一例患有Prune-Belly综合征的8岁儿童外科治疗面临的挑战

Rodrigo Alexandre Trivilato, Gabriela Dadalt, Deborah Lima Assenço, Débora Fernandes Barbalho, Márcio Rodrigues Costa, Nadin Chater, Rodrigo Rosa Lima, Bernardo Monteiro Antunes Barreira, João Paulo de Bessa Teixeira, Fernando Cruvinel, José Luiz Figueiredo
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引用次数: 0

摘要

Prune Belly综合征,也称为Eagle-Barret综合征,是一种罕见的疾病,每100000名新生儿中有3.8名活产婴儿患病。其主要特征是腹部肌肉发育不全,因此得名“梅干-腹部综合征”。黄金标准的治疗方法是手术,最好在6到18个月的生命中矫正隐睾和包茎。泌尿系统畸形的矫正和腹部整形术应在4岁之前进行。关于晚期治疗及其对预后的影响,文献中几乎没有证据。上述背景使我们提出了一个罕见的案例,即一名六岁儿童的手术时间比平时晚。
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Challenges in the surgical treatment of a 8-year old child living with Prune Belly syndrome: case report
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.
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来源期刊
Journal of Human Growth and Development
Journal of Human Growth and Development Social Sciences-Life-span and Life-course Studies
CiteScore
2.70
自引率
0.00%
发文量
37
审稿时长
22 weeks
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