Spinal cord edema preceding syringomyelia associated with Chiari I malformation--case report

A 38-year-old woman with Chiari I malformation presented with spinal cord edema preceding syringomyelia manifesting as a 5-month history of nuchal pain and numbness of the upper extremities. Magnetic resonance imaging showed spinal cord edema, a poorly defined syrinx at the C-2 to T-2 levels, and distorted cerebellar tonsils. Computed tomography revealed cerebrospinal fluid (CSF) density in the center of spinal cord edema, and positron emission tomography revealed no uptake of L-[methyl-11C]methionine, indicating a non-neoplastic lesion. Craniocervical decompression achieved excellent clinical and neuroradiological outcomes. The success of surgical treatment supports the theory that patients with Chiari I malformation have increased transmural flow of CSF, causing spinal cord edema that progresses to syringomyelia. Early treatment of patients with spinal cord edema is indicated to prevent permanent spinal cord injury due to progressive syringomyelia.     

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.     

"特发性"胸椎左侧凸患者并发脊髓病变及其临床意义

目的 探讨对临床上无明显神经损害的"特发性"胸椎左侧凸患者存在的脊髓病变情况,并分析其临床意义.方法 1997年10月至2003年10月共诊治"特发性"胸椎左侧凸59例,其中男性31例,女性28例;年龄7～44岁,平均15岁;Cobb角15°～108°,平均56°.所有病例经临床及影像学检查,排除神经纤维瘤及先天性脊椎发育异常等病因.对临床诊断为"特发性"胸椎左侧凸的患者行全脊髓磁共振成像(MRI)检查,对脊髓病变的发生率进行统计.结果 59例"特发性"胸椎左侧凸患者经全脊髓MRI检查后,共有33例发现髓内病变,髓内病变发生率为56%,其中Chiari畸形合并脊髓空洞24例,脊髓空洞5例,Chiari畸形、脊髓空洞、脊髓裂1例,Chiari畸形、脊髓空洞、脊髓栓系1例,脊髓空洞合并栓系1例,Dandy-Walker畸形1例.其余26例未发现脊髓病变.分析伴有脊髓病变的胸椎左侧凸患者中男性比例及平均Cobb角均显著大于无脊髓病变的胸椎左侧凸(P＜0.05).结论 对于胸椎左侧凸,尤其是男性、Cobb角较大的患者需高度怀疑伴有脊髓病变.对于此类患者术前常规全脊髓MRI检查具有重要意义.     

Surgical treatment of a syringomyelia associated with an idiopathic arachnoid malformation disclosed by preoperative MRI

BACKGROUND: We describe the very rare condition of an idiopathic spinal arachnoid malformation associated with syringomyelia (SM) and depicted on preoperative magnetic resonance imaging (MRI) whose features were confirmed at surgery. CASE DESCRIPTION: A 34-year-old female suffered from progressive gait impairment because of lower limb palsy and sensory disturbances. MRI demonstrated a bulging membrane at the T6 level that was transversely stretched between the dorsal aspect of the spinal cord and the posterior dura mater. At this level, the spinal cord appeared atrophic and pushed anteriorly against the dura with enlargement of the posterior subarachnoid spaces (SAS) and focal collapse of an associated panmedullar SM. Surgery consisted in releasing the arachnoid malformation and opening the inferior segment of the syringomyelic cavity. Pathological examination revealed a fibro-sclerotic tissue with cellular areas of meningo-endothelial cells. Postoperative neurological status progressively improved but slightly. Three-months and 1 year postoperatively, MRI showed the collapse of the whole SM and restoration of cerebrospinal fluid (CSF) flow at the treated T6 level. CONCLUSION: Spinal arachnoid malformations associated with SM are very rare and have never been described up to now on MRI. Surgical removal of the causative malformation allows spinal cord decompression and prevents the recurrence of the SM by restoring normal CSF circulation.     

