

Although the etiology of syringomyelia is not clearly understood, many surgical methods have been proposed for its treatment. One widely used technique in cases of communicating syringomyelia is that of posterior fossa decompression and plugging of the obex (Gardner's Operation). In this paper we present five cases of syringomyelia which were investigated using detailed myelo-computerized tomographic techniques, of which two appeared to be communicating syringomyelia and which were treated by posterior fossa decompression and obex plugging. We also discuss the place of computed tomography in the differential diagnosis of communicating syringomyelia.

Adolescent↗
PubMed2005

Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis.

BACKGROUND: Syringomyelia is a common intramedullary lesion associated with spinal arachnoiditis and obstruction of the foramen magnum such as in Chiari's malformation. Disturbance of cerebrospinal fluid flow around the spinal cord has an important role in the development of syringomyelia due to spinal arachnoiditis; however, the exact mechanisms have not been clarified. The purpose of this retrospective study is to understand the clinical features and pathomechanisms of syringomyelia secondary to spinal arachnoiditis and to provide the current choice of surgical treatment in this difficult clinical entity. METHODS: Clinical and radiological findings in 15 patients with syringomyelia associated with spinal arachnoiditis who underwent surgical treatment in our institutes between 1982 and 2000 were reviewed. All patients presented with paraparesis or tetraparesis on admission. RESULTS: Magnetic resonance imaging (MRI) or computed tomography-myelography revealed that the syrinx predominantly existed at the thoracic levels. Five patients showed complete block of the thoracic subarachnoid space by conventional myelography. T2-weighted MRI showed diffuse intramedullary hyperintensity at the level of arachnoiditis. As the first surgical treatment, 10 patients underwent syringo-peritoneal shunt placement. Three patients were treated with a syringo-subarachnoid shunt, and 2 patients were treated with a ventriculoperitoneal shunt. Eight patients required further shunting operations for syringomyelia 2 months to 12 years after the first surgery. Neurologic improvement was obtained in 9 patients (60%) with decreased size of the syrinx. One patient remained stable; 5 patients showed gradual deterioration. CONCLUSIONS: The syrinx originated from the thoracic levels where severe adhesion of the subarachnoid space was present. The mechanisms of syrinx formation may be based on the increased interstitial fluid in the spinal cord. Shunting procedures were effective in some population of the patients. Decompression procedures of the spinal subarachnoid space may be an alternative primary surgical treatment except for patients with longitudinally extensive arachnoiditis.

Adult↗
PubMed1988

Treatment of syringomyelia with a syringosubarachnoid shunt.

The surgical results in 40 patients with syringomyelia, treated with a syringosubarachnoid shunt or other procedures are reviewed. The principal indication for surgery was that of significant neurological deterioration. There were 12 patients with idiopathic syringomyelia without tonsillar ectopia, 12 with an associated Chiari malformation, 11 with post-traumatic syringomyelia and five patients with spinal arachnoiditis. There were 38 syringosubarachnoid shunts performed in 35 patients, and an excellent or good result was achieved in 26 patients (74.3%). In terms of the type of syringomyelia, the best results were obtained in the idiopathic group without tonsillar ectopia and in the post-traumatic group. A short duration of pre-operative symptoms favoured a better outcome, and in our opinion, early surgical treatment is indicated for all patients with neurological deterioration. All eight patients in whom a posterior fossa decompression was performed as the initial surgical procedure required a second operation, either a syringosubarachnoid or syringoperitoneal shunt to achieve neurological improvement or stabilization. Thus, the syringosubarachnoid shunt is an effective therapeutic modality for patients with syringomyelia, particularly for the idiopathic and post-traumatic groups. More than one surgical procedure may be required to achieve cessation of deterioration. Overall, excellent or good results were achieved in 29 (72.5%) of the 40 patients.

Adult↗
PubMed2001

Generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia.

OBJECTIVES: To report the simultaneous occurrence of generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia. SETTING: Vienna, Austria. CASE DESCRIPTION: An 83-year old woman presented with predominantly lower limb and distal tetraparesis, diffuse wasting, spasticity of both lower limbs, contractures, and severe kyphoscoliosis. Radiographic examinations revealed generalised spinal cord atrophy, Chiari-I malformation, and syringomyelia between C5 and C7. The generalised spinal cord atrophy was assumed to be due to either secondary atrophy or spontaneous collapse of a formerly more extensive syringomyelia. All other causes for generalised spinal cord atrophy were excluded. CONCLUSIONS: Generalised spinal cord atrophy in a patient with Chiari-I malformation and syringomyelia may be due to either secondary atrophy or spontaneous collapse of a formerly holocord syringomyelia. Myelography and MRI imaging of the spine are helpful in diagnosing this condition.

