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Published on: September 5, 2011
Currarino's syndrome in twins presenting as neonatal intestinal obstruction--identical presentation in non-identical
Ramnik V Patel1, Paolo De Coppi2, Edward Kiely3
1Department of Paediatric Urology, University College London Hospitals NHS Foundation Trust, London, UK Department of Paediatric Urology, Great Ormond Street Children Hospital NHS Trust, London, UK.
Abstract:
We report a case of non-identical twins who presented with identical neonatal intestinal obstruction with features of anorectal stenosis, presacral mass and sacral anomaly consistent with Currarino's syndrome or triad. Plain sacral radiograph, contrast enema and MRI were diagnostic. Initial management involved a defunctioning colostomy followed by a posterior sagittal anorectoplasty with excision of the teratoma ± anterior sacral meningocele and finally closure of colostomy in a staged multidisciplinary approach. The twins' father is also affected with features of Currarino's syndrome but was diagnosed during family screening. Currarino's syndrome presenting with identical neonatal low intestinal obstruction in a non-identical set of twins is rare and interesting. Antenatal diagnosis of Currarino's syndrome is difficult and may prove to be a challenge even in the postnatal period. Sacral spine radiograph, contrast enema and MRI are diagnostic. Management requires high index of suspicion, low threshold for MRI and multidisciplinary staged approach.
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