Congenital anomalies of the kidney and the urinary tract - leading cause for chronic kidney disease in children - a case report

Abstract

Introduction: The spectrum of congenital anomalies of the kidneys and the urinary tract is extremely broad and ranges from mild, asymptomatic malformations to severe, life-threatening ones. In young children congenital anomalies are the leading cause for kidney failure and for kidney transplantation or dialysis. Prenatal sonography has facilitated the detection of urological abnormalities presenting with hydronephrosis. The goal of management is preservation of renal function through mitigation of the risk for recurrent urinary tract infections (UTI) and/or obstruction. Material and methods: A case report of a 14-year-old male patient is discussed. He is diagnosed at the age of two months with congenital bilateral grade 2 hydronephrosis and megaureters, caused by ob­structive uropathy - Marion`s disease (congenital bladder neck sclerosis). Results: The diagnosis is confirmed via abdominal ultrasound, intravenous urography and comput­ed tomography (CT). At four months the patient is consulted with a pediatric urologist in Universi­ty Hospital for Emergency Medicine `N.I. Pirogov`, where corrective surgery is performed. He is ad­mitted multiple times at the Pediatric urology department in Sofia and at the Pediatric department in University Hospital `St. Marina` in Varna due to UTIs and an exacerbation of his kidney disease. The patient underwent five surgeries after the diagnosis in accordance with the severity of his anom­aly. The imaging studies demonstrated grade 1 hydronephrosis with improvement of urinary tract drainage. During the periodical follow-ups his renal laboratory test results indicated chronic kidney disease stage 2. Conclusion: Congenital anomalies of the kidney and urinary tract are the main cause for chronic kid­ney disease in pediatric patients. Advances in prenatal diagnostics are essential for early detection and diagnosis, requiring a multidisciplinary approach in management and prevention of this condi­tion.