title: Anorectal malformations with good prognosis: variables affecting the functional outcome.
abstract: OBJECTIVE
  The purpose of this study was to investigate the outcome of patients operated for anorectal malformations (ARMs) with good prognosis.
  
  
  METHODS
  Thirty patients underwent clinical evaluation by Rintala score and anorectal manometry recording anal resting pressure (ARP), rectoanal inhibitory reflex (RAIR), and rectal volume (RV). The results were analysed with regard to sex, type of ARM, surgical timing of posterior sagittal anorectoplasty (PSARP), neurospinal cord dysraphism (ND), neonatal colostomy, and institution where they underwent surgery.
  
  
  RESULTS
  6/30 (20%) presented ND despite normal sacrum. 17/30 (57%) patients had a normal Rintala score. ND and neonatal colostomy were significantly associated with a pathologic score (p=0.0029 and p=0.0016). Patients with ND had significantly lower ARP compared to patients with normal spine (23.5±7.2mmHg vs 32±7.9mmHg, p=0.023). ARP was significantly lower in patients with neonatal colostomy compared to patients with primary repair (25.22±10.24mmHg vs 32.57±6.68mmHg, p=0.026). RAIR was present in only 2/6 (33%) patients with ND, while in 21/24 (87.5%) without ND (p=0.015) and in 4/9 (44%) patients with neonatal colostomy, while in 19/21 (90.5%) patients submitted to primary repair (p=0.014).
  
  
  CONCLUSIONS
  Neurospinal cord dysraphism may be present despite normal sacral ratio. From a clinical point of view, patients with good prognosis ARMs are not completely comparable to healthy children. Neurospinal cord dysraphism and neonatal colostomy seem to worsen the clinical and manometric (ARP and RAIR) outcomes of these patients.
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy. Electronic address: rosella.arnoldi@gmail.com.
authors.forename: Rossella
authors.initials: R
authors.lastname: Arnoldi
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Macchini
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Valerio
authors.initials: V
authors.lastname: Gentilino
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Giorgio
authors.initials: G
authors.lastname: Farris
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Morandi
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Brisighelli
authors.affiliation: Department of Pediatric Surgery, Fondazione IRCCS Ca' Granda-Ospedale Maggiore Policlinico, Milano, Italy.
authors.forename: Ernesto
authors.initials: E
authors.lastname: Leva
mesh_terms: D001003:Anal Canal / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery*; D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000503:physiopathology / Q000601:surgery*; D003672:Defecation; D013505:Digestive System Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008365:Manometry; D011312:Pressure; D011379:Prognosis; D019651:Plastic Surgery Procedures / Q000379:methods*; D012007:Rectum / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery*; D012189:Retrospective Studies
keywords: Anorectal malformation; Anorectal manometry; Functional outcome