Summary: | The urachus is a tubular structure that extends cranially from the anterior dome of the bladder to the umbilicus. Urachal anomalies (UA) formation results from incomplete involution of this embryonic structure, leading to various pathologies. Congenital UA can be divided into five groups: patent urachus, umbilical-urachal sinus, vesicourachal diverticulum, urachal cyst, and alternating sinus. Typically, a patent urachus is detected around birth, while the other congenital UA tend to be asymptomatic. Due to the relatively rare incidence and presenting symptoms mirroring intraabdominal or pelvic diseases, UA is not typically considered in differential diagnosis. When viewed in the differential, diagnosis of congenital UA and acquired urachal remnant diseases is elusive because of the heterogeneous clinical presentation and no uniform diagnosis protocol. Herein, we provide a comprehensive review of urachal embryology, anatomy, clinical manifestations, and the current trends in diagnostic evaluation and management of UA, with an emphasis on pediatrics. Keywords: Urachus, Urachal remnant, Umbilical discharge, Umbilical mass, Vitelline duct, Hematuria
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