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[Late post-traumatic syringomyelia. Discussion apropos an atypical case].

Late post-traumatic syringomyelia is uncommon, the clinical signs appearing several years after severe trauma to the cervical spinal cord. It is due to progressive cavitation in the spinal cord. In the case reported here progress was atypical because there were no immediate neurological signs and the symptoms of syringomyelia developed only 3 weeks after injury. Several possibilities are open to discussion. Was the trauma responsible for the syringomyelia? Did it cause an aggravation of a pre-existing hydromyelia or were both lesions purely coincidental? A silicon tube draining the cavity in the cord to the subarachnoid space resulted in a real improvement in the neurological condition.

Adult↗

[Several clinical features of syringomyelia].

On the basis of clinical studies of 387 patients with syringomyelia the authors describe different forms of this disorder. The results of these studies conducted by means of one derivation echoencephalography depending upon the age and disease duration in patients with syringomyelia are demonstrated. The possible mechanisms of syringomyelia development are being discussed.

Adolescent↗

[The diagnosis of syringomyelia (author's transl].

Seven cases are reported in which syringomyelia was confirmed by neuroradiological studies and surgical procedures. Dissociated sensory loss was noted in four patients in this series. Peripheral paresis and muscular atrophy in arms and hands were found in all seven patients. In only two of the patients the wide spinal canal were found in the lateral view of plain cervical spine films. Assimilation of the atlas to the occipital bone was seen in two patients. Arnold-Chiari malformation was found by myelography and posterior fossa craniectomy in four patients. Metrizamide CT cisternography was found to be useful to demonstrate Arnold-Chiari malformation. Gas myelography was performed on six patients. A collapsing cord was demonstrated in four patients. In other two patients the study was not successful because of technical problem and the association of communicating hydrocephalus. Computed tomography, haad scanner, was done on six patients. Plain CT was not useful diagnostic study for the diagnosis of Arnold-Chiari malformation or syringomyelia. This study and other reports suggest that the most reliable radiological diagnostic method of syringomyelia is gas myelography and demonstration of the collapsing cord. CT of the spinal cord requires further improvement, however, CT with high resolution and metrizamide will improve diagnostic accuracy.

Adolescent↗

Terminal ventriculostomy for syringomyelia.

The pathogenesis of syringomylia is not yet well known and its surgical management still poses problems. Terminal ventriculostomy, as proposed by Gardner et al. (1977), in treating the communicating syringomyelia could be an alternative approach as opposed to the cranio-vertebral decompression or subarachnoid shunt of the syrinx. This operation was performed by the authors in 3 cases. In all of them there was postoperative improvement of the neurological symptoms. In the authors' opinion the main problem is the difficulty in preoperative diagnosis of communicating syringomyelia. This technique is simple and could present an efficient means of diagnosing the communicating syringomyelia, particularly when there are no associated malformations.

Adult↗

[Value of the study of somatosensory evoked potentials during surgical correction of scoliosis associated with syringomyelia. Apropos of 4 cases].

PURPOSE OF THE STUDY: The presence of a syringomyelia cavity increases the rate of neurological complications on the course of surgical treatment of scoliosis. We have evaluated the results of monitoring of somatosensory evoked potentials (SEP) in these situations. MATERIAL AND METHODS: Four patients presenting a scoliosis associated with syringomyelia have been operated through a posterior-approach with CD instrumentation. SEP monitoring was performed pre and intraoperatively. We studied the latency and the amplitude of P40. RESULTS: Preoperative SEP showed in all cases posterior spinal cord involvement (even without clinical manifestations). During monitoring, we noted in one case no variation. In one case a flattening of the response with normalisation within 5 minutes. In two cases a persistent flattening with normalisation within 10 and 15 minutes following modification of the instrumentation. In all cases, postoperative neurological status was identical to preoperative one. DISCUSSION: Preoperative SEP can make the diagnosis of posterior spinal cord involvement even when clinical status is normal. The extent of the preoperative SEP abnormalities may preclude the risk of intraoperative neurological complications. Intraoperative SEP can be performed with the same anesthetic protocol and the same technique used when operating idiopathic scoliosis. The results seem reliable. When alteration occur as for idiopathic scoliosis alteration of the amplitude appears earlier than alteration of the P40 latency. Restoration of normal responses appears later than in idiopathic scoliosis. CONCLUSION: SEP monitoring should diminish the risk for neurological complications in the course of surgical treatment of scoliosis associated with syringomyelia.

