Study of Prune Belly Syndrome: An Underreported Rare Disease
Introduction and objectives: Prune belly syndrome is a rare disease with an incidence rate of 1 per 40,000 live births and has a triad of clinical features like bilateral cryptorchidism, lax abdomen and urogenital defects. A 4 month old baby with prune belly syndrome who was admitted to the government hospital with complaints of recurrent urinary tract infection led us to do a short review of this disease. The review aims to evaluates the incidence of prune belly syndrome, identifies the common clinical presentation and evaluates the outcome of this syndrome published in the literature.
Methods: A literature search was conducted using keywords in different databases. Overall, 204 articles published from January 2010 to June 2021 on prune belly syndrome were screened, out of which 89 non duplicated articles were identified. Of these, 35 relevant articles were included.
Results: Most of the cases presented with multiorgan defects, in which the majority of children had gastrointestinal and genitourinary defects. The most common complications identified were renal failure and recurrent urinary tract infections. A small percent of patients have undergone surgical procedures as a part of the management. 16 (45.71%) patients died mostly due to the associated complications.
Conclusion: It has been noticed that at the time of detection, the disease has worsened or complications have developed leading to high mortality among affected children. Therefore, spreading awareness among parents and healthcare professions that early investigation and diagnosis along with a multidisciplinary approach can improve a patient's survival is extremely vital.