Percutaneous drainage and serial magnetic resonance imaging in the diagnosis of symptomatic posttraumatic syringomyelia: case report and review of the literature

A patient with high cervical tetraplegia with new-onset headaches and posttraumatic syringomyelia is presented. Percutaneous drainage of the syrinx resulted in a resolution of the headaches and collapse of the syrinx on follow-up magnetic resonance imaging (MRI). The return of the symptoms correlated with the re-expansion of the syrinx on MRI. The patient underwent syringopleural shunting with persistent resolution of the symptoms and collapse of the syrinx on MRI. The value of percutaneous drainage and serial MRI to determine the clinical significance of posttraumatic syringomyelia is discussed.     

Spontaneous resolution of syringomyelia: report of two cases and review of the literature

OBJECTIVE AND IMPORTANCE: The natural history of syringomyelia is highly unpredictable, and some patients experience improvement or stabilization without surgery. However, the mechanisms of the formation and spontaneous resolution of syringomyelia remain controversial. This report concerns two patients with syringomyelia who demonstrated spontaneous reductions in syrinx size, accompanied by symptomatic improvement. CLINICAL PRESENTATION: One patient was a 10-year-old girl with syringomyelia associated with a tight cisterna magna and basilar impression, who demonstrated a spontaneous decrease in syrinx size, accompanied by symptomatic improvement, in 22 months. The other patient was a 39-year-old man with syringomyelia associated with a Chiari I malformation, who demonstrated a spontaneous reduction in syrinx size and neurological improvement, accompanied by elevation of the cerebellar tonsils, 6 months after diagnosis. INTERVENTION: The patients were monitored. CONCLUSION: The mechanisms of spontaneous resolution of syringomyelia, as well as the factors leading to the cerebrospinal fluid flow disturbances that cause syringomyelia, may vary. Resolution of foramen magnum lesion-related syringomyelia may be the result of spontaneous correction of the abnormal cerebrospinal fluid flow, as observed in our cases, or of cavity fluid drainage into the spinal arachnoid space because of spinal cord fissuring.     

Syringomyelia associated with a cauda equina meningioma involving the conus medullaris

An unusual case of syringomyelia secondary to a cauda equina meningioma involving the conus medullaris is described. The tumour was totally removed with decompression of an adjacent cyst and syrinx resulting in resolution of the symptoms and radiological appearance.     

Spontaneous resolution of cervical syringomyelia. A case report

A 54-year-old man visited our hospital with complaints of severe pain in the neck and the right upper limb. His MR (magnetic resonance) imaging showed a cervical syringomyelia associated with Chiari I malformation. After one month, his complaints vanished smoothly, and the repeated MR imaging revealed shrinkage of the syrinx. The mechanisms of spontaneous resolution of syringomyelia were discussed according to some relevant literature.     

Symptomatic syringomyelia secondary to clinically obscure infratentorial tumour

The formation of a cervical spinal cord syrinx as a result of an infratentorial mass, even though uncommon, has been reported in international literature. In such cases, syringomyelia is usually asymptomatic, while the tumour-related symptoms and signs predominate. We report a patient with a posterior fossa tumour and secondary syringomyelia. In this patient, syringomyelia symptoms and signs were present, and a cervical spine Magnetic Resonance Imaging (MRI) showed a large cervical syrinx. A more careful clinical examination though, revealed a sub-clinical posterior fossa syndrome and brain MRI revealed a large infratentorial meningioma. A posterior fossa craniotomy was performed, followed by complete tumour resection and almost complete remission of the syrinx and its related symptoms. The authors discuss the role of posterior fossa tumour induced tonsillar herniation in the development of secondary syringomyelia, the mechanisms leading to syrinx formation and the conditions that must be fulfilled for that to happen.     

Treatment of Chiari malformation, syringomyelia and hydrocephalus by neuroendoscopic third ventriculostomy.

This short paper illustrates a case with cervical myelomeningocoele, a Chiari malformation (CM), hydrocephalus (HC) and cervical syringomyelia treated by neuroendoscopic third ventriculostomy (NTV) with resolution of both the hydrocephalus and the syrinx. Two similar cases are discussed. The technique is advocated for the treatment of such complex dysraphic conditions.     

