INTRODUCTION: Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in Currarino syndrome. Our aim was to evaluate the urological outcome in patients with Currarino syndrome. PATIENTS AND METHODS: We retrospectively reviewed all Currarino syndrome patients treated in our institution. Of 20 patients, we could evaluate the urological outcome in 16. This group of patients underwent clinical, radiological and urodynamic evaluation. RESULTS: All 16 patients had a sacral defect, fourteen of them presenting a presacral mass (87.5%), eight a tethered cord (50%), and 7 anorectal malformations (43.7%). Eight patients underwent neurosurgical treatment for neural tube defects. In 14 patients, the presacral mass was resected. One case presented detrusor overactivity, 2 recurrent urinary tract infections and 2 vesicoureteral refluxes. Both patients with lipomyeloschisis had a neuropathic bladder. All the other patients could void the bladder spontaneously. Renal function was normal in all. CONCLUSION: Currarino syndrome is a rare congenital disorder presenting a variable phenotype. Urological outcome is good in the majority of patients.
INTRODUCTION:Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in Currarino syndrome. Our aim was to evaluate the urological outcome in patients with Currarino syndrome. PATIENTS AND METHODS: We retrospectively reviewed all Currarino syndromepatients treated in our institution. Of 20 patients, we could evaluate the urological outcome in 16. This group of patients underwent clinical, radiological and urodynamic evaluation. RESULTS: All 16 patients had a sacral defect, fourteen of them presenting a presacral mass (87.5%), eight a tethered cord (50%), and 7 anorectal malformations (43.7%). Eight patients underwent neurosurgical treatment for neural tube defects. In 14 patients, the presacral mass was resected. One case presented detrusor overactivity, 2 recurrent urinary tract infections and 2 vesicoureteral refluxes. Both patients with lipomyeloschisis had a neuropathic bladder. All the other patients could void the bladder spontaneously. Renal function was normal in all. CONCLUSION:Currarino syndrome is a rare congenital disorder presenting a variable phenotype. Urological outcome is good in the majority of patients.