title: Spinal cord anomalies in patients with anorectal malformations without severe sacral abnormalities or meningomyelocele: outcomes after expectant, conservative management.
abstract: OBJECTIVE The goal of this study was to determine the significance of spinal cord anomalies (SCAs) in patients with anorectal malformations (ARMs) by comparing the outcomes for bowel function, lower urinary tract symptoms (LUTS), and lower-limb neurological abnormalities to these outcomes in patients with similar ARMs and a normal spinal cord. METHODS The spinal cord MRI records of female patients treated for vestibular and perineal fistula (VF/PF) and male patients with rectourethral fistula (RUF) at a single center between 1983 and 2006 were reviewed. Bowel function and LUTS were assessed by questionnaire. Patients with extensive sacral anomalies or meningomyelocele were excluded. RESULTS Of 89 patients (median age 15 years, range 5-29 years), MRI was available in 90% (n = 80; 40 male patients with RUF), and 80% of patients returned the questionnaire (n = 64; 31 male patients with RUF). Spinal cord anomalies were found in 34%, comprising a filum terminale lipoma in 30%, low conus medullaris in 10%, and thoracolumbar syrinx in 6%. Bowel functional outcomes between patients with SCAs (n = 23) and those with a normal spinal cord (n = 41) were not significantly different for soiling (70% vs 63%), fecal accidents (43% vs 34%), and constipation (57% vs 39%; p = not significant for all). The LUTS, including urge (65% vs 54%), urge incontinence (39% vs 24%), stress incontinence (17% vs 22%), and straining (32% vs 29%) were also comparable between groups (p = not significant for all). No patients developed lower-limb neurological abnormalities. CONCLUSIONS The results suggest that the long-term functional outcomes for patients with SCAs who had VF/PF and RUF may not differ significantly from patients with the same type of ARMs and a normal spinal cord. The results favor a conservative approach to their management in the absence of abnormal neurological findings in the lower limbs.
authors.affiliation: Department of Pediatric Surgery, University of Helsinki, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Kristiina
authors.initials: K
authors.lastname: Kyrklund
authors.affiliation: Department of Pediatric Surgery, University of Helsinki, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Mikko P
authors.initials: MP
authors.lastname: Pakarinen
authors.affiliation: Department of Pediatric Surgery, University of Helsinki, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Seppo
authors.initials: S
authors.lastname: Taskinen
authors.affiliation: Department of Pediatric Surgery, University of Helsinki, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Reetta
authors.initials: R
authors.lastname: Kivisaari
authors.affiliation: Department of Pediatric Surgery, University of Helsinki, Hospital for Children and Adolescents, Helsinki, Finland.
authors.forename: Risto J
authors.initials: RJ
authors.lastname: Rintala
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000071056:Anorectal Malformations / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D019468:Disease Management; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D015995:Prevalence; D012003:Rectal Fistula / Q000150:complications / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D013116:Spinal Cord / Q000002:abnormalities* / Q000000981:diagnostic imaging; D016896:Treatment Outcome; D055815:Young Adult
keywords: ACE = antegrade continence enema; ARM = anorectal malformation; ASARP, PSARP = anterior, posterior sagittal anorectoplasty; BFS = Bowel Function Score; CM = conus medullaris; LUTS = lower urinary tract symptoms; NS = not significant; RUF = rectourethral fistula; SCA = spinal cord anomaly; TCS = tethered cord syndrome; UTI = urinary tract infection; VF/PF = vestibular and perineal fistula; VUR = vesicoureteric reflux; anorectal malformations; congenital; spinal cord; tethering