Currarino syndrome

Other names: Currarino triad; ASP (Anal atresia, sacral anomalies, presacral mass); Sacral defect with Anterior Meningocoele Note: Currarino syndrome is a multiple congenital anomalies syndrome characterized by partial agenesis of the sacrum in association with pelvic malformation. Anal atresia and the presence of a pre-sacral mass (teratoma and/or anterior meningocoele) make up the so called Currarino triad. Other malformations, such as renal (35%) and gynaecological (19%) are common. Hirschsprungs disease has also been recorded. Other rarer complications include pelvic abcess, malignant degeneration of a pre-sacral teratoma, E Coli ascending meningitis and spinal cord tethering. Recurrence of a benign teratoma has been recorded. Urinary incontinence, dysmenorrhoea, dyspareunia, poor sphincter control, sacral anaesthesia and headaches precipitated by coughing or straining have been reported. Inheritance: Autosomal dominant. De novo mutations have been recorded and account for approximately 15% of cases.