Little is known about the natural history of scoliosis found in patients with syringomyelia, including the factors affecting scoliosis curve progression and the effect of syrinx drainage treatment. Twenty patients having scoliosis with syringomyelia diagnosed by MRI were followed up for 6.6 (range 2.0–12.6) years on an average. Various factors potentially influencing curve pattern or progression in these patients were then retrospectively reviewed. The convex side of major curve of scoliosis tended to be on the same side as the syrinx and as the unilateral neurologic abnormality. No correlation was found between the location and the size of the syrinx and the location and size of the major curve of the scoliosis, or between the severity of neurologic deficit and the size of the major curve of the scoliosis. In patients under the age of ten at the time of diagnosis of scoliosis and with a flexible curve, decompression of the syrinx improved or stabilized scoliosis. In most patients over the age of ten, surgical treatment of the scoliosis was necessary because of the large initial size of the curve or progression of the curve even after syrinx drainage. Other factors including gender, location of the syrinx, type of the curve, and severity of neurologic deficits did not correlate with the progression of the curve. The results of this retrospective study suggest that early diagnosis and decompression of a syrinx in scoliosis patients especially under the age of ten is crucial and may decrease the curve size and limit scoliosis curve progression.
Scoliosis; Syringomyelia; MRI; Syrinx drainage
Delayed Chiari malformation with syringomyelia is an uncommon and sparsely reported complication after lumbar cisternal shunting. A number of treatments have been implemented with varying degrees of success. After review of these modalities, a treatment plan was devised and implemented.
A 15-year-old girl was diagnosed with idiopathic intracranial hypertension (pseudotumor cerebri) and had placement of a Medtronic Spetzler lumboperitoneal shunt. She did well for 10 years and then developed a 3-week history of headache, balance problems, and sensory loss. Workup demonstrated a new Chiari malformation and cervical syrinx. She underwent initial placement of a ventriculoperitoneal shunt, followed by ligation of the lumboperitoneal shunt. She then had a suboccipital decompression and duraplasty. Follow-up at 6 months showed relief of her symptoms and MRI demonstrated resolution of the Chiari malformation and syrinx.
Delayed Chiari malformation and/or syringomyelia after lumbar shunting is best treated with placement of a ventricular device and ligation of the lumbar shunt. The Chiari malformation and/or syrinx should be surgically addressed for persistent symptoms or progression of the structural abnormality.
Acquired chiari malformation; idiopathic intracranial hypertension; lumboperitoneal shunt; pseudotumor cerebri; syringomyelia
First described in 1928, percutaneous aspiration of syringomyelia is infrequently performed to aid in diagnosis and surgical management. We describe a case of post traumatic syringomyelia successfully treated with direct fluoroscopic drainage with substantial resolution of syrinx-related neurologic symptoms.
The patient is a 36-year-old man involved in a motorcycle accident who sustained multiple vertebral fractures resulting in dense paraplegia below T4, ultimately treated with multilevel lami-nectomy and pedicle screw fixation. The patient began to experience phantom leg paresthesias and muscle spasm felt to be related to an extensive spinal cord syrinx extending to the conus.
Syrinx drainage was accomplished fluoroscopically at the level of the conus by percuntaneous needle drainage after cord localization with intrathecal myelographic contrast. Clear spinal fluid was drained from the syrinx cavity without complication. Immediately during and after drainage, the patient recognized a decrease in phantom pelvic and lower extremity paresthesias with significantly reduced spasticity. Syrinx collapse was documented with post-drainage CT imaging. Sustained relief of paresthesias and muscle spasms was achieved with gradual syrinx and symptom return requiring subsequent drainages performed at nine, 22 and 37 months following the initial drainage.
In addition to confirming the symptomatic nature of syringomyelia in those with atypical symptoms, fluoroscopically guided drainage of syrinx can in select instances provide sustained relief.
