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PubMed1980

Evaluation of syringomyelia with metrizamide computed tomographic myelography.

Nineteen cases of cervical syringomyelia, five surgically confirmed, were examined with metrizamide computed tomographic myelography. While a collapsed cord appeared to be a very reliable finding in this series, those cases with distended cords can be studied with sequential scans (at 3 and 24 hr), allowing the distinction between syrinx and cystic intramedullary tumor by the presence or absence of delayed cyst opacification.

Adult↗
PubMed1997

Intramedullary pressure in syringomyelia: clinical and pathophysiological correlates of syrinx distension.

OBJECTIVE: The pathophysiological effects of syrinx distension are incompletely understood. Although it is generally assumed that the accumulation of fluid within syrinx cavities can contribute to neurological dysfunction, there are no reports describing intramedullary pressure in syringomyelia. The purpose of the current study was to measure syrinx pressures in patients with progressive clinical deterioration and to correlate these data with neurological deficits and intraoperative physiological findings. METHODS: Intramedullary fluid pressure was measured manometrically in 32 patients undergoing syrinx shunting procedures. The data were correlated with syrinx morphology, intraoperative somatosensory evoked potentials, laser Doppler measurements of local spinal cord blood flow (six patients), and neurological findings before and after syrinx decompression. RESULTS: Syrinx pressures recorded under atmospheric conditions ranged from 0.5 to 22.0 cm H2O (mean = 7.7 cm). There was a significant elevation of the cardiac pulse (mean = 0.7 cm H2O) and the respiratory pulse (mean = 1.1 cm H2O) that was consistent with raised cerebrospinal fluid pressure. Syrinx pressures decreased to subatmospheric levels after surgical drainage. In 18 of 24 patients with predrainage somatosensory evoked potential abnormalities, syrinx decompression produced a consistent reduction of N20 latencies (mean change = 0.49 ms +/- 0.094 SE right, P = 0.002; 0.61 ms +/- 0.089 SE left, P = 0.001) and a similar but less consistent increase in N20 amplitudes (mean change = 0.17 mV +/- 0.103 SE right, P = 0.115; 0.31 mV +/- 0.097 SE left, P = 0.027). Measurements of local spinal cord blood flow revealed very low baseline values (mean = 12.2 arbitrary units +/- 13.9 standard deviation), which increased to intermediate levels (mean = 144.7 arbitrary units +/- 42.6 standard deviation) after syrinx decompression. Patients with syrinx pressures greater than 7.7 cm H2O tended to have more rapidly progressive symptoms, exhibited greater improvements after shunting, and had a higher incidence of postoperative dysesthetic pain. CONCLUSION: The current study is the first to measure intramedullary pressure in a human disease. Evidence is presented that distended syringes are associated with varying levels of raised intramedullary pressure that can accentuate or induce neurological dysfunction by the compression of long tracts, neurons, and the microcirculation. Symptoms referrable to raised intramedullary pressure are potentially reversible by syrinx decompression.

Adolescent↗
PubMed2000

Reevaluation of syringosubarachnoid shunt for syringomyelia with Chiari malformation.

OBJECTIVE: The purpose of this study was to evaluate the effectiveness of syringosubarachnoid (S-S) shunting for syringomyelia with Chiari malformation. The authors describe the technical methods of performing the S-S shunt and the clinical results, including shunt malfunction. METHODS: Forty-nine patients underwent S-S shunting. These patients were divided into three groups according to differences in the surgical technique used. Group I patients underwent laminectomy plus midline myelotomy and had a shunt tube placed in the dorsal subarachnoid space. Group II patients underwent laminectomy plus dorsal root entry zone myelotomy and had a shunt tube placed in the dorsolateral subarachnoid space. Group III patients underwent hemilaminectomy plus dorsal root entry zone myelotomy and had a shunt tube placed in the ventrolateral subarachnoid space. RESULTS: Clinical results were generally satisfactory, especially in terms of pain relief, in all three groups. However, 10 patients among Groups I and II required follow-up surgery because of shunt problems; no second surgery was necessary for any patient in Group III. CONCLUSION: The S-S shunt was very effective in deflating the syrinx, and the clinical results were satisfactory. Therefore, even though foramen magnum decompression is a very effective treatment, S-S shunting should be reevaluated and not rejected; it should be considered as one of the major surgical options. To prevent the possibility of cord injury by myelotomy or shunt malfunction, the dorsal root entry zone should be selected as the myelotomy site, and the shunt tube should be inserted into the ventral subarachnoid space at the cervical level.

Adolescent↗
PubMed1996

Unilateral blindness due to patient positioning during cervical syringomyelia surgery: unilateral blindness after prone position.

During spinal surgery using a horseshoe headrest with the patient in the prone position, the possibility of central retinal artery occlusion (CRAO) increases, and its cause can be attributed primarily to excessive extraocular pressure, a very rare complication. This report describes a case of CRAO, occurring in an adult, after cervical syringomyelia surgery in which a horseshoe headrest was used.

Adult↗
PubMed2003

Scoliotic curve patterns in patients with Chiari I malformation and/or syringomyelia.

