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PubMed1999

Pediatric syringomyelia with chiari malformation: its clinical characteristics and surgical outcomes.

BACKGROUND: Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia. METHODS: We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism. RESULTS: Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration. CONCLUSIONS: Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.

Adolescent↗
PubMed1995

Cerebrospinal fluid drainage for syringomyelia.

Twenty-eight years of experience with syringomyelia of various causes in the syringomyelia clinic at the Midland Centre for Neurosurgery and Neurology has provided a database of 723 patients, mostly adults, with either hindbrain herniation, syringomyelia or both. Treatment of syringomyelia by drainage has never been the optimum primary treatment on the basis that the cavity is usually secondary to some other disturbance of the cerebrospinal fluid pathways. Over this period 73 patients had either syrinx drainage (56 syringopleural, 14 syringo-subarachnoid shunts) or other procedure such as myelotomy and cord transection. Ten years after operation only 53.5% and 50% of these two groups respectively continued to remain clinically stable. A complication rate of 15.7% included fatal haemorrhage, infection and displacement of drains. At second operation or necropsy at least 5% of shunts were found to be blocked. All the shunts were inserted without a valve and the lowering of the intrasyrinx pressure has therefore been energetic when the lower end of the drainage tube has been taken to the pleural or peritoneal cavities. This produced collapse of the cord cavities around the tip of the drainage tube and increased the likelihood of blockage. If the mechanisms which were responsible for the syringomyelia were still operative then recurrence was likely to occur alongside the drainage tube leaving the tube immured in the wall of the syrinx cavity. When hydrocephalus was present, in addition treatment of the hydrocephalus by a valved shunt to the peritoneum or to the right atrium was often effective in improving the syringomyelia. This treatment has also been used in patients without hydrocephalus. The mechanisms of improvement were unclear but this treatment stratagem has nevertheless been employed in 45 cases. In 30 out of these 45 cases the drainage of cerebrospinal fluid from outside the syrinx cavities seemed to be worthwhile; 10 patients suffered some form of complication, most of which were reversible; 8 patients were worse following surgery. It is concluded that both drainage of the syringomyelia cavity and also extra-syrinx drainage may have a place in the management of difficult syringomyelia problems although the primary treatment should be to establish the patency of CSF pathways in both hindbrain-related and non-hindbrain-related cases.

Adult↗
PubMed2006

Ethnic differences in syringomyelia in New Zealand.

OBJECTIVE: To determine the prevalence of syringomyelia in a defined population in New Zealand and measure the prevalence of syringomyelia in the three main ethnic groups (Maori, Pacific people and Caucasians/others) living in this region. METHODS: A retrospective study of all confirmed cases of syringomyelia diagnosed in residents of northern New Zealand from 1961 to 2003. RESULTS: In all, syringomyelia was diagnosed in 137 patients. The mean age at onset of symptoms was 27.5 years and mean age at diagnosis was 32.6 years. The incidence of new cases increased from 0.76/100,000 a year between 1962 and 1971 to 4.70/100,000 a year by 1992-2001. The prevalence of syringomyelia in 2003 was 8.2/100,000 people: 5.4/100,000 in Caucasians or others, 15.4/100,000 in Maori and 18.4/100,000 in Pacific people (chi2 = 37.0, p<0.0001). Syringomyelia was more often associated with an isolated Chiari I malformation in Pacific people (84.4%) as compared with 42.9% of Maori and 38.2% of Caucasians or others (chi2 = 62.3, p<0.0001). CONCLUSION: The prevalence of syringomyelia is higher in northern New Zealand than in studies carried out before the advent of magnetic resonance imaging. The prevalence is particularly high in Maori and Pacific people. The cause of the ethnic differences in the prevalence of syringomyelia identified in this study is unexplained and warrants further investigation.

Adolescent↗
PubMed2003

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.

Adult↗
PubMed1989

[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.

Arnold-Chiari Malformation↗
PubMed1995

[Surgical treatment of syringomyelia].

