Prune Belly Syndrome (PBS) is a rare congenital disorder that affects the abdominal muscles, urinary tract, and genitalia. The spelling of this syndrome is represented using the International Phonetic Alphabet (IPA) as /pruːn ˈbɛli sɪndroʊm/. The first syllable, "pruːn," is pronounced like the word "prune," while the second part of the word, "ˈbɛli," uses the short vowel sound of "e" and an acute accent over the "i" to indicate stress on the second syllable. This spelling accurately represents the pronunciation of this disorder.
Prune Belly Syndrome, also known as Eagle-Barrett syndrome or abdominal muscle deficiency syndrome, is a rare congenital disorder that affects the development of a fetus, leading to various abnormalities in the abdominal region. This syndrome primarily affects males, and its exact cause is not yet fully understood.
The key characteristic of Prune Belly Syndrome is the underdevelopment or absence of the abdominal muscles, giving the abdomen a wrinkled and prune-like appearance. This lack of muscular support results in a protruding belly resembling a prune. Furthermore, the syndrome may cause abnormalities in other body systems, including urinary tract and gastrointestinal issues, as well as undescended testicles in males.
Medical professionals may diagnose Prune Belly Syndrome through ultrasound imaging during pregnancy or shortly after birth, based on physical examination and the presence of characteristic symptoms. There is currently no cure for the syndrome, and treatment focuses on managing the associated complications. This entails addressing urinary and kidney problems with medication or surgical interventions, as well as providing nutritional support and ensuring appropriate growth and development.
The severity of Prune Belly Syndrome can vary widely among individuals, with some experiencing relatively mild symptoms while others may face more complex health challenges. Due to the complexity of the disorder, a multidisciplinary approach involving pediatric surgeons, urologists, nephrologists, and geneticists is typically necessary to provide comprehensive care to individuals with Prune Belly Syndrome.