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PubMedDate not supplied

Syringomyelia and developmental scoliosis.

We attempted to characterize the signs and symptoms of patients presenting with scoliosis as a manifestation of occult syringomyelia, and to determine the effect of syrinx decompression on the deformity and neurologic deficits. This study represented 21 patients treated from 1981 through 1991 at the authors' institution. All patients with occult syringomyelia and no other central nervous system lesions underwent decompression of the syrinx, with improvement in neurologic signs and symptoms. Three of the four with scoliosis that were not treated by arthrodesis showed improvement of their deformity at an average follow-up of 35 months. Three of five patients with myelodysplasia, occult syringomyelia, and scoliosis not previously treated with spinal arthrodesis showed stabilization or improvement of their deformity at an average follow-up of 21 months. We conclude that syringomyelia is associated with a high incidence of developmental scoliosis, and that decompression of the syrinx leads to improvement in, or stabilization of, the majority of scoliotic curves, or postpones the need for fusion.

Adolescent↗
PubMed2006

Evidence of familial syringomyelia in discordant association with Chiari type I malformation.

We report a sister and two half brothers who presented with magnetic resonance imaging (MRI)-proven syringomyelia and associated Chiari type I malformation in two cases. The individuals have the same mother but two different fathers. The mother shows no clinical signs of syringomyelia. The two fathers died through unknown causes. In a third healthy son of the mother by a relationship with a third father syringomyelia was excluded by MRI. We believe that an autosomal-dominant predisposition is the primary factor in the appearance of syringomyelia in these cases.

Adult↗
PubMed2001

Generalized intestinal dysmotility in a patient with syringomyelia.

Syringomyelia is a rare degenerative disorder of the spinal cord that causes progressive neurological dysfunction. Patients with syringomyelia have a variety of gastrointestinal symptoms although there are limited data on the frequency or the severity of these complaints. In this case report we describe a 65-yr-old man with syringomyelia and gastrointestinal dysmotility at multiple levels as documented by esophageal motility study, gastric emptying scan, antroduodenal motility study, and anorectal manometry. The patient's nausea, recurrent vomiting, and fecal incontinence were treated successfully with dietary changes, a promotility agent, and a bowel training regimen. This report highlights the need for more objective studies of gastrointestinal motility in patients with syringomyelia.

Aged↗
PubMed2005

Syringomyelia hydrodynamics: an in vitro study based on in vivo measurements.

A simplified in vitro model of the spinal canal, based on in vivo magnetic resonance imaging, was used to examine the hydrodynamics of the human spinal cord and subarachnoid space with syringomyelia. In vivo magnetic resonance imaging (MRI) measurements of subarachnoid (SAS) geometry and cerebrospinal fluid velocity were acquired in a patient with syringomyelia and used to aid in the in vitro model design and experiment. The in vitro model contained a fluid-filled coaxial elastic tube to represent a syrinx. A computer controlled pulsatile pump was used to subject the in vitro model to a CSF flow waveform representative of that measured in vivo. Fluid velocity was measured at three axial locations within the in vitro model using the same MRI scanner as the patient study. Pressure and syrinx wall motion measurements were conducted external to the MR scanner using the same model and flow input. Transducers measured unsteady pressure both in the SAS and intra-syrinx at four axial locations in the model A laser Doppler vibrometer recorded the syrinx wall motion at 18 axial locations and three polar positions. Results indicated that the peak-to-peak amplitude of the SAS flow waveform in vivo was approximately tenfold that of the syrinx and in phase (SAS approximately 5.2 +/- 0.6 ml/s, syrinx approximately 0.5 +/- 0.3 ml/s). The in vitro flow waveform approximated the in vivo peak-to-peak magnitude (SAS approximately 4.6 +/- 0.2 ml/s, syrinx approximately 0.4 +/- 0.3 ml/s). Peak-to-peak in vitro pressure variation in both the SAS and syrinx was approximately 6 mm Hg. Syrinx pressure waveform lead the SAS pressure waveform by approximately 40 ms. Syrinx pressure was found to be less than the SAS for approximately 200 ms during the 860-ms flow cycle. Unsteady pulse wave velocity in the syrinx was computed to be a maximum of approximately 25 m/s. LDV measurements indicated that spinal cord wall motion was nonaxisymmetric with a maximum displacement of approximately 140 microm, which is below the resolution limit of MRI. Agreement between in vivo and in vitro MR measurements demonstrates that the hydrodynamics in the fluid filled coaxial elastic tube system are similar to those present in a single patient with syringomyelia. The presented in vitro study of spinal cord wall motion, and complex unsteady pressure and flow environment within the syrinx and SAS, provides insight into the complex biomechanical forces present in syringomyelia.

