Syringomyelia complicating myelomeningocele: review of the evidence

Piatt JH Jr. Syringomyelia complicating myelomeningocele: review of the evidence. Journal of Neurosurgery. 2004 Feb;100(2 Suppl Pediatrics):101-9. Review.

Syringomyelia is frequently found in association with myelomeningocele. Although often asymptomatic, it can, in exceptional cases, cause severe morbidity. The author performed a structured literature review to address the following aspects of this clinical problem. What is the natural history? There are data concerning the imaging prevalence, the autopsy prevalence, and the prevalence of clinically active syringomyelia among patients with myelomeningocele, but literature provides no description of the course of this condition over time. What is the clinical significance? That correlations among symptoms, signs, treatments, and imaging findings are poorly described is no surprise in view of the large fraction of patients with syringomyelia who are asymptomatic. There is, however, an impressive mass of anecdotal evidence associating progressive syringomyelia with untreated (or inadequately treated) hydrocephalus. How to make the diagnosis? There is no disagreement that magnetic resonance imaging is the diagnostic modality of choice, but the literature provides very little guidance about who should undergo this investigation. There is no analysis of the costs and benefits of screening or periodic surveillance. What is the best treatment? Many surgical procedures have been reported to be useful as components of complex algorithms relating clinical factors and imaging data to treatment recommendations. There have been no controlled studies. There have been no prospective studies. There have been no multiinstitutional studies. No studies in which outcomes have been evaluated using objective, validated instruments. No studies in which the robustness of treatment effects over time have been documented. The complexity of treatment selection in contemporary practice makes syringomyelia unsuitable for randomized controlled trials of different therapies. Prospective cohort studies are feasible, however, and hold the potential to address many important questions about natural history and patient outcomes.

PMID: 14758937