This modality may have additional advantages including serving as an adjunctive maneuver to improve operative access to the subarachnoid space by collapsing the cord, and serving as a temporizing measure for those patients who are poor surgical candidates. Maneuvers such as fluoroscopic table angulation provide an additional benefit in both the localization and drainage of syringomyelia and thus may be preferred over other minimally invasive procedures such as CT-guided drainage.
syringomyelia, syrinx, fluoroscopy, percutaneous drainage
Syringomyelia is characterized by a fluid-filled cavity within the spinal cord. While its pathogenesis is currently debated, the relationship of syringomyelia with other conditions, such as Chiari I malformation and cord/column trauma, is well accepted. Despite these common associations, a nidus for syrinx formation has not been identified in a subset of patients. We report 2 patients with idiopathic cervicothoracic syringomyelia who presented with progressive neurologic dysfunction. Diagnostic and treatment algorithms used in the care of these patients are presented.
Retrospective review, including preoperative and postoperative studies, intraoperative findings, and the patients' surgical outcomes.
Patients underwent laminectomy, lysis of adhesions, untethering of spinal cord, fenestration of syrinx, and duraplasty after preoperative studies demonstrated evidence of focal cerebrospinal fluid flow block at the level of the syrinx. One patient's neurologic condition improved after surgery, whereas the other's remained unchanged without further deterioration; both showed radiographic decrease in the syrinx on immediate postoperative magnetic resonance imaging.
These 2 cases illustrate patients who develop a cervicothoracic syrinx in the absence of any trauma, infection, previous manipulation of the neuraxis, or malformations known to be associated with a syringomyelia. Whereas there is no consensus on the optimal management of these patients, the patients reported here experienced arrest in deterioration or improvement of their neurologic examination, making the identification of this condition important as a potentially reversible cause of neurologic deficits. Long-term follow-up is required to determine the efficacy, durability, and lifestyle impact of the procedure.
Spinal cord injuries; Syringomyelia; idiopathic; Syrinx; Arachnoiditis; Central subarachnoid stenosis
Post-traumatic syringomyelia is a progressive degenerative disorder that is a well-recognized sequela of spinal cord injury. There is currently no optimal intervention capable of producing satisfactory long-term clinical results.
In this report, we present a 55-year-old Asian man with recurrent syringomyelia after shunt treatment. The syrinx extended from the thoracic cord into the medulla. We used a silicone tube to create a channel connecting the syrinx cavity directly to the fourth ventricle. The patient made a good recovery and follow-up magnetic resonance imaging revealed a considerable diminution in the size of the syrinx.
We present a new approach that has the potential to improve the outcome of patients with recurrent post-traumatic syringomyelia, who cannot be treated by conventional methods.
This paper describes MRI findings of upper cervical subluxation due to alar ligament disruption following a vehicular collision. Incidental findings included the presence of a myodural bridge and a spinal cord syrinx. Chiropractic management of the patient is discussed.
A 21-year old female presented with complaints of acute, debilitating upper neck pain with unremitting sub-occipital headache and dizziness following a vehicular collision. Initial emergency department and neurologic investigations included x-ray and CT evaluation of the head and neck. Due to persistent pain, the patient sought chiropractic care. MRI of the upper cervical spine revealed previously unrecognized clinical entities.
This case highlights the identification of upper cervical ligamentous injury that produced vertebral subluxation following a traumatic incident. MRI evaluation provided visualization of previously undetected injury. The patient experienced improvement through chiropractic care.
