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Basilar impression, Chiari malformation and syringomyelia: a retrospective study of 53 surgically treated patients.

The present study shows the results of 53 patients who have been treated surgically for basilar impression (BI), Chiari malformation (CM), and syringomyelia (SM). The patients were divided into two groups. Group I (24 patients) underwent osteodural decompression with large inferior occipital craniectomy, laminectomy from C 1 to C 3, dural opening in Y format, dissection of arachnoid adhesion between the cerebellar tonsils, medulla oblongata and spinal cord, large opening of the fourth ventricle and dural grafting with the use of bovine pericardium. Group II patients (29 patients) underwent osteodural-neural decompression with the same procedures described above plus dissection of the arachnoid adherences of the vessels of the region of the cerebellar tonsils, and tonsillectomy (amputation) in 10 cases, and as for the remainning 19 cases, intrapial aspiration of the cerebellar tonsils was performed. The residual pial sac was sutured to the dura in craniolateral position. After completion of the suture of the dural grafting, a thread was run through the graft at the level of the created cisterna magna and fixed to the cervical aponeurosis so as to move the dural graft on a posterior- caudal direction, avoiding, in this way, its adherence to the cerebellum.

Adult↗

Von Hippel-Lindau disease with multiple spinal cord hemangioblastomas, syringomyelia and pheochromocytoma.

A 31-year-old blind man presented with numbness and weakness in the left hand, and elevated blood pressure. Multiple hemangioblastomas in the spinal cord associated with syringomyelia were well demonstrated by gadolinium-enhanced magnetic resonance imaging (MRI). He also had pheochromocytoma in the right adrenal gland, which was disclosed by abdominal computed tomography, MRI and 131I-metaiodobenzylguanidine scintigraphy. MRI screening should be considered for patients with von Hippel-Lindau gene to detect the multiple lesions in this disease.

Adrenal Gland Neoplasms↗

[Post-traumatic syringomyelia. Report of three cases].

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.

Adult↗

Cerebellopontine angle meningioma causing asymptomatic syringomyelia--case report.

A 36-year-old female was admitted with a 3-month history of headache and gait disturbance. Magnetic resonance imaging demonstrated a large tumor in the right cerebellopontine angle and syringomyelia in the upper cervical cord associated with caudal displacement of the cerebellar tonsil. Complete removal of the tumor resulted in disappearance of these associated conditions. Herniation of the cerebellar tonsil and distortion of the brain stem had probably caused disturbance of cerebrospinal fluid flow, which combined with obstruction of the spinal canal, caused the syrinx.

Adult↗

MR imaging of syringomyelia and hydromyelia.

The relative effectiveness of plain computed tomography (CT), metrizamide CT, conventional myelography, and magnetic resonance (MR) imaging was compared for the examination of cystic spinal cord lesions. Intramedullary cavities in 18 patients were demonstrated by MR imaging: cavities were uncomplicated in 13 patients, associated with spinal tumors in two, and studied after occipital craniectomy for treatment of Chiari malformation and syringomyelia in two. Cavities were shown by MR imaging in all enlarged spinal cords, but a cavity was shown in only one of four small cords. The rostral limits of the cavities were demonstrated better than were the caudal extensions. Ventricular communication was not demonstrated. Chiari malformation was shown only in cavities that did not involve the medulla. Syringes associated with tumor were indistinguishable from uncomplicated cavities, but the tumor had abnormal signal on long spin-echo sequences in two cases. Cystic cord tumor (one case) had an inhomogeneous appearance. Caudal displacement of the cerebellar hemisphere through the surgical defect associated with compression of the fourth ventricle was shown in two cases after posterior fossa craniectomy. Thirteen patients were studied with metrizamide CT also. MR imaging proved to be as accurate as metrizamide CT in the diagnosis of intramedullary cavities that result in spinal cord enlargement, but it was less sensitive in detecting cavities within normal-sized or diminished spinal cords. It had the advantage that tumor tissue could be distinguished from associated syrinx cavities by differences in signal characteristics; and cerebellar ectopia was evaluated easily on sagittal MR views.

Arnold-Chiari Malformation↗

Mechanisms and treatment in post-traumatic syringomyelia.

The aetiology, pathophysiology, and treatment of post-traumatic syringomyelia is discussed. The data collected from clinical observation, radiodiagnostic methods, and operative findings are illustrated. It is concluded that at least five different processes may contribute to this progressive disease. A deeper understanding of these mechanisms could help in patient management.

Adolescent↗

Treatment of posttraumatic syringomyelia with extradural decompressive surgery.

The authors review the management of five patients with posttraumatic syringomyelia (PTS) associated with an uncorrected spinal deformity. Patients with evidence of progressive neurological deterioration underwent ventral spinal decompressive surgery. The mean patient age at the time of injury was 39 years, and the time between injury and the diagnosis of PTS ranged from 2 to 22 years. Mechanisms of injury consisted of fracture/subluxations in three patients and burst fractures in two. All patients experienced delayed neurological deterioration consistent with PTS. Magnetic resonance imaging revealed ventral deformities, and the spinal canal stenosis ranged from 20 to 50% (mean 39%). All patients underwent ventral epidural spinal decompressive surgery to correct the bone deformity and restore the spinal canal. The mean follow-up period was 38 months. The decompressive intervention was initially successful in treating the neurological deterioration in all patients. Symptoms resolved completely in four patients, and the other experienced neurological improvement. Postoperative magnetic resonance imaging revealed a reduction in the size of syrinx cavity in the patients whose symptoms resolved and no change in the remaining patient. Two patients required a subsequent second-stage posterior intradural exploration and duraplasty for recurrence of symptoms and/or syrinx. Posttraumatic spinal deformity may cause spinal canal stenosis and alter subarachnoid cerebrospinal fluid (CSF) flow in certain patients. Ventral epidural spinal decompressive surgery may result in neurological improvement and a reduction of the syrinx cavity, avoiding the need for placement of a shunt or other intradural procedures. However, some patients will also require reconstruction of the posterior subarachnoid space with duraplasty if the ventral decompressive procedure achieves only partial restoration of the subarachnoid CSF flow.

