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A 31-year-old woman presented with worsening numbness and pain in the arms and chest. Neurological findings at admission were decreased pain sensation and temperature sensation in the arms and chest. Magnetic resonance demonstrated a large cervical syrinx from the level of C4 to Th4 associated with Chiari I malformation. Occipital craniectomy and C1 laminectomy were performed for foramen magnum decompression. Intraoperative ultrasonography, performed after removal of the outer membrane of the dura mater at the level of the foramen magnum, revealed insufficient decompression. Therefore, the dura mater was completely opened and duraplasty was performed with a polyglycolic acid patch and fibrin glue. Sufficient decompression was thus achieved. The neurological symptoms and signs improved within the first postoperative month, and magnetic resonance showed a decrease in the size of the syrinx and no cerebrospinal fluid leakage. In patients undergoing foramen magnum decompression with duraplasty, the use of a polyglycolic acid patch and fibrin glue renders suturing unnecessary and avoids the common complications associated with suture duraplasty.

Clinical and radiological features of syringomyelia in 15 patients with spinal dysraphism are reported. There were 8 patients with occult spinal dysraphism (lumbosacral lipoma) and 7 with spina bifida aperta (meningomyelocele). Syringomyelia with spinal dysraphism can be radiologically divided into two types according to the dysraphic state. The syrinx in the patients with occult spinal dysraphism occurred immediately rostral to the lipoma and was localized to the lower thoracic to lumbar levels, while in the meningomyelocele patients the syrinx extended from the cervical to the thoracic level. Large syrinx formation was recognized in 1 of the 7 occult spinal dysraphism cases and 3 of the 8 meningomyelocele cases. For syringomyelia with occult spinal dysraphism, 4 patients underwent syringo-subarachnoid shunting (S-S shunt, 2 cases) or syringostomy (2 cases) during an untethering operation. In the case of meningomyelocele, S-S shunts were placed in 2 patients. Collapse of the syrinx was achieved in all 6 patients who underwent S-S shunting or syringostomy. Decreased size of the syrinx was also noted in 3 occult spinal dysraphism patients who underwent untethering operations alone. In conclusion, a large syrinx in the case of spinal dysraphism should be surgically treated. S-S shunting is effective in both types of syringomyelia. Foramen magnum decompression may be an alternative method of surgical treatment for syringomyelia in patients with meningomyelocele.

The surgical results of foramen magnum decompression in seven patients with syringomyelia associated with Chiari I malformations are reported. The patients were two men and five women, ranging in age from 23 to 54 years (mean, 30 yr). A bony foramen magnum decompression combined with the removal of the outer layer of the dura mater was performed in seven patients. In an average postoperative follow-up period of 2 years (range, 1-3 yr), neurological symptoms and signs improved in six of seven patients and were unchanged in one patient. Postoperative magnetic resonance imaging scans taken a few weeks after surgery showed a decrease in the size of the syrinx or a disappearance of the syrinx in five of seven patients; a few months were required for a similar reduction in the remaining two patients. In all seven patients, the decompression of the foramen magnum proved to be sufficient, as disclosed by postoperative magnetic resonance imaging. Foramen magnum decompression by our surgical technique is advantageous because all the procedures are extradural and there are, therefore, fewer postoperative complications than the foramen magnum decompression techniques previously reported. Foramen magnum decompression by the authors' surgical technique is effective as the initial surgical treatment for syringomyelia associated with Chiari I malformations.

The authors report nine cases of syringomyelia secondary to adhesive arachnoiditis treated in our institute from 1982 to 1991. Neurological signs, radiological features, and results of surgical treatment were reviewed. Common initial manifestations in association with syringomyelia were spastic paraparesis in 8 patients (88.9%), regional sensory loss in 4 (44.4%), neurogenic bladder in 4 (44.4%), and somatic pain in 2 (22.2%). All the neurological symptoms or signs progressed gradually for years. All the patients were treated with various modes of shunt operations, including syringo-peritoneal shunt in seven patients, syringo-subarachnoid shunt in one, and ventriculo-peritoneal shunt in one, respectively. Three patients who failed to resolve their major complaints after the first surgery further received syringo-peritoneal shunts. Postsurgical follow-up periods ranged from 18 months to 10 years. Serial MR imaging revealed a significant reduction of the size of syringomyelia in 8 of 9 patients. However only 6 patients showed certain improvement of neurological deficits. Three patients who failed to improve clearly had a long (beyond 15 years) history of syringomyelia before the first surgical treatment. A total of 11 syringo-peritoneal shunts were done in 8 patients of whom 5 patients improved neurologically. It was noticed that 4 of 5 successful syringo-peritoneal shunts were placed in the caudal level of the syrinx. In conclusion, syringo-peritoneal shunt may be at present an optimal mode of surgical management for syringomyelia secondary to adhesive arachnoiditis. In addition, we would like to recommend that follow-up serial MR imaging be done for patients having adhesive arachnoiditis in order to detect syringomyelia as early as possible.

