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PubMed1996

Lhermitte-Duclos disease associated with syringomyelia.

A 23-year-old man presented with a 2-week history of intracranial hypertension. CT showed a large, nonenhancing cerebellar mass with surrounding calcification and displacement of the fourth ventricle. MRI revealed a septate lesion, with low signal on T1-weighted and high signal on T2-weighted images. The cerebellar tonsils were displaced below the foramen magnum and there was associated syringomyelia. The MRI features were characteristic of Lhermitte-Duclos (LD) disease (dysplastic gangliocytoma) and the diagnosis was confirmed following surgery. In this case, we emphasise the usefulness of MRI in the diagnosis of LD disease and consider the possible pathogenesis of the associated syringomyelia.

Adult↗
PubMed1997

Syringomyelia and Arnold Chiari in scoliosis initially classified as idiopathic: experience with 25 patients.

The authors analysed the clinical and radiological findings and the surgical management of 25 patients admitted for scoliosis classified as idiopathic at first presentation, but in fact associated with spinal cord and/or brain stem anomalies. Twenty patients had syringomyelia, 19 had Chiari malformation. Scoliosis was the only presenting symptom when all these patients were referred to the orthopaedic surgeon. On examination, five patients had normal neurological findings, while the others showed very mild neurological deficits. The diagnosis of syringomyelia and Chiari malformation was established by MRI, which is the best form of neuroradiological examination for discovering spinal abnormalities. Neurosurgical treatment is strongly recommended as the first step in the management of "pseudo" idiopathic scoliosis.

Adult↗
PubMed1993

Surgical treatment of "hindbrain related" syringomyelia: new data for pathogenesis.

52 patients with "hindbrain related" syringomyelia underwent surgical treatment. All patients underwent primary reconstructive surgery at the craniovertebral junction. Terminal ventriculostomy was performed as the secondary operation in 2 cases. The surgical treatment arrested progression of signs in 33 (63.5%), stabilized disease in 9 (17%) cases. Postoperative deterioration occurred in 8 (15%) cases. Mortality was 4% (2 patients). Percutaneous or intra-operative injection of myodil and gas into the syrinx, as well as CT, revealed the existence of communication with the 4th ventricle in 14 patients. Investigation of cerebrospinal and syrinx fluid revealed increased level of IgG, IgM or IgA in the syrinx fluid in 16 out of 22 patients. Immunohistological examination of pia mater revealed specific staining for IgG. Thus, syrinx formation may be due to synergic action of hydrodynamic and immunopathological mechanisms. Results indicate that early surgical treatment is preferable to patients with hindbrain anomalies and hydromyelia. We consider primary reconstructive operation at the posterior fossa as the preferred surgical management of "hindbrain related" syringomyelia.

Adolescent↗
PubMed1990

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

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

Adolescent↗
PubMed1987

Syringosubarachnoid shunt for treatment of syringomyelia.

The experiences with a series of 9 patients with syringomyelia treated by syringosubarachnoid shunt (SSS) are presented. All of the cases were evaluated by myelography, CT scan or MNR. The cases showed satisfactory results which consisted in stabilization of the symptoms (55%) or clinical improvement (44%). These results should be continuously evaluated, due to the short follow-up period and to the variable natural history of the illness. Treatment by SSS is a technique of low morbidity. It is recommended for patients with syringomyelia without significant descent of the tonsils or in those cases where posterior fossa operations have failed to obtain good results.

Adolescent↗
PubMed1995

Association of idiopathic hypopituitarism and syringomyelia.

The magnetic resonance in an 8-year-old boy with hypopituitarism and cervical syringomyelia are presented. Magnetic resonance imaging of the brain clearly demonstrated syringomyelia in the cervical spinal cord and transection of the pituitary stalk, findings identical to those reported in the literature. Both disorders have been seen in association with a difficult labor, so we suggest that this is not a chance finding.

Child↗
PubMed1995

Syringomyelia in association with tumours of the posterior fossa. Pathophysiological considerations, based on observations on three related cases.

In this report we describe 3 patients with syringomyelia in association with tumours of the posterior fossa. In each patient the syrinx was demonstrated on pre-operative magnetic resonance imaging (MRI). After total or partial removal of the tumour the syrinx collapsed. It is concluded that the pathogenesis of syrinx formation in this entity requires interference with normal cerebrospinal fluid (CSF) flow at the foramen magnum. We suggest that the obstruction to the flow of CSF causes alterations in the passage of extracellular fluid (ECF) in the spinal cord which lead to syringomyelia.

Adolescent↗
PubMed1997

Surgical technique for cranio-cervical decompression in syringomyelia associated with Chiari type I malformation.