Aged↗
PubMed1996

Syringomyelia associated with Hajdu-Cheney syndrome: case report.

OBJECTIVE AND IMPORTANCE: Hajdu-Cheney syndrome is a rare idiopathic bone disease based on a generalized bone dysplasia accompanied by acro-osteolysis. We describe a surgical case of this syndrome that was accompanied by neurological signs associated with cervical syringomyelia. CLINICAL PRESENTATION: A 41-year-old woman was referred to our hospital with mild quadriparesis and sensory disturbance resulting from a car accident. There was a neck injury. She showed almost all of the major characteristic clinical features and roentgenographic findings of Hajdu-Cheney syndrome with syringomyelia. INTERVENTION: Surgical treatment was indicated because of the progressive neurological deficits. Foramen magnum decompression and C1 laminectomy were performed, and the dura was exposed. The dura was opened at the area of the foramen magnum and C1. The occipitocervical posterior fusion was carried out with an iliac bone graft and titanium wires. CONCLUSION: Postoperatively, quadriparesis and sensory disturbance improved and the patient showed improved ambulation. Magnetic resonance imaging disclosed the well-decompressed foramen magnum. The syringomyelia disappeared in the segmental area of C2 and was decreased in the segmental areas of C5-T6. The treatment of this syndrome is symptomatic. In this patient, magnetic resonance imaging disclosed compression of the brain stem by basilar invagination and platybasia, disturbance of cerebrospinal fluid flow at the level of the foramen magnum, and syringomyelia. It was suspected that the obstruction of cerebrospinal fluid flow at the level of foramen magnum caused the cervical syringomyelia. However, the long-term prognosis remains uncertain. Follow-up is necessary to assess the final result of the treatment.

Adult↗
PubMed1998

Syringomyelia persistence after Chiari decompression as a result of pseudomeningocele formation: implications for syrinx pathogenesis: report of three cases.

OBJECTIVE AND IMPORTANCE: We present the cases of three patients in whom pseudomeningocele (PSM) formations after posterior fossa decompression of hindbrain herniations (Chiari I malformations) were associated with persistence of syringomyelia. The physiological importance of correcting this complication has not been previously described. CLINICAL PRESENTATION: We identified three patients who developed suboccipital PSMs after undergoing posterior fossa decompression for hindbrain herniations and syringomyelia. All three patients experienced persistence of their symptoms and their syringomyelia, despite adequate posterior fossa decompression. TECHNIQUE: Subsequent exploration revealed cerebrospinal fluid leaking either from the dural suture line (one patient) or from perforations found within the bed of a polyglactin mesh dural graft (two patients). Correction of the PSM resulted in resolution of both the syringomyelia and the symptoms in all three patients. DISCUSSION: The persistence of syringomyelia in the presence of a PSM may be the result of dissipation of the cerebrospinal fluid systolic pressure wave into the distensible PSM cavity. This phenomenon suggests that the cerebrospinal fluid pressure exerted on the spinal cord surface favors resolution of the syrinx cavity.

Adult↗
PubMed1996

Post-traumatic syringomyelia.

STUDY DESIGN: This study retrospectively analyzed patients who developed post-traumatic syringomyelia secondary to spinal cord injury. OBJECTIVES: To identify an indicator that would predict the outcome of surgical treatment for post-traumatic syringomyelia. SUMMARY OF BACKGROUND DATA: Syrinx-subarachnoid shunting was chosen as a surgical treatment for post-traumatic syringomyelia. No previous study has been published concerning magnetic resonance imaging findings' ability to predict surgical results before surgery. METHODS: Nine patients diagnosed by magnetic resonance imaging with post-traumatic syringomyelia were the subjects of this study. The magnetic resonance imaging findings and surgical results were analyzed. RESULTS: Neurologic deterioration was found in five patients. Ascending dissociated sensory disturbances and motor weakness were noticed to occur characteristically above the level of the spinal cord injury. The other four patients complained of a slight worsening of numbness without displaying neurologic deterioration. The five patients with neurologic deterioration were treated with a syrinx-subarachnoid shunting. Two of the five patients experienced sustained neurologic improvement after a midline myelotomy, which allowed the fluid within the syrinx to spout out and cause the expanded spinal cord to collapse. This was called a "high-pressure syrinx." In these two patients, the preoperative magnetic resonance imaging demonstrated a positive flow-void sign. On the other hand, drainage of the syrinx in the three patients with a negative flow-void sign did not collapse the expanded spinal cord, and the surgical results were considered fair. This was called a "low-pressure syrinx." CONCLUSIONS: Post-traumatic syringomyelia was classified into two types. A preoperative distinction could be made based on the presence or absence of the flow-void sign on a T2-weighted magnetic resonance image.