Adolescent↗

[Syringomyelia associated with tentorial meningioma].

A 28-year-old male was admitted to our hospital with the complaints of numbness of the left upper limb and gait disturbance. Neurological examination disclosed slight left dysmetria, truncal ataxia and sensory disturbance at the dermatome of C8 and Th1. CT and MRI scans showed a large mass lesion in the left posterior fossa, ventricular dilatation, cavum septi pellucidi and cavum Vergae, empty sella, cervical syringomyelia and left tonsilar herniation. The tumor which attached to cerebellar tentorium was totally removed and a histological diagnosis of meningothelial meningioma was made. Postoperative MRI scan demonstrated a disappearing of syringomyelia with the improved tonsillar herniation. Association of syringomyelia with brain tumor is relatively rare, so its pathogenesis was discussed.

Adult↗

[An adult case of tethered cord syndrome with lipoma and thoraco-lumbar syringomyelia presenting slow progressive muscular atrophy in the lower limbs].

A 23-year-old man developed slowly progressive muscle weakness from the age of 10, followed by paresthesia and urinary disturbance. The physical examination showed bilateral muscular atrophy in the lower limbs and dissociated sensory disturbance below Th8. Although there was normal appearance of spinal X-P, MRI revealed conus medullaris at S2 involved thoraco-lumbar syringomyelia with extramedullary lipoma. After the operation for laminectomy of L5-S2 and untethering without direct surgery for syringomyelia, post-operated MRI exhibited definite shrinkage of the syrinx. It suggested that thoraco-lumbar syringomyelia in the present case was not congenital anomaly, but secondary outcome to tethered cord syndrome.

Adult↗

[Acute urine retention: a rare mode of revelation of cervico-dorsal syringomyelia caused by cyproheptadine].

BACKGROUND: Syringomyelia is rare in children aged less than 10 years, and bladder dysfunction is an unlikely first manifestation. This report describes a case of repeated episodes of acute urinary retention in a young girl revealing syringomyelia and Arnold-Chiari malformation. CASE REPORT: A 2.5 year-old girl was admitted because she was suffering from acute urinary retention. Her poor appetite had been treated with cyproheptadine, a histamine type I blocking drug. Clinical investigation revealed no local cause for this bladder dysfunction except moderate spasticity of the legs. Cystography showed no vesicoureteral reflux. Because the episodes of urinary retention recurred each day, magnetic resonance imaging (MRI) was performed; this showed the typical features of syringomyelia extending from C5 to T11 plus Arnold-Chiari malformation. The cyproheptadine was discontinued and the urinary retention disappeared. CONCLUSION: Cyproheptadine may have revealed latent neurogenic bladder in this case, although urodynamic studies, performed 3 months later, detected no bladder dysfunction.

Acute Disease↗

[Sudden exacerbation in atypical syringomyelia with a unilateral superficial sensory disorders as the principal manifestation].

We report a 22 year-old woman with syringomyelia, who complained of a sudden abnormal sensation in the left neck and upper extremity after maintaining her neck in flexion for sometime. Neurologic examination revealed superficial hypesthesia on the left from C2 down, but normal motor function. The mode of onset in our patient was atypical. The clinical manifestations of syringomyelia are usually slowly progressive. On the basis of X-P film of the cervical spine and cranial MRI, a diagnosis of syringomyelia with Chiari malformation (type 1) was made. The syrinx cavity extended from C1 to L1. On the transaxial image of the cervical cord, the syrinx cavity was demonstrated in the posterior horn area ipsilateral to the sensory disturbance. The CSF flow-void sign was present in the syrinx cavity, probably reflecting pulsation of the syrinx fluid. An abnormally high signal intensity area adjacent to the syrinx cavity on T2-weighted sequences indicated damaged cord. We speculate that dynamic factors produced by neck flexion and fluid pulsation explain the sudden exacerbation in our patient.

