The Currarino triad: What pediatric surgeons need to know

Abstract

© 2017 Elsevier Inc. Purpose We report our experience in managing a group of patients with Currarino syndrome, highlighting diagnostic challenges, surgical techniques, in addition to a review of current neurosurgical options. Patients and methods The study included patients with Currarino syndrome who presented to our pediatric surgery department during the period 2010 through 2016. The ‘sacral scimitar’ in plain X-ray provided the clue for the diagnosis; while MRI examination was essential to define the nature of the presacral mass and associated spinal anomalies. Results The study included 17 patients (13 girls and 4 boys). Their age at presentation ranged from 7 months to 10 years. We used posterior sagittal approach to correct anorectal anomalies, and excise presacral cysts that were subjected to histopathological examination. Two cases presented with a pelvic abscess (infected presacral dermoid cyst), which were initially drained followed by excision. The presacral mass consisted of either lipomyelocele (6), lipomyelomeningocele (3), or a developmental (dermoid) cyst (8). Tethering of the spinal cord was a common association (70%) Conclusion Apart from diagnostic challenges, the management of Currarino syndrome is similar to the usual management of ARM regarding the surgical approach and probably the prognosis that mainly depends on degree of associated sacral dysplasia. Level of evidence This is a case series with no comparison group (level IV).