title: Psychosexual Well-Being after Childhood Surgery for Anorectal Malformation or Hirschsprung's Disease.
abstract: BACKGROUND
  Anorectal malformations (ARMs) and Hirschsprung's disease (HD) are congenital malformations requiring pelvic floor surgery in early childhood, with possible sequelae for psychosexual development.
  
  
  OBJECTIVE
  To assess psychosexual well-being in adult ARM and HD patients related to health-related quality of life.
  
  
  METHODS
  Eligible for this cross-sectional two-center study were all patients aged ≥18 years who had been operated for ARM or HD. Exclusion criteria were intellectual disability, comorbidity affecting sexual functioning, and cloacal malformation.
  
  
  METHODS
  Participants completed the International Index of Erectile Functioning, Female Sexual Functioning Index, Female Sexual Distress Scale, Hirschsprung and Anorectal Malformation Quality of Life Questionnaire, and sexual education questionnaire.
  
  
  RESULTS
  Response rates were 32% and 37% for ARM and HD patients, respectively. We studied 70 participating ARM and 36 HD patients (median age 26 years). We excluded 10 patients with sexual inactivity in the past 4 weeks. Six of 37 men with ARM (16%) reported moderate to severe erectile dysfunction, vs. two of 18 men with HD (11%). Thirteen and 10 of 26 women with ARM (50% and 38%) reported sexual dysfunction or sexual distress, respectively, vs. eight and three of 15 women with HD (53% and 20%). Quality of life and type of malformation or operation were not associated with self-reported psychosexual problems. Addressing sexuality with special interest to the congenital anomaly during medical care was reported to be insufficient by 42 ARM (60%) and 22 HD patients (61%).
  
  
  CONCLUSIONS
  Approximately 13% of male ARM and HD patients reported erectile dysfunction, while 50% female ARM and HD patients reported sexual dysfunction not related to quality of life or type of malformation. Both ARM and HD patients felt a need for better addressing sexual concerns during medical care. Further research is needed to optimize form and timing of this education.
authors: Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Desiree
authors: D
authors: van den Hondel
authors: Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Cornelius E J
authors: CE
authors: Sloots
authors: Department of Reproduction and Development, Erasmus MC, Rotterdam, The Netherlands.
authors: Johanna M
authors: JM
authors: Bolt
authors: Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Rene M H
authors: RM
authors: Wijnen
authors: Department of Pediatric Surgery, Amalia Children's Hospital, Radboudumc, Nijmegen, The Netherlands.
authors: Ivo
authors: I
authors: de Blaauw
authors: Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: Hanneke
authors: H
authors: IJsselstijn
mesh_terms: D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D004414:Dyspareunia / Q000209:etiology* / Q000523:psychology; D007172:Erectile Dysfunction / Q000209:etiology* / Q000523:psychology; D006627:Hirschsprung Disease / Q000601:surgery*; D006801:Humans; D008297:Male; D011788:Quality of Life; D012725:Sexual Behavior; D018434:Sickness Impact Profile; D011795:Surveys and Questionnaires
keywords: Anorectal Malformation; Hirschsprung's Disease; Psychosexual Functioning; Sexuality