The clinical presentations and radiological features of scoliosis accompanying syringomyelia were analyzed in 14 cases of syringomyelia associated with a in 14 cases of syringomyelia associated with a Chiari malformation in children. Scoliosis was the initial symptom in 11 out of 16 patients (64%) with syringomyelia and present in 14 (88%) at the initial examination. The scoliosis associated with syringomyelia was characterized by a higher incidence of a single curve (6 cases, 43%) and convexity to the left (7 cases, 50%) than seen in idiopathic scoliosis. The syrinx was shifted to the convex side of scoliosis on the axial section at the middle or lower thoracic level in patients with a single curve, and at the cervical or upper thoracic level in patients with a double curve. The authors think that the scoliosis develops in children as a result of damage done to the anterior horn, which innervates the muscles of the trunk, by an asymmetrically expanded syrinx.

Three cases of post-traumatic syringomyelia are presented and the mechanism of syrinx formation is discussed. Two cases were examined radiologically. Computed tomography and magnetic resonance images (MRI) showed an expansive syrinx with adhesive arachnoiditis in the thoracic levels below the injury site and a localized syrinx on the posterolateral gray matter in the cervical levels above the injury site. These syrinxes existed below the C2 level and had no communication with the fourth ventricle. The other was an autopsy case. Postmortem examination revealed that a syrinx existed from C2 to Th6 and had no communication with the fourth ventricle or the central canal. It is concluded that small traumatic cavities in the gray matter evolve to an extensive syrinx by cerebrospinal fluid (CSF) entering via the posterior root entry zone, and adhesive arachnoiditis is an important factor in increasing the CSF which is entering. MRI was useful for the diagnosis.

Posttraumatic syringomyelia is an uncommon but significant late complication of spinal cord injury. It occurs in approximately 1.1 - 3.2% of cases of spinal injuries. With the increasing availability of CT and magnetic resonance imaging (MRI), the incidence of posttraumatic syringomyelia is increasing. The purpose of this report is to show MRI of posttraumatic syringomyelia and to assess the results of surgical treatment. Materials and Methods This series included 16 cases of posttraumatic syringomyelia studied with MRI. 9 out of 16 cases showed delayed deterioration of neurological symptoms following spinal injuries. The interval between the trauma and the delayed symptoms of deterioration was from 2 years 2 months to 32 years (mean, 8 years and 5 months). There were 13 men and 3 women. The age ranged from 22 to 69 years, with a mean age of 42 years. The initial spinal cord injury was located in the lower cervical region in 4 cases, the thoracic region in 8, and the upper lumbar region in 4. All the patients were studied with resistive 0.15T system (Toshiba MRT 15 A) or a superconductive 0.5T system (Toshiba MRT 50 A) or a superconductive 1.5T system (GE Sigma or Siemens Magnetom). Six patients underwent 8 operative procedures for posttraumatic syringomyelia. Syringoperitoneal shunt was performed in 4 patients, syringosubarachnoid shunt in 3 and ventriculoperitoneal shunt in one. Results 1. MRI In all cases, the posttraumatic syringomyelia was easily diagnosed by MRI. The syrinx extended superiorly and/or inferiorly from the area of the old trauma. In 4 out of 16 cases, the syrinx extended into the medulla oblongata.(ABSTRACT TRUNCATED AT 250 WORDS)

An autopsied case of amyotrophic lateral sclerosis complicated by cervical syringomyelia was reported. The case was a 59-year-old man, who first noticed weakness of both lower extremities at 54-year-old. The weakness spread to both upper extremities within 2 years. Cervical myelography revealed multi-level cervical spondylosis and anterior fusion of C5-C7 was done. But the weakness and atrophy of proximal muscle, diminished deep tendon reflex on upper extremities, hyperreflexia and pathological reflexes on both legs, tongue fasciculation and respiratory muscle weakness developed successively, and the patient died of respiratory distress at 59-year-old. Autopsy revealed multiple independent four syrinxes located at the level between C2-C7. One of these syrinxes had ependymal cell lining and thought to be idiopathic syringomyelia. The other three syrinxes were considered to be the cavitation in association with cervical spondylotic myelopathy. Degeneration and decreasing of spinal anterior horn cells, atrophy of medullary pyramis and Bunina bodies were observed as features of typical amyotrophic lateral sclerosis. Cervical spondylosis as causative lesion of multiple syrinxes was discussed, and relationship between ALS and the syrinxes was not indicated clearly.