Various surgical procedures for the treatment of post-traumatic syringomyelia have been introduced recently, but most surgical strategies have been unreliable. We introduce the concept and technique of a new shunting procedure, syringo-subarachnoid-peritoneal shunt. A 54-year-old patient presented to our hospital with a progressive impairment of motion and position sense on the right side. Sixteen years before this admission, he had been treated by decompressive laminectomy for a burst fracture of L1. On his recent admission, magnetic resonance (MR) imaging studies of the whole spine revealed the presence of a huge syrinx extending from the medulla to the L1 vertebral level. We performed a syringo-subarachnoid-peritoneal shunt, including insertion of a T-tube into the syrinx, subarachnoid space and peritoneal cavity. Clinical manifestations and radiological findings improved after the operation. The syringo-subarachnoid-peritoneal shunt has several advantages. First, fluid can communicate freely between the syrinx, the subarachnoid space, and the peritoneal cavity. Secondly, we can prevent shunt catheter from migrating because dural anchoring of the T-tube is easy. Finally, we can perform shunt revision easily, because only one arm of the T-tube is inserted into the intraspinal syringx cavity. We think that this procedure is the most beneficial method among the various shunting procedures.
Syringomyelia; Spinal cord injury; Shunt
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.
We report here a rare case of high cervical stenosis at the level of atlas who presented with progressively deteriorating quadriparesis and respiratory distress. A 10-year-old boy presented with above symptoms of one-year duration with a preceding history of trivial trauma prior to onset of such symptoms. Cervical spine MRI revealed a significant stenosis at the level of atlas from the posterior side with a syrinx extending above and below. High-resolution computed tomography of the above level yielded an ill-defined osseous bar compressing the canal at the level of C1 posterior arch, which appeared bifid in the midline. The patient was immediately taken up for surgery in view of his respiratory complaints. The child showed an excellent recovery after excision of the posterior arch of atlas and removal of the compressing osseous structure.
Atlas; posterior arch; spinal stenosis
To report on a female patient diagnosed with Currarino’s triad in adulthood.
This case presents an adult patient with a medical history of a congenital anal atresia, a partial sacral agenesis, and a surgically treated ectopic anus. After a coincidentally observed presacral mass by MRI, due to unexplained constipation later in adulthood, Currarino’s triad was suspected in this patient. This triad consists of anorectal malformation(s), sacrococcygeal defects and a presacral mass of various origin. Further investigation confirmed the mass to be a meningocele, and showed a tethered cord and a syrinx.
In (young) patients with anorectal malformations, although having no other symptoms, further examination might be required to exclude Currarino’s triad. Importance of early diagnosis and multidisciplinary assessment is recommended to establish adequate treatment if needed.
Currarino; Anorectal malformation; Sacrococcygeal agenesis; Presacral mass; Meningocele
Myelopathy is a form of neurological disease caused by compression of the spinal cord. Upper and lower quarter screens are commonly used in identifying myelopathy, although most of the screen components demonstrate poor or unstudied diagnostic value. The purpose of this case report is to describe the diagnostic process in detecting syringomyelia, an intramedullary lesion that may cause myelopathy. The patient was a 47-year-old female with a thoracic syrinx that was discovered by spinal magnetic resonance imaging (MRI) following a complicated and delayed clinical diagnostic course. Following surgical intervention and a two-week inpatient rehabilitation stay, the patient was discharged using a rolling walker for ambulation and was performing most transfers with modified independence. A complicating pattern of signs and symptoms combined with a diagnostic process guided by poorly studied screen components demonstrates the diagnostic dilemma associated with identifying the cause of myelopathy within the thoracic spine. This also indicates the need for further investigation of individual and clustered components of the neurological screen to improve the ability to identify patients in need of complete imaging studies in a more timely fashion.
Intramedullary Lesion; Myelopathy; Spinal Cord; Syrinx
Two cases of post-traumatic syringomyelia presenting 10 and 41 years after spinal injuries that had caused lumbar vertebral fractures but no lasting neurological deficits are reported. In both patients the caudal end of the syrinx cavities, as shown by MRI, corresponded to the level of the previous vertebral fractures. Patients presenting with post-traumatic syringomyelia after uncomplicated spinal fracture are very rare, and the significance of the past history of spinal trauma may be overlooked.
