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.

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Non-syndromal children with anorectal malformations are at risk for growth impairment, especially those with additional major comorbidity. Mental development is normal. Motor development is slightly impaired. Supportive care should focus on growth, dietary management, and motor development besides defecation problems.

OBJECTIVE:To determine the prevalence of hearing loss in school-age children who have undergone neonatal extracorporeal membrane oxygenation (ECMO) treatment and to identify any effects of hearing loss on speech-and language development. DESIGN:Prospective longitudinal follow-up study within the framework of a structured post-ECMO follow-up program.SETTING: Outpatient clinic of a level III university hospital. RESULTS:Tone audiometry was performed by standardized protocol in 136 children aged 5 to 12 years. Hearing loss was considered clinically significant when >20dB. Hearing was normal in 75.7% of children. Five children (3.7%) had bilateral sensorineural or combined hearing loss; 3 of them received special audiological care (2.2% of total sample). Of the 24 children with congenital diaphragmatic hernia, 19 (79.2%) had normal hearing; and only 2 (8.3%) had mild SNHL, unilateral in one of them.Follow-up at 24 months of age had shown normal verbal and non-verbal developmental scores.Language development and intelligence median (range) scores at 5 years of age were also normal: receptive language development 104 (55-133); syntactical development 104 (68-132); and lexical development 101 (50-141) for 89 children; intelligence quotient was 104 (68-132) in 106 children.Scores did not differ between those with normal hearing, and those with mild hearing loss, or those with moderate to severe hearing loss (p=0. 800, p=0.639, p=0.876, and p=0.886, for the respective developmental tests). CONCLUSIONS:We found normal language development and intelligence in a cohort of neonatal ECMO survivors. The prevalence of bilateral sensorineural hearing loss was in accordance with that of larger series in the United States -which exceeds the prevalence in the normal population.3