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PubMed1989

[Association of asymptomatic syringomyelia with tentorial meningioma].

A case of asymptomatic syringomyelia associated with tentorial meningioma is reported. A 53-year-old female was admitted to our hospital with the complaints of headache, decrease of bilateral visual acuity and gait disturbance. Neurological examination revealed no abnormality except for bilateral papilledema and left cerebellar sign. Motor and sensory systems were also intact. CT and MRI scans showed a left tentorial mass lesion, cervical syringomyelia and left tonsillar herniation. A relationship between the fourth ventricle and syringomyelia was not clearly identified. The tumor was totally removed and a histological diagnosis of fibroblastic meningioma was made. Postoperative radiological examination showed a diminishing of syringomyelia with the improved tonsillar herniation. Association of syringomyelia with brain tumor was reviewed, and its pathogenesis was discussed.

Cerebellar Neoplasms↗
PubMed1988

[Surgical treatment of syringomyelia associated with Chiari malformation].

There is now a controversy about the methods of surgical treatment in syringomyelia associated with Chiari malformation. In this report, the authors emphasize that syringosubarachnoid shunt is effective as a surgical procedure or syringomyelia associated with Chiari malformation. From 1982 to 1986, 17 patients with syringomyelia associated with Chiari malformation were surgically treated. Seventeen patients underwent 22 operative procedures for syringomyelia. Syringosubarachnoid shunt was used in eighteen patients, foramen magnum decompression with syringosubarachnoid shunt in one, foramen magnum decompression with terminal syringostomy in one, terminal syringostomy in one, and ventriculoperitoneal shunt in one. The average postoperative follow up period was 2 years and 3 months, from 4 months to 4 years and 11 months. Thirteen out of 17 patients showed neurological improvement and 3 patients were neurologically unchanged. In one patient, the symptoms deteriorated. Syringosubarachnoid shunt is an effective therapeutic procedure for the patient with syringomyelia associated with Chiari malformation. We think that shunt malfunction by arachnoiditis after operation could be prevented by inserting the shunt tube into the subarachnoid space and closing the arachnoid membrane.

Adolescent↗
PubMed1995

Absent superficial abdominal reflexes in children with scoliosis. An early indicator of syringomyelia.

We describe 12 children with idiopathic scoliosis who had a persistent absent superficial abdominal reflex (SAR) on routine neurological examination. MRI showed syringomyelia to be present in ten. The average age at detection of the scoliosis was 4.3 years and at diagnosis of syringomyelia 6.6 years. In all ten children the SAR was consistently absent on the same side as the convexity of the curve. In two it was the only abnormal neurological sign. An absent SAR in patients with scoliosis is an indication for investigation for underlying syringomyelia. In the children with syringomyelia, six had thoracic and four thoracolumbar curves. The clinical features differed in the two groups. Patients with thoracic curves were generally asymptomatic. Their neurological signs were subtle and none had any motor signs. By contrast, patients with thoracolumbar curves had symptoms and neurological signs. Abnormal gait was present in all four patients with thoracolumbar curves. In three this was due to considerable motor weakness. In eight children syringomyelia was associated with a Chiari-I malformation. In seven the syrinx was treated surgically by decompression of the foramen magnum.

Arnold-Chiari Malformation↗
PubMed1994

Syringomyelia. A potential risk factor in scoliosis surgery.

STUDY DESIGN: An 18-year-old patient with "idiopathic" adolescent scoliosis is presented. A thoracic syrinx was detected as an incidental finding during magnetic resonance imaging of the spine. OBJECTIVES: Syringomyelia may be a risk factor for neurologic injury during correction of scoliosis, and in these cases, spinal cord monitoring may be of particular value. BACKGROUND DATA: Spinal distraction and instrumentation carry a risk of neurologic damage in patients with scoliosis and associated syringomyelia. Syringomyelia is a cause of scoliosis, and although neurologic problems are the usual symptom, scoliosis may be the only sign at initial examination. A higher risk of neurologic injury has been reported in corrective surgical treatment of patients with syringomyelia. The mechanism of cord damage is unclear. Monitoring of spinal cord function is recommended to detect intraoperative neurological injury, which may be reversed on removing distraction and implants. RESULTS: Intraoperative somatosensory-evoked potential (SSEP) spinal cord monitoring detected possible cord damage during outrigger distraction. Reduction of distraction led to a recovery of SSEPs and a satisfactory operative outcome. CONCLUSION: Syringomyelia may be a risk factor for neurologic injury during correction of scoliosis, and SSEP spinal cord monitoring may identify and prevent intraoperative spinal cord injury.