Five patients with chronic arachnoiditis and syringomyelia were studied. Three patients had early life meningitis and developed symptoms of syringomyelia eight, 21, and 23 years after the acute infection. One patient had a spinal dural thoracic AVM and developed a thoracic syrinx 11 years after spinal subarachnoid haemorrhage and five years after surgery on the AVM. A fifth patient had tuberculous meningitis with transient spinal cord dysfunction followed by development of a lumbar syrinx seven years later. Arachnoiditis can cause syrinx formation by obliterating the spinal vasculature causing ischaemia. Small cystic regions of myelomalacia coalesce to form cavities. In other patients, central cord ischaemia mimics syringomyelia but no cavitation is present. Scar formation with spinal block leads to altered dynamics of cerebrospinal fluid (CSF) flow and contributes to the formation of spinal cord cystic cavities.
The severity and distribution of symptoms and signs in patients with syringomyelia is considered to be dependent on the longitudinal and transverse dimensions of the syrinx and it is thought that clinical examination can identify the extent of the cyst. Magnetic resonance imaging has made the anatomical localisation of intramedullary spinal lesions more exact and probably more specific than previous methods of investigation. Syrinx length, diameters, cyst:cord and cord:canal ratios have been studied in 12 patients with syringomyelia to assess whether the dimensions of the syrinx relate to the clinical findings. The length of syrinx appeared to be related to cyst diameter, cyst:cord and cord:canal ratios. Patients with a small syrinx tended to have a small cyst diameter, and small cyst:cord and cord:canal ratios. No significant relationship was found between muscle wasting or weakness, distribution of sensory loss, degree of disability or distress and the dimensions of the syrinx. These findings should be borne in mind when surgical management is being considered.
Spinal hemangioblastomas (HB) are rare, histologically benign, highly vascularized tumors often associated with von Hippel–Lindau (VHL) disease. The aim of the current study is to demonstrate the benefit of early surgical resection of large spinal HBs in selected asymptomatic patients with VHL.
Seventeen patients underwent microsurgical resection of 20 spinal HBs at the Department of Neurosurgery at Helsinki University Central Hospital (HUCH). Thirteen tumors were in the cervical spine, five in thoracic and one patient had two lumbar lesions. MRI tumor showed an associated syrinx in 16 patients (94%). Tumor volume ranged from 27 to 2730 mm3. Out of 17 patients, 11 (65%) tested positive for VHL in mutation analysis. Five of these patients with tumors ranging from 55 to 720 mm3 were treated prophylactically.
Complete tumor resection was performed in 16 patients (94%) who were followed up for a median of 57 months (range 2–165 months). No patient had neurological decline on long-term follow-up. Among the patients with VHL, five patients with preoperative sensorimotor deficits showed improvement of their symptoms but never regained full function. One patient who presented with tetraplegia remained the same. Otherwise, all five patients with prophylactic surgery remained neurologically intact.
Although documented growth on serial MRIs and the need for pathological diagnosis have been suggested as indications for surgery in otherwise asymptomatic patients, our series showed that a potentially larger group of asymptomatic patients with spinal HB associated with VHL would benefit from microsurgical resection. Long-term results of the surgical management of spinal HB are generally favorable. Our results suggest staging and early treatment for spinal HB larger than 55 mm3, especially in patients with VHL. Small spinal HBs may be followed up.
Spinal cord; spinal hemangioblastoma; McCormick classification; microsurgery; von Hippel–Lindau
Post-traumatic syringomyelia was previously thought to be an infrequent but serious sequel to spinal cord injury. Clinical and CT studies have shown an incidence of between 1% and 5%, but more recently MRI has suggested an incidence of up to 22%. Twenty spinal cords have been examined after death from two days to 43 years after injury. Four had syrinxes, 20% of the series, approaching the incidence found by MRI. The acute and chronic pathological changes after trauma are described. Post-traumatic syringomyelia seems to develop from cores of necrotic tissue (myelomalacic cores) rather than lysis of haematoma. The mechanism of extension of syrinxes remains unexplained.
