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PubMed1991

Percutaneous thecoperitoneal shunt for syringomyelia. Report of three cases.

Between January and April, 1990, three consecutive cases of syringomyelia were treated by percutaneous placement of thecoperitoneal shunts. Two of these patients had undergone craniovertebral decompression earlier at other centers and the third was treated primarily by a thecoperitoneal shunt. In each case, the syrinx was associated with Chiari I malformation, although the clinical presentation was due to a myelopathy. All three patients obtained unequivocal benefit from this simple procedure. Postoperative magnetic resonance images showed considerable shrinkage of the cysts corresponding with clinical improvement.

Adult↗
PubMed1992

Significance of positive Queckenstedt test in patients with syringomyelia associated with Arnold-Chiari malformations.

Ten patients with syringomyelia associated with Arnold-Chiari Type I malformations were evaluated. In each patient, a manometric Queckenstedt test was performed with the neck in various positions. No patient showed evidence of a block to the flow of cerebrospinal fluid (CSF) with the neck in the extended position; however, all showed a complete CSF block with the neck in a flexed position. Posterior fossa decompression with a C1-2 laminectomy was performed in nine cases, after which Queckenstedt test demonstrated free CSF communication in all nine with the neck in extension, in a neutral position, and in flexion. Postoperative magnetic resonance imaging showed shrinkage of the syrinx in the patients who underwent surgery. It is suggested that obstruction of the CSF pathway at the foramen magnum produced by neck movement is of importance in the formation and progression of a syrinx.

Adult↗
PubMed1995

Symptomatic syringomyelia following surgery to treat retethering of lipomyelomeningoceles.

The authors report the cases of three children in whom symptomatic syringomyelia occurred de novo following an operation to relieve retethering of a previously treated lipomyelomeningocele. No patient had a Chiari malformation. In two cases, magnetic resonance imaging performed before the first operation did not show a syrinx. At the time of surgery to relieve retethering, it was discovered that one of these patients had a minor degree of terminal hydromyelia and the other had a prominent central canal within the conus medullaris. The third patient was initially studied by means of myelography, which gave no indication of a syrinx, and one was not found at the time of the surgery to release the retethering. Neurological deficits appeared abruptly within several months of operation in two children, and insidiously after 12 to 18 months in the other symptomatic individual. In all three cases, the syrinx involved the distal spinal cord adjacent to the site of the lipoma. Treatment consisted of syringosubarachnoid shunting, which arrested the progression of deficits but only partially reversed them. The details of each case are presented and the possible mechanism of syrinx formation discussed. Early recognition and treatment of this unusual but important problem are emphasized.

Child↗
PubMed1995

Acquired Chiari I malformation and syringomyelia associated with bilateral chronic subdural hematoma. Case report.

The authors report a case of bilateral chronic subdural hematoma in a 25-year-old woman who had occipital and neck pain. Magnetic resonance imaging revealed progressive caudal descent of the cerebellar tonsils (acquired Chiari I malformation) and a large eccentric syrinx in the spinal cord from the C3-T7 levels. Spontaneous disappearance of the chronic subdural hematomas resulted in radiographic resolution of both lesions, as well as clinical improvement. Theories of syringomyelia formation, the relationship to acquired Chiari I malformation, and the implications of this case are discussed.

Adult↗
PubMed1996

Morphological study of experimental syringomyelia with kaolin-induced hydrocephalus in a canine model.

In this morphological study the authors investigated whether spinal cord cavitation, produced in young mongrel dogs that had been rendered hydrocephalic by cisternal injection of kaolin, consists of a dilated central canal or intramedullary cavities. Hydrocephalus was noted in 50 of 56 dogs treated with kaolin. Of the 50 hydrocephalic young dogs, 29 were shown to have central canal dilation that was prominent at the thoracic level and 21 to have cervical intramedullary cavities in the posterior column and/or the posterior horn. In 11 dogs from the latter group these cavities were demonstrated to have no communication with the central canal. This finding could not be explained by the hydrodynamic theory. On histopathological examination, myelomalacia and hemorrhagic infarction following ventricular shunting were noted adjacent to the cervical cavities, which suggested vascular impairment. A perfusion study revealed insufficient blood flow within the cervical cord at the level of the intramedullary cavities. A close correlation between the vascular insufficiency of the cervical cord and the pressure cone resulting from significant hydrocephalus was observed. The latter may cause cervicomedullary compression at the foramen magnum, affecting the venous drainage of the cervical cord below that level, resulting in intramedullary cavitation. Accordingly, vascular impairment was thought to play a significant role in the development of cervical syrinx formation in our kaolin model. The current results may provide a reasonable explanation for the formation of noncommunicating cervical syringomyelia in Chiari I malformation.