Dandy-Walker complex and syringomyelia in an adult: case report and discussion

OBJECTIVE AND IMPORTANCE: Syringomyelia in association with the Dandy-Walker complex is rare and only 17 cases have been reported, mostly as autopsy findings or incidental reports in series for other conditions. We present a further case report and discuss the possible pathogenetic mechanisms of syrinx formation in the presence of the Dandy-Walker complex. We emphasize the role of foramen magnum obstruction and alteration in cerebrospinal fluid flow dynamics in syrinx formation. The therapeutic importance of disimpaction of the foramen magnum is stressed. CLINICAL PRESENTATION: A 39-year-old man presented with headache. As a child, he had had cystoperitoneal and ventriculoperitoneal shunts inserted for hydrocephalus in association with the Dandy-Walker complex. On examination, the patient had limited upgaze but was otherwise normal. Magnetic resonance imaging revealed a large cervicothoracic syrinx and a Dandy-Walker cyst obstructing the foramen magnum. INTERVENTION: The cystoperitoneal shunt was revised. The patient's headaches resolved, and follow-up imaging demonstrated resolution of the syrinx and disimpaction of the foramen magnum. CONCLUSION: Disimpaction of the foramen magnum can resolve syringomyelia in patients with the Dandy-Walker complex. This can be achieved by a shunt procedure or by formal foramen magnum decompression. The rarity of the association between the Dandy-Walker complex and syringomyelia prevents firm conclusions from being made regarding the best management strategy for this condition.     

Radiographic assessment of surgical treatment of post-traumatic syringomyelia

Context: Symptomatic post-traumatic syringomyelia can affect the quality of life in patients whose neurologic function has already been impacted by a spinal cord injury.Objective: To investigate the radiographic and clinical outcomes following surgery for syringomyelia, we present a literature review along with a case series from a single surgeon’s experience.Methods: A retrospective review was conducted on patients with post-traumatic syringomyelia who were treated by a single surgeon. Thirty-four patients who underwent surgical treatment consisting of syrinx fenestration, lysis of adhesions, and duraplasty were identified. In addition, a narrative literature review was conducted with a primary focus on diagnosis and management of post-traumatic syringomyelia.Results: Literature review suggests that regardless of age, sex, vertebral location, or severity of trauma, patients who experience a spinal cord injury should be closely monitored for post-traumatic syringomyelia. Retrospective review of our 34 patients revealed 24 patients for whom pre- and post- operative MRI was available. The predominant location of the injury was cervical (15). The average syrinx length, measured in spinal segments, was similar when comparing pre- and post-operative MRIs; average syrinx length was 5.5 and 5.4 spinal segments, respectively. In contrast, syrinx axial dimension was decreased in 16 of the patients post-operatively and stable or increased in the other eight. The change in syrinx size did not correlate with clinical outcomes.Conclusion: Current surgical treatment of post-traumatic syringomyelia involves restoration of normal CSF flow dynamics; further prospective work is needed to correlate the clinical state, radiographic measures, and efficacy of surgical intervention.     

Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child

Summary A child with complete spontaneous resolution of a Chiari I malformation associated Syringomyelia without surgical intervention is presented. The child was followed clinically by serial magnetic resonance imaging (MRI) and remains neurologically stable after 8-years of follow-up. To our knowledge, only 6 pediatric cases with spontaneous resolution of a spinal cord syrinx documented by MRI without surgical intervention have been reported. This case is of interest in the light of the postulated theories to explain spontaneous resolution of syringomyelia.     