A 9 year-old girl with syringomyelia and scoliosis was treated with Echols’ procedure, a surgical technique that employs a metal stent to maintain drainage of fluid from the syrinx into the subarachnoid space. The patient presented to our institution 34 years later with a history of progressive myelopathy and surgically-treated deformities of the thoracic spine, lumbar spine, and right foot. Computer-assisted myelography indicated that the metal wire remained in place and that the syrinx had collapsed. Neurological examination and neurophysiological testing confirmed the presence of thoracic myelopathy, which may be due to the wire tethering the thoracic spinal cord to the dorsal dura. This is believed to be the sole long-term report of the effects of Echols’ procedure. The history of direct treatment of syringomyelia is reviewed and is contrasted with indirect treatment of syringomyelia, which relieves the condition by opening obstructed CSF pathways within the foramen magnum or spine.
Syringomyelia; Arnold Chiari Malformation; Type 1; History; Surgery
Like human infants, songbirds learn their species-specific vocalizations through imitation learning. The birdsong system has emerged as a widely used experimental animal model for understanding the underlying neural mechanisms responsible for vocal production learning. However, how neural impulses are translated into the precise motor behavior of the complex vocal organ (syrinx) to create song is poorly understood. First and foremost, we lack a detailed understanding of syringeal morphology.
To fill this gap we combined non-invasive (high-field magnetic resonance imaging and micro-computed tomography) and invasive techniques (histology and micro-dissection) to construct the annotated high-resolution three-dimensional dataset, or morphome, of the zebra finch (Taeniopygia guttata) syrinx. We identified and annotated syringeal cartilage, bone and musculature in situ in unprecedented detail. We provide interactive three-dimensional models that greatly improve the communication of complex morphological data and our understanding of syringeal function in general.
Our results show that the syringeal skeleton is optimized for low weight driven by physiological constraints on song production. The present refinement of muscle organization and identity elucidates how apposed muscles actuate different syringeal elements. Our dataset allows for more precise predictions about muscle co-activation and synergies and has important implications for muscle activity and stimulation experiments. We also demonstrate how the syrinx can be stabilized during song to reduce mechanical noise and, as such, enhance repetitive execution of stereotypic motor patterns. In addition, we identify a cartilaginous structure suited to play a crucial role in the uncoupling of sound frequency and amplitude control, which permits a novel explanation of the evolutionary success of songbirds.
Non-neoplastic intramedullary spinal lesion cases are rarely seen in the literature. We would like to present this case for differential diagnosis of intramedullary spinal tumors. The aim of this case report is to attract attention on the MRI findings with lack of contrast enhancement and long syrinx formation which differs these types of lesions from the intramedullary spinal tumors. Intraoperative, pathological and immunohistochemical findings of non-neoplastic intramedullary spinal lesion were discussed.
Spinal tumor; Non-neoplastic; Intramedullary
Posttraumatic syringomyelia may result from a variety of inherent conditions and traumatic events, or from some combination of these. Many hypotheses have arisen to explain this complex disorder, but no consensus has emerged. A 28-year-old man presented with progressive lower extremity weakness, spasticity, and decreased sensation below the T4 dermatome five years after an initial trauma. Magnetic resonance imaging (MRI) revealed a large, multi-septate syrinx cavity extending from C5 to L1, with a retropulsed bony fragment of L2. We performed an L2 corpectomy, L1-L3 interbody fusion using a mesh cage and screw fixation, and a wide decompression and release of the ventral portion of the spinal cord with an operating microscope. The patient showed complete resolution of his neurological symptoms, including the bilateral leg weakness and dysesthesia. Postoperative MRI confirmed the collapse of the syrinx and restoration of subarachnoid cerebrospinal fluid (CSF) flow. These findings indicate a good correlation between syrinx collapse and symptomatic improvement. This case showed that syringomyelia may develop through obstruction of the subarachnoid CSF space by a bony fracture and kyphotic deformity. Ventral decompression of the obstructed subarachnoid space, with restoration of spinal alignment, effectively treated the spinal canal encroachment and post-traumatic syringomyelia.
