Psychosexual Well-Being After Childhood Surgery for Anorectal Malformation or Hirschsprung's Disease

Abstract

Introduction Anorectal malformations (ARMs) and Hirschsprung's disease (HD) are congenital malformations requiring pelvic floor surgery in early childhood, with possible sequelae for psychosexual development. Aims 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. Main Outcome Measures 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%). Conclusion 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.

Lower urinary tract symptoms and sexual functions after endorectal pull-through for Hirschsprung disease: controlled long-term outcomes

“…Of the few studies on postoperative sexual problems among HD patients, Moore et al [7] noted that 5% of patients reported sexual dysfunction including infertility and erectile dysfunction in males, especially after Duhamel and Swenson procedures. Van den Hondel et al [12] showed in their recent uncontrolled study that 11% of the men with HD had severe erectile dysfunction but no ejaculation difficulties, and that 53% of the women with HD had sexual dysfunction and 20% sexual distress, which, however, were not associated with the length of the aganglionosis or type of operation. Our study did not identify any defects in physical sexual functioning after EPT compared to matched peers, supporting the safety of EPT with regard to preservation of such functions.…”

“…Pelvic dissection close to the rectal wall is the aim of all surgical approaches to HD. Also EPT carries a potential risk to other structures in the vicinity [12,26], although appropriate pelvic dissection within the wall of the distal rectum in EPT should prevent injury to the genitourinary tract.…”

“…The few uncontrolled studies available after EPT lend support to this notion [10,11]. There is very limited information concerning the outcomes for sexual function in adulthood in patients with HD [7,12,13]. This study has aimed to evaluate the long-term outcomes for LUTS and sexual function after EPT for HD in relation to ageand gender-matched peers from the general population, in order to provide further information on the safety of these procedures regarding preservation of the functional integrity of the genitourinary tract.…”

Long‐term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands

“…Patients with HD can also experience urinary dysfunction (with enuresis in 25%, more nocturnal than in daytime) [43] and sexual dysfunction (11% of male and 53% of female patients) [44]. Growth, urinary and sexual dysfunction were not assessed in this study because of our focus on bowel function.…”

Congenital cloaca: Long-term follow-up results with emphasis on outcomes beyond childhood

“…Schmidt et al 21 found that 65% of the females with anorectal malformations had active sexual life. In the report of van den Hondel et al, 22 87% of females with anorectal malformation were sexually active. In both these studies, the patients were older (all older than 18 years) than in reports concerning cloaca patients; this may influence the rate of sexual activity.…”