Prune-belly syndrome: an autopsy case report

Prune-belly syndrome: an autopsy case report

Prune-belly syndrome (PBS) is a rare congenital anomaly characterized by a spectrum of mild-to-severe presentations of urinary tract malformations, deficient abdominal wall musculature, and cryptorchidism in male newborns or genital abnormalities in the female newborns. Currently, antenatal diagnosi...

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Journal Title: Autopsy and Case Reports
First author: Marcela Arruda Pereira Silva Vasconcelos
Other Authors: Patrícia Picciarelli de Lima
Palabras clave:
Prune Belly Syndrome; Cryptorchidism; Urologic Diseases
Language: English
Get full text: http://www.revistas.usp.br/autopsy/article/view/98464
Resource type: Journal Article
Source: Autopsy and Case Reports; Vol 4, No 4 (Year 2014).
DOI: http://dx.doi.org/10.4322/acr.%y.98464
Publisher: Universidade de São Paulo
Usage rights: Reconocimiento - NoComercial (by-nc)
Categories: Health Sciences --> Pathology
Abstract: Prune-belly syndrome (PBS) is a rare congenital anomaly characterized by a spectrum of mild-to-severe presentations of urinary tract malformations, deficient abdominal wall musculature, and cryptorchidism in male newborns or genital abnormalities in the female newborns. Currently, antenatal diagnosis is feasible with ultrasound examination, and treatment is based on case report experience. More recently, intrauterine management has been undertaken with encouraging results. The authors report a case of PBS diagnosed at the seventeenth gestation week, when ultrasonographic examination revealed the presence of ascites, distended bladder, thickened bladder wall and posterior urethral valve. The fetus was submitted to an intrauterine intervention at the nineteenth gestational week. Delivery occurred at 34 weeks of gestation and the newborn examination was consistent with PBS. On the second day of life, the newborn was submitted to abdominoplasty, colostomy, and orchiopexy. However, the outcome was unfavorable with respiratory failure and death on the fifteenth day of life. The autopsy confirmed the diagnosis of PBS, but the immediate cause of death was attributed to aspiration pneumonia.

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