Urological outcome in patients with Currarino syndrome

Luca Pio, Gianluca Piatelli, Andrea Rossi, Pierluigi Scarsi, Elisa Merello, Valeria Capra, Armando Cama, Piero Buffa, Michele Torre

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)1643-1646
Number of pages4
JournalJournal of Pediatric Surgery
Volume49
Issue number11
DOIs
Publication statusPublished - Nov 1 2014

Fingerprint

Currarino triad
Neurogenic Urinary Bladder
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Neural Tube Defects
Urodynamics
Urinary Tract Infections
Urinary Bladder
Phenotype
Kidney
Incidence
Anorectal Malformations
Therapeutics
Vesicoureteral Reflux 2

Keywords

  • Currarino syndrome
  • Neuropathic bladder
  • Urological outcome

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Urological outcome in patients with Currarino syndrome. / Pio, Luca; Piatelli, Gianluca; Rossi, Andrea; Scarsi, Pierluigi; Merello, Elisa; Capra, Valeria; Cama, Armando; Buffa, Piero; Torre, Michele.

In: Journal of Pediatric Surgery, Vol. 49, No. 11, 01.11.2014, p. 1643-1646.

Research output: Contribution to journalArticle

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AU - Cama, Armando

AU - Buffa, Piero

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N2 - 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.

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