STUDY DESIGN: A retrospective radiographic review was performed on 41 patients with scoliosis associated with a Chiari I malformation and/or syringomyelia. OBJECTIVES: To characterize curve patterns and curve features in this population and possibly refine the radiographic indications for magnetic resonance imaging in patients with a normal history and physical examination. SUMMARY OF BACKGROUND DATA: A subset of patients with "idiopathic" scoliosis may have an underlying neurologic abnormality. The radiographic indications for magnetic resonance imaging in asymptomatic patients with a normal clinical examination are not well defined. METHODS: Data were collected from standing posteroanterior and lateral radiographs. The curve pattern and specific curve features were recorded and compared with historic controls. Thoracic kyphosis and total lumbar lordosis were also measured. RESULTS: Fifty-one percent of patients were male. Ten curve patterns were identified, and, based on our criteria, approximately 50% of patients had an "atypical" pattern (left thoracic, double thoracic, triple, long right thoracic). A subset of those with "typical" patterns (right thoracic, right thoracic/left lumbar) had atypical features including a superior or inferior shift of the apex and/or the upper or lower end vertebrae. The mean kyphosis (T3-T12) was 41.8 degrees. CONCLUSIONS: Although the decision to obtain magnetic resonance imaging in a patient with scoliosis should be based on both clinical and radiographic criteria, we suggest that a heightened index of suspicion is warranted with certain curve patterns (left thoracic, double thoracic, triple, and a long right thoracic curve with end vertebra caudal to T12), and with a high or low apex and/or end vertebra, especially in males and patients with a normal to hyperkyphotic thoracic spine.

Adolescent↗
PubMed2005

Post-traumatic syringomyelia: a review of the cases presenting in a regional spinal injuries unit in the north east of England over a 5-year period.

STUDY DESIGN: Retrospective study of the management of patients with a confirmed diagnosis of post-traumatic syringomyelia (PTS) in a regional spinal injuries unit. OBJECTIVES: To investigate the incidence of PTS in a regional spinal injuries unit, and to assess mode of presentation, management, and subsequent outcome. SUMMARY OF BACKGROUND DATA: Majority of patients male, mean age 44 years (range 33-60 years), with thoracic spinal cord injury (SCI). Most PTS developed within 5 years after injury (range 6 months to 25 years) and presented with reduced sensation. Fifty percent had surgical intervention at the time of SCI, and 50% managed conservatively. Four patients had additional spinal injury pathology not corrected at time of surgery. METHODS: Retrospective analysis of the case notes of all patients with a confirmed diagnosis of PTS (n = 16). Demographic details obtained and details of the original injury and subsequent clinical course and management noted. RESULTS: Incidence of PTS = 0.02%. Magnetic resonance imaging scanning performed in 56% with PTS. The majority of PTS developed around the site of the original lesion. The most common method of management was insertion of a syringoperitoneal shunt (44%). Thirty-one percent improved after surgery, 31% remain stable. One patient died. The symptoms of 3 patients continue to deteriorate slowly despite surgical intervention. CONCLUSIONS: Incidence of PTS are lower in our study than that quoted in the literature. Benefits of initial surgical management of SCI in reducing development of PTS are unclear. Benefits of surgical management of PTS are unclear. Additional studies are required as this may influence future management of spinal cord injured patients.

Adult↗
PubMedDate not supplied

Syringomyelia and scoliosis: a review of twenty-five pediatric patients.

A retrospective review of 25 patients with scoliosis secondary to syringomyelia was conducted. All patients had large syrinxes, but only 10 patients had abnormal neurologic findings, suggesting that subtle neurologic findings in patients with scoliosis should not be underestimated and supporting liberal use of magnetic resonance imaging (MRI). The results of three approaches to the care of these patients were reviewed: passive observation, bracing, and spinal fusion. Curves progressed > or = 5 degrees in patients aged < 10 years who were observed, but in children aged > 10 years, curves progressed > or = 10 degrees. Curves responded well to bracing, but correction was not maintained out of brace for patients with progressive and/or severe curves. Good corrective was achieved safely during operation.

Adolescent↗
PubMed1990

Syringomyelia and syringobulbia: pathophysiology, surgical treatment and nursing implications.

Syringomyelia (central cavitation of the spinal cord) and syringobulbia (cavitation of the medulla) are relatively rare disorders. These conditions are often found in association with congenital abnormalities such as Chiari malformations, with neoplasms or as sequelae to spinal cord trauma. The pathology progresses from minor sensory changes to weakness and wasting and, in the case of syringobulbia, to respiratory compromise and even death. Surgical treatment involves drainage or decompression of the syrinx cavity with diversion of fluid to the subarachnoid space or peritoneal cavity. Posterior fossa decompression may also be used. These procedures attempt to halt destruction of the nervous system, but will not reverse damage that has already occurred. A case presentation and associated nursing diagnoses are discussed to illustrate current medical and nursing interventions.

Adult↗
PubMed1989

MRI--the investigation of choice in syringomyelia?

