Currarino Syndroma, initially misdiagnosed and treated like megacolon congenitum

  • Ivona Djordjevic
  • Danica Jovanovic
  • Blagoje Grujic
Case Report


Currarino syndrome was first described as a triad by Guido Currarino, an American radiologist in 1981. It is an autosomal dominant hereditary disease known by the triad of anorectal stenosis, anterior sacral defect, and a presacral mass that is most often an anterior sacral meningocele.

We represent a 3 year boy with refractory constipation from birth, which had been wrongly diagnosed and treated as Hirschsprung’s disease since early childhood. The patient underwent urgent colostomy because of water intoxication due to bowel irrigation, and following investigations (CT, MRI) revealed anorectal anomaly and presacral mass compatible with Curarrino’s syndrome.

Definitive operation was done with perineal approach, posterior sagittal anorectoplasty, with interval colostomy closure 2 months after perineal operation. An uncomplicated postoperative recovery ensued. The patient’s bowel habits successfully returned to normal soon after the operation. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are rectal examination, sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of Currarino syndrome families, CT scan, magnetic resonance (MR) evaluation of patient spine and pelvis and suction rectal biopsies. If the mass is a meningocele, colostomy and neurosurgical exploration should precede anoplasty due to the risk of meningitis.


Currarino’s triad Constipation Anterior meningocela 


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Copyright information

© © Versita Warsaw and Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • Ivona Djordjevic
    • 1
  • Danica Jovanovic
    • 2
  • Blagoje Grujic
    • 2
  1. 1.Clinic for Pediatric Surgery and OrthopedicsNisSerbia and Montenegro
  2. 2.Institute for mother and child “Vukan Cupic”New BelgradeSerbia and Montenegro

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