Subarachnoid pressure-dependent change in syrinx size in a patient with syringomyelia associated with adhesive arachnoiditis. Case report

The pathophysiology of syringomyelia is still not well understood. Current prevailing theories involve the assumption that cerebrospinal fluid (CSF) flows into the syrinx from the subarachnoid space through the perivascular space of Virchow-Robin. Reported here is the case of a patient with syringomyelia in which this course is clearly contradicted. This patient with a holocord syrinx associated with adhesive arachnoiditis was treated 3 years previously with insertion of a subarachnoid-peritoneal shunt and had recently experienced worsening myelopathy. On surgical exploration, the shunt system was functioning normally. The medium-pressure shunt valve was replaced with an adjustable valve with a higher closing pressure setting, thus increasing the CSF pressure in the subarachnoid space. Contrary to prevailing theories, this procedure markedly reduced the size of the syrinx. This case provides direct evidence that the syrinx size is inversely related to subarachnoid CSF pressure and supports the hypothesis that the pressure gradient across the spinal cord parenchyma is the force that generates syringes in syringomyelia.     

Unraveling the riddle of syringomyelia

The pathophysiology of syringomyelia development is not fully understood. Current prevailing theories suggest that increased pulse pressure in the subarachnoid space forces cerebrospinal fluid (CSF) through the spinal cord into the syrinx. It is generally accepted that the syrinx consists of CSF. The here-proposed intramedullary pulse pressure theory instead suggests that syringomyelia is caused by increased pulse pressure in the spinal cord and that the syrinx consists of extracellular fluid. A new principle is introduced implying that the distending force in the production of syringomyelia is a relative increase in pulse pressure in the spinal cord compared to that in the nearby subarachnoid space. The formation of a syrinx then occurs by the accumulation of extracellular fluid in the distended cord. A previously unrecognized mechanism for syrinx formation, the Bernoulli theorem, is also described. The Bernoulli theorem or the Venturi effect states that the regional increase in fluid velocity in a narrowed flow channel decreases fluid pressure. In Chiari I malformations, the systolic CSF pulse pressure and downward motion of the cerebellar tonsils are significantly increased. This leads to increased spinal CSF velocities and, as a consequence of the Bernoulli theorem, decreased fluid pressure in narrow regions of the spinal CSF pathways. The resulting relatively low CSF pressure in the narrowed CSF pathway causes a suction effect on the spinal cord that distends the cord during each systole. Syringomyelia develops by the accumulation of extracellular fluid in the distended cord. In posttraumatic syringomyelia, the downwards directed systolic CSF pulse pressure is transmitted and reflected into the spinal cord below and above the traumatic subarachnoid blockage, respectively. The ensuing increase in intramedullary pulse pressure distends the spinal cord and causes syringomyelia on both sides of the blockage. The here-proposed concept has the potential to unravel the riddle of syringomyelia and affords explanations to previously unanswered clinical and theoretical problems with syringomyelia. It also explains why syringomyelia associated with Chiari I malformations may develop in any part of the spinal cord including the medullary conus. Syringomyelia thus preferentially develops where the systolic CSF flow causes a suction effect on the spinal cord, i.e., at or immediately caudal to physiological or pathological encroachments of the spinal subarachnoid space.     

Syringomyelia and syringobulbia associated with an ependymoma of the cauda equina involving the conus medullaris: case report

A patient with syringomyelia and syringobulbia secondary to an asymptomatic ependymoma of the cauda equina involving the conus medullaris is described. Delayed metrizamide computed tomography myelography was decisive for making the diagnosis of a highly extended syrinx associated with the tumor. Syringomyelic symptoms improved remarkably following the removal of the tumor, and the syrinx was not visualized in a postoperative study. The pathogenesis of syringomyelia associated with a caudally located spinal cord tumor is discussed.     

Decompression of the spinal subarachnoid space as a solution for syringomyelia without Chiari malformation

