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Syringomyelia of the distal spinal cord in children
H Meltzer1, H E James, D Trauner
1Division of Neurosurgery, School of Medicine, University of California, San Diego 92103, USA.
Insights
This study highlights distal cord syringomyelia in children presenting with unusual symptoms like bowel/bladder issues or severe leg pain. Surgical shunting effectively resolved these symptoms, indicating its importance in diagnosis.
Area of Science:
- Pediatric Neurology
- Neurosurgery
- Spinal Cord Imaging
Background:
- Syringomyelia commonly affects the cervical and thoracic spinal cord.
- Distal cord syringomyelia is less common and may present atypically.
Observation:
- Two pediatric cases presented with unique symptoms: bowel/bladder dysfunction and paroxysmal leg pain/incontinence.
- Magnetic resonance imaging (MRI) revealed a syrinx localized to the distal spinal cord in both patients.
- These cases were not associated with Arnold-Chiari malformation, trauma, tumors, or spinal dysraphism.
Findings:
- Syringomyelia-peritoneal shunting led to complete resolution of symptoms in both children.
- Postoperative MRI confirmed complete collapse of the syringomyelia cavity.
- This suggests surgical intervention is effective for distal cord syringomyelia.
Implications:
- Clinicians should consider distal cord syringomyelia in the differential diagnosis for children with unexplained bowel/bladder disturbances or lower extremity pain/spasms.
- Early diagnosis and treatment via shunting can significantly improve patient outcomes.
- This finding expands the understanding of syringomyelia presentation and management in pediatric populations.
Abstract:
The syndrome of syringomyelia in children has been extensively described in the literature and is said to most commonly involve the cervical and thoracic spinal cord. We here present two children who had an unusual constellation of signs and symptoms, characterized by bowel and bladder disturbance in one, and in the other with intermittent paroxysmal severe pain and cramping and flexion of the lower extremities accompanied at times by episodes of incontinence. Both were found on magnetic resonance imaging (MRI) to have a syrinx localized to the most distal cord. This was not associated with Arnold-Chiari malformation, trauma, tumors or any other form of spinal dysraphism. Both patients underwent placement of a syringomyelia-peritoneal shunt with complete resolution of symptomatology. Postoperative MRI revealed a complete collapse of the syringomyelia cavity. In those children presenting with bowel or bladder disturbances, associated or not with intermittent pain, flexion attacks, or cramping in the lower extremities, the differential diagnosis of a syringomyelia of the distal cord should be part of the clinical considerations.