The clinical presentation, radiological features, and results of surgical treatment were analyzed in 17 cases of hydrosyringomyelia associated with a Chiari malformation, in children and adolescents younger than 20 years of age. The initial symptoms were a skeletal abnormality (71%), such as scoliosis (11 patients) or pes cavus (1 patient), pain or numbness (24%), and motor weakness (6%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (85%), muscle weakness (64%), muscle atrophy (35%), and lower cranial nerve palsy (35%). The characteristic neurological findings were unilateral sensory and motor deficits (65%) with decreased or absent deep tendon reflexes on the same side. The localization of the syrinx on the axial section varied according to the level, even in the same patient. In 11 patients with unilateral sensory disturbances or unilateral sensory and motor deficits, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance at the cervical or thoracic level. On the other hand, in 6 patients with bilateral sensory and motor deficits, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side. A syringosubarachnoid shunt was placed in 16 patients, foramen magnum decompression without closure of the obex was performed in 1 patient, ventriculoperitoneal shunt in 1 patient, terminal syringostomy in 1 patient, and foramen magnum decompression with terminal syringostomy in 1 patient. In 15 of 17 patients (88%), the neurological symptoms improved after an average follow-up of 4 years and 1 month. We think that as a surgical treatment, placement of a syringosubarachnoid shunt is effective.

The clinical presentation and radiological features were analyzed in 30 cases of syringomyelia associated with Chiari malformation. None of the patients had spinal dysraphism. The age on admission ranged from 6 to 59 years with a mean of 27 years. Syringomyelia was diagnosed by CT myelography and or MRI from 1982 to 1988. The initial symptoms were skeletal abnormality (43%) such as scoliosis (12 cases) or pes cavus (one case), unilateral pain or numbness (40%) and unilateral motor weakness (17%). Frequently seen signs on admission were sensory deficit (100%), scoliosis (57%), muscle weakness (57%), muscle atrophy (37%) and lower cranial nerve palsy (40%). The neurological findings were asymmetrical in all patients. The characteristic neurological findings in the cases presenting under 20 years of age were unilateral sensory and motor deficits (61%) with decreased or absent deep tendon reflex on the same side. The localization of the syrinx in axial section varied according to the level even in the same case. In 15 cases with unilateral sensory disturbance or unilateral sensory and motor deficit, the syrinx was located in the region corresponding to the posterolateral portion on the same side as that of sensory disturbance in the cervical or thoracic level. On the other hand, in 15 cases with bilateral sensory and motor deficit, the syrinx was located in the central portion and extended into the posterolateral portion of the more affected side.(ABSTRACT TRUNCATED AT 250 WORDS)

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.

Thirteen cases of syringomyelia which was confirmed by metrizamide CT myelography (MCTM) were reported. Nine cases were associated with Chiari malformation, and four were idiopathic type. Delayed CTM which were performed in several hours after the metrizamide myelography disclosed not only the existence of the syrinx but its shape and location. From the investigation of their clinical findings together with the appearance of their syrinx on CT scan, the following results were obtained. The syrinx was not always situated centrally within the cord, but had laterality at some spinal level in nine out of thirteen cases. The main laterality of the syrinx seemed to be located in the posterior horn. In the cases which had unilateral dissociated sensory disturbance, the laterality of sensory disturbance corresponded with the side of syrinx. These results suggested the possibility that the intrathecal injected contrast materials entered into the syrinx via the dorsolateral sulcus of the cord rather than via IV ventricle in some cases.

There is now a controversy about the methods of surgical treatment in syringomyelia associated with Chiari malformation. In this report, the authors emphasize that syringosubarachnoid shunt is effective as a surgical procedure or syringomyelia associated with Chiari malformation. From 1982 to 1986, 17 patients with syringomyelia associated with Chiari malformation were surgically treated. Seventeen patients underwent 22 operative procedures for syringomyelia. Syringosubarachnoid shunt was used in eighteen patients, foramen magnum decompression with syringosubarachnoid shunt in one, foramen magnum decompression with terminal syringostomy in one, terminal syringostomy in one, and ventriculoperitoneal shunt in one. The average postoperative follow up period was 2 years and 3 months, from 4 months to 4 years and 11 months. Thirteen out of 17 patients showed neurological improvement and 3 patients were neurologically unchanged. In one patient, the symptoms deteriorated. Syringosubarachnoid shunt is an effective therapeutic procedure for the patient with syringomyelia associated with Chiari malformation. We think that shunt malfunction by arachnoiditis after operation could be prevented by inserting the shunt tube into the subarachnoid space and closing the arachnoid membrane.