Eighty cases of surgically treated syringomyelia were retrospectively reviewed. The cases were classified into following 4 types, type 1: syringomyelia with Chiari malformation (54 cases), type 2: syringomyelia with basal arachnoiditis (15 cases), type 3: syringomyelia with an obstruction of the foramen Magendie (1 case), and type 4: syringomyelia with spinal arachnoiditis (14 cases). Foramen magnum decompression (FMD) was performed in patients with type 1, in type 2 fourth ventricle-subarachnoid shunt was additionally performed. Gardner's operation was performed in patients with type 3. Syrinx-peritoneal shunt was performed in patients with type 4. Surgical procedures for syringomyelia which we selected were thought to be appropriate, based on postoperative syrinx collapse rate in MRI. However, postoperative clinical course was much different in each type of syringomyelia after the collapse of syrinx had been equally achieved. Neurological disorders were stopped in deterioration after surgery in all cases of type 1. However, motor weakness was still deteriorated in half cases of type 2, and in 60% of type 4. When clinical severity of the patients with type 1 and 2, based on the distribution of dissociated sensory loss and motor weakness, were classified into 5 grades. The rate of improvement of patient's symptoms and signs was higher in the lower grades. We concluded that a surgical treatment for syringomyelia was essentially a preventive one, therefore it should be done in early stage of disorders.

Arachnoiditis↗
PubMed2000

Evaluation of syringomyelia with three-dimensional constructive interference in a steady state (CISS) sequence.

The purpose of this study was to evaluate a three-dimensional (3D) constructive interference in steady state (CISS) sequence in the assessment of syringomyelia. Eleven patients with syringomyelia were prospectively studied with magnetic resonance imaging. All patients underwent sagittal imaging with T1- and T2-weighted spin-echo (SE), and 3D-CISS sequences. The SE and 3D-CISS images, as well as multiplanar reconstruction (MPR) images of the 3D-CISS sequence, were analyzed with regard to image quality, degree of artifacts, visualization of the extent and internal structure of the syringomyelia, and contrast-to-noise ratio (CNR) of the fluid within the syringomyelia. Contrast between the spinal cord and cerebrospinal fluid (CSF), as well as delineation was significantly poorer for the T1-weighted SE sequence than for the 3D-CISS sequence (P < 0.01), while there was no significant difference between the T2-weighted SE sequence and the 3D-CISS sequence. Artifacts induced by CSF flow were significantly more for the T2-weighted SE sequence than for the 3D-CISS sequence (P < 0.01). Although the extent of syringomyelia was delineated equally among the three sequences in 9 of 11 patients, it was better for the 3D-CISS sequence than for the SE sequences in the remaining two. Septation and communication between the cavities were best detected by the 3D-CISS MPR images. The CNR of the 3D-CISS sequence was significantly higher than that of the SE sequence (P < 0.01). The 3D-CISS sequence demonstrates the extent and internal structures of syringomyelia better than conventional SE sequences and should be added to SE sequences in the evaluation of syringomyelia.

Adult↗
PubMed2006

Tonsillar herniation and cervical syringomyelia in association with posterior fossa tumors in children: a case-based update.

BACKGROUND: Posterior fossa tumors most commonly occur in children. They frequently present with raised intracranial pressure and may have tonsillar herniation. The symptomatology is predominantly directed towards the tumor. The occurrence of syringomyelia in such cases is most unusual. Symptomatic syringomyelia is rare. CASE REPORT: We describe a case of a pilocytic astrocytoma of the cerebellum in a 13-year-old girl who presented with clinical features of progressively worsening raised intracranial pressure and secondary tonsillar herniation and cervical syringomyelia. Magnetic resonance (MR) imaging showed a large midline inhomogenously enhancing vermian tumor causing moderate obstructive hydrocephalus. In addition, the cerebellar tonsils herniated down to the C2 level, and there was a centrally located syrinx from C2-T1. The tumor was resected through a suboccipital craniectomy. At follow-up after 3 months, MR imaging demonstrated total resolution of tonsillar herniation and cervical syringomyelia. CONCLUSIONS: The occurrence of tonsillar herniation and syringomyelia in association with a slow growing benign tumor like pilocytic astrocytoma of the cerebellum is uncommon. The blockade of normal cerebrospinal fluid circulation pathway at the foramen magnum is the crucial factor. Surgical extirpation of the tumor restores the normal cerebrospinal fluid circulation at the foramen magnum and produces an excellent outcome. The need for an additional surgical procedure for treatment of associated tonsillar herniation and syringomyelia can be avoided. Volumetric assessment of the posterior fossa may provide a better understanding of the pathophysiology of syringomyelia in such patients.