Adolescent↗
PubMed1995

[Epidemiology of syringomyelia in Japan--the nationwide survey].

The nationwide epidemiological survey of syringomyelia was carried out in Japan by sending inquiries to neurologists, child neurologists, neurosurgeons and orthopedic surgeons for the period of 1991 and 1992. A total of 1,243 cases of syringomyelia were ascertained. Among them, 622 were men and 619 women, and the average age of onset was 28 years old. The classification by Barnett et al was used, presenting syringomyelia with Chiari malformation in 684 cases (51.2%), dysraphism in 47 (3.7%), post traumatic syringomyelia in 139 (11%), post-spinal arachnoiditis in 76 (6%), spinal cord tumor in 132 (10.5%) and others in 204. Its predominant clinical course was slowly progressive, but 202 cases (17.9%) showed rather stable course including spontaneous resolution in 29 cases. The main initial symptoms were numbness in 522 cases (42%), motor disturbance in 504 (40.5%), and pain in 296 (23.8%). Neurologic signs noted in the abnormality of deep tendon reflexes in 836 cases (67.3%), motor disturbance in 763 (60.4%) and positive pathological reflexes in 383 (30.1%). Sensory disturbance was found in 942 cases (75.8%) and the dissociated type were 559 out of them (59.3%). It is noteworthy that 982 out of 1,243 were documented by MRI and surgical operations such as foramen magnum decompression, syringo-subarachnoid shunt and others were performed in 829 cases. Syringobulbia was confirmed on MRI in 101 cases of syringomyelia in which spinal cord tumors were most frequently associated.

Adolescent↗
PubMed1988

Syringomyelia: how MRI aids diagnosis and management.

MRI is an invaluable tool in the diagnosis and assessment of treatment of syringomyelia. It also confirms the theory of the pathogenesis of the disease and suggests why some operations on the condition fail. A series of patients with Chiari I, with and without syringomyelia, have been studied with MRI both prior to surgery and following treatment. In four cases, with Chiari I alone and minor symptoms only, management has been conservative. No progression of symptoms or signs have been seen, MRI allowing syringomyelia to be excluded, both at diagnosis or development during follow-up. Patients with Chiari I, six with and one without syringomyelia, were split into two groups; those with symptoms and signs of cervico medullary compression (CMC) and those with pure cord amyotrophy with upper limb weakness and numbness of typical "cape type". In three CMC patients, foramen magnum decompression (FMD) with fascia lata grafting and high syringotomy, and in two FMD without syringotomy, were carried out. The procedure improved pain, CMC symptomatology and clinical signs, but had only minor effects on amyotrophic symptoms. Some improvement was seen after surgery in distention of the syrinx on MRI studies, but there was no progression in the syrinx cavities. In two patients with pure amyotrophic the syrinx cavities. In two patients with pure amyotrophic symptoms, primary syringoperitoneal shunting (SPS) was carried out, and a further two patients with primary FMD subsequently went on to SPS for amyotrophic symptoms and signs.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Magnetic resonance imaging (MRI) in syringomyelia.

Based on an own material of 19 patients with syringomyelia and on the related literature a survey is given on the diagnosis, differential diagnosis, postoperative evaluation and the dynamics of CSF and cyst fluids, using magnetic resonance imaging (MRI). The following conclusions can be drawn: 1. MRI is the preferred method of investigation for diagnosis and differential diagnosis of syringomyelia. 2. Using MRI, it is possible to study fluid flow in the vertebral canal and the movements of the syrinx fluid. The data are as yet limited, but in the future will form an important contribution to our understanding of the pathogenesis of syringomyelia and to the choice of a treatment method. 3. MRI is important for the postoperative follow-up of patients with syringomyelia, including tracking the cyst and detecting complications.

