Hydroureteronephrosis Caused by a Retrocaval Ureter

A Rare Case Report

Abstract

A rare congenital abnormality of the inferior vena cava is known as the retrocaval ureter. During embryogenesis, the ureter descends
posteriorly to the inferior vena cava. While this anomaly can occur in pediatric patients, it most frequently presents symptoms in individuals during their third and fourth decades of life, such as hydronephrosis, typically caused by ureteric obstruction. Retrocaval ureter may also be asymptomatic, which could explain its low occurrence worldwide and underreporting of cases. In this particular case, we reviewed the medical record of a 21-year-old male patient who had right Pelviureteric junction obstruction (PUJO) due to a right retrocaval ureter. This condition was clinically managed through a right pyeloplasty procedure and the placement of a double J stent.

Introduction

Since its first description in 1893, the circumcaval ureter, also referred to as the retrocaval ureter, has been documented in fewer than 200 cases [1]. This anomaly leads to the proximal ureter inserting posteriorly to the inferior vena cava (IVC) and subsequently at the renal pelvis. The abnormal development of the infrarenal IVC is attributed to the ventrally positioned subcardinal vein, which confines the ureter posteriorly. Typically, between weeks four and eight of fetal development, the infrarenal IVC grows dorsally from the supra cardinal vein [1]. Prior to reaching the bladder, the distal ureter typically passes superficially alongside the common iliac vessels [2]. Patients with IVC compression of the proximal ureter, resulting in hydronephrosis or hydroureteronephrosis, often remain asymptomatic until their third or fourth decades of life, despite the anomaly being present from birth [3].