Our purpose is to present our results with the surgical treatment of syringomyelia associated with Chiari type I malformation. Between October 1989 and October 1995, twenty-eight patients underwent a sub-occipital craniotomy and a C1 laminectomy. After dura mater opening the cerebellar tonsils were mobilised. Neither catheter, nor plugging of the obex, nor tonsillar tissue removal was performed. The dura mater was enlarged by means of a wide graft to create a new cisterna magna of adequate size. Postoperative MRI scans showed an ascent of the cerebellum of 4.3 +/- 4.8 mm (measured by the fastigium to basal line), as well as of the brainstem (mean migration of the mesencephalon-pons junction of 4.3 +/- 3.3 mm). The tonsils emigrated cranially 6.5 +/- 4.8 mm. While preoperative mean syringo-cord ratio was 66.3% +/- 13.3, post-operatively was 12.1% +/- 12.7 (p < 0.0001). A complete collapse of the syrinx was observed in 39% of the patients. Long-term improvements were obtained in 73% of the cases and 27% were unchanged. No patient got worse. We conclude that in the treatment of syringomyelia associated with Chiari I malformation an artificial cisterna magna of sufficient size must be created. This is achieved by means of an extensive sub-occipital craniotomy and C1 laminectomy, followed by dural opening. Small bone removal with limited enlargement of the posterior fossa often results in failures of treatment and recurrences. Tonsillar removal is not necessary to obtain a good reconstruction of the cisterna magna.

Adolescent↗
PubMed2000

Spontaneous drainage in syringomyelia: magnetic resonance imaging findings.

We present five cases of syringomyelia associated with Chiari I or other causes of partial obstructions at the cervicomedullary junction, with spontaneous disruption of the wall of a cervical syrinx and formation of a communication between the cavity and the subarachnoid space, shown on axial MRI. MRI can be used to investigate the hydrodynamics, showing the liquid inside the disrupted syrinx wall and the pathway of drainage. The finding of spontaneous drainage may be important for understanding the pathogenesis of syringomyelia and may be helpful for choosing a surgical approach.

Adult↗
PubMed2003

Neuropathic arthropathy caused by Arnold-Chiari malformation with syringomyelia.

We report a case of neuropathic arthropathy caused by Arnold-Chiari malformation with syringomyelia, with details of the clinical and radiologic aspects. The patient had pain and stiffness in his left upper limb. MRI of the left shoulder revealed neuropathic arthropathy. Upon examination, his left elbow also had the characteristic findings for neuropathic arthropathy. Examination of the spinal cord by MRI confirmed Arnold-Chiari malformation associated with syringomyelia. Neuropathic arthropathy requires evaluation of the spinal cord to assess for occult causal lesions.

Adult↗
PubMed2006

Winged scapula as the presenting symptom of Chiari I malformation and syringomyelia.

CASE REPORT: We report a 19-year-old girl with a 4-month history of an inability to fully elevate her upper extremity past the horizontal position. Physical examination revealed a winged scapula. MRI was demonstrative of a Chiari I malformation with a small cervical syrinx eccentrically placed to the same side as the dysfunctional extremity. DISCUSSION: We believe this to be the first report of dysfunction of the long thoracic nerve via a hindbrain hernia-induced syrinx with a resultant winged scapula. We would hypothesize that enough anterior horn motor neurons and their axons destined for the long thoracic nerve were injured by the syringomyelia to result in isolated deinnervation of the serratus anterior muscle. CONCLUSION: The clinician may wish to include syringomyelia in the differential diagnosis of a winged scapula.

Adult↗
PubMed2000

Can hindbrain decompression for syringomyelia lead to regression of scoliosis?

Scoliosis in childhood develops secondary to syringomyelia in some children. The existing literature does not provide a clear answer as to whether surgical treatment of the syrinx can allow subsequent improvement of the spinal deformity, thus preventing the need for scoliosis surgery. This series comprised 16 patients with syringomyelia who presented with significant scoliosis in the absence of major neurological deficit. All underwent a hindbrain decompression, and follow-up ranged from 1 to 6 years (mean 2.5 years). Subsequent deformity surgery was necessary in eight cases, but the scoliosis was seen to improve or arrest its progression in six (37.5%). Improvement was found to be statistically more likely in children of younger age at the time of syrinx surgery and in those with left thoracic curves. Improvement occurred in 71.4% of those under the age of 10 at the time of hindbrain decompression.

Adolescent↗
PubMed2002

The pathophysiology of syringomyelia - historical overview and current concept.

Various ideas and hypotheses have been brought forward to explain the development of syringomyelia in the past two centuries. None of them offers a sufficient basis to serve as a concept for the treatment of all affected patients. Apart from a discussion of the different hypotheses this paper proposes a new pathophysiological concept based on clinical, experimental and literature studies. Syringomyelia is understood as a state of chronic interstitial edema of the spinal cord due to accumulation of extracellular fluid (ECF). This accumulation is caused by a cascade of events starting with obstruction of cerebrospinal fluid (CSF) flow and/or spinal cord tethering which ultimately alter ECF flow and increase ECF volume. Treatment should be targeted against the pathological process which causes CSF flow obstruction and cord tethering to inhibit this pathophysiological process at a decisive point.

