PRUNE BELLY SYNDROME AND ITS ASSOCIATION WITH CHRONIC KIDNEY DISEASE: A CASE REPORT

Abstract

This case report discusses Prune-Belly syndrome (PBS), a rare congenital condition that primarily affects males and is characterized by abdominal wall muscle deficiencies, cryptorchidism, and urinary tract malformations, often associated with genetic mutations. Diagnosis is often made prenatally via ultrasound, which can indicate complications such as hydronephrosis and oligohydramnios, increasing postnatal mortality, especially in girls with urethral atresia. Treatment involves both prenatal interventions, such as urinary diversion, and postnatal interventions, including urinary tract reconstruction and antimicrobial prophylaxis due to the risk of pyelonephritis. The objective of this study is to associate Prune Belly Syndrome with kidney disease through a case report. PBS can lead to chronic kidney disease, increasing the risk of anemia, cardiovascular disease, growth retardation, bone disorders, and cognitive impairment. This study reports the case of a patient with PBS who developed chronic kidney disease and explores clinical information from medical records and laboratory and imaging test results, following appropriate ethical and regulatory guidelines. The patient was diagnosed at birth and developed multiple urinary tract infections. A surgical intervention, such as a ureterostomy, was performed, but the patient developed non-dialysis-requiring chronic kidney disease. At six months of age, the patient developed severe refractory sepsis and acute kidney injury. However, due to hemodynamic instability, he was unable to receive renal replacement therapy, ultimately leading to death.