STUDY DESIGN: Review and analysis of seven cases of syringomyelia treated surgically. OBJECTIVE: To demonstrate the beneficial role of decompressive surgery for the altered cerebrospinal fluid (CSF) flow dynamics in syringomyelia not associated with Chiari I malformation. A comparison between the pre- and post-operative syrinx size and CSF flow in the subarachnoid space was made using cine-mode magnetic resonance imaging (cine-MRI) and then correlated with clinical improvement. SETTING: University Hospital, Seoul, Korea. METHODS: Conventional spinal MRI and cine-MRI were performed in the region of CSF flow obstruction preoperatively in seven patients with syringomyelia not associated with Chiari I malformation. The group consisted of one case of syrinx with post-traumatic compression fracture, one case of post-traumatic arachnoiditis, two cases of holocord syrinx associated with hydrocephalus without Chiari malformation, one case of syrinx with post-traumatic pseudomeningeal cyst, one case of post-laminectomy kyphosis-associated syringomyelia and one case of post-tuberculous arachnoiditis syringomyelia. Based on the preoperative cine-MRI, the types of surgery appropriate to correct the CSF flow obstruction were chosen: decompressive laminectomy-adhesiolysis and augmentation duraplasty in arachnoiditis cases, ventriculoperitoneal shunt for hydrocephalus, cyst extirpation in pseudomeningeal cyst and both anterior and posterior decompression-fusion in the case of post-laminectomy kyphosis. A syrinx-draining shunt operation was performed in three cases; where the syringomyelia was associated with post-traumatic compression fracture refractory to a previous decompression, where hydrocephalus was present in which the decompression by ventriculoperitoneal shunt was insufficient and where post-traumatic arachnoiditis was present in which the decompression was impossible due to diffuse adhesion. Change in syrinx size was evaluated with post-operative MRI in all seven cases and restoration of flow dynamics was evaluated with cine-MRI in three of the cases, two patients with clinical improvement and one patient with no change of clinical status, respectively. RESULTS: Four out of seven patients showed symptomatic improvement after each decompressive operation. In the remaining three cases, reconstruction of the spinal subarachnoid space was not possible due to diffuse adhesion or was not the main problem as in the patient with syrinx associated with hydrocephalus who had to undergo a shunt operation. One of these three patients showed clinical improvement after undergoing syringosubarachnoid shunt. A decrease of syrinx size was observed in only two out of the five patients who showed clinical improvement after treatment. Of these five patients, two patients underwent post-operative cine-MRI and the restoration of normal CSF flow dynamics was noted in both patients. Of the remaining two patients, one underwent post-operative cine-MRI and there was no change in the CSF flow dynamics evident. CONCLUSION: These results suggest that the restoration of CSF flow dynamics between the syrinx and the subarachnoid space by decompressive operation is more effective than simple drainage of the syrinx cavity itself in the treatment of syringomyelia without Chiari malformation.     

A successful treatment for a case of idiopathic syringomyelia with syrinx located in the anterior horn

Idiopathic syringomyelia not associated with Chiari malformation, tumor, nor tetherd cord syndrome has not been frequently reported. So the clinical significance and optimum treatment strategy for this disease remains unclear. We report a 37-year-old man who was diagnosed as having idiopathic syringomyelia and was treated successfully using implantation of a syringo-subarachnoid shunt. The patient suffered gradual worsening of sensory disturbance in the upper extremities, and Magnetic resonance imaging demonstrated a large syrinx located in the cervical spinal cord (C2-3), located mainly at the left anterior horn. We performed syringo-subarachnoid shunt for syringomyelia under partial hemilaminectomy. During the surgery, the spinal cord was markedly rotated and dorsal root entry had shifted to the midline because of the large syrinx. In order to perform myelotomy at the dorsal root entry zone, we needed additional drilling to the medial portion of the lamina. After this procedure, we were able to perform implantation of the syringo-subarachnoid shunt safely. After the operation, the syrinx collapsed immediately and no neurological deficit was observed. The patient was discharged 2 weeks after the operation. Idiopathic syringomyelia in which the syrinx has deviated to the anterior horn is rare. In those cases, we consider that sufficient hemilaminectomy for the midline should be performed.     

脊髓空洞症的手术治疗与临床评价

目的：对脊髓空洞症应用外科手术治疗进行探讨,以提高该种病变的临床疗效。方法：129例脊髓空洞症经CT或MRI扫描检查确诊,应用外科手术治疗,并进行平均1．5年以上的随访及评判治疗效果。结果：86例经受空洞－蛛网膜下腔分流术或空洞切开术的病人,空洞腔均显闭合,43例单纯行枕－颈减压的Amold-Chiari畸形或脊髓闭合不全的病人,其空洞腔亦见明显缩小。结论：在脊髓空洞症病人宜采取外科手术治疗,而枕－颈减压,空洞切开术或空洞－蛛网膜下腔分流术是可供选择有效治疗方法。     

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.     