Animals↗
PubMed1996

Deformation of the cervicomedullary junction and spinal cord in a surgically treated adult Chiari I hindbrain hernia associated with syringomyelia: a magnetic resonance microscopic and neuropathological study. Case report.

The lower brainstem and cervical spinal cord from an ordinarily treated case of Chiari Type I hindbrain hernia associated with syringomyelia was examined using high-resolution magnetic resonance microscopy and standard neuropathological techniques. Magnetic resonance microscopy allows total screening and visualizes the disturbed internal and external microanatomy in the three orthogonal planes with the resolution of low-power optical microscopy. An additional advantage is the in situ visualization of the shunts. Afterwards the intact specimen is still available for microscopic examination. Part of the deformation of the medulla is caused by chronic tonsillar compression and molding inside the foramen magnum. Other anomalies, such as atrophy caused by demyelination, elongation, and unusual disturbances at the level of the trigeminal and solitary nuclear complexes contribute to the deformation. At the level of the syrinx-free upper part of the cervical cord, anomalies of the dorsal root and the dorsal horn are demonstrated.

Arnold-Chiari Malformation↗
PubMed1997

Treatment of syringomyelia associated with arachnoid scarring caused by arachnoiditis or trauma.

The authors conducted a retrospective study of 107 patients treated for syringomyelia associated with arachnoid scarring between 1976 and 1995 at the Departments of Neurosurgery at the Nordstadt Hospital in Hannover, Germany, and the University of California in Los Angeles, California. Twenty-nine patients have not been surgically treated to date because of their stable neurological status. Seventy-eight patients with progressive neurological deficits underwent a total of 121 surgical procedures and were followed for a mean period of 32 (+/- 37) months. All patients demonstrated arachnoid scarring at a level close to the syrinx. In 52 patients the arachnoid scarring was related to spinal trauma, whereas 55 had no history of trauma and developed arachnoid scarring was a result of an inflammatory reaction. Of these, 15 patients had undergone intradural surgery, eight had suffered from spinal meningitis, three had undergone peridural anesthesia, and one each presented with a history of osteomyelitis, spondylodiscitis, and subarachnoid hemorrhage. No obvious cause for the inflammatory reaction resulting in arachnoid scarring could be ascertained for the remaining 26 patients. The postoperative neurological outcome correlated with the severity of arachnoid pathology and the type of surgery performed. Shunting of the syrinx to the subarachnoid, pleural, or peritoneal cavity was associated with recurrence rates of 92% and 100% for focal and extensive scarring, respectively. Successful long-term management of the syrinx required microsurgical dissection of the arachnoid scar and decompression of the subarachnoid space with a fascia lata graft. This operation stabilized the preoperative progressive neurological course in 83% of patients with a focal arachnoid scar. For patients with extensive arachnoid scarring over multiple spinal levels or after previous surgery, clinical stabilization was achieved in only 17% with this technique.

Adult↗
PubMed2007

Immediate resolution of tonsillar herniation and severe cervicothoracic syringomyelia after third ventriculostomy for hydrocephalus caused by a brainstem tumor. Case report.

The authors report the case of a patient who harbored a large intrinsic brainstem tumor associated with tonsillar herniation, hydrocephalus, and severe cervicothoracic syringomyelia. This 15-year-old girl had a 2-month history of progressively worsening headaches. Magnetic resonance imaging showed a large tumor in the dorsolateral brainstem accompanied by hydrocephalus as well as a cervicothoracic syrinx extending to the level of T-8. She underwent endoscopic third ventriculostomy that resulted in a marked decrease in the ventricle size as well as complete resolution of the syrinx and tonsillar herniation within 4 days. Clinical and neuroimaging findings of the case along with a pertinent literature review are presented.

