Abstract
BackgroundThe Currarino triad is a rare hereditary syndrome comprising anorectal malformation, sacral bony defect, and presacral mass. Most of the patients are diagnosed during infancy.Case presentationA 44-year-old man was diagnosed with Currarino triad, with a huge presacral teratoma and meningocele. One-stage surgery via posterior approach was successful.ConclusionsTreatment of the presacral mass in the Currarino triad, diagnosed in adulthood, is challenging. Multidisciplinary management and detailed planning before surgery are important for a satisfactory outcome.
Highlights
The Currarino triad is a rare hereditary syndrome comprising anorectal malformation, sacral bony defect, and presacral mass
This syndrome is an autosomal dominant condition resulting from mutations in the homeobox HLXB9 gene on chromosome 7
We present a rare case of the Currarino triad, which was diagnosed in mid-forties with constipation secondary to the huge presacral teratoma, with meningocele, and was successfully treated with one-stage surgery
Summary
The Currarino triad was first described by Currarino et al in 1981 [1] This syndrome is an autosomal dominant condition resulting from mutations in the homeobox HLXB9 gene on chromosome 7. It comprises an anorectal malformation, a sacral bony defect, and a presacral mass. An elastic hard presacral mass was palpable at 4 cm from the anal verge. CT scan showed a partial defect in the right side of the sacrum with deformity, a 60 × 30 × 21-mm meningocele which was protuberated anteriorly from the level of the S3 vertebra, and a 90 × 85 × 68-mm cystic mass which was located anterior to the meningocele, compressing the rectum ventrally (Fig. 1).
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