Currarino Syndrome: lower urinary tract function, treatment plan and outcomes

Abstract

Currarino syndrome (CS) is a rare congenital disorder characterized by anorectal malformation, sacral agenesis and presacral mass. We performed video-urodynamics (VUDS) assessment of patients with CS to characterize the lower urinary tract function, individualize management plans and to follow outcomes. We conducted a cross-sectional study on 11 patients diagnosed with CS at the spina bifida multidisciplinary clinic. Lower urinary tract function was assessed by VUDS from three months to 12 months after neurosurgery. All patients had sacral agenesis; nine patients had anorectal malformation (9/11, 81.8%) and five patients had a presacral mass (5/11, 45.5%). The average age at neurosurgery was 7.9 months (range, 2–19). One patient had bilateral vesicoureteral reflux (VUR) with increasing detrusor pressure at the end of filling. In two patients, the detrusor activity showed weakening during urination, while no other lower urinary tract abnormality was identified on urodynamic evaluation. Six patients underwent VUDS before and after surgery; of these, two patients showed improved bladder function after surgery, while the remaining four patients showed no change in urodynamics. The average duration of follow-up was 27.5 months (range, 9–51). Renal function was normal in all patients. We can conclude that patients with CS often exhibit associated neurological abnormalities. VUDS assessment may help detect lower urinary tract dysfunction at an early stage and facilitate timely urological intervention to avoid kidney damage.