The Currarino triad is a hereditary transmitted syndrome, originally defined by 
Currarino as ASP-association, consisting of an anorectal malformation, a sacral 
bony defect and a presacral mass. In most cases autosomal dominant transmission 
is suggested. In family members one or two features of the syndrome may be 
missing, indicating an incomplete form of this complex. We describe two 
unrelated girls at the age of 8 and 9 months respectively with ASP-association. 
Family screening in both patients showed 8 additional cases with a complete or 
incomplete Currarino triad, four of them being asymptomatic. A review of the 
literature up to 1991 revealed 48 patients with ASP-association. In more than 
80% of cases, this complex is diagnosed in the first decade, whereas incomplete 
Currarino syndrome is diagnosed predominantly in adults. Most frequently the 
presacral mass in ASP-association was reported to be an anterior meningocele 
(47%) and a benign teratoma (40%). The number of patients with Currarino 
syndrome has been underestimated so far. We recommend anorectal examination, 
pelvic ultrasound and pelvic x-rays in all patients with a history of chronic 
constipation since early childhood. Positive findings should lead to further 
investigations such as barium enema, MRI, myelography and family screening. 
Close cooperation between pediatric surgeons and neurosurgeons is required to 
ensure adequate surgical treatment, considering both the risk of malignant 
degeneration as well as the risk of intraoperative nerve damage. Thus, radical 
excisional surgery is not obligatory in every case of Currarino syndrome.