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Neurological Disease
Published in John S. Axford, Chris A. O'Callaghan, Medicine for Finals and Beyond, 2023
In this rare condition, a central fluid-filled cavity develops in the spinal cord (syringomyelia) or brainstem (syringobulbia). It may be associated with congenital abnormalities at the foramen magnum or occur secondary to a tumour of the cord or to trauma. The expanding cavity compresses sensory fibres crossing the midline on their way to join the lateral spinothalamic tract; later, it may also compress the corticospinal tracts. There is loss of pain and temperature (but not light touch, proprioception or vibration) sensation at the segmental levels of the syrinx along with reflex loss at that level. As symptoms progress UMN signs may develop in the legs.
Kyphotic Deformity of the Spine
Published in Benjamin Joseph, Selvadurai Nayagam, Randall T Loder, Anjali Benjamin Daniel, Essential Paediatric Orthopaedic Decision Making, 2022
A 17-year old-male presented for evaluation of a progressive spinal deformity in the upper thoracic spine (Figure 19.1a–e). Diagnoses of cutis marmorata telangiectatica congenita syndrome, congenital hydrocephalus, macrocephaly, growth hormone deficiency, hypothyroidism, and Chiari malformation with basilar invagination had been made previously. He also had a history of a vertebro-basilar ischemic stroke at 1 year of age. At the age of 1 year, he underwent C1–2 laminectomy with Chiari decompression and partial resection of his cerebellar tonsils. At 12 years of age, a repeat intradural decompression was abandoned due to adhesions. The following year, he was diagnosed with a syrinx and underwent a T4 laminectomy and placement of a syringo-pleural shunt. A follow-up MRI revealed that the syrinx was still present but was slightly smaller (Figure 19.2a, b).
Syringomyelia and Lower Urinary Tract Dysfunction
Published in Jacques Corcos, Gilles Karsenty, Thomas Kessler, David Ginsberg, Essentials of the Adult Neurogenic Bladder, 2020
When performing an MRI on a patient with syringomyelia, a few things are clearly seen. The syrinx is tube shaped and extends beyond the spinal cord injury (SCI) site to at least two vertebral levels. The signal is homogeneous and clearly delimits the upper and lower limits of the syringomyelia. The extension is always much more significant than the clinics suppose.
Association between subarachnoid hemorrhage-induced hydrocephalus and hydromyelia: pathophysiological changes developed in an experimental model
Published in Neurological Research, 2023
A ‘craniospinal pressure dissociation’ theory was postulated by Williams [6]. This theory posited that significant pressure changes during daily activities may increase the intrathoracic pressure, as reflected in the spinal CSF through the epidural spinal veins [6]. The CSF flow from the cranial to the SAS reflects expansile brain motion during the cardiac cycle, especially in the arterial pulse. A syrinx cavity is frequently accompanied by a myelomalacia area. The progression of a syrinx cavity transmits the pressure to the medulla spinalis surface. The syrinx does not require communication between the central channel and the ventricular system. The dorsal root entry zone and the perivascular spaces can influence the communication between the central channel and extracellular spaces of the medulla spinalis. This mechanism can play a primary role in compensating for the changes occurring in the perimedullary CSF flow. However, pathologies and iatrogenic conditions, such as spinal meningitis, SAH, peridural anesthesia, intramedullary spinal surgery, infarction, trauma, and spinal injuries, frequently cause arachnoid scarring that impaired this mechanism [24,25]. Both parenchymal (extracanalicular) syringes and paracentral dissections of central channel syringes are lined with glial or fibroglial tissues that are frequently ruptured into the SAS. The fibroglial tissue is characterized by Wallerian degeneration, neuronophagia, and central chromatolysis. The clinical presentation in patients varies depending on the affected tracts and nuclei with this cavity [2,24,25].
Experience with revision craniovertebral decompression in adult patients with Chiari malformation type 1, with or without syringomyelia
Published in British Journal of Neurosurgery, 2022
Adikarige Silva, Gopiga Thanabalasundaram, Ben Wilkinson, Georgios Tsermoulas, Graham Flint
Our revision surgery appeared to be particularly successful in the presence of syringomyelia, with all 15 syringomyelia patients demonstrating resolution of their symptoms. This, too, is reflected in the literature, with 95% improvement rates being reported following revisional craniovertebral decompression.15 For this reason, some authorities advocate secondary decompressive surgery with intradural exploration, in preference to syrinx shunting, after failed primary craniovertebral decompression for hindbrain-related syringomyelia.6,15,18 The counter view is that revision surgery is more hazardous than placing a shunt into the syrinx cavity. Our results would suggest that revision surgery does not carry undue risk and can be effective in a majority of cases. It avoids leaving a patient dependent upon implanted shunt tubing, which can block after a period of time.27 With those of our patients for whom follow-up imaging was available, syrinx resolution or reduction was observed in all cases. This result at least matches other published outcomes.28
Rapid progression of acute cervical syringomyelia: A case report of delayed complications following spinal cord injury
Published in The Journal of Spinal Cord Medicine, 2022
Chenghua Yuan, Jian Guan, Fengzeng Jian
The incidence of post-traumatic syringomyelia varies between 0.3–3.2% and a varying interval between an SCI and the initial presentation of syringomyelia from months to years has been reported,6 but there are limited reports of delayed post-traumatic syringobulbia.7,8 Briefly, a patient with syringobulbia was reported,8 in whom symptomatic regression and a significant reduction in syrinx size occurred after a syringo-peritoneal shunt surgery. Fourteen years before admission, the patient underwent spinal instrumentation surgery for thoracolumbar fractures. A similar case was described involving a T12 compression fracture 30 years prior to the development of new symptoms, with weakness and sensory impairment in the patient’s left arm for 5 years.9 MRI results revealed a syringobulbia. During surgery, the thickened arachnoid membrane was cut, and the foramen of Magendie was explored. Post-operatively, symptoms abated and the syrinx diminished. Unfortunately, the patient's symptoms gradually worsened; it is not clear why the surgery did not target the level of fracture.