Primary spinal syringomyelia (original) (raw)
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Pathophysiology of primary spinal syringomyelia
Journal of Neurosurgery: Spine, 2012
Object The pathogenesis of syringomyelia in patients with an associated spinal lesion is incompletely understood. The authors hypothesized that in primary spinal syringomyelia, a subarachnoid block effectively shortens the length of the spinal subarachnoid space (SAS), reducing compliance and the ability of the spinal theca to dampen the subarachnoid CSF pressure waves produced by brain expansion during cardiac systole. This creates exaggerated spinal subarachnoid pressure waves during every heartbeat that act on the spinal cord above the block to drive CSF into the spinal cord and create a syrinx. After a syrinx is formed, enlarged subarachnoid pressure waves compress the external surface of the spinal cord, propel the syrinx fluid, and promote syrinx progression. Methods To elucidate the pathophysiology, the authors prospectively studied 36 adult patients with spinal lesions obstructing the spinal SAS. Testing before surgery included clinical examination; evaluation of anatomy on ...
Treatment of syringomyelia in patients with arachnoiditis at the cranio-cervical junction
World Neurosurgery, 2017
Objective: Cranio-cervical junction arachnoiditis (CCJA) is an uncommon cause of syringomyelia. The pathophysiology of syrinx formation is uncertain and the appropriate management unclear. A series of cases is reported to demonstrate variations in etiology, uniformity of functional CSF obstruction at the foramen magnum, and results of surgical intervention. Methods: We retrospectively analyzed the clinical and radiological features of a consecutive series of patients treated for syringomyelia related to CCJA. Results: Eight patients (5 male, 28-66 years old) were treated from 2000-2016. MRI demonstrated cervicothoracic syringomyelia in all cases, with the rostral extension of the syrinx suggesting communication with the fourth ventricle in all but one case. There was reduction of foramen magnum CSF space in all cases, cerebellar ectopia in five cases, and fourth ventricular entrapment in three cases. Treatment consisted of posterior fossa decompression with either a GoreTex or pericranial patch graft. Six patients had a fourth ventricle-spinal subarachnoid shunt. Two patients had titanium mesh cranioplasty. The immediate postoperative period was associated with reduction in syrinx cavity size, and improvement in neurological symptoms in all cases. At follow-up 10-60 months post-operatively, three patients exhibited recurrence of the syrinx, and underwent successful re-operation at the cranio-cervical junction. One patient with persistence of the inferior component of the syrinx was treated with a syrinx-spinal subarachnoid shunt. Conclusions: Most syrinx cavities associated with CCJA communicate with the fourth ventricle. Posterior fossa decompression and fourth ventricle to spinal subarachnoid space shunting appears a reasonable treatment for this form of syringomyelia.
Surgical treatment of syringomyelia: Favorable results with syringosubarachnoid shunting
Surgical Neurology, 1989
The authors review the clinical course of 29 patients who underwent syringosubarachnoid shunting for syringomyelia. Twenty-two patients presented hindbrain-related syringomyelia; seven patients had non-hindbrain-related syringomyelia secondary to trauma (four cases) and to spinal arachnoiditis (three cases). The surgical technique is described in detail. All patients showed postoperative deflation or collapse of the syrinx at follow-up magnetic resonance imaging evaluation. Symptoms stabilized in 17 cases (59%); 9 cases (31%) showed improvement in the neurological function; 3 cases (10%) presented delayed neurological deterioration, probably owing to spinal cord ischemia.
Spinal syringomyelia following subarachnoid hemorrhage
Journal of Clinical Neuroscience, 2012
Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.
Pathophysiology of persistent syringomyelia after decompressive craniocervical surgery
2010
S y r i n g o m y e l i a is more often associated with CM-I than any other etiology. The objective of surgical treatment of syringomyelia associated with the CM-I is to decompress the cerebellar tonsils, eliminate the syrinx, and prevent the progression of myelopathy. A reduced syrinx size, which is associated with disease stabilization, occurs after craniocervical decompression or syrinx luid shunting. Unlike craniocervical decompression, syrinx shunting does not address the underlying cause of the syringomyelia, requires invasion of the spi-J Neurosurg: Spine / Volume 13 / December 2010 a n c a r l o su f f r e D i n i , M.a., 1 re n é sM i t h , r.n., 1 he t t y l. DeVr o o M , r.
