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Prune belly syndrome: Approaches to its diagnosis and management
Radhouane Achour1, Wafa Bennour2, Imen Ksibi2
1Emergency Department of Gynecology and Obstetrics, Maternity and Neonatology Center, Faculty of Medicine, Tunis-El Manar University, Tunis, Tunisia.
Insights
Prune Belly syndrome (PBS) is a rare condition affecting males, characterized by abdominal muscle deficiency, urinary tract, and undescended testes malformations. Renal dysplasia severity is the main prognostic factor, impacting patient outcomes.
Area of Science:
- Pediatric Surgery
- Urology
- Medical Genetics
Background:
- Prune Belly syndrome (PBS), also known as Eagle-Barrett syndrome, is a rare congenital disorder.
- It is defined by a triad of abdominal muscle deficiency, urinary tract abnormalities, and bilateral cryptorchidism.
- The syndrome's presentation varies widely, from stillbirth to near-normal development.
Purpose of the Study:
- To present clinical characteristics, diagnostics, therapy, and outcomes of 3 patients with Prune Belly syndrome.
- To highlight the diagnostic and therapeutic challenges associated with this rare condition.
- To emphasize the prognostic significance of renal dysplasia in PBS.
Main Methods:
- Retrospective case review of 3 patients diagnosed with Prune Belly syndrome.
- Analysis of clinical findings, diagnostic imaging, surgical interventions, and patient outcomes.
- Evaluation of the correlation between renal malformations and overall prognosis.
Main Results:
- All 3 patients exhibited congenital abdominal wall aplasia and diverse urogenital malformations.
- Two patients presented with cryptorchidism and mega-bladder; one had renal dysplasia.
- One patient required urgent vesicotomy for anuria; two newborns died from renal failure.
Conclusions:
- The management of Prune Belly syndrome is complex due to its varied presentation and the need for individualized surgical approaches.
- Severe renal dysplasia is the primary determinant of prognosis in PBS.
- Despite severe manifestations, some patients may achieve a good quality of life.
Abstract:
Prune Belly syndrome (PBS) or Eagle-Barrett syndrome is an anatomo-radiological syndrome consisting of a complex and rare malformation characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract, and bilateral cryptorchidism. The exact etiology is unknown, though PBS predominantly occurs in males. The clinical manifestations can vary widely, from stillbirth to renal and major respiratory dysplasia to almost normal children. The current study included a total of 3 patients. The findings included clinical characteristics, diagnostics, therapy, and clinical outcomes. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism and a mega-bladder were observed in 2 patients and distinctive renal malformations, such as renal dysplasia, were observed in 1 patient. Treatment varies but usually includes surgical management of symptoms. One patient required urgent urinary surgery; a vesicotomy was urgently performed due to anuria. These aspects explain the great diversity of opinions on the approach to this syndrome, but the severity of renal dysplasia is the main prognostic factor. Two newborns died a few days later due to severe renal failure. Despite these concerns, many patients with PBS report being in physical and mental health and having a good quality of life.
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