During a 12 month period of operation of a 0.3 Tesla MRI iron cored resistive scanner 74 cases of syringomyelia were diagnosed on clinical, radiological and/or surgical grounds. Without knowledge of any clinical or radiological data the syrinxes were classified into five groups--idiopathic, idiopathic associated with Chiari malformation, tumour associated, post-traumatic and arachnoiditis associated--and the lesion characteristics within each group were compared. Although MRI was extremely sensitive in picking up even small syrinxes, there was considerable overlap of MRI characteristics across the sub-groups, so that two post-traumatic syrinxes had lesion characteristics identifiable with those of tumour syrinx and one intramedullary tumour syrinx had the MRI characteristics of a benign, idiopathic syrinx. It is concluded that meticulous attention to technique, including axial as well as sagittal T1 weighted sequences, and the administration of intravenous paramagnetic contrast media are necessary for detection and accurate classification of syrinxes.

Adult↗
PubMed1980

Syringomyelia in pigs.

Syringomyelia was present in 3 of 6 pigs examined in an outbreak of disease in which a respiratory and an ataxic syndrome were recognized. Lesions were found in cervical spinal cord segments in pigs 3 to 5 months old. The aetiology was not determined.

Animals↗
PubMed2003

Pressure wave propagation in fluid-filled co-axial elastic tubes. Part 2: Mechanisms for the pathogenesis of syringomyelia.

Our aim in this paper is to use a simple theoretical model of the intraspinal cerebrospinal-fluid system to investigate mechanisms proposed for the pathogenesis of syringomyelia. The model is based on an inviscid theory for the propagation of pressure waves in co-axial, fluid-filled, elastic tubes. According to this model, the leading edge of a pressure pulse tends to steepen and form an elastic jump, as it propagates up the intraspinal cerebrospinal-fluid system. We show that when an elastic jump is incident on a stenosis of the spinal subarachnoid space, it reflects to form a transient, localized region of high pressure within the spinal cord that for a cough-induced pulse is estimated to be 50 to 70 mm Hg or more above the normal level in the spinal subarachnoid space. We propose this as a new mechanism whereby pressure pulses created by coughing or sneezing can generate syrinxes. We also use the same analysis to investigate Williams' suck mechanism. Our results do not support his concept, nor, in cases where the stenosis is severe, the differential-pressure-propagation mechanism recently proposed by Greitz et al. Our analysis does provide some support for the piston mechanism recently proposed by Oldfield et al. and Heiss et al. For instance, it shows clearly how the spinal cord is compressed by the formation of elastic jumps over part of the cardiac cycle. What appears to be absent for this piston mechanism is any means whereby the elastic jumps can be focused (e.g., by reflecting from a stenosis) to form a transient, localized region of high pressure within the spinal cord. Thus it would seem to offer a mechanism for syrinx progression, but not for its formation.

Animals↗
PubMed1977

Three cases of communication syringomyelia secondary to midbrain gliomas.

Three cases of midbrain gliomas are descrbied clinically and pathologically. In each case high pressure symptoms were followed by visual disturbance and the onset of syringomyelia symptoms before death. All the patients had hydrocephalus. In one case with concomitant syringobulbia, the syrinx appeared to due to CSF communicating with the cord cavity through the tissues of the brain stem. In the other cases the communication between the CSF pathways and the syrinx was at the usual site, through the central canal at the obex.

Adolescent↗
PubMed1981

Clinical features, investigation and treatment of post-traumatic syringomyelia.

Thirteen patients who sustained spinal cord trauma causing persisting disability, developed new symptoms, the chief one of which was severe pain unrelieved by analgesics. The clinical diagnosis of post traumatic syringomyelia was confirmed in each case by means of myelography, as well as endomyelography in seven patients. In every case exploration of the spinal cord syrinx was performed. Ten patients were troubled by severe pain while three patients were mainly subject to altered sensation in the upper limbs. Of the six patients who had initially sustained complete cord transections, three were treated by cord transection and three were treated by syringostomy. The seven patients who sustained incomplete cord lesions were all treated by syringostomy. The patients who initially sustained incomplete sensory motor spinal cord damage had a better symptomatic response to surgery than hose who had sustained a complete spinal cord lesion. The ten patients whose main symptom was severe pain were completely relieved of their symptoms by surgery.

Adult↗
PubMed1981

Syringomyelia and its surgical treatment--an analysis of 75 patients.

A consecutive series of 75 patients with syringomyelia is presented, all of whom were treated by cranio-vertebral operations. Attention is drawn to the difficulty in assessing the results of treatment but 56 stabilised or showed modest improvement after surgery. Occluding the central canal appeared to have no greater influence on the progression of the disease than did simple decompression and did have a higher incidence of complications. Upper motor neurone weakness, joint position sense and central neck pain are the features most likely to improve and it is concluded that relieving the medullary compression resulting from a Chiari type 1 malformation, rather than influencing the syrinx, is the means by which this may occur. Simple decompression with preservation of the arachnoid membrane, combined with syringostomy in certain cases, is recommended.

Adolescent↗