Neurosurgical Review, 2015
Surgical intervention is not so commonly deployed for idiopathic syringomyelia, even the symptomatic ones; is only undertaken, in the current era, after thorough clinical evaluation and extensive high-resolution neuroimaging; and is more assuredly performed, high-tech, in health facilities in the developed world with cutting edge logistic supports. In the practice environment of a low-resource African developing country, a 41-year-old young man recently presented in severe clinical-neurological deficit, Nurick grade 5, with an extensive cervical-medullary idiopathic syringomyelia. In spite of severe preoperative and intraoperative resource limitations, he successfully underwent spinal surgical decompression, fenestration of syrinx, and expansile duraplasty. He improved clinically postoperatively and achieved mobility, Nurick grade 3, before hospital discharge. He sustained this improvement, Nurick grade 3 to 2, 20 months post-op. He is now independent, needing only a walking stick to aid ambulation, and an MRI at this time showed only minimal focal post-op changes but total disappearance of the syrinx.
Surgical treatment of ?Hindbrain Related? syringomyelia: New data for pathogenesis
Acta Neurochirurgica, 1993
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 existense 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. Immunohystological 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.
Intraoperative measurement of spinal cord blood flow in syringomyelia
Clinical Neurology and Neurosurgery, 2000
The role of spinal cord ischemia in the pathophysiology of syringomyelia remains undetermined. Previous reports in the literature suggest that shunting of syringes can improve spinal cord blood flow. In order to determine the effects of syrinx decompression on spinal cord blood flow in patients with syringomyelia, we prospectively measured regional spinal cord blood flow (RSCBF) intraoperatively pre and post shunting in patients with symptomatic syringomyelia using laser doppler flowmetry. Six patients with MRI documented syringomyelia were studied (three with Arnold Chiari I malformation and associated syrinx and three with post-traumatic syringomyelia). Surgery was performed on all patients with either a syringopleural or syringoperitoneal shunt. Laser doppler blood flow and somatosensory evoked potentials were monitored prior to myelotomy and after shunt insertion. Results indicate that there was a significant increase in RSCBF after decompression of the syrinx. This study supports the hypothesis that spinal cord ischemia is important in the pathophysiology of syringomyelia and confirms previous reports in the literature regarding RSCBF in syringomyelia.
Syringomyelia associated with intradural arachnoid cysts
Journal of Neurosurgery: Spine, 2006
Object Intradural arachnoid cysts are relatively uncommon pouches of cerebrospinal fluid (CSF) found within the subarachnoid space. The authors present a series of eight symptomatic patients in whom syrinx cavities were associated with arachnoid cysts, and they discuss treatment strategies for this entity. Methods The population comprised eight men whose mean age was 50 years (range 35–81 years). All patients experienced gait difficulty, and it was the chief complaint in seven; arm pain was the primary complaint in one. No patient had a history of spinal trauma, meningitis, or previous spinal surgery at the level of the syrinx cavity or arachnoid cyst. In each patient imaging revealed a syrinx cavity affecting two to 10 vertebral levels. Posterior thoracic arachnoid cysts were found in proximity to the syrinx cavity in each case. There was no evidence of cavity enhancement, Chiari malformation, tethered cord, or hydrocephalus. All patients underwent thoracic laminectomy and resectio...
Spinal arteriovenous malformation associated with syringomyelia
Journal of Neurosurgery: Spine, 2009
The authors describe 4 cases of syringomyelia-associated spinal cord arteriovenous malformation (AVM). All cases were managed with embolization of the AVM. Treatments were aimed to stabilize the AVM itself and not directed toward the syrinx. In 3 of the 4 cases the syringomyelia resolved after treatment. Reports concerning AVM as a cause of syringomyelia is very scarce and lacks posttreatment clinical information. In light of the clinical course and imaging findings, the authors propose a theory that venous hypertension in the spinal cord is the trigger for the development of syringomyelia, which may reverse after AVM treatment.