Syringomyelia; Magnetic resonance imaging; Trauma; Subarachnoid space; Obstruction
Two cases are reported of syrinx associated with an intramedullary metastasis. The first presented with hyperaesthesia in the right arm and bilateral pyramidal leg weakness, the second with a partial right sided Brown Sequard syndrome with a sensory level at D6. A possible pathological mechanism for the development of such syrinxes is discussed.
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.
This study aimed to estimate the prevalence and incidence of cerebrovascular disease (CVD) and stroke in Korean male adolescents.
Materials and Methods
The authors reviewed all medical certificates, medical records, and radiologic images from the examinations of Korean military conscription from January 2008 to May 2011.
Of the 101156 examinees, 40 had CVD and stroke during adolescence. The overall prevalence and incidence of CVD and stroke was 39.54 cases per 100000 adolescents and 2.08 cases per 100000 adolescents per year, respectively and these were similar to the worldwide data. There were 3 cases of aneurysm, 3 cases of dural arteriovenous fistula, 11 cases of arteriovenous malformation, 4 cases of cavernous hemangioma, 4 cases of cerebrovascular infarction, 16 cases of Moyamoya disease, and 1 case of missing data. The incidence of arteriovenous malformation (0.57 cases per 100000 adolescents per year) was lower than the incidence for the worldwide general population. The incidence of Moyamoya disease was higher than that in any other country (15.82 cases per 100000 adolescents, vs. 0.83 cases per 100000 adolescents per year).
We observed ischemic and hemorrhagic stroke, each accounting for approximately half of cases, and high incidence of Moyamoya disease with low incidence of arteriovenous malformation in Korean male adolescents.
Cerebrovascular disease; stroke; adolescent; male; Korea; conscription
The sound-generating mechanism in the bird syrinx has been the subject of debate. Recent endoscopic imaging of the syrinx during phonation provided evidence for vibrations of membranes and labia, but could not provide quantitative analysis of the vibrations. We have now recorded vibrations in the intact syrinx directly with an optic vibration detector together with the emitted sound during brain stimulation-induced phonation in anaesthetized pigeons, cockatiels, and a hill myna. The phonating syrinx was also filmed through an endoscope inserted into the trachea. In these species vibrations were always present during phonation, and their frequency and amplitude characteristics were highly similar to those of the emitted sound, including nonlinear acoustic phenomena. This was also true for tonal vocalizations, suggesting that a vibratory mechanism can account for all vocalizations presented in the study. In some vocalizations we found differences in the shape of the waveform between vibrations and the emitted sound, probably reflecting variations in oscillatory behaviour of syringeal structures. This study therefore provides the first direct evidence for a vibratory sound-generating mechanism (i.e. lateral tympaniform membranes or labia acting as pneumatic valves) and does not support pure aerodynamic models. Furthermore, the data emphasize a potentially high degree of acoustic complexity.
To investigate the role of magnetic resonance imaging (MRI) in neurological disorders, 115 children were studied in two groups. Group A (78 patients) was studied by paired computed tomography and MRI cranial scans. Group B (37 patients) was studied by paired computed tomography assisted myelography (CTM) and MRI spinal scans. In group A, the scans were generally equivalent for supratentorial tumours and for investigating fits, hydrocephalus, benign intracranial hypertension, and cerebral atrophies, but MRI scanning was superior for posterior fossa tumours and cysts. In group B, MRI scans were superior for intramedullary spinal tumours, spinal dysraphic problems with tethering or syrinx, and were complementary to CTM in diastematomyelia.