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PubMed1982

Percutaneous puncture of spinal cysts in the diagnosis and therapy of syringomyelia and cystic tumors.

The experience with percutaneous puncture of intraspinal cystic lesions is reported in four cases: an extradural cyst demonstrated with air, a spinal cord tumor with lipiodol and two cases of syringomyelia with metrizamide. Percutaneous puncture of cystic lesions makes possible an accurate delineation of the lesion and a histological approach through biochemical and cytological analysis of the aspirated cystic fluid, while aspiration of the fluid of cystic tumors decompresses the spinal cord and thus plays a therapeutic role.

Adult↗
PubMed1980

Radiographic studies of the ventricles in syringomyelia.

Radiographic investigations of 171 patients with "communicating' syringomyelia have been reviewed. Hydrocephalus was found in one-third of the cases and has occasionally progressed after operation on the posterior fossa, sometimes with accompanying clinical deterioration. The outlets of the fourth ventricle were usually abnormal; tonsillar descent, arachnoiditis and both together were seen. Arachnoiditis correlated strongly with a history of difficult birth. The foramen of Magendie was sometimes patent and sometimes blocked. There was no consistent level of occlusion corresponding to a persistent roof of the fourth ventricle. The cisterna magna was usually small or obliterated but some examples of large cisterns or subarachnoid pouches were found. Radiological demonstration of a communication from the fourth ventricle to the syrinx occurred in only seven patients by positive contrast material and not by air. It is suggested that a sizable communication is rare at the time when patients seek treatment.

Adult↗
PubMed1993

Segmental hyperhidrosis in syringomyelia with Chiari malformation.

Segmental hyperhidrosis was seen in three cases of syringomyelia with Chiari malformation confirmed by magnetic resonance imaging. Using the iodine-starch reaction, all three cases showed hyperhidrosis in the areas roughly corresponding to those of sensory disturbances. Two possible modes of pathogenesis are discussed: stimulation of preganglionic neurons and interference to the inhibitory tract.

Adolescent↗
PubMed1993

Foraminal syringomyelia: suggestion for a grading system.

The standard treatment of foraminal syringomyelia includes foramen magnum decompression and duraplasty. Improvement or stabilization of the disease are achieved in most of cases. However, at least one third of patients are reported to receive little or no benefit. In this paper we retrospectively reviewed a series of 40 consecutive foramen magnum decompressions in order to identify the possible pre-operative outcome predictors. Based on clinical evolution, neurological impairment and radiological features, a scale of severity was fixed and retrospectively tested. A pre-operative score was obtained for each patient and was correlated with the surgical results. Then a four level grading system was derived. All grade I and grade II patients achieved good results (improvement or stabilization), whereas grade III patients showed intermediate behaviour and grade IV invariably worsened. On this basis, surgical results of foramen magnum decompression might be further improved provided that a careful pre-operative selection is made.

Adult↗
PubMed1991

Clinico-morphological correlation in syringomyelia: a statistical study assisted by computer measurement of magnetic resonance images.

A computer-assisted measurement of synrinxes was performed on MR images in a series of 26 patients with syringomyelia-Chiari complex. Syrinx data such as length, surface area, contour, and greater diameter were statistically correlated to each other and with the main clinical symptomatology of the patients. In general, a direct relationship between the various measurements of the syrinxes exists. Nevertheless, although a slight relationship could be established between the presence of muscular atrophy and a greater length and contour of the syrinxes, the present study suggests that the evaluation of syrinx dimensions cannot be brought into relation with the nature or distribution of clinical symptomatology. Because a reduction in the syrinx is generally the aim of surgery, this conclusion should be born in mind when a surgical treatment is offered to syringomyelic patients.

Adult↗
PubMed1988

Foramen magnum decompression versus terminal ventriculostomy for the treatment of syringomyelia.

The A.A review 30 consecutive cases of syringomyelia operated on during the last seven years. Six terminal ventriculostomies (TV) and twenty-seven procedures for foramen magnum decompression (FMD) were performed. All patients of TV group had CT-myelography (CTM) and/or NMR controls at different times after surgery. Clinical results are as follows: 1) of the 6 patients who had TV, only one showed an improvement while five continued to deteriorate and three of them needed a FMD, one a cysto-peritoneal shunt and the last one died from lung cancer. 2) of the 27 patients who had FMD, twenty improved, four were unchanged and three worsened. 3) no surgical deaths occurred in this series. Postoperative NMR monitoring represents an effective non-invasive neuroradiological procedure that allows follow-up of syrinx evolution over the years.

