title: Impact of spinal dysraphism on urinary and faecal prognosis in 25 cases of cloacal malformation.
abstract: OBJECTIVE
  Urinary and faecal continence are key challenges goal of cloacal malformation management. Most well-known prognostic factors are the length of common channel (CC) and the presence of a sacral defect, but the impact of associated spinal dysraphism is less well documented. The aim of this study was to investigate the impact of different types of dysraphism on urinary and faecal continence in this patient population.
  
  
  METHODS
  From 1991 to 2011, charts and office notes of 25 patients with cloacal malformation were retrospectively reviewed. At last clinic visit, urinary and faecal continence status according to Krickenbeck criteria were correlated with the length of CC, the presence of a sacral defect (sacral ratio), and the presence of different types of spinal cord dysraphism using magnetic resonance imaging (MRI) and Fisher's exact test.
  
  
  RESULTS
  Mean follow-up was 8 years (4 months-21 years). The sacral ratio was abnormal (below 0.74) in 18 cases out of 25 (72%). MRI review showed normal spinal cord in eight out of 23 cases (Group 1), spinal cord anomaly in 15 out of 23 cases (65%) including nine cases of tethered cord complex (Group 2) and six cases of a short spinal cord (Group 3). While statistical analysis showed a difference regarding urinary prognosis between the groups (p=0.005), no significant difference was found regarding faecal prognosis. None of the six patients with short spinal cord were continent for both urinary and faecal prognosis.
  
  
  CONCLUSIONS
  This is the first study, which highlights the impact of different types of spinal dysraphism on functional outcome in patients with cloaca. Short spinal cord seemed to carry the worst prognosis. A prospective study with a larger series is mandatory to confirm these preliminary results.
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France. Electronic address: cessolivia@gmail.com.
authors.forename: Cécile O
authors.initials: CO
authors.lastname: Muller
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France; National Reference Centre for Rare Diseases on Anorectal Malformations and Rare Pelvic Anomalies (MAREP), Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Célia
authors.initials: C
authors.lastname: Crétolle
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Blanc
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France; National Reference Centre for Rare Diseases on Anorectal Malformations and Rare Pelvic Anomalies (MAREP), Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Ilona
authors.initials: I
authors.lastname: Alova
authors.affiliation: Department of Statistics, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Jais
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Stephen
authors.initials: S
authors.lastname: Lortat-Jacob
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Aigrain
authors.affiliation: Department of Paediatric Neurosurgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, INSERM U745, Paris, France.
authors.forename: Michel
authors.initials: M
authors.lastname: Zérah
authors.affiliation: Department of Paediatric Surgery, Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France; National Reference Centre for Rare Diseases on Anorectal Malformations and Rare Pelvic Anomalies (MAREP), Necker-Enfants Malades Hospital, APHP, Paris Descartes University, Paris, France.
authors.forename: Sabine
authors.initials: S
authors.lastname: Sarnacki
mesh_terms: D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000145:classification / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D005242:Fecal Incontinence / Q000145:classification / Q000453:epidemiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011379:Prognosis; D016135:Spinal Dysraphism / Q000453:epidemiology*; D014549:Urinary Incontinence / Q000453:epidemiology*
keywords: Caudal regression syndrome; Cloacal malformation; Dysraphism; Urinary and faecal prognosis