Arnold-Chiari Malformation↗
PubMed2001

Cesarean section in a patient with syringomyelia.

PURPOSE: To describe the anesthetic management of Cesarean section in a patient with syringomyelia. CLINICAL FEATURES: A 27-yr-old pregnant woman with syringomyelia was scheduled to undergo elective Cesarean section. At the age of 25 yr, she had begun to experience headaches, and at the age of 26 yr, a diagnosis of syringomyelia of the upper spinal cord was made on the basis of magnetic resonance imaging findings. No symptoms other than headache were noted preoperatively. General anesthesia was used for the Cesarean section. After the administration of 1 mg vecuronium as a priming dose, 5 mg vecuronium were injected. At the onset of clinical muscle weakness, 225 mg thiamylal were promptly administered as the induction agent and the patient was intubated (timing principle with priming method) and pressure on the cricoid cartilage applied to prevent regurgitation of stomach contents. Anesthesia was maintained with oxygen, nitrous oxide and isoflurane at a low concentration. Mild hyperventilation was used throughout the procedure. Anesthesia and surgery proceeded without any problem, response to vecuronium was clinically normal and recovery was uneventful. Neurological status remained normal. CONCLUSION: We report the safe use of general anesthesia for Cesarean section in a patient with syringomyelia. Precautions were taken to avoid increases in intracranial pressure and our patient experienced no untoward neurologic event.

Adult↗
PubMed2005

Familial Chiari type I malformation with syringomyelia in two siblings: case report and review of the literature.

OBJECTIVE: Familial cases of Chiari malformation with syringomyelia are rare. The majority of the reported series and case reports detail sporadic cases. The authors report two siblings who presented with Chiari type I malformation and syringomyelia (CMI+S). CLINICAL PRESENTATION: We report two sisters who each presented with scoliosis on routine school physicals. Their clinical examination was unremarkable; however, imaging studies demonstrated a Chiari malformation with syringomyelia. Both underwent cervicomedullary decompression, and follow-up imaging studies revealed resolution of the syringomyelia. CONCLUSION: A review of the literature reveals fewer than ten previous reports of familial CMI+S in the past 30 years. Although rare, the existence of familial cases of CMI+S suggests a genetic component to the pathogenesis of this condition in at least a proportion of patients. Neurosurgeons should be aware of the familial aggregation of CMI+S.

Adolescent↗
PubMed2006

Diastematomyelia in children: treatment outcome and natural history of associated syringomyelia.

OBJECTIVE: To quantify the long-term outcome of children with diastematomyelia and the implication of syringomyelia in the natural history. MATERIALS: Retrospective study of 17 children (nine girls and eight boys) with diastematomyelia operated during 1989-2004. Mean age at diagnosis was 3.4 years (range 5 days-12 years), mean follow-up was 5 years; 14 had excision of spur and cord untethering, 3 had excision of spur alone. RESULTS: The bony spur was in the lumbar region in 12 and thoracic in 5 patients. Syringomyelia was present in eight (47%), associated with bony spur between L1 and 4 (p=0.088), spina bifida occulta in ten (58.8%), and spinal lipoma in one (6%). Preoperative Necker Enfants Malades (NEM) scores were 17 in eight patients, 16 in four, 15 in three, 13 in one, and 12 in one patient. Presenting neurological deficits were motor in eight, sensory deficits in three, and anal incontinence in one patient. There was improvement of NEM scores postoperatively in five patients only, but still with residual deficit. Repeat spur excision and cord untethering was performed in three patients for neurological deterioration 1-4 years after first operation. On postoperative MRI scans syringomyelia remained unchanged in all eight patients. There were one skin infection, one transient motor deterioration, and one patient with sensory deficit after surgery. CONCLUSION: Prophylactic operations were associated with the best clinical outcome. Despite improvement, all patients with established preoperative deficit still had residual neurological deficits at their last follow-up. The associated syringomyelia remained unchanged after surgery, indicating that it does not contribute to the neurological syndrome.