Adolescent↗
PubMed2001

The role of acute decompression and restoration of spinal alignment in the prevention of post-traumatic syringomyelia: case report and review of recent literature.

STUDY DESIGN: Case report. INTRODUCTION: Acute post-traumatic syringomyelia formation after spinal cord injury has been considered a rare complication. At this writing, most recent reports have surfaced in neurosurgical journals. As an entity, post-traumatic syringomyelia has not been widely appreciated. It has been confused with conditions such as Hansen's disease or ulnar nerve compression at the cubital tunnel. One study also demonstrated that the occurrence of syrinx is significantly correlated with spinal stenosis after treatment, and that an inadequate reduction of the spine may lead to the formation of syrinx. This reported case describes a patient in whom post-traumatic syringomyelia began to develop 3 weeks after injury, which improved neurologically after adequate decompression. SUMMARY OF BACKGROUND DATA: A 30-year-old man sustained a 20-foot fall at work. He presented with a complete spinal cord injury below T4 secondary to a T4 fracture dislocation. The patient underwent open reduction and internal fixation of T1-T8. After 3 weeks, the patient was noted to have ascending weakness in his bilateral upper extremities and some clawing of both hands. METHODS: A computed tomography myelogram demonstrated inability of contrast to pass through the T4-T5 region from a lumbar puncture. An incomplete reduction was noted. The canal showed significant stenosis. A magnetic resonance image of the patient's C-spine showed increased signal in the substance of the cord extending into the C1-C2 area. The patient returned to the operating room for T3-T5 decompressive laminectomy and posterolateral decompression including the pedicles, disc, and posterior aspect of the body. Intraoperative ultrasound monitoring showed a good flow of cerebrospinal fluid past the injured segment. RESULTS: On postoperative day 1, the clawing posture of the patient's hands was significantly diminished, and the patient noted an immediate improvement in his hand and arm strength. Over the next few days, the patient's strength in the bilateral upper extremities increased to motor Grade 4/5 on manual testing. A magnetic resonance image 4 weeks after decompression showed significant improvement in the cord diameter and signal. CONCLUSIONS: Post-traumatic syringomyelia has not been reported at so early a stage after injury. This disorder is an important clinical entity that must be recognized to prevent potentially fatal or devastating complications. As evidenced by the reported patient and the literature, if this disorder is discovered and treated early, permanent deficit can be avoided. The prevention of post-traumatic syringomyelia requires anatomic realignment and stabilization of the spine without stenosis, even in the case of complete injuries, to maintain the proper dynamics of cerebrospinal fluid flow.

Accidents, Occupational↗
PubMed2002

Chiari I malformation associated with syringomyelia and scoliosis: a twenty-year review of surgical and nonsurgical treatment in a pediatric population.

STUDY DESIGN: Retrospective review of patients with Chiari I malformation with or without associated scoliosis. OBJECTIVES: Determine the effect of decompression of Chiari I malformation with syringomyelia on stabilization or improvement of associated scoliosis. SUMMARY OF BACKGROUND DATA: Chiari malformations are often associated with spinal deformities, including scoliosis. Studies have suggested a causal relation between syringomyelia and scoliosis. METHODS: Patients with Chiari I malformation and syringomyelia with or without scoliosis treated over the last 20 years were reviewed. Patients with any other anomalies were excluded. Scoliotic curves were classified by magnitude and curve type. All patients were treated with surgical decompression of the Chiari malformation with or without drainage of the syringomyelia. RESULTS: Twenty-five patients were identified, ranging in age from 19 months to 16.5 years. Nineteen patients (76%) had associated scoliosis. The majority of the patients with scoliosis (13 of 19) sought treatment for spinal deformity, and only 6 had for pain or neurologic symptoms. Eleven of 19 patients with scoliosis (58%) underwent fusion. Eight of 19 (42%) patients have not undergone fusion: 3 have experienced progress, 1 remains in a stable condition, and 4 have experienced improvement of curvature since undergoing decompression. The mean age of patients who experienced progress after decompression was 14.5 years, compared to 6 years for patients who experienced improvement. CONCLUSION: Early decompression of Chiari I malformation with syringomyelia and scoliosis resulted in improvement or stabilization of the spinal deformity in 5 cases. Each of these patients underwent decompression before 8 years of age and before the curve was severe. However, this series represents a few patients demonstrating this trend, and further follow-up and investigation are warranted.

Adolescent↗