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Prune belly syndrome
1Department of Surgery, Harvard Medical School and Children's Hospital of Boston, Massachusetts 02115, USA.
Insights
Prune Belly Syndrome, also known as Eagle-Barrett syndrome, is a rare condition characterized by a deficient abdominal wall, urinary tract dilation, and undescended testes. It often involves other system malformations, affecting many patients.
Area of Science:
- Pediatric Medicine
- Medical Genetics
- Developmental Biology
Background:
- Prune Belly Syndrome (PBS) presents as a lax, wrinkled abdominal wall.
- It is frequently associated with other congenital anomalies.
- Also known as Eagle-Barrett syndrome or triad syndrome.
Purpose of the Study:
- To define the characteristic features of Prune Belly Syndrome.
- To highlight associated anomalies in affected individuals.
- To provide a comprehensive overview of this rare condition.
Main Methods:
- Review of existing literature on Prune Belly Syndrome.
- Analysis of case studies and clinical observations.
- Synthesis of diagnostic criteria and associated conditions.
Main Results:
- Prune Belly Syndrome involves a triad of abdominal wall deficiency, urinary tract dilation, and bilateral cryptorchidism.
- Approximately 75% of patients exhibit additional malformations.
- Affected systems include cardiopulmonary, gastrointestinal, and orthopedic.
Conclusions:
- Prune Belly Syndrome is a complex congenital disorder with multisystemic implications.
- Early recognition and management are crucial for affected infants.
- Further research is needed to understand the underlying pathogenesis.
Abstract:
Prune belly is a lax, wrinkled abdominal wall and frequently is associated with other anomalies. The prune belly syndrome is a specific constellation of anomalies consisting of an abdominal wall deficient in muscular tissue, dilated urinary tract, and bilateral cryptorchidism. This group of anomalies is also called the Eagle-Barrett syndrome and the triad syndrome. In about 3 of 4 patients with the prune belly syndrome, there are associated malformations of the cardiopulmonary, gastrointestinal, and orthopedic systems.