Predicting sexual problems in young adults with an anorectal malformation or Hirschsprung disease.

AIM: The aim of this study was to examine the prevalence of sexual dysfunction and distress and to assess whether sexual functioning could be predicted by psychosocial factors in childhood and adolescence in patients with an anorectal malformation or Hirschsprung disease. MATERIAL AND METHODS: In 1998 patients completed a psychosocial questionnaire: The Self-Perception profile. To assess the prevalence of sexual distress and sexual functioning in adulthood (2015) the Female Sexual Function Index (FSFI), The Female Sexual Distress Scale (FSDS) and the International Index of Erectile Functioning (IIEF) were used. RESULTS: In total 74 patients returned the questionnaires (26.2%). 36.8% of women reported sexual dysfunction and 45% experienced sexual distress. In our male sample 8.8% reported mild to moderate erectile dysfunction. In females perceived self-competence in adolescence was associated with sexual distress (p<.01). In male adolescents associations were found between perceived self-competence in romantic relations (p<.01) and in close friendships (p<.05) and sexual desire in adulthood. CONCLUSION: Pediatric surgeons should be more aware of sexual problems patients may face at older age as a result of their congenital disease and treatment. More standardized care and follow-up are needed. Prognosis study-Level II.

Cloacal malformation patients report similar quality of life as female patients with less complex anorectal malformations.

PURPOSE: 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. MATERIALS AND 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. CONCLUSION: 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.