Unilateral renal agenesis and duplication of inferior vena cava in an adult female

A 62-year-old female patient with a one-year history of dysuria and no accompanying symptoms was referred to Radiology for a pre-endoscopic assessment of the abdomen. No distinct facies were observed, nor were previous surgical interventions noted.

Abdominal contrast-enhanced computed tomography (CT) showed no right kidney in the right renal fossa, its vasculature and corresponding urinary collecting system (Figure 1). The left kidney is of normal position and configuration (Figures 1 and 2). It measures approximately 12.5 x 4.9 x 6.2 cm (cephalocaudal x width x anteroposterior).

Two inferior vena cavae (IVC) are detected (pointed by green and yellow arrowheads in Figure 1). The right IVC is seen draining its ipsilateral common iliac vein and terminating at the right atrium. The left IVC is also draining its ipsilateral common iliac vein and terminating at the left renal vein (pointed by the orange arrow in Figure 1).

The uterus and the other abdominal organs appear unremarkable.

Background

The inferior vena cava (IVC) embryogenesis occurs during the fourth to eighth weeks of gestation. At this stage, three sets of paired veins, namely the supracardinal veins, the posterior cardinal veins, and the subcardinal veins, exist. These veins develop and regress sequentially until the final mature IVC is formed. Persistence of supracardinal veins is the most common cause of duplicated IVC [1–3].

Double IVC is relatively rare, with a prevalence of 0.2–3%. The incidence of unilateral renal agenesis ranges from 1:1100 to 1:5000. Additionally, the association of double IVC with unilateral renal agenesis has rarely been reported in the literature [4].

Clinical Perspective

Recognising and reporting such inferior vena cava anomalies is vital to prevent complications during abdominal operations, particularly retroperitoneal-associated operations and IVC filter placement [5]. Laparoscopic live donor nephrectomy also requires extra caution in the presence of duplicated IVC [1].

Imaging Perspective

Post-processing reconstructions, such as multiplanar reconstruction, 3D reconstruction, and maximum intensity projection, can be utilised to better assess the vasculature.

Outcome

Careful investigation of the abdominal vasculature through imaging is crucial to avoid mistaking it for other conditions such as pathological lymphadenopathy, saccular aortic aneurysm, left pyelouretic dilatation, retroperitoneal cyst, or an abnormal form of a gonadal vessel.

Teaching Points

Missed or misdiagnosed abnormal development of IVC can lead to complications during vascular and retroperitoneal interventions, hence the need for proper diagnosis [2,3,5]. Careful investigation of the abdominal vasculature is crucial to avoid such circumstances.

All patient data have been completely anonymised throughout the entire manuscript and related files.