Adult↗

Surgical treatment of type I Chiari malformation with syringomyelia in adults.

The surgical treatment of six adult patients with Type I Chiari malformation associated with syringomyelia is reported. Two patients presented with acute respiratory insufficiency, which is common in Type II and rare in the Type I anomaly. One patient initially appeared with unilateral vocal cord palsy that was followed by wasting and fasciculation of the tongue and limb muscles. Two patients had a typical central cord syndrome and the last patient had hemiparesis and gait disturbance. The first patient was diagnosed clinically. Two patients were studied by computed tomographic metrizamide myelogram (CTMM). The last three patients were diagnosed by magnetic resonance imaging (MRI) scan. All patients were operated on with a modified 'Gardner procedure'. Symptoms related to cervico-medullary compression improved satisfactorily. Signs of central cord syndrome, however, remained stationary event though the follow-up MRI examinations revealed good resolution of the syringomyelia. One patient died within one week of surgery. MRI makes the diagnosis of this anomaly much easier and is the ideal tool for follow-up study. Clinicians should include this malformation in the differential diagnosis when considering cases of syringomyelia.

Adult↗

Sensory loss in syringomyelia: not necessarily dissociated.

The pattern of sensory loss was assessed in 210 cases of syringomyelia. Dissociated sensory loss occurred in 49% of cases, indicating that its occurrence is not a necessary finding for the diagnosis of syringomyelia. Syringomyelia should be considered in the differential diagnosis of all cases of spinal cord disease.

Humans↗

[Surgical treatment for syringomyelia associated with Chiari malformation].

Two major surgical procedures, i.e., foramen magnum decompression (FMD) and syringo-subarachnoid shunt (S-S shunt) have been performed as the treatment for syringomyelia with Chiari malformation in our department. FMD was done principally in patients with symptoms due to Chiari malformation and for small size or localized syrinx, and S-S shunt was applied to patients with main symptoms due to syringomyelia, especially with local pain and for large syrinx in size. Eighty patients of syringomyelia with Chiari malformation were surgically treated. FMD was performed in 38 patients, and S-S shunt in 39 patients as the first operation. FMD were done with craniectomy of posterior fossa, C1 laminectomy and resection of epidural band, but without opening dura, and S-S shunt were done with hemipartial laminectomy and DREZtomy. The clinical outcome was evaluated, comparing the two major surgical procedures. Satisfactory neurological improvement was obtained in the two groups, but S-S shunt group took better improvement than FMD group. These results suggest that both procedures are effective, but should be selected by preoperative clinical and radiological findings.

Adolescent↗

Syringomyelia and hydrocephalus in a dog.

A 7-year-old spayed female Pomeranian with a 6-month history of progressive paraparesis was determined to have syringomyelia accompanied with hydrocephalus. Magnetic resonance imaging clearly revealed severe syringomyelia in the cervical portion of the spinal cord, which was directly connected to the marked dilated fourth ventricle. Acetazolamide was prescribed for 3 weeks, but the neurologic deficits progressed. Laminectomy of the first and second vertebrae, which is one method of treating this type of syringomyelia in human beings, was performed, and then, acetazolamide was continued. The dog had partial amelioration, and further deterioration was not evident in the 12 months after surgery.

Animals↗

[Syringomyelia associated with posterior fossa cyst: a case report].

