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PubMed2006

Scoliosis in children with Chiari I-related syringomyelia.

OBJECTIVE: To study the relationship between scoliosis and Chiari I malformation, with reference to the possible role of cranio-vertebral decompression in preventing the need for scoliosis correction. MATERIAL AND METHODS: Out of a total of 36 patients with symptomatic Chiari I, who underwent primary cranio-vertebral decompression by a single paediatric neurosurgeon with an interest in Chiari malformation between 1998 and 2003, 13 had clinically detected scoliosis. Of these, ten had no other structural spine abnormality, which could influence the natural history of scoliosis and were included in this study. RESULTS: In all but one patient, syringomyelia improved significantly after cranio-vertebral decompression. Of the ten patients, eight had levoscoliosis (left convexity), all single curves, and two had curves to the right (both double curves). Six patients did not require corrective scoliosis surgery after cranio-vertebral decompression. The mean Cobb angle for those not requiring scoliosis correction was 29 degrees in contrast to a mean of 76 degrees for those requiring correction [p=0.001, one-way analysis of variance (ANOVA)]. The mean age of patients requiring corrective surgery was 158 months against 125 months for those not requiring correction (p=0.084, one-way ANOVA). These findings were confirmed by multivariate analysis, which also confirmed that symptom duration, syrinx length and site were not significant in predicting the need for corrective surgery following cranio-vertebral decompression. CONCLUSIONS: Cranio-vertebral decompression for Chiari I may prevent the need for corrective scoliosis surgery when performed before the age of ten and below a Cobb angle of 30 degrees.

Adolescent↗
PubMed2000

Expansion of Chiari I-associated syringomyelia after posterior-fossa decompression.

UNLABELLED: Chiari I malformation (CMI) is an abnormality that involves caudal herniation of the cerebellar tonsils into the foramen magnum. CMI has been shown to be closely associated with the development of syringomyelia (SM). OBJECTS: Several theories have emerged to explain the apparent correlation between the existence of CMI with subsequent development of SM. However, the exact mechanism of the evolution of SM is still subject to controversy. We report here the case of a 12-year-old girl admitted to hospital with headache, vomiting, ataxia, and moderate pyramidal signs. METHODS: Radiological evaluation revealed the presence of CMI, accompanied by a small SM. The patient underwent posterior fossa decompression and improved significantly. She was re-admitted 6 months later with clinical evidence of progressive spinal cord dysfunction. MR revealed gross expansion of the syrinx. CONCLUSIONS: This case raises questions regarding the pathophysiology of CMI and its association with SM. The case indicates the need for neurological and radiological follow-up for patients undergoing posterior fossa decompression due to CMI, even for those without an initial syrinx. This is the first report known to us of expansion of a syrinx following decompression of an associated CMI.

Arnold-Chiari Malformation↗
PubMed2000

Syringomyelia in children with primary scoliosis.

The clinical notes of 35 children presenting with scoliosis were reviewed; all 35 had been investigated with MRI. Seven were found to have syringomyelia, and six of these had Chiari malformation. Correction of the syrinx resulted in improvement or stabilisation of the spinal curvature. We recommend that all cases presenting with primary scoliosis should have MRI and should be treated if a syrinx is found.

Arnold-Chiari Malformation↗
PubMed2003

Syringomyelia and the arachnoid web.

Three cases of syringomyelia associated with arachnoid webs are reported. Each patient presented with progressive myelopathy and had thoracic syringes detected on magnetic resonance imaging (MRI). In one patient the web was also visible. At operation a thoracic arachnoid web was found, obstructing the subarachnoid compartment in each patient. One patient had intraoperative ultrasound, which demonstrated caudal web movement with each cardiac systole. The webs were divided and shunts inserted into the syringes. All patients improved clinically, and on follow-up MRI. Arachnoid webs are likely to represent a focal band of arachnoiditis and are difficult to visualise on standard preoperative MR imaging. A reduction in the subarachnoid space compliance with resultant increase in pulse pressure and potentiation of an arterial pulsation driven perivascular flow could explain the associated syringes. Treatment should be aimed at restoring compliance, and involve division of the web with or without shunt insertion.

Adult↗
PubMed2004

Syringomyelia caused by cervical spondylosis.

Syringomyelia is generally associated with Chiari type malformations, spinal tumors, or spinal trauma. Cervical spondylosis is only rarely involved. We here present a case of a 64-year-old woman with severe radicular pain in the right arm and the syringomyelic syndrome. Lateral radiographs of the cervical spine demonstrated spondylotic change at the C4/5 and C6/7 levels, and instability at C4/5. Dynamic magnetic resonance (MR) imaging revealed the spinal cord to be compressed at C5 and C6 with the body in extension, and the syrinx extended from C2 to the Th3 level on sagittal images. It was reduced remarkably after anterior decompression and stabilization at C4/5 and C6/7, and her symptoms also improved after surgery. We concluded that the syrinx in this case might have developed due to craniospinal pressure dissociation caused by intermittent spinal cord compression.