Adolescent↗
PubMed2000

Intraoperative measurement of spinal cord blood flow in syringomyelia.

The role of spinal cord ischemia in the pathophysiology of syringomyelia remains undetermined. Previous reports in the literature suggest that shunting of syringes can improve spinal cord blood flow. In order to determine the effects of syrinx decompression on spinal cord blood flow in patients with syringomyelia, we prospectively measured regional spinal cord blood flow (RSCBF) intraoperatively pre and post shunting in patients with symptomatic syringomyelia using laser doppler flowmetry. Six patients with MRI documented syringomyelia were studied (three with Arnold Chiari I malformation and associated syrinx and three with post-traumatic syringomyelia). Surgery was performed on all patients with either a syringopleural or syringoperitoneal shunt. Laser doppler blood flow and somatosensory evoked potentials were monitored prior to myelotomy and after shunt insertion. Results indicate that there was a significant increase in RSCBF after decompression of the syrinx. This study supports the hypothesis that spinal cord ischemia is important in the pathophysiology of syringomyelia and confirms previous reports in the literature regarding RSCBF in syringomyelia.

Adult↗
PubMed2002

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.

Adolescent↗
PubMed1997

Structural scoliosis model in dogs with experimentally induced syringomyelia.

STUDY DESIGN: Animal scoliosis model associated with syringomyelia. OBJECTIVE: To investigate the pathogenesis of scoliosis produced in dogs with kaolin-induced syringomyelia. SUMMARY OF BACKGROUND DATA: Kaolin injected into the cisterna magna produces basilar arachnoiditis, leading to hydrocephalus and syringomyelia. There have been no reports on scoliosis associated with kaolin-induced syringomyelia. METHODS: Kaolin was injected percutaneously into the cisterna magna of 11 beagles 6-8 weeks after birth. Roentgenograms, computed tomography, and magnetic resonance imaging were obtained. The spinal cord and the paraspinal muscles were examined histologically. Structural changes of the vertebral column were analyzed with calcein and tetracycline labeling. RESULTS: Hydrocephalus occurred in nine dogs. A communicating syringomyelia appeared in five dogs. Mild scoliosis developed in two dogs, and severe cervical scoliosis in one dog. In the syringomyelia cases, acute or subacute inflammatory changes were found in the spinal cord. Damage of the anterior and posterior horn cells was more marked in the scoliotic animals than in the nonscoliotic animals. In three of the syringomyelia cases, including two scoliosis cases, the paraspinal muscles revealed neurogenic changes. The deformed vertebrae appeared to diminish rather than to increase the deformity in severe scoliosis. CONCLUSION: The exact mechanism of the development of scoliosis could not be identified, although an etiologic relation with malfunction of the central nervous system was noted. This model may be useful to study scoliosis experimentally.

Animals↗
PubMed1997

Chiari I malformation associated with syringomyelia and scoliosis.

STUDY DESIGN: A retrospective review of a series of 12 children who underwent suboccipital foraminotomy and duroplasty for Chiari I malformation. OBJECTIVE: To assess the effects of this surgery on associated syringomyelia and scoliosis. SUMMARY OF BACKGROUND DATA: Suboccipital foraminotomy for the treatment of syringomyelia associated with Chiari I malformation was greatly stimulated by Gardner's hydrodynamic theory, and its results proved to be encouraging. However, several authors reported improvement or stabilization of associated scoliosis after this surgery. METHODS: A retrospective review was conducted on 12 patients who underwent suboccipital foraminotomy for Chiari I malformation associated with syringomyelia. Neurologic Impairment, extent of syringomyelia, and severity of associated spinal deformity were assessed preoperatively and at a 4.5-year average follow-up (range, 2.1-12 years). Anomaly of superficial abdominal reflexes was found in all cases, and para or tetraparesis in three cases. Syringomyelia was of variable localization and extent. Scoliosis was present in 7 cases (greater than 40 degrees in 5 cases). RESULTS: Diminution or complete disappearance of syringomyelia was observed in 11 cases, 3 months to 1 year after surgery. Superficial abdominal reflexes anomaly improved in four cases. Minimal neurologic deficit persisted in one case. Scoliosis improved in one case, remained unchanged in one case, and progressed in the five cases with preoperative severe deformity, requiring instrumentation and fusion. CONCLUSIONS: Improvement of syringomyelia and neurologic deficit, observed with suboccipital foraminotomy, supports the theory that abnormal hydrodynamics of the cerebral spinal fluid is most likely to cause these deficits.

