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

Síndrome de Currarino: función del tracto urinario inferior, plan de tratamiento y resultados.

  • Hongliang Xia Children’s Hospital of Soochow University
  • Mingcui Fu Children’s Hospital of Soochow University
  • Hangzhou Wang Children’s Hospital of Soochow University
  • Xu Cao Children’s Hospital of Soochow University
  • Xiangming Yan Children’s Hospital of Soochow University
  • Yun Zhou Children’s Hospital of Soochow University
  • Jian Wang Children’s Hospital of Soochow University
Keywords: Currarino syndrome (CS), video-urodynamics (VUDS), neurogenic bladder, children, multidisciplinary

Abstract

Currarino syndrome (CS) is a rare congenital disorder char-acterized by anorectal malformation, sacral agenesis and presacral mass. We performed video-urodynamics (VUDS) assessment of patients with CS to char-acterize the lower urinary tract function, individualize management plans and to follow outcomes. We conducted a cross-sectional study on 11 patients diag-nosed with CS at the spina bifida multidisciplinary clinic. Lower urinary tract function was assessed by VUDS from three months to 12 months after neuro-surgery. All patients had sacral agenesis; nine patients had anorectal malforma-tion (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 uro-dynamic 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 dura-tion 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.

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Published
2021-06-10
How to Cite
Xia, H., Fu, M., Wang, H., Cao, X., Yan, X., Zhou, Y., & Wang, J. (2021). Currarino Syndrome: lower urinary tract function, treatment plan and outcomes.: Síndrome de Currarino: función del tracto urinario inferior, plan de tratamiento y resultados. Investigación Clínica, 62(2), 103-111. https://doi.org/10.22209/IC.v62n2a01