Adolescent↗
PubMed2005

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.

Adult↗
PubMedDate not supplied

[Type I Chiari malformation coexisting with syringomyelia: pathogenesis, treatment choice and prognostic factors for remote outcome in the light of own case].

The Chiari malformation, condition characterized by herniation of posterior fossa contents below the level of foramen magnum coexists very often with solitary or multiple syringomyelic cysts in spinal cord. The authors, on the basis of current opinions presented in literature and an own case present considerations concerning pathogenesis, operative procedures and prognostic factures which are of value in prediction of long-term outcome in patients which Chiari type I-Syringomyelia complex. The presence or absence of three preoperative signs as: muscular atrophy, ataxia and scoliosis facilitate, with confidence of 95%, prediction of long-term postoperative result. Preoperative incidence of all three above signs is much less favourable for outcome. In the presented case all unfavourable signs were observed preoperatively and, despite of good MRI-result, long-term outcome was only fair.

Adult↗
PubMed1999

[Syringomyelia and Chiari abnormality in the adult. Analysis of the results of a cooperative series of 285 cases].

This chapter discusses the retrospective data found in 285 patients with syringomyelia associated with Chiari abnormality and collected from 18 neurosurgical departments. A pre and postoperative MRI study and a minimum follow up of at least 2 years were required. A scale of severity was fixed and tested before and after treatment. The size of the cyst, the degree of the foraminal obstruction were analyzed. The mean age at diagnosis was about 39 years and the duration of symptoms about 6.7 years. Sensory disorders were present in 91% of cases, pain in 66% and motor deficit in about 60%. According to our functional classification, the majority of our patients were moderately disabled and only 10.8% showed a severe impotence. Results of the two major surgical procedures, foramen magnum decompression (FMD) (88% of cases) and cyst shunting procedures (SP) (32% of cases) were evaluated with a mean follow-up period of 6.7 years (ranged from 2 to 14 years). Better clinical and morphological results (87% of stabilization or improvement for FMD versus 71% for SP) were obtained by FMD procedure comparing to SP, with the same rate of complications.

Adult↗
PubMed1999

[Post-traumatic syringomyelia].

Clinical and neuroradiological data were recorded in a series of 73 spinal cord injured patients (33 in Nantes, 40 in Paris-Bicêtre) in whom a post traumatic syringomyelia (PTS) developed. These findings and a review of the literature allow to point out some of the main characteristics in such a pathology. Clinical symptoms are frequent, the commonest of them are pain and sensory loss but any alteration of the neurological status after spinal cord injury has to be considered. Magnetic resonance imaging (MRI), sagittal and axial T1 and T2 weighted images, confirms the diagnosis of syrinx (area with the same signal intensity as CSF extending beyond the site of the initial lesion at least on 2 vertebral segments). MRI allows the diagnosis when it is performed in the follow up of asymptomatic patients. So PTS is not infrequent in spinal cord injured patients, for some of them in the first year after the trauma. The highest incidence is found in patients with complete thoracic lesions. Pathophysiology and surgical management have to take into account post traumatic residual stenosis of the vertebral canal.

Adult↗
PubMed1999

[Non-traumatic arachnoiditis and syringomyelia. A series of 32 cases].

We conducted a retrospective study of 32 patients treated for syringomyelia associated with non-traumatic arachnoid scarring. We selected the cases with documented history of arachnoiditis with pre and post-operative clinical evaluation of the neurological status and anatomical study on MRI with a minimal follow-up of one year. Extensive arachnoid scarring (Group I) was noted in 18 cases, after spinal meningitis in 15 cases (tuberculosis in 9 cases, listeria in 3 cases, pyogenic meningitis in 3 cases), subarachnoid hemorrhage in 3 cases. Focal arachnoid scarring (Group II) occurred in 10 cases, related to spinal surgery in 5 cases (meningiomas: 2, neurinomas: 2, thoracic discectomy: 1), to peridural anesthesia in 1 case, thoracic disc herniation in 1 case, Pott's disease in 1 case, no obvious cause in 2 cases. Basal arachnoid scarring without hindbrain herniation (Group III) was associated with birth injuries in 4 cases. Shunting of the syrinx to the subarachnoid or peritoneal cavity was associated with a recurrence rate of 60% whereas microsurgical dissection of the arachnoid scar and decompression of the subarachnoid space with a recurrence rate of 33%, with a mean follow-up period of 28 months. Successful long-term management of the syrinx was associated with basal or focal spinal arachnoid scarring, no history of spinal meningitis, microsurgical dissection of the arachnoid scar and decompression of the subarachnoid space.