Adult↗
PubMed1981

Some observations on the pathogenesis of syringomyelia.

The pathogenesis of most cases of syringomyelia remains obscure although a modification of the hydrodynamic theory of Gardner allows a logical surgical approach to treatment. Data are presented confirming a high incidence of traumatic birth in patients with syringomyelia who have a Chiari malformation or basal arachnoiditis, but demonstrating no increase in traumatic birth in patients with the Chiari malformation but no syringomyelia. A traumatic birth may be the factor responsible for creating the potential for syringomyelia in those individuals with the embryological defect of the Chiari anomaly.

Adolescent↗
PubMedDate not supplied

Asymptomatic syringomyelia in the course of medulloblastoma.

Syringomyelia is frequently associated with Chiari malformation or one of many other pathological conditions. Its co-occurrence with medulloblastoma is rare, and to our knowledge, only 4 patients have been reported, although some reports have documented on syringomyelia associated with intracranial processes or intramedullary tumor. The authors describe an unusual case of asymptomatic thoracic syringomyelia complicated by an intrasyringal hemorrhage in a child with medulloblastoma. This report illustrates that, although unusual, syringomyelia is a potential complication in the natural history of medulloblastoma, and the authors consider the possible pathogenesis of syrinx enlargement.

Cerebellar Neoplasms↗
PubMed1995

Syringomyelia of the distal spinal cord in children.

The syndrome of syringomyelia in children has been extensively described in the literature and is said to most commonly involve the cervical and thoracic spinal cord. We here present two children who had an unusual constellation of signs and symptoms, characterized by bowel and bladder disturbance in one, and in the other with intermittent paroxysmal severe pain and cramping and flexion of the lower extremities accompanied at times by episodes of incontinence. Both were found on magnetic resonance imaging (MRI) to have a syrinx localized to the most distal cord. This was not associated with Arnold-Chiari malformation, trauma, tumors or any other form of spinal dysraphism. Both patients underwent placement of a syringomyelia-peritoneal shunt with complete resolution of symptomatology. Postoperative MRI revealed a complete collapse of the syringomyelia cavity. In those children presenting with bowel or bladder disturbances, associated or not with intermittent pain, flexion attacks, or cramping in the lower extremities, the differential diagnosis of a syringomyelia of the distal cord should be part of the clinical considerations.

Child, Preschool↗
PubMed1999

Involuntary movements and abnormal spontaneous EMG activity in syringomyelia and syringobulbia.

OBJECTIVE: To describe different types of involuntary movements and abnormal spontaneous electromyographic (EMG) activity in patients with syringomyelia. BACKGROUND: A comprehensive study on involuntary movements in patients with syringomyelia has not yet been undertaken, to these authors' knowledge. METHODS: One hundred adult patients with syringomyelia were examined over the last 15 years. Involuntary movements were videotaped and evaluated by two independent observers. Electromyographic recordings were made using bipolar surface electrodes. The H-reflex recovery curve was obtained after stimulation of the median nerve at the elbow and recording from the flexor carpi radialis. RESULTS: Involuntary movements or abnormal postures were observed in 22 patients. Three patients showed segmental spinal myoclonus, nine minipolymyoclonus, and four propriospinal myoclonus. Five patients had unilateral or bilateral hand postural tremor (8-10 Hz). Focal or segmental dystonia was observed in three patients. Electromyography showed spontaneous bursts of grouped action potentials synchronous in muscles innervated by the same spinal segment, synchronous firing of neurogenic motor unit potentials, or continuous motor unit activity. Increased H-reflex responses to conditioning stimuli were found in patients with spinal myoclonus. Long latency responses were obtained during peripheral nerve stimulation in four patients. Four patients had rigidity and abnormal upper limb posture. Respiratory synkinesis was observed in three patients. One patient developed inverse masticatory muscle activity. CONCLUSIONS: Patients with syringomyelia showed a wide spectrum of involuntary movements. An increased excitability of spinal motor neurons was probably the basic underlying mechanism.

