RESUMEN
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.
Asunto(s)
Cuidadores/psicología , Estado de Salud , Síndrome del Abdomen en Ciruela Pasa/psicología , Calidad de Vida , Adolescente , Adulto , Niño , Preescolar , Emociones , Femenino , Estudios de Seguimiento , Humanos , Masculino , Estudios Retrospectivos , Encuestas y CuestionariosRESUMEN
Prune-belly syndrome is a congenital disorder characterized by abdominal wall musculature deficiency, urinary tract anomalies, and bilateral cryptorchidism. Because of the defect in the musculature, the abdominal skin has a peculiar wrinkled appearance. The syndrome is commonly associated with pulmonary, skeletal, cardiac, and gastrointestinal defects. Developmental delays and growth retardation have also been reported. The incidence of prune belly syndrome is approximately 1:40,000 live births. Over 95% of patients are men. Urinary tract disease is the major prognostic factor, with the complications of pulmonary hypoplasia and end stage renal disease resulting in a mortality rate of 60%. Treatment involves surgical correction of the abdominal wall defect and urinary tract abnormalities, early orchiopexy, and supportive management of associated defects.
Asunto(s)
Síndrome del Abdomen en Ciruela Pasa/diagnóstico , Anomalías Cutáneas/diagnóstico , Pared Abdominal/cirugía , Adolescente , Femenino , Humanos , Lactante , Discapacidad Intelectual/etiología , Masculino , Síndrome del Abdomen en Ciruela Pasa/epidemiología , Síndrome del Abdomen en Ciruela Pasa/psicología , Síndrome del Abdomen en Ciruela Pasa/cirugía , Resultado del TratamientoRESUMEN
Over the past 10 years, we have seen sixteen children (15 males and 1 female) with the prune-belly syndrome. We have managed 12 of these children, but 4 of them were never reviewed. Abdominal wall reconstruction was performed in 11 children by dissection of spermatic vessels and fixing the testis in the scrotum. Because of our conservative approach to the management of urinary tract dilatation, we performed only one nephrostomy and 3 unilateral nephro-ureterectomies. Seven children were reviewed. The result of abdominal wall reconstruction was good in terms of cosmetic appearance and psychologic effect for the parents; we have observed no atrophic testes after orchidopexy. One of these children suffers from chronic renal failure, while the other children have good renal function. Prune-belly syndrome is a disease with a broad spectrum. The prognosis depends on the degree of renal dysplasia. These children require long-term follow-up.
Asunto(s)
Síndrome del Abdomen en Ciruela Pasa/cirugía , Músculos Abdominales/cirugía , Niño , Preescolar , Côte d'Ivoire , Dilatación Patológica/cirugía , Disección , Estética , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Enfermedades Renales/cirugía , Fallo Renal Crónico/etiología , Masculino , Nefrectomía , Complicaciones Posoperatorias , Pronóstico , Síndrome del Abdomen en Ciruela Pasa/psicología , Procedimientos de Cirugía Plástica , Estudios Retrospectivos , Escroto/cirugía , Cordón Espermático/cirugía , Testículo/cirugía , Uréter/cirugía , Enfermedades Ureterales/cirugía , Derivación UrinariaRESUMEN
A total of 6 boys with the prune belly syndrome underwent total abdominal wall reconstruction by a technique that permits simultaneous bilateral orchiopexy and/or urinary tract reconstruction. Until now, the psychosocial implications of the abdominal wall disfigurement caused by this syndrome have been ignored. This procedure provides an excellent cosmetic result that, in turn, promotes psychological health and a positive body image in these children.