Idiopathic syrinx in the pediatric population: a combined center experience

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Discovery of a syrinx in a child, without a readily identifiable proximate cause such as a Chiari malformation, tumor, or site of tethering, is often a cause of concern for families and a source of consternation for clinicians. There is a paucity of data describing the natural history of an idiopathic syrinx in the pediatric population. The authors present the combined data of 2 major pediatric neurosurgical centers to describe their experience with this condition.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>Data were collected at Children's Hospital Boston and St. Louis Children's Hospital according to institutional review board–approved protocols and captured visits over a 2.5-year interval (October 2006–March 2009), with records reviewed if the patient had a preexisting diagnosis of syrinx. Patients were identified by ICD-9 codes derived from departmental databases. All pediatric patients (age &lt; 19 years) in whom idiopathic syrinx had been diagnosed, as defined by MR imaging findings (dilated central canal in the spinal cord of ≥ 1 mm in axial dimension and extending over at least 2 vertebral levels), were included.</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Forty-eight patients met the criteria for idiopathic syrinx during this period, and in 32 of them detailed follow-up imaging was available. Discovery of a syrinx was incidental in 6 patients, whereas the others were referred for imaging because of the presence of pain, neurological symptoms, scoliosis, or skin markings. The average age at the first MR imaging session was 9.7 years, with a mean syrinx size of 4 mm (range 1.2–9.4 mm). The majority (52%) of patients had a thoracic syrinx, with the average lesion spanning 7.1 vertebral levels. The average follow-up was 23.8 months (range 2–64 months), and subgroups of patients with &lt; 3 years and ≥ 3 years of follow-up were independently reviewed. Overall, symptoms improved in 34% and worsened in 9%; 57% of the patients remained asymptomatic or stable. Radiographically (in the subgroup of 32 patients with detailed follow-up imaging), syrinx size decreased in 25% of patients, increased in 12.5%, and remained unchanged in 62.5%, with no apparent correlation between change in syrinx size and clinical symptoms.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Clinically, children with an idiopathic syrinx remained asymptomatic, stable, or improved in 91% of cases. The majority of syringes (87.5%) remained stable or shrank over time, with no apparent correlation between changes in size and changes in symptoms. Although longer follow-up is needed, these data suggest that the natural history of an idiopathic syrinx in children is benign, and that repeated imaging may not be necessary.</jats:p></jats:sec>

Idiopathic syringomyelia: retrospective case series, comprehensive review, and update on management

“…In particular, a nonoperative approach is justified when a patient is either asymptomatic or experiences relatively mild symptoms. In their study on IS, Magge et al 26 also suggested that surgical management of the syrinx may not offer much utility since both their surgical cases either worsened or showed no changes clinically. We routinely may obtain follow-up MR images on an annual basis or more frequently as dictated by symptoms.…”

“…Specifically, regarding the issue of pain, Magge et al 26 noted that there does not appear to be any correlation between syrinx size or location and the symptom of pain. Although we did find an overall increased syrinx diameter in the 2 surgical cases, the relevance is unclear given the few cases we have.…”

“…In a population of 48 children with IS, Magge et al 26 divided the presentation into the following 5 groups: scoliosis, cutaneous marker/developmental anomaly, pain, neurological findings, and screening/incidental finds. Chern et al…”

“…15,26,34 Although not clearly defined, the general consensus seems to be that IS is not associated with any of the conditions mentioned previously. 3,28,31,34,37 The management and natural history of such idiopathic syringes remain unclear.…”

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.…”