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Natural history of untreated syringomyelia in pediatric patients
Ash Singhal1, Tim Bowen-Roberts, Paul Steinbok
1Department of Surgery, Division of Pediatric Neurosurgery, British Columbia’s Children’s Hospital and University of British Columbia, Vancouver, British Columbia, Canada. ash.singhal@cw.bc.ca
Insights
Pediatric syringomyelia often remains stable without surgery. Periodic imaging is recommended, as a small percentage of nonoperative cases show syrinx enlargement or symptom progression.
Area of Science:
- Pediatric Neurosurgery
- Neurology
- Radiology
Background:
- The natural history of pediatric syringomyelia, particularly in nonoperative cases, is not well-documented.
- Existing research often focuses on symptomatic or surgically treated patients, leaving a gap in understanding the course of untreated syrinxes.
Purpose of the Study:
- To investigate the natural history of untreated syringomyelia in pediatric patients with minimal neurological symptoms.
- To analyze the clinical and radiological course of nonoperative syrinxes in children.
Main Methods:
- Retrospective review of a neurosurgery database for pediatric patients (<18 years) with spinal syrinxes on MRI.
- Inclusion criteria: at least two spinal MRIs at least one year apart during nonoperative management.
- Analysis of MRI for syrinx size changes and clinical notes for symptom evolution.
Main Results:
- 17 pediatric patients with nonoperative syringomyelia were analyzed.
- Most patients presented with mild symptoms; neurosurgeons deemed the syrinx unrelated to symptoms in all cases.
- Syrinxes remained unchanged or diminished in 88% of patients; 94% showed no symptom worsening.
- Two patients (12%) experienced syrinx enlargement, with one worsening clinically; both had Chiari malformations and underwent surgery.
Conclusions:
- Pediatric syringomyelia managed nonoperatively often demonstrates stability.
- Despite overall stability, a small proportion of cases show syrinx enlargement, necessitating periodic imaging for monitoring.
- The findings support continued surveillance, including imaging, for nonoperative pediatric syringomyelia.
Object:
The natural history of syringomyelia in pediatric patients remains uncertain. Although symptomatic and operative cases of syringomyelia are well studied, there are fewer articles in the literature on the nonoperative syrinx and its clinical and radiological course. The purpose of this research was to analyze the natural history of untreated syringomyelia in pediatric patients presenting with minimal neurological symptoms.
Methods:
A review of the neurosurgery database at British Columbia's Children's Hospital identified all pediatric patients (< 18 years of age) with syringes identified on MR imaging. Patients were included in this study if they had at least 2 MR images of the spine, at least 1 year apart, while receiving nonoperative treatment. Magnetic resonance imaging was used to determine changes in the size of the syrinx over time. Clinic notes were analyzed to establish demographic and clinical features and to determine any clinical changes over time.
Results:
A total of 17 patients were included in the study. Symptoms at presentation were often mild and included limb numbness (3 cases), headaches (2 cases), mild sensory deficits (2 cases), mild motor deficits (3 cases), and intermittent incontinence (7 cases). The consultant neurosurgeon believed that the syrinx was not contributing to the symptoms in these 17 patients. The syrinx either remained unchanged (7 cases) or diminished in size (8 cases) in a total of 15 patients (88%). In the remaining 2 patients the authors noted an increase in syrinx size, in 1 of whom the clinical course also worsened. Both of these patients had a Chiari malformation and subsequently underwent craniocervical decompression. Overall, the mean change was -0.7 mm of maximal axial diameter (range -2.6 to +2.7 mm). Sixteen patients (94%) exhibited no worsening of symptoms over time.
Conclusions:
Syringomyelia often remains stable in patients receiving nonoperative treatment. However, given that 2 (12%) of 17 syringes in this series enlarged, it is likely appropriate to include periodic imaging in the follow-up of these cases.
