• Pieter J Emans, Jasper van Aalst, Ernest L W van Heurn, Carlo Marcelis, Gauke Kootstra, Regina G H Beets-Tan, Johannes S H Vles, and Emile A M Beuls.
• Department of Pediatric Surgery, University Hospital Maastricht, Maastricht, The Netherlands. pj.emans@orthop.unimaas.nl
• Neurosurgery. 2006 May 1;58(5):924-9; discussion 924-9.

ObjectivesThe Currarino triad, a relatively uncommon hereditary disorder, is often associated with tethered cord and anterior myelomeningocele. Little is known of the implications of these neuroanatomic malformations or of the neurosurgical attitude. The objective of this study is to identify the spinal cord and meningeal malformations associated with the Currarino triad and to discuss the risks and benefits of surgical intervention.MethodsWe analyzed the spinal cord malformations and the neurosurgical involvement with the Currarino triad by retrospective chart review.ResultsThe Currarino triad neuroanatomic malformations were identified in five patients. The Currarino triad was associated with a tethered cord in three patients, a myelomeningocele in five patients, a syrinx in two patients, a fistula between the colon and spinal canal in two patients, and an Arnold-Chiari Type 1 malformation in one patient.ConclusionFull spine imaging is required for all patients diagnosed with the Currarino triad. Magnetic resonance imaging of the head should be performed in every patient with neuroanatomic anomalies. Surgery of an anterior myelomeningocele is not necessarily indicated, only in the rare case in which the space-occupying aspect is expected to cause constipation or problems during pregnancy or delivery. Constipation directly after birth is seen in virtually all patients with the triad. Therefore, constipation cannot be used to diagnose a tethered cord syndrome nor indicate tethered cord release. Fistulas between the spinal canal and colon have to be operated on directly.

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