Makindo Medical Notes.com

Related Subjects: |Syringomyelia |Syringobulbia |Dandy Walker syndrome

Syringobulbia is a neurological condition characterized by the presence of a fluid-filled cavity, or syrinx, within the brainstem, specifically in the medulla oblongata. This condition is often associated with syringomyelia, where a similar syrinx forms within the spinal cord. Syringobulbia can lead to various neurological symptoms due to its location in the brainstem, affecting vital functions and cranial nerve nuclei.

About

• Fluid filled cavity in brainstem,

Aetiology

• Longitudinal cyst in the brainstem usually medulla
• Blockage of outflow from IVth ventricle with Arnold-Chiari malformation
• May be a continuation of syringomyelia

Causes

• Chiari Malformation: A congenital condition where brain tissue extends into the spinal canal, leading to syrinx formation.
• Spinal Cord Injury: Trauma to the spinal cord can lead to syrinx formation, which may extend into the brainstem.
• Tumours: Intramedullary tumours in the spinal cord or brainstem can cause syringobulbia by obstructing cerebrospinal fluid (CSF) flow.
• Post-Infectious or Post-Inflammatory Changes: Infections or inflammatory conditions affecting the central nervous system can lead to the formation of a syrinx.
• Arachnoiditis: Inflammation of the arachnoid membrane, often due to surgery, trauma, or infection, can result in syringobulbia.

Clinical

• Pain/temperature loss over the face in an onion skin like distribution sparing the nose
• Facial pain and loss of facial pain/temperature
• Horner's syndrome, Bulbar palsy
• Cerebellar signs if connections involved, Dysphagia
• Facial, palatal or laryngeal palsy - VII, IX, X, XI cranial nerves dysfunction
• Wasted tongue - XII cranial nerve dysfunction

Investigations

• MRI: The imaging modality of choice, which can reveal the extent of the syrinx within the brainstem and spinal cord.
• CT: May be used if MRI is unavailable, though it is less sensitive in detecting syrinx cavities.

Management

• Conservative Management: In asymptomatic/mild cases, monitoring with MRI
• Surgical Intervention:
  • Decompression Surgery: Often performed for Chiari malformation to relieve pressure and improve CSF flow, potentially reducing the size of the syrinx.
  • Syrinx Shunting: A surgical procedure to drain the syrinx and prevent further expansion.
  • Tumour Resection: If a tumour is the cause, surgical removal may be necessary.
• Rehabilitation: PT/OT, SLT to manage neurological deficits and improve quality of life.
• Symptomatic Treatment: Medications to manage pain, muscle spasticity, and other symptoms.