Currarino Triad

Currarino triad is a rare constellation of anorectal malformation, sacrococcygeal defect, and a presacral mass. It is also known as ASP syndrome (Anorectal, Sacrococcygeal, Presacral), is inherited in an autosomal dominant manner in more than 50% of cases, and is diagnosed by age 16 in more than 80% of cases. Incomplete forms also exist.

• Anorectal malformations: Anal stenosis (with or without fistulas to the spinal canal) or anal atresia.
• Sacrococcygeal bony defect: Range from a small lateral deviation of the coccyx to asymmetric hypoplasia of multiple sacral segments ("scimitar sacrum").
• Presacral mass: Mature teratomas, dermoid or neurenteric cysts, lipomas, among others, often in combination with an anterior meningocele.

In addition to the above, other malformations may be seen in patients with Currarino triad, including tethered cord (and its neurological sequelae), intraspinal lipomas, rectovaginal fistulas, and partial duplication of the vagina, uterus or urinary tract.

While patients with caudal regression syndrome can also have anorectal, spinal and urogenital findings, a presacral mass is only found in patients with the Currarino triad.

References

Riebel T, Mäurer J, Teichgräber UK, Bassir C. The spectrum of imaging in Currarino triad. Eur Radiol. 1999;9(7):1348-53.