Cervical Vertebrae↗
PubMed1999

Surgical treatment of Chiari I malformation with or without syringomyelia.

102 patients with Chiari I malformation with or without syringomyelia underwent primary reconstructive operation at the craniocervical junction (CCJ). We present here the indications, methods of surgical management with clinical, radiological and operative findings. Here we present the results of a retrospective review. The authors discuss the possible ways of improvement in the results of "hindbrain related" syrngomyelia treatment.

Adolescent↗
PubMed2000

Syringomyelia--as a late complication of tuberculous meningitis.

The aim of this paper is to demonstrate the unusual MR features of thoracic syringomyelia following TB meningitis and to discuss the neurosurgical aspect of the treatment of this rare entity. Four years after a TB meningitis episode, a 30 year-old female patient developed a progressive spastic paraparesis. MR studies revealed multiloculated syrinxes throughout the thoracic cord. She had a syringo-subarachnoid shunt with a silastic "T" tube inserted. On the first postoperative day, she showed a dramatic neurological improvement, but unfortunately her paraparesis progressed to the preoperative level within a month despite diminished size of the syrinxes on the control MRI examination. Two and a half years after the operation the patient complained of having a burning type of central pain, and further deterioration in neurological function. Thoracic spinal MRI examination demonstrated enlarged syringomyelic cavities. At the second operation syringo-peritoneal shunt insertion was performed via right T10-11 hemilaminectomy using a "T" tube. At present, 4 months after the second operation, the patient's neurological examination demonstrated decreased spasticity, and improved strength in the legs compared to the preoperative level. MRI is the first choice of investigation in detecting TB related myelopathy as it provides a greater detail of pathological changes within and around the spinal cord such as syrinx formation and arachnoiditis. The MR findings are also helpful in deciding the management and predicting the outcome. Presence of multifocal loculations and arachnoid adhesions is the likely cause of treatment failures and poor prognosis.

Adult↗
PubMed2005

Syringomyelia, neuropathic arthropathy and rheumatoid arthritis as diagnostic dilemmas in two different cases: confounding factor and true coexistence.

Two patients with neuropathic arthropathy (NA) of hands and wrists due to cervical syringomyelia are described. Articular deformities resembling rheumatoid arthritis (RA) were present in both cases. RA was misdiagnosed in the first case, leading to incorrect treatment with iatrogenic consequences, and there was real coexistence of RA and NA in the second one. Physicians must be aware of this rare condition in the differential diagnosis of wrist and hand deformities.

Arthritis, Rheumatoid↗
PubMed1986

F-responses in syringomyelia.

Motor and sensory nerve conduction and various F-response parameters have been examined in the median and ulnar nerves bilaterally in 22 patients with syringomyelia. Excluding those nerves with isolated peripheral lesions, motor and sensory conduction was normal in the distal nerve segments, except for one subject in whom severe wasting of the muscles was associated with slowed motor velocities. Minimum and/or maximum F-response latencies were increased in one or more nerves in 16 of the 22 cases, which was attributed to disturbed function of anterior horn cells and the intraspinal segment of the motor fibres, or mild subclinical nerve trauma. There was a tendency for F-response amplitude and duration to be increased, probably reflecting the combined effects of spasticity and enlargement of motor units due to reinnervation.

Adult↗
PubMed1990

Tangier disease: central nervous system impairment in a case of syringomyelia-like syndrome.

A neuroradiological investigation of the central nervous system (CNS) in a case of Tangier disease presenting as a syringomyelia-like syndrome is reported. No syringomyelinic cavities were found. However, MRI showed cervical spinal cord atrophy and scattered foci of greater density with T2 weighted images in the white matter of the frontal, parietal and occipital lobes. Cerebral and cervical spinal cord involvement in the course of Tangier disease is now shown for the first time. The authors postulate that the MRI detected alterations are related to the underlying illness.

Brain↗
PubMed1991

Pathology of roots, spinal cord and brainstem in syringomyelia-like syndrome of Tangier disease.

We report here a post-mortem examination of a 46-year-old patient who died after a 23-year-long syringomyelia-like syndrome of Tangier disease. The L5 dorsal root and the superficial peroneal nerve showed fiber loss and lipid vacuole accumulation in Schwann cell cytoplasm. The L5 ventral root had moderate fiber loss without lipid vacuoles. In the cervical roots, fiber loss was intense and there were no foamy Schwann cells. Motor neuron loss was severe in the cervical spinal cord and the facial nerve nucleus and slight at the lumbar level. Under electron microscopy, some neurons of the lower spinal cord showed atypical inclusions. These data suggest that an unknown metabolic defect is responsible for a primary neuronopathy. Lipid accumulation in Schwann cells, resulting from fiber degeneration is probably transient, accounting for the absence of foamy cells in regions with longstanding involvement.

Autopsy↗
PubMed1982

Intraoperative ultrasound imaging of the spinal cord: syringomyelia, cysts, and tumors--a preliminary report.