Adolescent↗
PubMed1999

Respiratory disturbances during sleep in syringomyelia and syringobulbia.

OBJECTIVE: To determine the frequency and types of abnormalities of respiratory control during sleep in syringomyelia and syringobulbia and to provide a basis to predict patients at risk of sudden death. METHODS: Thirty patients (15 male and 15 female; mean age 39.0 +/- 12.6 years) with communicating syringomyelia were divided into two groups: those with evidence of syringobulbia (17 patients) and those without compromise of the medulla or syringomyelia (13 patients). Patients were studied with pulmonary function studies and polysomnography. Respiratory center sensitivity to CO2 (rebreathing technique) was measured in 9 patients. RESULTS: Severely affected patients had mild-to-moderate restriction and individual patients had bilateral diaphragmatic or vocal cord palsy, abnormal respiratory rhythm, prolonged inspiratory time, or an abnormal respiratory response to CO2. Very prolonged central, obstructive, and mixed sleep apneas with low O2 saturation values and a fixed heart rate were recorded in most patients with syringobulbia. Five patients developed severe respiratory complications and died during a follow-up period of 10 years. Respiratory abnormalities failed to correlate with syrinx size. CONCLUSIONS: Severe abnormalities in respiratory rhythm generation during sleep occur in patients with syringobulbia. The respiratory disturbances are not due to muscle weakness and they are not correlated with the size of the cavity. The combination of dysphagia and dysphonia in patients with longstanding syringomyelia and syringobulbia predicted likelihood of respiratory disturbances during sleep.

Adolescent↗
PubMed1985

Magnetic resonance imaging in syringomyelia.

Myelography and myelography assisted with computed tomography have been the most commonly used radiographic methods in the study of syringomyelia. These studies have never been entirely reliable in demonstrating the syrinx cavity and its relationship to other intracranial structures. During the 1st year of operation of the magnetic resonance imaging facility, the syringomyelic cavity was demonstrated in 15 patients who all had typical clinical signs and symptoms associated with syringomyelia. Nine cases were syringomyelia with Chiari malformation. One case showed additional hydrocephalus. Four cases were idiopathic, and 1 case was remotely posttraumatic. Magnetic resonance imaging, although it is in its infancy, already promises to be the most important radiographic technique for syringomyelia because it provides an anatomically truthful visualization of the sagittal plane of the cervical cord and can demonstrate the syrinx cavity and its relationship with the cerebellar tonsils, the 4th ventricle, and other related structures.

Adolescent↗
PubMed1993

Anatomical basis of syringomyelia occurring with hindbrain lesions.

Hindbrain lesions that distort or compress the cervicomedullary junction are commonly associated with syringomyelia. As a basis for discussing pathogenetic mechanisms, the upper end of the central canal of the spinal cord was examined histologically in six aborted fetuses and 14 adults dying of natural causes; the results were correlated with magnetic resonance images in 40 normal subjects. The central canal of the medulla, which extends from the cervicomedullary junction to the fourth ventricle, was found to migrate dorsally, elongate in dorsoventral diameter, and dilate beneath the tip of the obex to form a large, everted aperture. This opening communicates directly with the subarachnoid space through the foramen of Magendie and is indirectly continuous with the main body of the fourth ventricle. In adults, the aperture of the central canal is located approximately 1.0 cm below the tela choroidea inferior and 3.5 cm below the midpoint of the fourth ventricle. Analysis of magnetic resonance imaging scans in 45 patients with syringomyelia and simple hindbrain lesions revealed two patterns of cavity formation: 1) lesions that obstructed the upper end of the central canal or its continuity with the subarachnoid space produced a noncommunicating type of syringomyelia; and 2) lesions that obstructed the basilar cisterns or the foraminal outlets of the fourth ventricle produced a communicating type of syringomyelia (hydromyelia) in association with hydrocephalus. Evidence is presented that syrinxes occurring with hindbrain lesions are not caused by a caudal flow of cerebrospinal fluid from the fourth ventricle into the central canal of the spinal cord.

Aged↗
PubMed2001

Complete spontaneous resolution of childhood Chiari I malformation and associated syringomyelia.

The diagnosis of Chiari I malformation and associated syringomyelia is often made in childhood. Since the advent of magnetic resonance imaging, these abnormalities are increasingly detected incidentally. Despite incomplete understanding of the natural history of asymptomatic Chiari I malformations, the current recommendation is to consider prophylactic surgical intervention in those with an associated syringomyelia. This case report presents a complete spontaneous resolution of a Chiari I malformation and syringomyelia in a child. It illustrates the possibility that asymptomatic children with Chiari I malformations and syringomyelia may be followed conservatively.

