MRI and radiographic findings in Currarino's triad

Abstract

Currarino's triad is a rare complex of a congenital sacral bony abnormality, anorectal malformation and a presacral mass. Intractable constipation since birth is the leading symptom of this triad, which follows an autosomal dominant mode of heredity. We report conventional radiographic and MR findings in one family consisting of a mother and her two daughters. In all three cases, radiography revealed an abnormality of the os sacrum, the so-called scimitar sacrum. MR examination, undertaken next in our institution, was applied with T1-, T2- and proton density weighted sequences in all three orientations before and after i. v. application of gadolinium diethylene-triamine-pentaacetic acid (Gd-DTPA). In two patients we were able to diagnose the complete form of the triad and in one patient an incomplete form. In any case of a radiographically diagnosed scimitar sacrum in combination with constipation, Currarino's triad should be considered. MRI, as the method of choice, should be the next step to detect a presacral mass and any anomalies of the spinal canal. The importance of early recognition lies in the high morbidity and mortality rates resulting from this disorder.

The spectrum of imaging in Currarino triad

“…Presently, MRI answers many questions of complex anorectal pathology including enteric fistulae, in which previously MRI was considered to be unreliable (as in two of our cases) as well as for genital abnormalities. It is therefore very valuable especially for surgical planning [14,17,18]; therefore, presently radiation-exposing modalities, such as plain films, enema, CT, myelo-CT and genitography, should only be used if MRI is not available. With regard to one of our patients with complex cloacal and urinary tract malformations, it seems questionable if a comparable exact demonstration of all the abnormalities, including ectopic refluxive ureters, found by genitography, could have been obtained by MRI with an even more optimal and adequate technique.…”

Currarinos syndrom ved Rikshospitalet 1961 – 2012

“…With backing in the literature, we now recommend that asymptomatic mutation carriers should be examined with MRI (3,14,25,34), and that presacral tumours (except for small meningoceles) should be removed (3,25,34). Figure 3b and Tom Monclair (born 1942) MD PhD and specialist in paediatric and general surgery.…”

MR imaging of anorectal malformations and associated anomalies

“…Magnetic resonance imaging, as the method of choice, is able to accurately detect all the different aspects of this triad, and, therefore, should be performed in the preoperative phase, to optimize therapy planning (Fig. 18) [56,57].…”