Adolescent↗
PubMed2003

Spinal arachnoid cysts associated with syringomyelia: report of two cases and a review of the literature.

We describe two cases of spinal arachnoid cyst associated with syringomyelia and report the clinical results after surgical treatment using excision of the cyst without a shunt operation for the syringomyelia. Case 1 is a 73-year-old woman who presented with a spastic gait and numbness of her bilateral lower extremities. Magnetic resonance imaging (MRI) showed the presence of a spinal arachnoid cyst extending from T3 to T8 and syringomyelia from T8 to T10. The cyst had compressed the spinal cord anteriorly. We excised the cyst without applying a shunt tube for the syringomyelia. Case 2 is a 68-year-old woman who presented with gait disturbance and numbness of her left lower extremity. MRI indicated that the spinal cord had been compressed anteriorly by a spinal arachnoid cyst extending from T10 to T11. Syringomyelia existed just caudal to the cyst at T11. In our surgical treatment, we excised only the cyst. In both cases, neurologic examination after the operation showed amelioration of the condition. Postoperative MRI indicated that the spinal cord had moved to the center, its original position, and the syringomyelia had decreased in size. Conclusively, spinal arachnoid cyst associated with syringomyelia can be treated by simple excision of the cyst without shunting the syrinx if the decompression effect resulting from removal of the cyst is sufficient.

Aged↗
PubMed2003

Sagittal plane deformity in the thoracic spine: a clue to the presence of syringomyelia as a cause of scoliosis.

STUDY DESIGN: A retrospective review of scoliosis radiographs of 93 patients with either idiopathic scoliosis or syringomyelia-associated scoliosis were assessed, defining their sagittal alignment. OBJECTIVE: To validate an observation regarding the absence of Dickson's sagittal deformity of the thoracic spine in patients with syringomyelia-associated scoliosis. SUMMARY OF BACKGROUND DATA: Patients with adolescent idiopathic scoliosis have a classic sagittal deformity. Dickson described that patients with adolescent idiopathic scoliosis (AIS) have an associated lordotic deformity at the apex of their coronal deformity. MATERIALS AND METHODS: Retrospective reviews of standard scoliosis series radiographs of 93 patients with idiopathic or syringomyelia-associated scoliosis from two institutions were compared. Particular attention was given to the lateral radiograph of the spine assessing presence or absence of Dickson's apical lordosis. Nine patients had to be excluded because of inadequate imaging. The study group consisted of 30 patients with scoliosis from TSRH with documented syringomyelia identified between 1985 and 1997. The demographic and radiographic features of this group were compared with those of a control group consisting of a consecutive series of 54 patients from HSS with adolescent idiopathic and normal MRI. The groups were comparable for age (mean age: control 13 y; syrinx 12 y) and curve pattern but differed in curve magnitude (mean Cobb: control 50 degrees; syrinx 40 degrees ). RESULTS: Apical lordosis was present in 97% of patient with AIS and a normal MRI but was absent in 75% of patients with syringomyelia-associated scoliosis (P < 0.0001). The results also confirmed that male patients with scoliosis and left-side curves have a predisposition to having a syringomyelia (P < 0.0001). CONCLUSIONS: Sagittal plane deformity in scoliosis can be an indicator of the presence of a syringomyelia. Our results reinforce the necessity of assessing sagittal plane deformity when treating scoliosis. If apical lordotic deformity is absent, a diagnosis of idiopathic scoliosis should be made with caution.

Adolescent↗
PubMed2002

Syringomyelia without hindbrain herniation: tight cisterna magna. Report of four cases and a review of the literature.