Adult↗
PubMed1997

[Clinical observations on syringomyelia treated by syrinx-peritoneal shunting with "T" tube].

We 22 cases of syringomyelia were verified by MR and treated by syrinx-peritoneal shunting with "T" tube. After the operation, the patients were reexamined by MR and B ultrasound and confirmed that their shunting was functional. Symptoms of the patients disappeared. But four patients got slight numbness in one leg or at the saddle area. One patient suffered from a difficult movement in one hand. With the exception of 6 patients, 16 showed that both of the newly and original symptoms improved significantly. Follow up for 2 years with regular MR and B ultrasound reexaminations was made. The patients resumed their work and study. MR and B ultrasound reexaminations showed that all of syrinxes collapsed significantly as the same as that after the operation immediately. These mean that this kind of operation is useful and effective for most syringomeylias.

Adolescent↗
PubMed2000

Non-hindbrain-related syringomyelia. Obstruction of the subarachnoid space and the central canal in rats. An experimental study.

BACKGROUND: The aim is to determine the mechanism of non-hindbrain-related syringomyelia in experimental models. The effects of obstruction of central canal and subarachnoid space on occurrence of cavities were discussed. METHODS: 31 Sprague-Dawley rats were used with eight (Group D) as a control. In 10 rats (Group A) 1.5 microl kaolin was microinjected into the dorsal columns and central gray matter of the spinal cord at the level of Th6-10. In 10 rats (Group B) 0.1 cc kaolin was injected into the subarachnoid space at the same level. In 3 rats (Group C), 1.5 microl kaolin was administered into both dorsal midline of the spinal cord and the subarachnoid space. RESULTS: In Group A, histological examination revealed cystic cavity and dilatation of the central canal in five rats; denuded ependymal line and multicystic formations in ependymal and periependymal areas in seven rats. In Group B, denuded ependymal line in three rats and microcystic formations in ependymal and periependymal areas in four rats were revealed. In Group C, there were microcystic formations in two rats and syrinx cavity in one rat. CONCLUSIONS: Developments leading to occurrence of cavities are focused on the central canal in all groups. These models indicate that the CSF-flow is from the subarachnoid space to the central canal leading to changes of cavities. In cases of obstruction of the subarachnoid space or the central canal, the occurrence of syrinx cavity initially is due to increased CSF (cerebrospinal fluid) pressure in the central canal. Flow changes in spinal cord is indicated by this study.

Animals↗
PubMed2001

Orofacial pain as the sole manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformation.

This is a case report of a male patient who presented with orofacial pain for a year as the only manifestation of syringobulbia-syringomyelia associated with Arnold-Chiari malformation. This article places emphasis on the clinical presentation and possible differential diagnoses. The pain was continuous and affected the left side of the face. It was exacerbated by coughing and physical effort, possibly as a consequence of an increase in intracranial pressure. Paroxysmal pain crises developed over this background of continuous pain, compatible with neurogenic trigeminal pain of the left second branch, together with pain episodes similar to cluster headache on the same side. The symptoms were resolved following neurosurgical management with amplification of the foramen magnum.

Arnold-Chiari Malformation↗
PubMed2001

[Acute onset syringomyelia: two cases].

Two cases of acute presentation of cervical syringomyelia associated with Chiari malformation are reported. In the first case, dysphagia was the main symptom; the second patient had symptoms suggesting acute brain stem ischemia. The mechanism of this acute onset of the illness is discussed.

Acute Disease↗