Prune-Belly Syndrome: An Update

Title:Prune-Belly Syndrome: An Update

Volume: 20

Author(s): Ana Flávia Conegundes, Isadora Garcia, Bárbara Miranda, Arthur Borges, André Sanglard, Gabriel Ferreira, Rafael Borges and Ana Cristina Simões e Silva*

Affiliation:

• Interdisciplinary Laboratory of Medical Investigation, Unit of Pediatric Nephrology, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, Minas Gerais, Brazil

Keywords: Prune-belly syndrome, congenital anomalies of the kidney and urinary tract, chronic kidney disease, mesodermal developmental defect, urethral obstruction, abdominal wall defect, bilateral cryptorchidism.

Abstract: The Prune-Belly (Eagle-Barrett) syndrome (PBS) is a congenital and genetically heterogeneous disease, more prevalent in males, defined by the clinical triad (1) deficiency of abdominal muscles, (2) bilateral cryptorchidism, and (3) urinary tract abnormalities. The abdomen of an infant with PBS has a typical appearance, similar to the aspect of a prune, which gives it its name. Although the etiology of this disorder is still unknown, numerous theories, mutations, and genetic disturbances have been proposed to explain the origin of PBS. Prognosis can differ a lot from one patient to another, since this condition has a wide spectrum of clinical presentation. Despite being a rare condition, the importance of PBS should not be underestimated, in the light of the potential of the disorder to lead to chronic kidney disease and other severe complications. In that regard, this review gathers the most up-to-date knowledge about the etiopathogenesis, clinical features, diagnosis, management and prognosis of PBS.

Cite this article as:

Conegundes Ana Flávia, Garcia Isadora, Miranda Bárbara, Borges Arthur, Sanglard André, Ferreira Gabriel, Borges Rafael and Simões e Silva Ana Cristina*, Prune-Belly Syndrome: An Update, Current Pediatric Reviews 2024; 20 () . https://dx.doi.org/10.2174/0115733963285237240524042142