We report a case of a 47-year-old woman with a posterior fossa cyst associated with syringomyelia and hydrocephalus. Her birth was traumatic and she had suffered a fractured skull. About 10 years prior to coming to our department she had occipitalgia and a pain had developed over a month from her right shoulder to hand. Shortly before presenting occasional electric-like shocks were felt in her right hand associated with coughing and hiccuping. Magnetic resonance imaging (MRI) revealed a large midline posterior fossa cyst, hydrocephalus and syrinx (C1-Th11). Cisternography could not demonstrate communication between the cyst and the subarachnoid space and the 4th ventricle was present but without communication with the cyst. The cyst was tentatively diagnosed as an arachnoid cyst. One week after placement of a cyst-peritoneal shunt, CT scans showed a decrease in cyst and ventricle size and 2 months later a follow-up MRI revealed resolution of the syringomyelia. We suspected that cyst-peritoneal shunt was effective for this patient who had syringomyelia associated with incommunicated posterior fossa cyst.

Cranial Fossa, Posterior↗

Syringomyelia.

Syringomyelia is not a single disease, but rather a descriptive term for any fluid cavity in the spinal cord. Regardless of the underlying etiology, the signs and symptoms of syringomyelia are related to the location, size, and extent of the cavity. With the advent of magnetic resonance imaging (MRI) and improved surgical procedures for decompression of the cavity, syringomyelia is an increasingly recognized cause of disability and even death in patients with clinical signs and symptoms of central spinal cord lesions. In this review, the etiology, pathophysiology, imaging, and treatment will be concisely discussed.

Cerebrospinal Fluid↗

[Syringomyelia--is it only a problem in neurosurgery?].

Three cases of arthroplasty in the course of syringomyelia are presented. These patients have been diagnosed in orthopedic-traumatologic department due to their bony/articular symptoms. After having established the diagnosis the patients were referred to Neurosurgery. Final diagnosis of syringomyelia requires computerized tomography and magnetic resonance imaging. Syringomyelia necessitates an early surgical treatment.

Adult↗

[A huge retrocerebellar arachnoid cyst with syringomyelia: case report].

The authors report a case of a huge posterior fossa arachnoid cyst with syringomyelia. This 22-year-old man presented with a history of drop attacks. Magnetic resonance imaging demonstrated ventricular hydrocephalus, a huge posterior fossa arachnoid cyst and syringomyelia. Cyst-peritoneal shunting was installed. The patient had an uneventful postoperative course, and his drop attacks disappeared. Magnetic resonance imaging one month after surgery showed reduction of the ventricular size and size of the arachnoid cyst and syrinx. Large arachnoid cysts in the posterior fossa are reported occasionally, but rare with syrinx. The authors described this case to illustrate the possible contribution of the pathogenesis of the syringomyelia associated with a huge posterior fossa arachnoid cyst.

Adult↗

[A new operative technique of posttraumatic syringomyelia: thecoperitoneal shunt].

The authors report a successful case of operative treatment for a patient with a traumatic syringomyelia. A 33-year-old male presented with arm pain and right sided sensory loss due to posttraumatic syringomyelia. Magnetic resonance image showed syringomyelia from the upper cervical cord to the lower thoracic cord. Based on the hypothesis of Ball and Dayan, and Williams, a thecoperitoneal shunt operation was performed. The proximal shunt catheter was placed in the subarachnoid space rostral to the injury level and the distal shunt catheter was introduced percutaneously into the peritoneum. Postoperative radiological studies showed improvement and progressive clinical deterioration stopped. The advantages of this surgery are that it is less invasive to the spinal cord, and that there is a lower shunt malfunction rate because of the use of a D-L catheter which develops less shunt obstruction. Furthermore, we were able to evaluate shunt flow from the valve. In spite of multicystic syrinx, we were easily able to determine the placement of the shunt catheter for this operation. For these reasons, the thecoperitoneal shunt can be placed before further expansion of the syrinx. We think that this method is safer for patients with incomplete cord injury than S-P shunt or S-S shunt.

Adult↗