British Journal of Radiology (2007) 80, e88-e92
© 2007 British Institute of Radiology
doi: 10.1259/bjr/13099777
Inferior vena cava duplication and left renal aplasia: incidental findings?
Y Hama, MD
Department of Radiology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-0042, Japan and Radiation Biology Branch, NCI/NIH, Building 10, Room B3 B69, Bethesda, MD 20892-1002, USA
Correspondence: Yukihiro HamaMD, Radiation Biology Branch, NCI/N H, Building 10, Room B3 B69, Bethesde, MD 20892-1002, USA. E-mail: yjhama{at}yahoo.co.jp
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Abstract
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A duplicated inferior vena cava and renal aplasia is rare and absence of one kidney has been considered to occur exclusively on the right side. We report an unusual case of a duplicated inferior vena cava and left renal aplasia and discuss the interrelationship between these developmental anomalies.
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Introduction
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The association of congenital anomalies of the inferior vena cava (IVC) with renal aplasia or hypoplasia is very rare and has been reported in only 11 patients [1, 2]. Interestingly, absence of one kidney occurs exclusively on the right side and most patients were male [1]. We present the intravenous pyelography (IVP) and CT findings of a unique female case of duplicated IVC with left renal aplasia, and discuss the pathogenesis and possible aetiology.
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Case report
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A 58-year-old woman was referred to our institution for evaluation of microscopic haematuria, which was pointed out in an annual health check up. Her past history and family history were not remarkable. Laboratory data and general physical examination were unremarkable except for microscopic haematuria. Abdominal ultrasound showed dilatation of the right renal pelvis, thus IVP was performed to rule out urinary calculi. IVP demonstrated no dilatation or obstruction of the urinary tract; however the left urinary tract was not visualized (Figure 1
). CT scan revealed the hypertrophied right kidney, but the left kidney was not detected (Figure 2a). The left adrenal gland was present as a typical linear shape. Duplication of the IVC was also noted and the left IVC crosses anterior to the aorta, uniting with the right IVC to form a normal right-sided IVC (Figure 2b). Based on the findings of IVP and CT, a diagnosis of duplicated IVC and left renal aplasia was made. 2 weeks later, the microscopic haematuria disappeared completely with fluid treatment.
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Figure 1. Intravenous pyelography demonstrated the normal functioning right kidney and the non-functioning left kidney.
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Figure 2. (a) At the level of the right kidney, contrast-enhanced CT shows that the right kidney (arrowheads) is large, but the left kidney is undetectable. Both right inferior vena cava (IVC; black arrow) and left IVC (white arrow) are identified. (b) At the level of the upper pole of the right kidney, contrast-enhanced CT shows that the left IVC (arrow) crosses anterior to the aorta and unites with the right IVC. The right accessory renal vein (arrowhead) is draining directly into the crossing left IVC.
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Discussion
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Duplication of the IVC results from persistence of both supracardinal veins [3]. The prevalence is 0.2–3% [3]. The exact incidence of unilateral aplasia of the kidney is not known because the anomaly is often clinically silent. It ranges from 1:1100 to 1:5000 in various series using different methods including autopsy, excretory urography and ultrasound screening [1]. However, the association of IVC anomalies with renal aplasia is very rare and has been reported in only 11 patients [1, 2]. It has been suggested that the IVC anomaly may be associated with the right kidney aplasia, because there has been no reported case of IVC anomaly and absence of the left kidney in the literature. It has been postulated that the developing kidneys profoundly affect the formation of the IVC [4]. Lack of space for the expanding metanephric tissue for a few days during the fifth to seventh weeks of gestation causes the regression of some of the venous structures draining the caudal end of the embryo [5]. The special relationship between the kidney and the laterally located posterior cardinal veins [3] profoundly influences the fate of the primitive venous system and determines the asymmetric right vena cava [5]. We consider that the absence of the left kidney in the pelvis during embryological organogenesis allowed the primitive veins to persist, thus causing an anomalous development to occur. It may be possible to apply this hypothesis to the aplasia of the right kidney, too [5]. In that case, how can we explain the fact that most unilateral renal aplasia with IVC anomalies occurrs on the right side? Was it just by chance, or was this the result of an acquired insult [7, 8]? There is no exact answer to the question now [1].
Although IVC and renal anomalies may be clinically silent, each condition may be associated with morbidity. An IVC anomaly may produce a variety of symptoms, in particular deep vein thrombosis [1, 3]. Patients with unilateral renal aplasia are susceptible to hypertension and to renal failure [1]. Thus, it is important to be aware of the possible concomitant presence of these two significant anomalies. Vascular structure and renal configuration can usually be identified on contrast-enhanced CT scans and/or MR angiography [3, 6].
In conclusion, this case shows imaging of rare anomalies using contrast-enhanced CT scans and IVP. Although the exact aetiology of IVC and renal anomalies is not clear, knowledge of both anomalies is essential to avoid diagnostic pitfalls.
Dr Y Hama is partially funded by a grant from the Japan–US Radiological Exchange Association and the National Defense Medical College, Japan.
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Acknowledgments
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The author thanks Prof. Shoichi Kusano, National Defense Medical College, Japan, Dr Toshiyuki Nakagawa and Dr Ryuhei Miyakawa, Toyookadaiichi Hospital, Japan for their great assistance.
Received for publication April 26, 2005.
Revision received June 23, 2005.
Accepted for publication July 11, 2005.
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References
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Abstract
Introduction