Adolescent↗
PubMed1999

Effect of cerebrospinal fluid shunting on experimental syringomyelia: magnetic resonance imaging and histological findings.

The histological changes associated with syringomyelia after reduction of the syrinx size were investigated after cerebrospinal fluid shunting in experimental syringomyelia in the rabbit. Five weeks after syringomyelia was induced by the injection of kaolin into the cisterna magna in Japanese white rabbits, ventriculosubgaleal shunting or syringoepidural shunting were performed. After 1 week magnetic resonance (MR) imaging and histological examination were then carried out. Five of 11 shunted animals showed postoperative reduction of syrinx size on MR imaging. Grossly, some specimens showed cavity collapse and parenchymal healing, and others showed a small residual syrinx in the dorsal horn. The most dramatic histological changes occurred in the gray matter. Specimens with syrinx collapse showed rarefaction and tearing of the gray matter, with mild glial reaction. The edematous gray matter showed both degeneration and regeneration, with neuronal processes surrounded by edema fluid. Reactive astrocytes were observed mainly at the margin of the residual syrinx. Some astrocytic processes invested the extraaxonal space and gray matter lacked supportive tissue. Greater reduction of the syrinx after shunting operation was correlated with more regeneration and less degeneration, and the white matter was edematous and histological changes were milder. Syrinx shrinkage occurred after shunting in this experimental model of syringomyelia. The selective vulnerability of gray matter even after shunting may explain discrepancies between imaging findings and clinical features in this disease. The study supports the potential benefit from early treatment, considering the associated morphological findings of regeneration.

Animals↗
PubMed1987

Surgical treatment of syringomyelia with syringopleural shunting.

The clinical course of 21 patients who underwent syringopleural shunting for syringomyelia is summarised. All the patients were continuing to deteriorate at the time of the operation. Objective improvement was seen in nine patients following the procedure but three subsequently deteriorated. Contralateral syrinx symptoms have appeared in two patients. No change was seen in six patients who did not deteriorate later. Three were worse following the procedure. In syringomyelia with marked hydrocephalus, ventricular drainage by a valved shunt may be the preferred first procedure. Craniovertebral decompression is recommended for syringomyelia with hindbrain herniation without dense arachnoiditis. In appropriate cases syringopleural shunting may be performed in combination with craniovertebral decompression, and may be the procedure of choice in cases with marked hindbrain arachnoiditis. In cases with a sizeable syrinx who have subsequently deteriorated following craniovertebral decompression, syringopleural shunting may be considered the preferred second procedure. Syringopleural shunting is suggested in amenable cases of syringomyelia associated with spinal tumour, trauma or arachnoiditis.

Adult↗
PubMed1984

Surgical treatment of syringomyelia. Favorable results with syringoperitoneal shunting.

The authors reviewed the clinical findings, radiological evaluation, and operative therapy of 39 patients with syringomyelia. Syringoperitoneal (SP) shunting was used in 15 patients and other procedures were used in 24 patients. Follow-up periods ranged from 1 1/2 to 12 years. During the period of this study, metrizamide myelography in conjunction with early and delayed computerized tomography scanning replaced all other diagnostic procedures in patients with syringomyelia. Preoperative accuracy for the two procedures was 87%. The most common symptoms were weakness (79%), sensory loss (67%), pain (38%), and leg stiffness (28%). Surgery was most effective in stabilizing or alleviating pain (100%), sensory loss (81%), and weakness (74%); spasticity, headache, and bowel or bladder dysfunction were less likely to be reversed. Approximately 80% of patients with idiopathic and posttraumatic syringomyelia and 70% of those with arachnoiditis improved or stabilized. Better results were obtained in patients with less severe neurological deficits, suggesting the need for early operative intervention. A higher percentage of patients had neurological improvement with SP shunting than with any other procedure, especially when SP shunting was the first operation performed. Patients treated with SP shunts also had the highest complication rate, most often shunt malfunction. These results indicate that SP shunting is effective in reversing or arresting neurological deterioration in patients with syringomyelia.

Adolescent↗
PubMed1995

A critical appraisal of drainage in syringomyelia.

