title: Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations.
abstract: OBJECTIVE
  Cloacal malformations are the most complex type of anorectal malformation in females. This study aimed to report quality of life (QoL) of patients with a cloacal malformation for the first time in literature.
  
  
  METHODS
  Female patients with an anorectal malformation participating in a follow-up program for congenital malformation survivors aged 5 or older were eligible for this study. QoL was assessed with the PedsQL™ 4.0 inventory. Scores of patients with a cloacal malformation (CM) were compared with those of female patients with rectoperineal or rectovestibular fistulas (RP/RV) and with reference data.
  
  
  RESULTS
  A total of 59 patients (67% response rate; 13 patients with cloacal malformation) were included, QoL was assessed by patient self-report at median age of 12years (8-13), and by parent proxy-report at median age of 8years (5-12). There were no differences between groups regarding the presence of associated anomalies, with also no differences regarding anomalies in the urinary tract (CM vs. RP/RV=31% vs. 15%, p=0.237). Scores of the cloacal malformations group were similar to those of the comparison group, except the proxy-reported scores on school functioning (60.0 vs. 80.0, p=0.003). Proxy-reported scores of cloacal malformation patients were significantly lower than reference values on total QoL-score, psychosocial health, and emotional and school performance. Patients (irrespective of type of ARM) who suffered from fecal soiling reported significantly lower scores with regard to psychosocial health (71.7 vs. 81.7, p=0.034) and its subscale school performance (65.0 vs. 80.0, p<0.001). QoL-scores reported by cloacal malformation patients did not differ significantly from the reference values of the healthy population. Parents of cloacal malformation patients reported significantly lower total QoL, emotional and school performances, as well as a lower general psychosocial health for their children relative to reference data of healthy children.
  
  
  CONCLUSIONS
  Patients with cloacal malformations and females with less complex anorectal malformations report similar QoL. Parents of cloacal malformation patients report more problems on several psychosocial domains relative to the healthy reference group. To monitor these matters, long-term follow-up protocols should contain multidisciplinary treatment including periodical assessment of psychosocial well-being.
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands. Electronic address: h.versteegh@erasmusmc.nl.
authors.forename: H P
authors.initials: HP
authors.lastname: Versteegh
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: D
authors.initials: D
authors.lastname: van den Hondel
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: H
authors.initials: H
authors.lastname: IJsselstijn
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: R M H
authors.initials: RM
authors.lastname: Wijnen
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands.
authors.forename: C E J
authors.initials: CE
authors.lastname: Sloots
authors.affiliation: Department of Pediatric Surgery, Sophia Children's Hospital-Erasmus Medical Center, Rotterdam, the Netherlands; Department of Pediatric Surgery, Amalia Children's Hospital-Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: I
authors.initials: I
authors.lastname: de Blaauw
mesh_terms: D000293:Adolescent; D001003:Anal Canal / Q000002:abnormalities*; D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000523:psychology*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D002988:Cloaca / Q000002:abnormalities*; D005260:Female; D005500:Follow-Up Studies; D006305:Health Status Indicators; D006306:Health Surveys; D006801:Humans; D010290:Parents; D011446:Prospective Studies; D011788:Quality of Life; D012006:Rectovaginal Fistula / Q000151:congenital / Q000523:psychology; D012007:Rectum / Q000002:abnormalities*; D057566:Self Report
keywords: Anorectal malformations; Cloacal malformations; Quality of life