Challenges in the surgical treatment of a 8-year old child living with Prune Belly syndrome: case report / Desafios no tratamento tardio da síndrome de Prune Belly: relato de caso

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.

Mucoepidermoid carcinoma of the penis: Case report and literature review.

We describe the fifth case of mucoepidermoid carcinoma. The patient had penile ulcer with bilateral inguinal and pelvic lymphadenopathy and underwent total penectomy. After antibiotic therapy, the patient began outpatient chemotherapy, but the treatment was discontinued due to his intolerance. The patient died due to infectious complications of the inguinal lymphatic fistula 7 months after the histological diagnosis. Notably, the periurethral area was involved in the anatomopathological evaluation of the excised penis. The penile mucoepidermoid carcinoma was aggressive and the perimeatal region was involved. This case helps demystify the origins and prognosis of this rare case. More reports documenting patient characteristics and their evolution with penile mucoepidermoid carcinoma are needed.