[The Currarino triad. An autosomal-dominant inherited complex of anorectal malformation, sacrococcygeal defect and presacral tumor. Observation of 9 further cases].
Nine cases of a syndrome are described, which is known as the "Currarino triad" and which belongs to the group of malformations in which there is a persistent neurenteric communication. The features of the triad consist of ano-rectal anomalies (particularly ano-rectal stenoses), a curved, but limited, sacro-coccygeal defect ("scimitar sacrum") and a presacral tumour, which may be an anterior sacral meningocoele, a teratoma, a cyst (dermoid or neurenteric a cyst (dermoid or neurenteric cyst) or a mixture of these. In at least 50% of cases, the triad is familial and autosomal dominant. Of our nine cases, seven were familial. A dangerous complication is meningitis due to infection of the cystic component, or fistula formation between the colon and spinal canal (one of our cases). Other complications are a fixed filum terminale ("tethered cord") and malformations of the urogenital tract. The risk of malignancy in a teratoma is low, but exists. When considering the indications for surgery, this must be kept in mind, as must the risk of infection, and damage to neural structures during operation. In all cases of early obstipation the sacrum should be x-rayed in order to exclude a Currarino triad. The finding of a sacral defect and a presacral mass makes it essential to obtain CT of the pelvis with contrast in the distal gut and in the meningeal sac. The same is true for screening of the family (including views of the sacrum).
Holthusen
W
W
Birtel
T
T
Brinkmann
B
B
Gunkel
J
J
Janneck
C
C
Richter
E
E
D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000235:genetics*; D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000235:genetics*; D011859:Radiography; D012007:Rectum / Q000002:abnormalities*; D012447:Sacrum / Q000002:abnormalities*; D013577:Syndrome
Die Currarino-Triade. Ein autosomal-dominant erblicher Komplex von anorektaler Missbildung, Sakrokokzygealdefekt und präsakralem Tumor. Beobachtung von 9 weiteren Fällen.