Surgical treatment of syringomyelia. Selection of surgical procedures

The surgical approach to syringomyelia is controversial. In this study, the authors evaluated the results of various operative procedures applied in 31 patients with syringomyelia. Syringomyelia was associated with Chiari malformation in 17 cases, with spinal adhesive arachnoiditis in nine, with trauma in three, and with epidural arachnoid cyst in one. One case was idiopathic. A total of 38 operations were performed. Syringosubarachnoid shunting was applied in 17 patients, syringoperitoneal shunting in 11, terminal syringostomy in three, ventriculoperitoneal shunting in three, lumboperitoneal shunting in two, foramen magnum decompression (suboccipital craniectomy plus upper cervical laminectomy) and terminal syringostomy in one, and foramen magnum decompression with syringosubarachnoid shunting in one. The postoperative follow-up period ranged from 2 to 55 months (average, 26 months). Of the 31 patients, 23 showed neurological improvement, five were unchanged, and three deteriorated. Among the last three, one patient with Chiari malformation developed shunt malfunction due to arachnoiditis after syringosubarachnoid shunting. In two patients with syringomyelia secondary to adhesive arachnoiditis, the spinal cord was damaged by extensive separation of the arachnoid membrane at surgery. On the basis of the results in these 31 cases, the authors conclude that syringosubarachnoid shunting is effective for syringomyelia associated with Chiari malformation if syringomyelia is responsible for the clinical symptoms. Post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis should be treated by syringoperitoneal shunting. If hydrocephalus is present, ventriculoperitoneal shunting is indicated. Finally, terminal syringostomy is no more effective than syringosubarachnoid or syringoperitoneal shunting.     

Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis

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

Syringo-subarachnoid shunt for syringomyelia associated with Chiari malformation (type 1)

Summary The authors report the surgical results of 28 patients with syringomyelia associated with Chiari malformation (type 1). 28 patients underwent 34 operative procedures. Syringo-subarachnoid shunt was performed in 28 patients, foramen magnum decompression with syringo-subarachnoid shunt in three, ventriculo-peritoneal shunt in one, terminal syringostomy in one, and foramen magnum decompression with terminal syringostomy in one. In an average postoperative follow-up period of 3 years and 9 months ranging from one year to 7 years and one month, neurological symptoms and signs improved in 24 out of 28 patients (82%). Some improvement was noted in sensory deficit and motor weakness. In 3 patients, the symptoms did not change. In 3 patients whose symptoms were unchanged, preoperative studies demonstrated atrophy of the spinal cord, in which irreversible changes were shown. The symptoms deteriorated in one patient. In one patient whose symptoms increased 3 months after syringo-subarachnoid shunt, shunt insufficiency due to postoperative adhesive arachnoiditis was responsible for neurological deterioration.The authors propose that syringo-subarachnoid shunt is effective as a surgical procedure for syringomyelia associated with Chiari malformation (type 1) if the patient does not have symptoms due to Chiari malformation or has only mild signs and symptoms which do not require foramen magnum decompression, such as nystagmus or atrophy of sternocleidomastoid muscle.     

Surgical treatment of syringomyelia: favorable results with syringosubarachnoid shunting

The authors review the clinical course of 29 patients who underwent syringosubarachnoid shunting for syringomyelia. Twenty-two patients presented hindbrain-related syringomyelia; seven patients had non-hindbrain-related syringomyelia secondary to trauma (four cases) and to spinal arachnoiditis (three cases). The surgical technique is described in detail. All patients showed postoperative deflation or collapse of the syrinx at follow-up magnetic resonance imaging evaluation. Symptoms stabilized in 17 cases (59%); 9 cases (31%) showed improvement in the neurological function; 3 cases (10%) presented delayed neurological deterioration, probably owing to spinal cord ischemia.     

Spontaneous resolution of Chiari I malformation and syringomyelia: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Indications for surgery and the surgical technique of foramen magnum decompression for patients with Chiari I malformation and syringomyelia are controversial issues. This case report supports the view that observation may be adequate for patients without progressive symptoms or with mild clinical symptoms. CLINICAL PRESENTATION: A 37-year-old woman presented with a 3-month history of burning dysesthesias and hypesthesia in her right arm. A neurological examination revealed hypesthesia in the right trigeminal distribution. A magnetic resonance imaging scan revealed a Chiari I malformation with syringomyelia between C2 and T2. No hydrocephalus was observed. CLINICAL COURSE: Because the patient's symptoms regressed spontaneously, surgery was not performed. Thirty-two months after her initial examination, the patient was asymptomatic. A second magnetic resonance imaging scan was obtained, which demonstrated complete spontaneous resolution of the Chiari I malformation and syringomyelia. CONCLUSION: We attribute the regression of the patient's symptoms to spontaneous recanalization of cerebrospinal fluid pathways at the foramen magnum, which most likely was due to rupture of the arachnoid membranes that had obstructed cerebrospinal fluid flow.     

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.     

Terminal syringomyelia: is it as innocent as it seems?--Case report

A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.     

Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia: case report

BACKGROUND: Chiari I malformation, accompanied by superposed bony anomaly of the craniovertebral junction, is comparatively rare. We report a case of Chiari I malformation accompanied by assimilation of the atlas, Klippel-Feil syndrome, and syringomyelia. CASE DESCRIPTION: The patient was a 61-year-old woman demonstrating numbness of the extremities, sensory impairment, muscular weakness, and tendon hyper-reflexia. X-ray images and CT scans demonstrated assimilation of the atlas to the occipital bone, C2 and C3 fusion, abnormal passage of the vertebral arteries, and an anomalous bony mass on the right lateral mass of the atlas protruding into the spinal column. The odontoid process was also deviated to the left. Magnetic resonance images demonstrated bilateral descent of the cerebellar tonsils and syringomyelia extending from C6 to T8. Computed tomographic scans with the head rotated to the right demonstrated increased narrowing of the vertebral column caused by the right lateral mass of the atlas, and MR images confirmed exaggerated deformation of the spinal cord at the same region. This deformation manifested no neurologic symptoms, and we therefore performed foramen magnum decompression and duraplasty using Gore-Tex (W.L. Gore & Associates, Inc., Flagstaff, AZ). In the early postoperative period, neurologic symptoms improved. CONCLUSION: We believe it is important that a treatment plan for Chiari I malformation accompanied by bony anomaly of the craniovertebral junction be determined based on morphologic investigation of the region supplemented by dynamic imaging-based evaluation of instability, or a careful inspection for atypical passage of the vertebral arteries, a frequent site of complication.     

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.     

Syringomyelobulbia associated with cervical spondylosis. Pathophysiology and therapeutic implications

Spinal spondylosis is rarely implicated in syringomyelia. We report the case of a 70-year-old patient with a 10-year history of gait disturbance; constrictive pain of lower limbs and urinary incontinance. Physical examination disclosed spastic tetraparesis. In the upper limbs, deep tendon reflexes were abolished, with hyposthesia and hands amyotrophy. Brain and cervical MRI showed syringomyelobulbia with cervical spondylotic myelopathy. Extensive cervical laminectomy induced a mild clinical improvement. A second MRI performed 6 months after surgery depicted a complete disappearance of the bulbo-medullar cavitation with secondary atrophy. Extradural spondylotic compression of the spinal cord should be firmly considered as an etiology of syringomyelia. A purely extradural decompression could be sufficient to induce regression of the medullary cavitation.     

Advances in diagnosis and treatment of spinal hemangioblastomas

Spinal hemangioblastoma usually leads to large intramedullary cysts. Until recently clinical and conventional radiological examination was often not able to detect single or multiple tumor niduses at the wall of a secondary syringomyelia cyst. Thus during surgical exploration the tumor not infrequently was missed, leading to misinterpretation of the pathological entity as syringomyelia, spinal gliosis or low grade spinal astrocytoma. This often resulted in explorative biopsy or decompressive laminectomy. Clinical deterioration postoperatively was not uncommon due to the remaining tumor and increasing spinal cord enlargement. Now magnetic resonance imaging allows the exact preoperative localization of the medullary lesion apart from the accompanying cysts and facilitates differential diagnosis of primary syringomyelia or other spinal cord neoplasms. Microsurgical techniques and laser-assisted resection have improved the outcome. We report on eight patients with spinal hemangioblastoma treated in our clinic since the installation of magnetic resonance imaging and laser-assisted microneurosurgery.     

Neuraxial analgesia during labor in a patient with Arnold-Chiari type I malformation and syringomyelia

Chiari type I malformation is a caudal displacement of the cerebellum with tonsillar herniation through the foramen magnum, frequently associated with syringomyelia, a syndrome characterized by cyst-like cavities in the spinal cord; each of the conditions leads to characteristic neurologic abnormalities. Pregnant patients with these types of malformation are considered to have an increased risk of brainstem compression and/or progression of the disease during labor. We present the case of a patient in labor with a diagnosis of syringomyelia associated with Chiari type I malformation and describe the anesthetic management. The patient revealed during labor that she had syringomyelia associated with Chiari type I malformation, after having made no mention of it in previous history taking. Finally, we review the pathology observed in these patients.     