Adult↗

Syringosubarachnoid shunt for syringomyelia associated with Chiari I malformation.

OBJECT: The authors describe the surgical procedures for placing syringosubarachnoid shunts and the results of surgery, as well as the prevention of shunt malfunction. METHODS: The series consisted of 59 patients with syringomyelia associated with Chiari I malformation in whom syringosubarachnoid shunts were placed. Their ages ranged from 4 to 62 years (median 28 years). The follow-up period ranged from 13 to 219 months. The authors principally implanted the shunts in patients with large-sized syringes. Neurological improvement was satisfactory, and postoperative magnetic resonance imaging demonstrated that the syringes had resolved or decreased in size in all patients. Reoperation was necessary in 10 patients who were treated before 1993. CONCLUSIONS: To prevent shunt malfunction, both dorsal root entry zone myelotomy and placement of the syringosubarachnoid shunt tube into the ventral subarachnoid space are useful.

Adolescent↗

Experimental hydrosyringomyelia, ischemic myelopathy, and syringomyelia.

The authors found that cavities in the spinal cord of dogs appearing after the intracisternal administration of kaolin are not due to ischemic softening, but rather to distention and subsequent rupture of the central canal. Ischemic lesions could be produced and were found to have a quite different histopathology; this supports the hydrodynamic hypothesis of cavitary myelopathy following adhesive arachnoiditis, probably as a compensatory mechanism to the associated hydrocephalus. The similarities to human syringomyelia are discussed.

Animals↗

Syringomyelia: long-term results after posterior fossa decompression.

A retrospective study of the surgical results in 31 adult patients with syringomyelia associated with Arnold-Chiari malformation was carried out. The standard surgical procedure consisted of a posterior fossa craniectomy. The vallecula was opened in 28 patients, with plugging of the central canal in 19. In three patients, severe arachnoidal scarring was present, and dissection was not attempted. The postoperative follow-up period ranged from 5 to 10 years. More than half of the patients considered themselves improved by surgery; however, only five cases showed objective improvement. Despite the operation, six patients experienced progressive neurological deterioration.

Adolescent↗

Posttraumatic syringomyelia. Case report.

The ninth case of posttraumatic syringomyelia with postmortem confirmation is presented. Onset of symptoms occurred 19 years after the original trauma. The review of the literature with a discussion of postulated mechanisms of syrinx distention follows. The present case differs from previously reported postmortem cases in that there was communication through the medullary parenchyma with the fourth ventricle.

Adult↗

Neurocutaneous melanosis with hydrocephalus and syringomyelia. Case report.

The authors report the investigation, treatment, and progress of a unique case of neurocutaneous melanosis with syringomyelia. The suspected etiology, the pathology, and the variable clinical manifestations of this rare phakomatosis are recounted. The potential for malignant change and the possible mechanisms of syrinx development in this syndrome are discussed.

Child↗

Syringomyelia as a complication of Paget's disease. Case report.

Cranial settling and basilar invagination with medullospinal compression is believed to have resulted in the production of a syrinx in a case of Paget's disease of the cranium. This mechanism of compression at the craniovertebral junction resembles the development of syringomyelia in Chiari malformations presenting in adult life.

Female↗

Chiari I malformation with syringomyelia. Evaluation of surgical therapy by magnetic resonance imaging.

Five patients with a Chiari I-syringomyelia complex of adult onset were evaluated by magnetic resonance (MR) imaging. All patients underwent suboccipital craniotomy with upper cervical (C-1 and part of C-2) laminectomy, arachnoid retraction, and duraplasty. Postoperative MR studies of four patients disclosed collapse of the syringomyelic cavity, even when the cavity extended into the thoracic region. This appeared to be a progressive process taking place over several weeks. Operative complications are noted and physiological implications are discussed.

Adolescent↗

Syringomyelia extending to the basal ganglia. Case report.

A 10-year-old girl was admitted to the hospital with complaints of progressive right hemiparesis and sensory disturbance. Magnetic resonance imaging revealed a Chiari Type I malformation and syringomyelia from T-10 to C-1. The syrinx extended from the medulla to the right putamen along the pyramidal tract.

Basal Ganglia↗

Syringomyelia-Chiari complex: magnetic resonance imaging and clinical evaluation of surgical treatment.

Thirty patients with syringomyelia-Chiari complex who underwent posterior fossa decompression or syringosubarachnoid shunting were studied clinically and by magnetic resonance (MR) imaging to assess the changes in the syrinx and in their clinical picture after surgery. When symptoms of posterior fossa compression were present, posterior fossa decompression was performed; however, when symptoms of posterior fossa compression were absent, the choice of posterior fossa decompression or syringosubarachnoid shunting depended, respectively, on whether the syrinx was narrow or wide on MR imaging. At least 1 year after surgery, subjective improvement or arrest of disease was recorded in 73% of the patients. The present study suggests that: 1) the symptoms attributed to spinal cord damage have no significant relationship to the size of the syrinx on MR images; 2) the surgical techniques employed in this series (posterior fossa decompression or syringosubarachnoid shunt) were equally useful in inducing syrinx collapse; and 3) when posterior fossa decompression is performed, plugging of the obex is not necessary for syrinx collapse.

Adolescent↗