Adolescent↗
PubMed1979

Subarachnoid pouches of the posterior fossa with syringomyelia.

Three men are presented, all of whom were born with difficulty and later developed severe syringomyelia with collapsing cords on air myelography. Each had hydrocephalus and a sizable subarachnoid pouch in the posterior fossa. The clinical features and investigations are presented with a discussion of the aetiology and possible mechanisms concerned in pathogenesis and treatment. All three men had posterior fossa exploration over fifty years after birth, and the first two were also treated by valved ventriculo-atrial shunts. Each patient has improved a little since operation, but no certainty that this was due to surgery has been proved in any case; none has progressed since the last operation.

Cerebrospinal Fluid Shunts↗
PubMed1989

Multicystic syringomyelia treated with a single, non-valved syringoperitoneal shunt: fast and near-complete MRI normalization.

Four patients with multi-cystic syringomyelia were treated by a single, non-valved syringoperitoneal shunt. In all the patients, the cavitations were separated by a segment of apparently normal cord. In two of the patients, the cavitations also contained multiple transverse septa. In three patients the shunt was inserted in the most caudal cavitation, causing the shunted compartment to collapse, usually within a few weeks. The width of the more cranial cavitations normalized on postoperative MRI scans over several months. This was the case also in one patient where the most cranial cavitation was shunted. No complications or side effects were observed that could be attributed to the use of a non-valved shunt.

Adult↗
PubMed1983

CT in syringomyelia: three different aspects.

Three different CT aspects of patients with myelographic and clinical syringomyelia patterns are presented. In the first the CT pattern was atypical. The second case is a typical example of opacification of the cavity after contrast enhancement and the third shows re-formation of the cavity 7 years after surgical damage.

Contrast Media↗
PubMed1992

The association of hypopituitarism with small pituitary, invisible pituitary stalk, type 1 Arnold-Chiari malformation, and syringomyelia in seven patients born in breech position: a further proof of birth injury theory on the pathogenesis of "idiopathic hypopituitarism".

We report seven cases of hypopituitarism all having a history of breech delivery, asphyxia at birth, and syringomyelia. A small pituitary gland was found on MRI or CT in six cases, invisible pituitary stalk on MRI in five cases, and type 1 Arnold-Chiari malformation in six cases. A constellation of these abnormalities are best explained by traction of brain and spinal cord of the subjects exerted during breech delivery and further support the primary role of birth trauma in the genesis of "idiopathic hypopituitarism".

Adolescent↗
PubMed1991

Natural history and postsurgical outcome of syringomyelia.

The surgical treatment of syringomyelia is still debatable and the result are often poor. Several surgical procedures, based on various proposed etiopathologies, have been developed but in many cases have proved completely ineffective. We have evaluated the follow-up of 69 syringomyelic patients, some operated on, some not, in the search for clues to the management of the disease. For this purpose we devised a rating system, which we describe. 31 patients underwent surgery while 38 received no treatment. We found that half of the patients deteriorated, whether they were operated on or not; only 1 in 5 improved and the rest remained stable. For surgical treatment to be successful, the disease must be in rapid evolution but without definite paraparesis.

Adult↗
PubMed2001

Syringomyelia associated with adhesive spinal arachnoiditis: MRI.

We reviewed the MRI of seven patients with syringomyelia associated with surgically proven adhesive spinal arachnoiditis to describe clinical and MRI findings in this condition. Using 0.5, 1.0 or 1.5 tesla units, we obtained sagittal T1- and T2-weighted spin-echo and axial T1-weighted images. Additional sagittal T2-weighted images were obtained without using motion-artefact suppression. Contrast medium was given intravenously to five patients. The syrinx cavities were thoracic in five cases, cervicothoracic in one, and extended from C4 to L1 in one. No Chiari malformation or craniovertebral junction anomaly was demonstrated. Meningeal thickening was seen on T2-weighted sagittal images only in one case. Cord deformity due to adhesion or displacement due to an associated arachnoid cyst was seen in all cases best demonstrated on axial images. Focal blurring of the syrinx wall on axial images was seen in six patients. Flow voids were seen in the syrinx fluid in all cases on T2-weighted images obtained without motion-artefact suppression. No abnormal contrast enhancement was demonstrated.