Surgery for posttraumatic syringomyelia： a retrospective study of seven patients

Objective： To analyze retrospectively the clinical symptoms, signs, radiological findings and results of treatment of posttraumatic syringomyelia. Methods： The data of 7 patients with posttraumatic syringomyelia confirmed by computerized tomography （CT） and magnetic resonance imaging （MRI） in our hospital between 1999 and 2004 were reviewed retrospectively. The patients underwent decompressive laminectomy or syringo-subarachnoid （S-S） shunting with microsurgery. Long-term follow-up was available （range： 13-65 months）. Results： The major dinical manifestations of posttraumatic syringomyelia usually included the onset of increasing signs and the development of new symptoms after an apparently stable period. The clinical symptoms included pain, sensory disturbance, weakness, and problems in autonomic nerves. Syrinx existed merely at the cervical level in 4 cases and extended downward to the thoracic levels in the other 3 cases. One case underwent decompressive laminectomy, 6 cases were treated by S-S shunting. During the early postoperative period, all the patients showed an improvement of symptoms of syrinx without major complication or death. The decreased size or collapse of the syrinx was demonstrated by postoperative MRI. Conclusions ： Posttraumatic syringomyelia is a disabling sequela of spinal cord injury, developing months to years after spinal injury. MRI is the standard diagnostic technique for syringomyelia. The patients with posttraunmtic syringomyelia combined with progressive neurological deterioration should be treated with operations. S-S shunting procedure is effective in some patients with posttraumatic syringomyelia. Decompressive procedure may be an alternative primary surgical treatment for patients with kyphosis and cord compression.     

A myelographic study of idiopathic scoliosis--with special reference to its clinical significance

The purpose of this study was to investigate the concealed anomaly of the spinal cord in the so-called idiopathic scoliosis. Thirty-three patients with idiopathic scoliotic curvatures underwent metrizamide myelography before surgery from 1979 through 1985. Spinal cord anomalies were found in 8 of the 33 patients (abnormal group). They were 3 patients with Arnold-Chiari malformation with syringomyelia, 3 with syringomyelia and 2 with "narrowed dural tube". The remaining 25 patients (normal group) gave us the standard values for myelographic measurements with which we compared quantitatively the size of dural tube and spinal cord in the normal and abnormal groups. The abnormal group was classified into three types: type I was swollen spinal cord with normal dural tube; type II was swollen spinal cord with narrowed dural tube; and type III was normal spinal cord with narrowed dural tube. In conclusion myelography and myelographic measurements are essential for idiopathic scoliotic patients who will undergo spinal correction and fusion operations.     

先天性脊柱侧凸患者中的脊髓畸形和脊椎畸形

目的:探讨先天性脊柱侧凸患者的各类脊髓畸形与脊椎畸形之间的关系。方法:对我院2003年～2005年收治的211例已确诊的先天性脊柱侧凸患者的临床资料进行回顾性分析,统计先天性脊柱侧凸患者脊髓畸形、脊椎畸形的发生率及其好发部位,观察两者间的关系。结果:211例先天性脊柱侧凸患者中脊髓畸形44例(20.85%),其中脊髓空洞症25例次(11.85%),脊髓纵裂30例次(14.22%),马尾终丝栓系28例次(13.27%),低位圆锥12例次(5.69%),其他类型少见,多种畸形常并发存在;脊髓畸形好发于脊柱胸段、胸腰段、腰段、腰骶段。44例伴有脊髓畸形的先天性脊柱侧凸病例中不良分节34例(77.27%),孤立性半椎体1例(2.27%),非孤立性半椎体21例(47.73%),脊柱裂30例(68.18%),混合型41例(93.18%);脊椎畸形好发于脊柱胸段、腰段、腰骶段。结论:先天性脊椎畸形常合并脊髓畸形,总体上脊髓畸形与脊椎畸形的好发部位相似,但各种脊髓畸形、脊椎畸形间缺少显著的、特定的对应关系。     

Acute syringomyelia: case report

OBJECTIVE AND IMPORTANCE: Syringomyelia is generally regarded as a chronic, slowly progressive disorder. We describe a case of acute dilation of the central canal of the spinal cord that presented with rapidly progressive segmental signs. CLINICAL PRESENTATION: A 29-year-old female patient who had previously undergone surgical treatment for a Chiari I malformation, syringomyelia, and hydrocephalus presented with an 8-day history of headaches, progressive paraparesis, and urinary retention. Magnetic resonance imaging scans demonstrated panventricular hydrocephalus in association with a holocord syrinx that extended to the obex. Magnetic resonance imaging scans that had been coincidentally obtained just 3 days before the onset of symptoms had revealed no evidence of hydrocephalus or syringomyelia. INTERVENTION: The patient underwent emergency revision of a failed ventriculoperitoneal shunt. Postoperatively, there was prompt resolution of the syringomyelia, hydrocephalus, and associated neurological deficits. CONCLUSION: Among patients with communicating syringomyelia, the central canal of the spinal cord participates as a "fifth ventricle" and can undergo rapid dilation in association with acute hydrocephalus. Appropriate treatment in such cases involves placement of a ventriculoperitoneal shunt.