Idiopathic syringomyelia, which is not associated with any definite pathogenic lesions, has been treated mainly by shunting of the syrinx and rarely by craniocervical decompression. The authors report four cases of syringomyelia thought to be idiopathic syringomyelia but treated by craniocervical decompression with favorable results. Syringomyelia was present without hindbrain herniation. In such cases, the subarachnoid space anterior to the brainstem at the level of the foramen magnum is usually open but the cisterna magna is impacted by the tonsils, a condition the authors term "tight cisterna magna." All patients underwent foramen magnum decompression and C-1 laminectomy, and the outer layer of the dura was peeled off. Further intradural exploration was performed when outflow of cerebrospinal fluid (CSF) from the fourth ventricle was deemed to be insufficient. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in three patients, and a reduction in ventricle size was shown in two. Syringomyelia associated with tight cisterna magna should not be classified as idiopathic syringomyelia; rather, it belongs to the category of organic syringomyelia such as Chiari malformation. A possible pathogenesis of cavitation is obstruction of the CSF outflow from the foramen of Magendie, and the cavity may be a communicating dilation of the central canal. Ventricular dilation may depend on the extent to which CSF drainage is impaired from the foramina of Luschka. These cavities may respond to craniocervical decompression if it results in sufficient CSF outflow from the foramen of Magendie, even in cases with concomitant hydrocephalus.

Adolescent↗
PubMed1993

Syringomyelia: a brief review of ontogenetic, experimental and clinical aspects.

The generally accepted definition of syringomyelia is that it is a chronically progressive illness characterized by the presence of cavities or syrinxes in the spinal cord. As manifold as the terminology of syringomyelia are the hypotheses of the etiology. Nowadays with MRI without and with gadolinium it is possible to recognize intramedullar cavities safely, the MR especially the cine-MR provides information on pathophysiological details of the flow and intracavitary pressure dependent pulsations of the CSF. Animal models and the findings of own experimental studies have enabled us to study a form of syringomyelia which very closely resembles that brought about by dysrhaphic malformations in the human being and to examine the effectiveness of certain types of surgical therapy. In this paper the term syringomyelia is only used for dysrhaphic cavities in the medulla. After our experience with 61 patients with syringomyelia now we perform the operative decompression of the craniocervical transition as the first step in the operative treatment of the progressive syringomyelia combined with severe craniocervical malformations. In cases with insufficient treatment response we suggest the syringoarachnoid shunting of persisting large intramedullar cavities.

Animals↗
PubMed2004

International survey on the management of Chiari I malformation and syringomyelia.

INTRODUCTION: The availability of magnetic resonance imaging (MRI) has resulted in an increasing number of asymptomatic, minimally symptomatic, and doubtfully symptomatic patients being diagnosed with a Chiari I malformation with or without syringomyelia. In an attempt to clarify how neurosurgeons manage these clinical problems, an international survey on the Chiari I malformation and related syringomyelia was undertaken. METHOD: A questionnaire on the expected natural course of the disease and on aspects of the surgical technique for a number of hypothetical cases relating to Chiari I malformation with and without syringomyelia was used to survey Pediatric Neurosurgeons worldwide. RESULTS: Of 246 questionnaires distributed, 76 (30.8%) were completed and returned. There was a consensus that no operation should be carried out in asymptomatic patients with a Chiari I malformation, unless there is associated syringomyelia. There was a consensus that decompression of the Chiari malformation should be performed in patients with scoliosis when syringomyelia is present, and the majority decompressed the Chiari malformation in scoliotic patients even in the absence of syringomyelia. Suboccipital decompression was the standard surgical procedure for Chiari I malformations. The majority of respondents favored routine dural opening at surgery and closure with a pericranial or synthetic patch graft. In the case of a persistent or progressive syrinx after suboccipital decompression, the majority recommended shunting of the syrinx to the subarachnoid space or to the pleural cavity. CONCLUSION: There continues to be much variation in the management of the Chiari I malformation.

Arnold-Chiari Malformation↗
PubMed1989

[MRT in primary and tumor-induced syringomyelia].

The MRI findings in 26 patients with primary syringomyelia and eight patients with a syrinx due to an intramedullary tumour were studied retrospectively. In general, the syrinx in the tumour cases resembled the appearance of primary syringomyelia. However, a specific and sensitive sign of tumour-induced syringomyelia was irregularity of the spinal cord close to the tumour (eight cases of eight), eccentric position of the lumen (seven of eight), and a diffuse increase of signal intensity on T2-weighted images (eight of eight). Primary syringomyelia also showed increased signal intensity on T2-weighted images outside the lumen, but this was closely related to the cavity. The use of Gd-DTPA improved demarcation of the tumour from the syrinx in all cases. Widening of the spinal canal and ectopia of cerebellar tonsils was a typical finding in primary syringomyelia. MRI proved a reliable method for distinguishing between a tumour-induced syrinx and primary syringomyelia.

Adolescent↗