The use of drains in the treatment of syringomyelia has a simple and immediate appeal and has been practiced widely since the report of Abbe and Coley over 100 years ago. Good short-term results have been claimed in the past, but long-term outcome is largely unknown. An experience in Birmingham, England is reviewed in which 73 patients who had had some form of syrinx drainage procedure performed were subsequently followed up. In these cases, a total of 56 syringopleural and 14 syringosubarachnoid shunts had been inserted. Ten years after the operations, only 53.5% and 50% of the patients, respectively, continued to remain clinically stable. A 15.7% complication rate was recorded, including fatal hemorrhage, infection, and displacement of the drain from the pleural and syrinx cavities. At second operation or necropsy, at least 5% of shunts were discovered to be blocked. The effect of other drainage procedures that do not use artificial tubing, such as syringotomy and terminal ventriculostomy, was analyzed but found not to offer any substantial benefit. These results indicate that drainage procedures are not an effective solution to remedying the progressive, destructive nature of syringomyelia. It is suggested that, rather than attempting to drain the syrinx cavity, disabling the filling mechanism of the syrinx is more appropriate. Most forms of syringomyelia have a blockage at the level of the foramen magnum or in the subarachnoid space of the spine. Surgical measures that aim to reconstruct the continuity of the subarachnoid space at the site of the block are strongly recommended. Lowering the overall pressure of the cerebrospinal fluid is advocated when reestablishment of the pathways proves impossible. Syrinx drainage as an adjuvant to more physiological surgery may have a place in the treatment of syringomyelia. If two procedures are done at the same time, however, it is difficult to ascribe with certainty a success or failure, and it is suggested that the drainage procedure be reserved for a later attempt if the elective first operation fails.

Adolescent↗
PubMed2004

Persistent syringomyelia following pediatric Chiari I decompression: radiological and surgical findings.

OBJECT: The incidence of syringomyelia in patients with Chiari I malformation is reported to be between 20 and 75%. In the series reported herein 10.6% of patients with both clinical entities have continued to have a significant syrinx following their first decompressive procedure. All but one patient had resolution of their syrinx after a second posterior fossa operation. The authors have analyzed this smaller group of patients to look for possible radiological or surgical findings that may aid in predicting which patients are less likely to respond to the initial first decompressive procedure. METHODS: The authors retrospectively reviewed radiological and operative data in eight patients who continued to have syringomyelia following a decompressive procedure. Seven (88%) of these patients had complete resolution of their syrinx following a second operation. At repeated operation, obstruction at the foramen of Magendie was seen in six patients. In one patient in whom the dura was not opened during first operation, the second operation revealed an arachnoid veil that occluded the foramen of Magendie. CONCLUSIONS: No single radiological measurement was found to aid in the prediction of which patients would not respond to the first decompressive procedure. Furthermore, no operative finding was extraordinarily unique to any single patient. All but one patient in whom confirmation of a patent foramen of Magendie was made at repeated operation-that is, lysing of arachnoid veils, stent placement, unilateral tonsillar coagulation-had resolution of their syringomyelia. Surgical reexploration should be considered in cases of persistent syringomyelia.

Adolescent↗
PubMed2005

Progressive syringomyelia controlled by treatment of associated hydrocephalus in an infant with birth injury. Case report.

The author's aim in reporting this case is to extend awareness of the importance of management of associated hydrocephalus among patients with syringomyelia to the posttraumatic state. A full-term infant was delivered by cesarean section because of transverse lie. In the 2nd week of life, hypotonia affecting the lower limbs and the left upper limb was recognized. On MR imaging, posterior fossa subdural hemorrhage and spinal cord injury at the T-1 and T-2 levels were evident. Sequential imaging studies demonstrated progressive myelomalacic changes in the cervical spinal cord and eventual development of syrinx cavities as far cephalad as C-3. Hydrocephalus developed simultaneously as well. A ventriculoperitoneal cerebrospinal fluid shunt was inserted at 22 months of age. Six months after shunt insertion, MR imaging demonstrated regression of the posttraumatic syringomyelia. The patient was stable from an imaging standpoint at 16 months after shunt insertion, and she has continued to make developmental progress. The importance of treatment of associated hydrocephalus is widely recognized in the management of syringomyelia associated with the Chiari malformations. It should be considered in the management of posttraumatic syringomyelia as well.

Birth Injuries↗