Animals↗
PubMed2004

Spontaneous resolution of a Chiari I malformation associated syringomyelia in one child.

A child with complete spontaneous resolution of a Chiari I malformation associated Syringomyelia without surgical intervention is presented. The child was followed clinically by serial magnetic resonance imaging (MRI) and remains neurologically stable after 8-years of follow-up. To our knowledge, only 6 pediatric cases with spontaneous resolution of a spinal cord syrinx documented by MRI without surgical intervention have been reported. This case is of interest in the light of the postulated theories to explain spontaneous resolution of syringomyelia.

Arnold-Chiari Malformation↗
PubMed2000

Surgery for syringomyelia: an analysis based on 163 surgical cases.

OBJECT: The authors analyzed the cases of 163 patients with syringomyelia to assess the appropriate surgical procedure. METHODS: Depending on the aetiological factors and treatment considerations the series was classified into three groups. Group I were cases where there was no definite demonstrable aetiological factor; Group II cases had basilar invagination and/or Chiari malformation; and Group III consisted of cases where the syrinx was secondary to an obvious aetiology, such as a mass lesion either in the posterior cranial fossa or in the spine or a severe kyphotic spinal deformity. Post-traumatic syringomyelia and syrinx in association with spina bifida were not studied. CONCLUSIONS: We concluded that for Group I cases syringosubarachnoid shunting is the ideal form of treatment. In Group II cases foramen magnum bony decompression is satisfactory and physiological. Good results were obtained even in cases where either a foramen magnum decompression alone or in combination with a syringo-subarachnoid shunt was done. Only syringosubarachnoid shunt (without a foramen magnum decompression) in Group II cases was found to produce poor outcome. Group III cases should be treated for the primary aetiological problem. Only syrinx drainage procedure without treatment of aetiology in these cases produced poor results. It was observed that clinical outcome rather than radiological improvement is the reliable indicator of the surgical result.

Adolescent↗
PubMed2004

A simple technique for expansive suboccipital cranioplasty following foramen magnum decompression for the treatment of syringomyelia associated with Chiari I malformation.

The benefits of osteoplastic suboccipital craniotomies over the traditional suboccipital craniectomies have been recognized. We describe a simple method of expansive suboccipital cranioplastic craniotomy using a free bone flap and report satisfactory clinical results in 16 patients with syringomyelia associated with Chiari I malformation. A free suboccipital bone flap is created from the rostral part of the occiput by placing two to four burr holes and connecting them with a craniotome. The posterior bony margin of the foramen magnum and the posterior arch of C1 are removed thereafter. Then dural plasty using a patch graft of dural substitutes is performed. The expansive suboccipital cranioplasty is performed by positioning the free bone flap caudal to the original location and fixing it with titanium miniplates to construct a bony frame to cover the foramen magnum. The rostral part of the cranial defect is filled with bone chips created during the craniotomy. Sixteen patients underwent this procedure. There was no operative mortality and no major complication, such as persistent pseudomeningocele. Preoperative symptoms improved significantly in all patients except for one who had persistent dysesthetic pain. Our simple method of expansive suboccipital cranioplasty for the treatment of syringomyelia associated with Chiari I malformation proved useful and achieved satisfactory long-term results.

Adolescent↗
PubMed1994

Late onset of syringomyelia after traumatic brain injury: association with Chiari 1 malformation.

Syringomyelia should be suspected when progressive weakness or sensory loss develops. It most commonly occurs as a congenital malformation but has also been associated with spinal cord injury and the Chiari malformation. Symptomatic Chiari malformation after head injury is rare and usually presents with signs of acutely increased intracranial pressure. This article describes syringomyelia as a late onset complication of head injury in the presence of an underlying Chiari malformation. A possible causal relationship between head injury, the brain malformation, and an extensive syrinx is postulated.

Arnold-Chiari Malformation↗
PubMed1978

Familial communicating syringomyelia.

Calssical cervical syringomyelia was found in 3 members of one family. All 3 underwent air myelogram, and a Chiari malformation type I and postural collapse of the spinal cord was found in each case. An affected 7-year-old boy was discovered after a clinical and radiological survey of 8 first-degree relatives on the basis of mild scoliosis, pyramidal tract signs in the lower limbs and enlarged sagittal diameter of the cervical canal. One other member had basilar impression of the skull but no neurological abnormalities. No positive correlation was found between either the size of the cystic cord enlargement or descent of the ectopic tonsils with the duration or severity of the neurological findings. Suboccipital decompressive craniotomy and upper cervical laminectomy in one case was followed by improvement in strength and sensation 1 year later. Progression in familial syringomyelia appears to occur through a mechanism identical to that in the sporadic form and surgery is therefore also indicated. In affected families, routine survey of close relatives for abnormal neurological signs, and radiological evidence of scoliotic deformity of the spine, enlarged cervical canal and bone abnormalities at the craniovertebral junction may prove valuable for early detection. A dominantly inherited, genetically determined malformation seems to be the probable mechanism of inheritance in this family.

Adolescent↗