A Rare Syndromic Coexistence: Bilateral Duplicated Collecting Systems with Bilateral Ureterovesical Junction Obstruction in a Child with Heterotaxy Syndrome and Polysplenia: A Case Report

Abstract The simultaneous occurrence of bilateral duplicated collecting systems and heterotaxy syndrome is extremely rare, with no previously reported cases from our setting to our knowledge. In this report, we present the case of a 2-year-old girl who experienced recurrent urinary tract infections, lower abdominal pain, and incontinence. An ultrasound revealed bilateral hydroureteronephrosis, and a contrast-enhanced computed tomography intravenous urography showed enlarged kidneys on both sides with duplicated collecting systems, moderate hydroureteronephrosis of the upper moiety, and dilated ureters due to stricture at the uretovasical junction. Additionally, the imaging revealed features consistent with heterotaxy syndrome (situs ambiguous) with polysplenia, including a right-sided stomach, hypoplastic pancreas, an elongated left liver lobe that extends laterally (beaver tail liver), a normal morphological variant of the liver, and azygous continuation of the inferior vena cava with absence of intrahepatic and infrahepatic segments. The heart and sigmoid colon were in their usual positions, with no associated cardiac abnormalities identified. The patient was referred to pediatric urology and nephrology for further evaluation. This case highlights the diagnostic value of cross-sectional imaging in identifying complex congenital anomalies and emphasizes the importance of a multidisciplinary approach in managing such rare presentations.