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Related Subjects: |Syringomyelia |Syringobulbia |Dandy Walker syndrome

Syringomyelia is a chronic, progressive neurological condition characterized by the formation of a fluid-filled cavity or cyst, known as a syrinx, within the spinal cord. This cavity can expand over time, leading to damage to the spinal cord and disruption of the normal flow of cerebrospinal fluid (CSF). The condition can result in a range of neurological symptoms depending on the location and extent of the syrinx.

Dark pool without drain
Fingers burned, lost sense of pain
Cored out spinal cord
Named for nymph turned hollow reed
Neurosurgery in need
@DrCindyCooper

About

• Shawl like distribution of spinothalamic sensory loss with pain loss

Aetiology

• Developmental abnormality with an enlarged cavity in the cord begins in adolescence
• May be part of other developmental structural abnormalities that affect CSF drainage.
• Lower cervical and high thoracic longitudinal cyst or syrinx in the cervical cord anterior to the central canal
• Expanding cyst damages area around anterior commissure where the spinothalamic tracts cross
• There is a gradual enlargement of Syrinx with time
• Blockage of outflow from IVth ventricle with Arnold-Chiari malformation

Causes

• Chiari Malformation: A congenital condition where brain tissue extends into the spinal canal, often leading to the formation of a syrinx.
• Spinal Cord Trauma: Injury to the spinal cord, such as from a car accident or fall, can lead to the development of a syrinx, sometimes years after the initial injury.
• Spinal Cord Tumours: Tumours within or near the spinal cord can obstruct the normal flow of CSF, leading to syrinx formation.
• Post-Infectious or Post-Inflammatory Changes: Infections, such as meningitis, or inflammatory conditions affecting the central nervous system can result in the development of a syrinx.
• Arachnoiditis: Inflammation of the arachnoid membrane, often due to surgery, trauma, or infection, can lead to the formation of a syrinx.

Clinical

• Clinically a central cord syndrome with dissociated sensory loss
• Classically burns on fingers due to loss of pain sensation.
• Central sensory impairment has a cape-like distribution over the patient's shoulders
• Preservation of the dorsal columns proprioception and vibration.
• Hand and arm muscle LMN weakness and wasting as cord grey matter damaged as cavity expands.
• Upper motor neurone lesions below the lesion
• Bladder and bowels usually spared
• Horner's syndrome due to cervical cord damage
• Facial numbness (Trigeminal nerve nucleus down to C2)
• Extension into medulla causes syringobulbia with brainstem and even cerebellar signs
• Can be asymmetrical

Investigations

• Magnetic Resonance Imaging (MRI): MRI is the imaging modality of choice for diagnosing syringomyelia. It can clearly show the syrinx within the spinal cord and help assess its size and extent.
• Computed Tomography (CT) Myelography: This imaging technique may be used in cases where MRI is contraindicated, providing information on the CSF flow and the anatomy of the spinal cord.

Management

• Observation: In asymptomatic cases or those with mild symptoms, regular monitoring with MRI and clinical evaluation may be sufficient.
• Surgical Intervention:
  • Decompression Surgery: In cases related to Chiari malformation, surgery to enlarge the opening at the base of the skull can relieve pressure and improve CSF flow, potentially reducing the size of the syrinx.
  • Syrinx Shunting: A surgical procedure to drain the syrinx and prevent its expansion may be considered in certain cases.
  • Tumour Resection: If a spinal cord tumour is causing the syrinx, surgical removal of the tumour may be necessary.
• Rehabilitation: Physical therapy and occupational therapy may be beneficial to manage symptoms, improve function, and enhance quality of life.
• Pain Management: Medications, such as analgesics or anticonvulsants, may be used to manage chronic pain associated with syringomyelia.