Child↗
PubMed2005

Results of the section of the filum terminale in 20 patients with syringomyelia, scoliosis and Chiari malformation.

BACKGROUND: Spinal cord traction caused by a tight filum terminale may be considered a pathogenic mechanism involved in the development of syringomyelia, the Chiari malformation (type I) and scoliosis. Section of the filum terminale is proposed as a useful surgical approach in these conditions. METHODS: Between April 1993 and July 2003, a total of 20 patients (8 men and 12 women) with a mean age of 33.5 years underwent section of the filum terminale with or without opening of the dural sac through a standard sacrectomy. Eight patients suffered from scoliosis, 5 from syringomyelia, 2 from Chiari malformation and 5 with a combination of these conditions. FINDING: After section of the filum terminale, patients with syringomyelia showed an early clinical improvement of dysaesthesia, thermo-anaesthesia, hypo-aesthesia and walking difficulties. Rising of the medullary conus was also observed. In patients with scoliosis, back pain improved dramatically and a curve reduction was noticed, although progression of the curve was observed in one case. In patients with Chiari malformation, headache, dysaesthesia and paraparesis disappeared. CONCLUSIONS: Section of the filum terminale is a useful strategy in the treatment of scoliosis, syringomyelia and the Chiari malformation, and offers a new aetiological basis for the understanding of these three disorders.

Adolescent↗
PubMed2005

[Charcot-Marie-Tooth disease associated with syringomyelia revealed by urinary disorders].

PURPOSE: Charcot-Marie-Tooth (CMT) disease is characterized by genetic and clinical heterogeneity. The occurrence of urinary disorders is unusual and requires further investigation. CMT disease and syringomyelia are not usually associated. Moreover, primary urinary signs in syringomyelia are not frequent. CASE REPORT: An 11-year-old girl with CMT disease 1A duplication presented with motor deficit of the leg associated with urinary disorders. Urodynamic study revealed neurogenic detrusor overactivity, and magnetic resonance imaging confirmed syringomyelia extending from T11 to an L1 located conus. DISCUSSION: Review of the literature confirmed the possibility of primary urinary signs in syringomyelia, with only one case of CMT and urinary signs described. In this case, the diagnosis was radicullar and medullar compression by a hypertrophic nerve formation called an "onion bulb" reflecting the demyelinisation and remyelinisation cycle. CONCLUSION: Urinary signs in CMT disease are exceptional and require investigation into the possibility of a central lesion.

Charcot-Marie-Tooth Disease↗
PubMed2004

The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis.

Syringomyelia is frequently accompanied by an extramedullary lesion at the foramen magnum, particularly a Chiari I malformation. Although syringomyelia associated with foramen magnum obstruction has characteristic clinical, radiological, and neuropathological features, its pathogenesis remains unclear. Currently prevalent hydrodynamical theories assert that obstruction of the subarachnoid space at the foramen magnum interferes with flow of cerebrospinal fluid (CSF) between the spinal and the intracranial subarachnoid compartments. As a result, spinal CSF is driven into the spinal cord through the perivascular spaces to form a syrinx. These theories are implausible biophysically because none postulates a pump adequate to drive fluid through these spaces. None of the theories can explain why syrinx pressure is higher than CSF pressure; why extensive gliosis, edema, and vascular wall thickening regularly occur; and why the composition of syrinx fluid is not identical with that of CSF. A new theory of pathogenesis is proposed to address these difficulties. In the presence of subarachnoid obstruction at the foramen magnum, a variety of activities, such as assuming the erect posture, coughing or straining, and pulsatile fluctuations of CSF pressure during the cardiac cycle, produce transiently higher CSF pressure above the block than below it. There are corresponding changes in transmural venous and capillary pressure favoring dilation of vessels below the block and collapse of vessels above the block. The spatially uneven change of vessel caliber produces mechanical stress on the spinal cord, particularly caudal to the block. The mechanical stress, coupled with venous and capillary dilation, partially disrupt the blood-spinal cord barrier, allowing ultrafiltration of crystalloids and accumulation of a protein-poor fluid. The proposed theory is consistent with the neuropathological findings in syringomyelia and with the pressure and composition of syrinx fluid. It also accounts for the prolonged course of syringomyelia and its aggravation by cough, strain, and assumption of an erect posture. It contributes to understanding the low incidence and the morphology of syringobulbia. It explains the poorly understood presentation of foramen magnum meningiomas with symptoms of a mid- to low-cervical myelopathy. The theory also affords an understanding of the late recurrence of symptoms in children with hydromyelia who are treated with a ventricular shunt.