Terminal ventriculostomy as an adjuvant treatment of complex syringomyelia: a case report and review of the literature

Complex syringomyelia is multifactorial, and treatment strategies are highly individualized. In refractory cases, sectioning of the filum terminale, also known as terminal ventriculostomy, has been described as a potential adjuvant treatment to alleviate syrinx progression. A 10-year-old boy with a history of arachnoiditis presented with complex syringomyelia, progressive lower extremity motor weakness, and spasticity. Previously, he had failed spinal cord detethering and direct syrinx shunting. Imaging studies demonstrated a holocord syrinx extending to the level of his conus medullaris and into the filum terminale. The patient underwent an uncomplicated lumbar laminectomy and transection of the filum terminale. Operative pathologic specimens demonstrated a dilated central canal within the filum. Postoperative imaging demonstrated significant reduction in the diameter of the syrinx. At follow-up, the patient’s motor symptoms had improved. Terminal ventriculostomy may be a useful adjuvant in treating caudally placed syringes refractory to other treatments. This procedure carries low neurological risk and involves no hardware implantation. In select cases, terminal ventriculostomy may help preserve neurological function in the face of otherwise progressive syringomyelia.     

An unusual presentation and resolution of syringomyelia after cervical spine injury. Case report

The authors describe a case of noncommunicating syringomyelia associated with Chiari malformation Type I in a patient in whom acute symptomatic exacerbation occurred following cervical spine trauma. Surgical stabilization and realignment of the spine resulted in marked resolution of the neurological abnormalities, and subsequent magnetic resonance imaging demonstrated persistent collapse of the syrinx. The authors review the various factors in the pathogenesis of this unusual sequence of events.     

Resolution of syringomyelia and Chiari I malformation by ventriculoatrial shunting in a patient with pseudotumor cerebri and a lumboperitoneal shunt

A sneeze caused acute left arm pain in a 36-year-old woman with a lumboperitoneal (LP) shunt that had been placed 3 years earlier for relief of headaches caused by pseudotumor cerebri. Numbness progressed up the left arm, neck, and back of the head and finally into the left face along with weakness of the hand and arm. Magnetic resonance imaging (MRI) and computed tomography revealed new tonsillar herniation and a large eccentric syrinx extending from C2 to T6. The functioning LP shunt was clamped, and a ventriculoatrial shunt was placed. Pain lessened and motor function improved slightly. MRI revealed complete resolution of the syrinx and resolution of the tonsillar herniation. Theories of syringomyelia formation, the relationship to Chiari I malformation, and the implications of this case are discussed.     

Syringomyelia associated with arachnoid septum at the craniovertebral junction, contradicting the currently prevailing theory of syringomyelia formation

Despite a number of various hypotheses in the literature, the pathophysiology of syringomyelia is still not well understood. In this article, we report two cases of cervical syringomyelia not associated with Chiari I malformation. Both cases had a septum-like structure in the subarachnoid space on the dorsal side of the cord at the craniovertebral junction. Cardiac-gated phase-contrast cine-mode magnetic resonance imaging (MRI) demonstrated decreased cerebrospinal fluid (CSF) flow on the dorsal side of the spinal cord. Surgical excision of this septum, restoring the CSF flow, resulted in a prompt reduction of the syrinx size in both cases. Findings in these cases contradict the currently prevailing hypothesis of syrinx formation that postulate that the piston-like movement of the cerebellar tonsils enhance the pulsatile CSF flow in the spinal subarachnoid space, driving the CSF into the syrinx through the perivascular space of Virchow and Robin. The authors propose that a mechanism based on the decreased pulsatile CSF flow in the spinal subarachnoid space will be more suitable as a hypothesis in studying the pathophyisiology of syringomyelia. These cases also provide an important lesson in managing the patients with syringomyelia not associated with Chiari I malformation.