The surgical approach to syringomyelia is controversial. In this study, the authors evaluated the results of various operative procedures applied in 31 patients with syringomyelia. Syringomyelia was associated with Chiari malformation in 17 cases, with spinal adhesive arachnoiditis in nine, with trauma in three, and with epidural arachnoid cyst in one. One case was idiopathic. A total of 38 operations were performed. Syringosubarachnoid shunting was applied in 17 patients, syringoperitoneal shunting in 11, terminal syringostomy in three, ventriculoperitoneal shunting in three, lumboperitoneal shunting in two, foramen magnum decompression (suboccipital craniectomy plus upper cervical laminectomy) and terminal syringostomy in one, and foramen magnum decompression with syringosubarachnoid shunting in one. The postoperative follow-up period ranged from 2 to 55 months (average, 26 months). Of the 31 patients, 23 showed neurological improvement, five were unchanged, and three deteriorated. Among the last three, one patient with Chiari malformation developed shunt malfunction due to arachnoiditis after syringosubarachnoid shunting. In two patients with syringomyelia secondary to adhesive arachnoiditis, the spinal cord was damaged by extensive separation of the arachnoid membrane at surgery. On the basis of the results in these 31 cases, the authors conclude that syringosubarachnoid shunting is effective for syringomyelia associated with Chiari malformation if syringomyelia is responsible for the clinical symptoms. Post-traumatic syringomyelia and syringomyelia secondary to adhesive arachnoiditis should be treated by syringoperitoneal shunting. If hydrocephalus is present, ventriculoperitoneal shunting is indicated. Finally, terminal syringostomy is no more effective than syringosubarachnoid or syringoperitoneal shunting.

Thermographic findings in 10 cases of syringomyelia were reported. 7 cases were associated with Arnold-Chiari malformation and 3 cases with spinal adhesive arachnoiditis. The cases included 3 men and 7 women, aged 7 to 56 years (mean age 27.7 years). Comparing the right side of the body with the left side, in 9 cases, thermography demonstrated asymmetric skin temperature of the trunk or extremities. MRI revealed laterality of the syrinx at the level of cervical or lumbo-sacral cord in 9 cases. The side with the lower temperature corresponded with the side of lateralized syrinx in 8 cases. Neurologically, 9 cases had laterality of sensory disturbance and 6 cases had asymmetric motor weakness. The side with the lower temperature also corresponded with the laterality of sensory disturbance in 8 cases, and the side of the motor weakness in 4 cases. Considering these findings, many of the patients with syringomyelia are thought to have asymmetric skin temperature. Intramedullary involvement of sympathetic pathway by the syrinx may cause such a change. As thermography is non-invasive and easy to carry out, it should be a useful method for estimating or even detecting cases of syringomyelia, especially when applied to out-patients.

The authors report one autopsy case of syringomyelia associated with Chiari malformation and basilar impression. The pathogenesis of syringomyelia in our case is discussed. This 37-year-old man complained of progressive difficulty in swallowing and walking for two years. He had noticed dysarthria for six months before admission. (Examination) Neurological examination showed dysarthria, down beat nystagmus, disturbance of IXth nerve, Xth nerve and XIth nerve, and cerebellar ataxia. Deep tendon reflexes were hyperactive in the upper and lower extremities. Babinski's sign was positive bilaterally. Neuroradiological examination demonstrated basilar impression and Chiari malformation. (Operation) Suboccipital craniectomy and laminectomy of upper cervical vertebra were performed with dural plasty. Postoperatively he acquired some improvement, but soon after he was worse. He died of respiratory disturbance. (Postmortem examination) Though the central canal was obliterated at the C4 level, the syrinx extended from the C5 to Th7 level. From the C5 to C8, the syrinx was present in the areas of central gray matter, extending into the left dorsal horn, where it communicated with subarachnoid space. Furthermore, the abnormal vessels were noticeable around the syrinx. At the Th2 level, they were also shown in central grey matter where no syrinx existed. (Conclusion) The etiology of syringomyelia in our case was not explained by Gardner's hydrodynamic theory. We suggested that intramedullary abnormal vessels played an important part for the formation of the syringomyelia.

The clinical features of 31 cases of syringomyelic syndrome, confirmed by metrizamide CT myelography (CTM) or magnetic resonance imaging (MRI), were analysed. Bilateral suspended sensory disturbance, previously regarded as a characteristic feature, was encountered in only 2 cases (6.5%). Thirteen patients (41.9%) showed no motor deficits of the upper limbs and 7 patients (22.6%) did not have weakness or atrophy in any of the four extremities. Cervical segmental signs were absent in 7 cases (22.6%) and in 2 (6.5%) signs were only present in the lower limbs. Scoliosis and foot deformity, such as pes cavus or equinovarus, were seen in 17 (54.8%) and 5 cases (16.1%) respectively. These skeletal abnormalities were initial symptoms in some childhood cases. In syringomyelic syndrome, clinical features are variable with many atypical clinical manifestations, apart from the previously recognized characteristic clinical features.