Arnold-Chiari Malformation↗
PubMed1997

Experimental syringomyelia: late ultrastructural changes of spinal cord tissue and magnetic resonance imaging evaluation.

BACKGROUND: In human hydrosyringomyelia and in the late stage of experimental syringomyelia, the spinal cord tissue adjacent to the syrinx is exposed to a similar pathophysiologic condition. We investigated the ultrastructural changes in the late stages of kaolin-induced syringomyelia, and in addition, we presented magnetic resonance imaging (MRI) findings of the cervicomedullary junction and syrinx, and the nature of edema in the spinal cord of this experimental model. METHODS: Syringomyelia was induced in rabbits by intracisternal injection of kaolin. MRI was performed at 6 weeks, and 6 and 12 months following injection, and the animals were killed by transcardial perfusion of formaldehyde solution and examined by transmission electron microscopy. Evans blue was injected intravenously in six rabbits, 6 weeks and 12 months following kaolin injection and was examined by confocal laser scanning microscopy. RESULTS: MRI showed that the syrinx communicated with the fourth ventricle in most animals. Demyelination of varying degrees and slight edematous change were seen in the perisyrinx white matter. No extravasation of Evans blue was seen by confocal microscopy. Abundant astrocytic proliferation with a large number of glial filaments was seen at the margin of the syrinx and between the axons in the perisyringeal region. The perivascular space enlargement occurred in both the gray and white matter. The endothelial junctions appeared intact. Regenerating axons and remyelination by oligodendrocytes were seen occasionally. CONCLUSIONS: The MRI confirmed the communication between the fourth ventricle and the syrinx. The ultrastructural changes were almost identical to those of the early stage syrinx, but the astrocytic proliferation was more severe, and the edema was less in the late stage. The perisyrinx edema appeared to be of the interstitial type, as in hydrocephalus. Axonal degeneration and demyelination continued with abortive attempt at regeneration and remyelination in the less edematous late stage, which might be the cellular basis for the persistence or worsening of clinical symptoms and signs in the chronic stage of syringomyelia even after surgical treatment.

Animals↗
PubMed1977

Difficult labour as a cause of communicating syringomyelia.

Although some causes of communicating syringomyelia are known most cases of the disease can only be described as idiopathic. The results of a questionnaire suggest a high incidence of difficult labour in the mothers of syringomyelia patients. A high proportion of patients had forceps deliveries, and a high proportion were the first born in their families. Because the validity of taking a birth history at the age of presentation (mean age 40 y, S.D. 14 for this sample) might be questioned, patients admitted with other disorders (mean age 40 y, S.D. 16) were used as a control group. These patients matched the syringomyelia patients in social class and place of birth. It seems likely that birth trauma may be a cause of tonsillar descent through the foramen magnum (ectopia) and of arachnoiditis; both conditions are often present. Once the tonsils become engaged in the foramen magnum, difference between the cranial and spinal pressure may, over the course of several years, cause the tonsils to descend further, thus leading to communicating syringomyelia. Over half these patients have a history of difficult labour and it is possible that birth injury may be a factor even where birth is regarded as normal.

Adult↗
PubMed1996

Syringomyelia as a cause of body hypertrophy.

