title: Urological outcome in patients with Currarino syndrome.
abstract: BACKGROUND
  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.
  
  
  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.
  
  
  CONCLUSIONS
  Currarino syndrome is a rare congenital disorder presenting a variable phenotype. Urological outcome is good in the majority of patients.
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy; DINOGMI, University of Genoa, Genova, Italy. Electronic address: lucakeats@hotmail.it.
authors.forename: Luca
authors.initials: L
authors.lastname: Pio
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Gianluca
authors.initials: G
authors.lastname: Piatelli
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Rossi
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Scarsi
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Elisa
authors.initials: E
authors.lastname: Merello
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Valeria
authors.initials: V
authors.lastname: Capra
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Armando
authors.initials: A
authors.lastname: Cama
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Piero
authors.initials: P
authors.lastname: Buffa
authors.affiliation: Istituto Giannina Gaslini, Genova, Italy.
authors.forename: Michele
authors.initials: M
authors.lastname: Torre
mesh_terms: D001003:Anal Canal / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D004065:Digestive System Abnormalities / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012007:Rectum / Q000002:abnormalities*; D012189:Retrospective Studies; D012447:Sacrum / Q000002:abnormalities*; D013595:Syringomyelia / Q000150:complications*; D001750:Urinary Bladder, Neurogenic / Q000209:etiology* / Q000503:physiopathology; D014563:Urodynamics
keywords: Currarino syndrome; Neuropathic bladder; Urological outcome