Dynamic intraoperative imaging of the spinal cord was done using a real-time ultrasound scanner. Prior to opening the dura mater the neurosurgeon is able to "explore" the intradural space and with a 7.5 MHz transducer visualize the normal spinal cord, including the central canal and the dentate ligaments. Anterior and posterior spinal arteries may be seen in certain patients. In syringomyelia the fluid-filled cavities may be visualized with ultrasound and drained or shunted with intraoperative ultrasound guidance. Spinal cord cysts or cystic components of tumors can be precisely identified and drained using ultrasound. Intramedullary tumors can be demonstrated sonographically, as can the location and extent of intradural extramedullary tumors and tumors that have both extradural and intradural components. Experience to date with intraoperative imaging of the spinal cord and with the real-time ultrasound scanner is very encouraging, and in the future it should allow for a more precise characterization of and operative approach to lesions of the spinal cord.

Cysts↗
PubMed1986

Use of the syringoperitoneal shunt in the treatment of syringomyelia.

A syringoperitoneal shunt is a one-way drain that empties cerebrospinal fluid from the pathological syringomyelic cavity into the abdomen. This treatment technique is based on the principle that the signs and symptoms of syringomyelia are caused by insidious interference with the condition of nerve signals in the spinal cord tracts due to either tearing or compression of the tracts by the distended cavity. A series of eight patients suffering from this disorder were operated upon using a syringoperitoneal shunt. The initial results seem to indicate that this technique is especially useful when pain, atrophy, or both dominate the clinical picture. Clinical signs, diagnostic studies, and surgical considerations are discussed.

Adolescent↗
PubMed1987

Syringomyelia after decompressive laminectomy for cervical spondylosis.

We describe a case of cervical spondylotic myelopathy in which deterioration occurred a month after decompressive laminectomy. Syringomyelia was then in evidence as seen by myelography and delayed metrizamide computed tomography scanning. The etiology of this finding and its possible relation to the course and treatment of cervical spondylotic myelopathy are discussed.

Adult↗
PubMed1989

Surgical management of syringomyelia--current results.

A retrospective study of the surgical results of 98 patients with syringomyelia was carried out. As primary surgical management, 38 cases were treated by craniovertebral decompression, 28 cases by craniovertebral decompression with syringotomy, and 22 cases in which Gardner's operation was performed. Primary syringoperitoneal shunt was done on six patients and the other primary procedures were performed on four. As the secondary operation, syringostomy was performed on nine patients and syringoperitoneal shunt on seven. Better results were obtained in patients managed by craniovertebral decompression with syringotomy or Gardner's operation. However, Gardner's operation had a higher mortality rate and a higher incidence of complication. If a second operation was required, syringoperitoneal shunt had a higher rate of stabilization than syringostomy.

Abnormalities, Multiple↗
PubMed1991

Syringomyelia secondary to congenital intraspinal lipoma.

Preoperative evaluation using serial magnetic resonance imaging was performed on an infant with lumbosacral lipoma. Syrinx formation arising just above the lipoma was observed in conjunction with rapid growth of the lipoma. Definite shrinkage of syringomyelia was obtained after radical excision of the lipoma. The syrinx formation was possibly caused by compression from the extramedullary lipoma.

Humans↗
PubMed1991

Management of syringomyelia associated with Chiari malformation: comparative study of syrinx size and symptoms by magnetic resonance imaging.

The authors reviewed the postoperative alteration of symptoms and syrinx size by magnetic resonance imaging (MRI) in 14 consecutive patients with syringomyelia associated with Chiari malformation. The patients were treated according to our treatment regimen and were divided into five groups according to operative modalities: (1) only foramen magnum decompression (D) for small syrinx; (2) D with ventriculoperitoneal (VP) shunt for small syrinx with hydrocephalus; (3) D with syringosubarachnoid (SS) shunt or (4) D with syringoperitoneal (SP) shunt for large syrinx; and (5) only VP shunt for syrinx with hydrocephalus and atlantoaxial dislocation, respectively. From the preoperative and postoperative sagittal MR images, the areas of the spinal cord and syrinx were measured by a digitizer and the syringo-cord (S-C) ratio was calculated. Out of 14 patients, 12 showed a reduction of syrinx size and a stabilization or improvement of symptoms after surgery. In the other two patients, the syrinx size did not change and their symptoms worsened. Magnetic resonance imaging follow-up showed that foramen magnum decompression without shunt is effective for patients with a small syrinx below 35% of the S-C ratio, and foramen magnum decompression with shunt was effective for patients with a large syrinx over 35% of the S-C ratio, but there was no significant difference between the SS and SP shunt group.

Adolescent↗
PubMed1994

Paraganglioma of the cauda equina with associated syringomyelia: case report.

We present an association not previously described in which the rare entity of paraganglioma of the cauda equina was found in association with syringomyelia of the cervico-thoracic cord. The patient, a 50-year-old woman presented with a long history of lower back pain with recent onset of sciatic pain and altered sensation in the right leg. Surgical resection of the tumor with intraoperative syringostomy of the syrinx has led to eradication of all symptoms and at 5 months shows no evidence of residual pathology.

Cauda Equina↗