INTRODUCTION AND OBJECTIVES: Prader-Willi Syndrome (PWS) is characterised by mental retardation, short stature, obesity and hypogonadism. The behavioral phenotype includes eating problems, temper outbursts, affective disorders, stereotypies and speech abnormalities. Urinary incontinence (UI) is common in children with mental retardation in general, but has not been reported systematically in children with PWS so far. The aim of the study therefore was to assess and identify the frequency and type of UI, as well as associated symptoms in patients with PWS.

METHODS: 118 parents of children with PWS completed the Dutch version of the “Parental Questionnaire: Enuresis/Urinary Incontinence”. This questionnaire includes items referring to day- and nighttime wetting, toilet habits, observable voiding behaviors and reactions, urinary tract infections, stool habits and behavioral symptoms.

RESULTS: The rate of nocturnal enuresis in persons with PWS was 13.6% at a mean age of 15.1 years. 3.8% had additional daytime urinary incontinence, and 3.3% had fecal incontinence. Lower urinary tract symptoms were common indicative of overactive bladder, dysfunctional voiding and postponement. Also, the rate of internalizing and externalizing behavioral problems was high.

CONCLUSIONS: Urinary incontinence is more common in persons PWS than in typically developing children, adolescents and adults. As lower urinary tract symptoms are common, detailed assessment and specific treatment of UI should be part of the care of all persons with PWS.

Source of Funding: None