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Prune-belly syndrome: an autopsy case report
Marcela Arruda Pereira Silva Vasconcelos1, Patricia Picciarelli de Lima1
1Department of Pathology - Hospital das Clínicas - Faculdade de Medicina - Universidade de São Paulo, São Paulo/SP - Brazil.
Autopsy & Case Reports
|June 3, 2017
Summary
Prune-belly syndrome (PBS), a rare congenital condition, was diagnosed prenatally. Despite intrauterine intervention and postnatal surgery, the infant with prune-belly syndrome unfortunately died from aspiration pneumonia.
Area of Science:
- Pediatrics
- Medical Genetics
- Fetal Medicine
Background:
- Prune-belly syndrome (PBS) is a rare congenital anomaly affecting the urinary tract, abdominal muscles, and testes in males or genitalia in females.
- Antenatal diagnosis is possible via ultrasound, with treatment historically based on case reports.
- Intrauterine management for PBS is a recent development showing promising outcomes.
