ABSTRACT
Prune Belly syndrome, also known as Eagle-Barret syndrome, is a rare disease, with a prevalence of 3.8 live births per 100,000 births. Its main characteristic is the hypoplasia of the abdominal muscles, giving rise to the name "prune belly syndrome". The gold standard treatment is surgery, ideally with correction of cryptorchidism and phimosis between 6 to 18 months of life. Correction of urinary malformations and abdominoplasty should be performed up to 4 years of age. Little evidence exists in the literature about late treatment and its implications for prognosis. The context mentioned above led us to present an uncommon case of a six years old child in which a surgical approach was performed later than usual.
Introdução: A síndrome de Prune Belly é uma doença rara, com prevalência de 3,8 nascidos vivos a cada 100 mil nascimentos. Tem como principal caraterística a hipoplasia da musculatura abdominal gerando a origem do nome "síndrome da barriga de ameixa". O tratamento padrão ouro é cirúrgico, idealmente com correção da criptorquidia e fimose entre 6 a 18 meses e a correção das malformações urinárias e abdominoplastia devem ser realizadas até os 4 anos. Aqui descreve-se um caso ainda menos comum, em que foi realizada abordagem cirúrgica de forma mais tardia que o habitual. Há pouca evidência na literatura acerca do tratamento tardio e suas implicações em relação ao prognóstico.
ABSTRACT
Prune-belly syndrome may be related to lower urinary tract obstruction (LUTO). LUTO in the early gestational age exacerbates fetal renal function and may require intrauterine intervention. If early developed LUTO causes bladder distension and abdominal musculature deficiency, it will result in prune belly syndrome. Therefore, early detection of the disease and proper treatment before the renal impairment is important. However, there are few literatures concerning the treatment of prune belly syndrome in the first trimester. We report a case of prune belly syndrome diagnosed at 11+6 weeks of gestation and the value of vesicocentesis as a modality of treatment. Ultrasound showed dilated fetal bladder and vesicocentesis was successful in reducing the volume of the bladder. However, the pregnancy was terminated upon request.
Subject(s)
Female , Humans , Male , Pregnancy , Cryptorchidism , Gestational Age , Pregnancy Trimester, First , Prune Belly Syndrome , Urinary Bladder , Urinary TractABSTRACT
Presentamos un caso de síndrome de Prune-Belly que se manifestó en un feto de sexo masculino a las 20 semanas de edad gestacional como megavejiga, megauretra, dilatación piélica bilateral y aumento de circunferencia abdominal. El complejo malformación-obstrucción uretral se refiere a la secuencia: • obstrucción uretral • megavejiga que, a su vez, produce: a) distensión abdominal con subsiguiente atrofia o hipoplasia de los músculos abdominales o abdomen en ciruela pasa; b) imposibilidad de descenso testicular o criptorquidia; c) dilatación ureteral por presión retrógrada o por reflujo desde la vejiga; d) displasia renal. La tríada de malformaciones urinarias, ausencia o hipoplasia de los músculos abdominales y criptorquidia bilateral se conoce con el nombre de síndrome de Prune-Belly o síndrome de Eagle-Barrett.
We present a case of Prune Belly Syndrome in a male fetus at 20 weeks of gestation with renal pelvis dilatation, massive bladder, mega urethral and an increase of the abdominal circumference. The complex urethral malformation-obstruction is about this sequence: urethral obstruction and massive bladder distention that causes: a) abdominal distention with subsequent atrophy or hypoplasia of abdominal muscles or Prune-Belly; b) impossibility of testicular reduction or cryptorchidism; c) megaureter by retrograde pressure or reflux from bladder; d) renal dysplasia. The triad of urinary malformations, absence or hypoplasia of abdominal muscles and cryptorchidism bilateral is knows as Prune-Belly syndrome or Eagle-Barrett syndrome.