Adult↗
PubMed2000

Polysomnographic respiratory findings in patients with Arnold-Chiari type I malformation and basilar invagination, with or without syringomyelia: preliminary report of a series of cases.

Patients with craniocervical disorders (CCD) show a wide variety of symptoms and signs suggesting cerebellar and/or high cervical lesion. The anatomic localization of respiratory centers and their possible injury may explain the presence of respiratory disturbances in these diseases. The aim of this preliminary study was to evaluate the polysomnographic findings in a group of patients with CCD, most of them with Arnold-Chiari malformation type I, since sleep apnea has been referred to in isolated cases in the literature. Eleven patients (seven females and four males) with CCD diagnosed by magnetic resonance imaging referred from the neurosurgery unit were submitted to clinical history, physical examination with sleep questionnaires, and scored on the Epworth Sleepiness Scale. Full night polysomnography was performed in an Oxford SAC system where EEG, electro-oculography, electrocardiography, chin and leg electromyography, chest and abdominal efforts, airflow, and oximetry were recorded continuously. Nine patients presented with Arnold-Chiari type I malformation, of whom six showed associated syringomyelia. The other two had basilar invagination. Ninety percent of these patients complained of sleep problems (snoring, choking, and witnessed apneas) and 72% presented hypersomnolence (ESS >9). The polysomnographic findings showed sleep fragmentation in 81% of the patients and a reduction of rapid eye movement sleep in 63%. The apnea/hypopnea index was above 5 in 72%, with a predominance of central apnea. Patients with craniocervical disorders present a higher probability of displaying sleep respiratory disturbances. Their sleep complaints should be assessed and patients should be submitted to an overnight sleep recording in order to identify sleep apnea.

Adult↗
PubMed1997

Syringomyelia in mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): imaging findings following bone marrow transplantation.

We present the imaging findings in a patient with mucopolysaccharidosis (MPS) type VI (Maroteaux-Lamy syndrome) who developed holocord syringomyelia. This represents the only reported case of syrinx formation in a child with MPS VI. Clinical, neurologic and spinal magnetic resonance imaging findings are presented. The patient has maintained a stable clinical and neurologic course over the period following allogeneic bone marrow transplant.

Bone Marrow Transplantation↗
PubMed2000

Neurocutaneous melanosis with hydrocephalus, intraspinal arachnoid collections and syringomyelia: case report and literature review.

Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome, characterised by large or numerous congenital pigmented nevi and excessive proliferation of melanin-containing cells in the leptomeninges. We report the MR findings in the brain and spine of a child with NCM who underwent neurosurgical treatment and was followed up for 8 years. The findings in this child (small hyperintense collections of melanocytes in both temporal lobes, mild meningeal enhancement along the spine and the development of an extensive subarachnoid CSF accumulation with cord compression and syringomyelia) are believed to be exceptionally rare.

Arachnoid↗
PubMed2002

Posttraumatic syringomyelia: volumetric phantom and patient studies using MR imaging.

The purpose of this study was to determine the volume of posttraumatic syringomyelia (PTS) based on standard MRI data acquisitions, and to compare the volumes with the neurological deficits of the patients. Firstly, phantom studies were performed using routine T1- (T1W; TR 500 ms, TE 13 ms) spin-echo (SE) images, 3D gradient-echo (GE) images and T2-weighted (T2W) turbo spin-echo (TSE) images (TR 3000 ms, TE 130 ms), in the sagittal plane. The slices were interleaved so that there was no gap. Twelve phantoms simulating a PTS were constructed and filled with fluid. Each volume was exactly measured immediately prior to filling (volumes: 3600-74,000 mm(3), mean 27,500 mm(3)). In the clinical study 32 patients with PTS were examined using the same protocol. Patients were supine and a phased-array coil was used. The phantom studies revealed measurement errors of within 35%. There were problems defining the boundaries in the small and irregular phantoms as well as in small and irregular PTS, and due to the partial-volume averaging effect. The two small irregular phantoms could only be measured on the axial images. The T2W images in the axial plane showed the best results: measurement accuracy 92%. In the clinical study all examinations were technically successful. The volumes of the PTS ranged between 200 and 19,800 mm(3); the mean volume was 4075 mm(3). Our initial results show that the volume measurement of a PTS using standard MRI sequences can help generate more objective and accurate measures of spinal cord lesions, and this may enhance the sensitivity of MRI in detecting disease progression or regression after treatment.

Adult↗