Health-related Quality of Life in Children With Prune-belly Syndrome and Their Caregivers.

OBJECTIVE: To compare health-related quality of life (HRQoL) in children with prune-belly syndrome (PBS) and their caregivers to healthy controls, as children and adolescents with PBS face numerous potential physical and psychosocial challenges. MATERIALS AND METHODS: Study participants completed the Pediatric Quality of Life Inventory Generic Core Scales (PedsQL) 4.0 generic core scales (children) or Quality of Life Enjoyment and Satisfaction Questionnaire Short Form (Q-LES-Q-SF) (caregivers) in an online, anonymous format. The PedsQL 4.0 is a 23-item, age-adjusted, validated questionnaire that assesses physical, emotional, social, and school functioning in pediatric patients. The Q-LES-Q-SF is a validated, self-report measure that assesses various areas of daily functioning in adults. RESULTS: PedsQL 4.0 was completed by 32 children with PBS. Individual physical (66.3 ± 20 vs 84.4 ± 17.3; P < .0001), emotional (68.4 ± 23.4 vs 80.9 ± 19.6; P < .01), social (63.1 ± 21.3 vs 87.4 ± 17.2; P < .0001), and school (53 ± 21.7 vs 78.6 ± 20.5; P < .0001) functioning scales were all significantly lower than in healthy children. Nineteen caregivers completed the Q-LES-Q-SF. Caregivers had a mean raw score of 54.8 ± 9.6, which was significantly lower (P = .02) than the comparative healthy adult cohort (59.8 ± 11.3). CONCLUSION: PBS profoundly impacts HRQoL in children, negatively affecting physical, emotional, social, and school functioning. Caregivers of PBS patients also report an overall lower quality of life, highlighting the challenges that families with chronically ill children often face.

Phantom urinary incontinence in children with bladder-bowel dysfunction.

OBJECTIVE: To report a previously undescribed condition in which children present with the sensation of wetness because of presumed urinary incontinence when they are actually completely dry. We have termed this entity "phantom" urinary incontinence (PUI). MATERIALS AND METHODS: Twenty children referred to our pediatric urology clinic were diagnosed with PUI between 2009 and 2013. Patient demographics, associated bladder and bowel symptoms, concomitant diagnoses, imaging, management, and treatment outcomes were evaluated. RESULTS: Twenty children (18 females and 2 males) were diagnosed with PUI over a 5-year interval. Mean age at diagnosis was 6.9 ± 2.5 years (range, 4-12 years). Nineteen patients (95%) had concomitant lower urinary tract symptoms, and all were also diagnosed with constipation. Urgency (75%) and frequency (50%) were the most common associated bladder symptoms. Of the 18 girls, 13 (72%) had associated vaginitis. Fourteen children (70%) carried a parent-reported diagnosis of obsessive-compulsive disorder or obsessive-compulsive disorder personality traits. Patients were managed with timed voiding, dietary modifications, and a bowel regimen. Ninety percent children experienced improvement of bladder-bowel dysfunction and resolution of PUI at a mean follow-up of 14.4 months. CONCLUSION: Children with PUI have a high incidence of obsessive-compulsive traits. Phantom incontinence as well as associated lower urinary tract symptoms resolve with adherence to a strict bladder-bowel regimen.