Natural history of untreated syringomyelia in pediatric patients

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>A review of the neurosurgery database at British Columbia's Children's Hospital identified all pediatric patients (&lt; 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.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>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.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>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.</jats:p></jats:sec>

Chiari 1 Malformation and Holocord Syringomyelia in Hunter Syndrome

“…In 90 % of cases, syringomyelia is associated with CM1 (Fernández et al 2009). From a clinical point of view, syringomyelia typically present in children with insidious onset and slow progression (Singhal et al 2011). Most often, scoliosis, motor or dissociate sensory impairment are noted due to sensory tract or corticospinal tract disruption and/or anterior horn cell involvement (Isu et al 1990;Feldstein and Choudhri 1999;Attenello et al 2008).…”

“…The latter presents usually with a progressive cavitation (syringomyelia) that might involve the entire length of the cord and/or the medulla oblongata. Typically, syringomyelia appears in adulthood (Masson and Colombani 2005); children might be affected and present with insidious onset and slow progression (Singhal et al 2011). Signs and symptoms of CM1 (e.g., headache, neck pain, or lower brainstem dysfunction) (Wu et al 1999;Greenlee et al 2002) and syringomyelia (e.g., scoliosis, motor or dissociate sensory impairment) (Isu et al 1990;Attenello et al 2008) also overlap or might be difficult to ascertain in MPS patients.…”

Management of idiopathic pediatric syringohydromyelia

“…Median clinical follow-up was 20.5 months, and median radiological follow-up was 13 months. The median maximum syrinx diameter was 2 mm (range 0.5-17 mm) and the median number of vertebral levels spanned was 5 (range [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. Eighty-one of the patients had a syrinx diameter equal to or greater than 2 mm.…”

Incidental Findings on Brain and Spine Imaging in Children

“…When no primary disorder is identified, the syrinx is considered idiopathic. The untreated natural history of idiopathic syringomyelia is excellent, 158,159 and the majority of such cases should not be considered for surgical treatment. Small spinal syringes may be difficult to distinguish from minimal dilations of the central canal of the spinal cord.…”

“…150,151,156 Although most cases of clinically relevant syringomyelia are associated with CM, it may also be seen in patients with spinal cord tumor, tethered cord, or arachnoiditis. 157,158 In general, the management of syringomyelia should be directed at the primary disorder. When no primary disorder is identified, the syrinx is considered idiopathic.…”