BACKGROUND: Among 26 patients with communicating syringomyelia who came to our out-patient clinic from April, 1989, to March, 1995, three (11.5%) had hypertrophy in limbs, hands, or feet. One had crossed hypertrophy. We considered the possibility that syringomyelia caused body hypertrophy. METHODS: We searched MEDLINE for articles which mention body asymmetry or hypertrophy, and examined the findings in our own patients. FINDINGS: The site of hypertrophy in our three patients coincided with the site of the neurological and magnetic resonance imaging findings. In addition, the horizontal and vertical location of the syrinx corresponded with the site of all four hypertrophic limbs. We located ten articles in which a diagnosis of syringomyelia was made, and five in which other diagnoses were made. INTERPRETATION: From studying our patients as well as those previously reported, we speculate that some types of body hypertrophy are due to damage, accompanied by stimulation, of the sympathetic neurons in the ipsilateral lateral horn of the spinal cord. Although there are many causes of hypertrophy, we suggest that the possibility of syringomyelia be investigated in patients with body hypertrophy, especially in those with any accompanying neurological abnormality.

Adult↗
PubMed2002

Syrinx size and duration of symptoms predict the pace of progressive myelopathy: retrospective analysis of 103 unoperated cases with craniocervical junction malformations and syringomyelia.

OBJECTIVE: the clinical course of syringomyelia associated with craniocervical junction abnormalities is variable. About half of the unoperated patients have benign course. This is difficult to explain on the basis of the present pathogenetic theories. Therefore, to understand the mechanism of syrinx progression better, we studied the value of syrinx size, syrinx morphology, and the syrinx/spinal cord size ratio in predicting the rate of progression and the severity of myelopathy in these patients. METHODS: 103 adult patients with syringomyelia associated with Chiari 1 malformation and/or radiographic signs of underdeveloped posterior cranial fossa were clinically and MRI examined. Patients were subdivided according to the type of disease progression. Severity of neurological deficits, and MRI features (the extent of cerebellar tonsillar displacement, anteroposterior diameter of cavities, the spinal cord and cyst/cord ratio and the shape of cavities) were measured. RESULTS: a significant relationship was found between the mid-sagittal diameter of the syrinxes and the type of disease course; patients with short duration and rapid progression had the largest diameters of cavities, whereas patients with longer duration as well as with slow rate of progression had smaller diameters (chi(2)=28.90, P<0.05; chi(2)=29.89, P<0.01; r=-0.24, P<0.05). In addition, a correlation was found between the anteroposterior diameter of the spinal cord and cyst/cord ratio and the disease duration (r=0.52, P<0.05 and r=0.48, P<0.05, respectively). CONCLUSIONS: the initial years for the development of symptomatic syringomyelia associated with CCJ malformations are characterized by relatively rapid clinical progression accompanied with distended cavities. In the later periods of unoperated syringomyelia, some patients show delay in the progress of syrinxes accompanied with collapse of cavities, probably either due to a spontaneous formation of drainage between the syrinx and the subarachnoidal space, or due to the restoration of abnormal CSF dynamics at the CCJ level.

Adult↗
PubMed2000

Syringomyelia presenting as ulnar neuropathy at the elbow.

OBJECTIVES: Syringomyelia may present with confusing, unilateral patterns of segmental muscle involvement and dissociated sensory loss. The objective of this study was to report a patient with Chiari malformation type 1 (CM1) and syringomyelia who had an unusual presentation suggesting ulnar neuropathy at the elbow. RESULTS: A 24-year-old woman presented with clinical evidence of ulnar neuropathy at the elbow except that there was disproportionate abductor digiti mini (ADM) atrophy and weakness, equivocal ipsilateral abductor pollicis brevis weakness and hyporeflexia in both arms. Nerve conduction studies revealed marked amplitude reduction of the left ulnar ADM-compound muscle action potential (ADM-CMAP) with a normal first dorsal interosseous-CMAP amplitude, no focal slowing or conduction block, and a normal ulnar sensory response amplitude. Electromyography (EMG) showed multi-segmental, left C7-T1 fibrillations and chronic reinnervation changes. Magnetic resonance imaging (MRI) of the cervical spine demonstrated CM1 and syringomyelia. CONCLUSIONS: Syringomyelia may clinically mimic ulnar neuropathy at the elbow.

Adult↗