|Year : 2009 | Volume
| Issue : 1 | Page : 27-29
Double inferior vena cava in a patient with bilateral testicular tumor: A case report with review of literature
B Satheesan, D Suresh Kumar, N Kathiresan
Department of Surgical Oncology, Division of Genito-Urinary Oncology, Cancer Institute (W.I.A.), Sardar Patel Road, Adayar, Chennai - 600 020, India
|Date of Submission||16-Dec-2008|
|Date of Acceptance||02-Feb-2009|
Division of Genito-Urinary Oncology, Department of Surgical Oncology, Cancer Insti tute (W.I.A.), Sardar Patel Road, Adyar, Chennai - 600 020
| Abstract|| |
Double inferior vena cava (IVC) is a rare developmental anomaly of IVC with a prevalence rate of 0.2-0.3%. Preoperative identification of major vascular anomalies is of paramount importance for a retroperitoneal surgeon. In addition, more anomalies are to be expected in those situations. Radiological studies in similar cases may be reported as retroperitoneal lymph nodes or masses if the radiologist is unaware. In this case report, a patient with bilateral testicular tumor with double IVC who underwent extensive retroperitoneal and pelvic lymph node dissection is described for rarity of the condition and technical difficulties associated with such a condition.
Keywords: Double inferior vena cava, testicular tumors, vascular anomaly
|How to cite this article:|
Satheesan B, Suresh Kumar D, Kathiresan N. Double inferior vena cava in a patient with bilateral testicular tumor: A case report with review of literature. Urol Ann 2009;1:27-9
|How to cite this URL:|
Satheesan B, Suresh Kumar D, Kathiresan N. Double inferior vena cava in a patient with bilateral testicular tumor: A case report with review of literature. Urol Ann [serial online] 2009 [cited 2019 Aug 23];1:27-9. Available from: http://www.urologyannals.com/text.asp?2009/1/1/27/48784
| Introduction|| |
Congenital anomalies of the inferior vena cava (IVC) are rare. The IVC originates in the abdominal and pelvic venous system in the embryo between six and ten weeks.  The complex mechanisms of vascular development translate into large number of possible abnormalities. These congenital anomalies of the IVC are associated with other anomalies, especially cardiac anomalies.  Congenital anomalies of the IVC have become more commonly recognized in asymptomatic patients with newer imaging modalities. The prevalence of IVC anomalies identified by computerized tomography (CT) scan is 5.6% in asymptomatic individuals.  Proper imaging studies are essential in planning retroperitoneal surgeries as vascular anomalies may cause embarrassment and unpleasant surprises for the surgeon. Massive hemorrhagic complications have been reported in 10% of patients with unidentified vascular anomalies.  These vascular anomalies at times mimic retroperitoneal nodes or masses. Modern imaging allows us to identify and discern between vascular structures and enlarged lymph nodes.  Here, we present a case of double IVC in a patient with bilateral testicular tumor who underwent post chemotherapy retroperitoneal lymph node dissection (RPLND) for residual tumor masses.
| Case Report|| |
A 38-year-old gentleman presented with history of bilateral testicular tumors for which bilateral inguinal orchidectomy was performed elsewhere. He had large retroperitoneal, supraclavicular, right inguinal, right popliteal, and mediastinal nodal masses. These were confirmed with imaging studies. Serum markers were elevated (serum alphafeto protein: 400 ng/ml and beta HCG: 550.13 u/l). Histopathological study revealed nonseminomatous mixed germ cell tumor - mature and immature teratoma with yolk sac tumor and a minor component of embyonal carcinoma (NSGCT). Evaluation entitled the clinical stage as stage III C (TNM). He was treated with four cycles of cisplatin-based chemotherapy at a local hospital (three cycles of cisplatin, bleomycin, and etoposide; and one cycle of etoposide and cisplatin). Postchemotherapy evaluation revealed persistence of peripheral, retroperitoneal, and thoracic masses with normalization of tumor markers. CT scan of abdomen and pelvis revealed double IVC with multiple large retroperitoneal and pelvic nodal masses [Figure 1]. He was referred to our center for further management. Echocardiography and CT scan of the chest failed to reveal any other major vascular or cardiac structural anomaly. Subsequently, he underwent resection of peripheral nodal mass (popliteal and right inguinal nodal masses) and suprahilar RPLND with bilateral pelvic lymphadenectomy in separate sittings. During RPLND double IVC was confirmed. The left IVC was found crossing the aorta at the level of left renal vein and the latter was draining into left IVC. There were three pairs of lumbar veins draining into left IVC and two into right IVC. There were two separate right renal veins 2 cm apart draining into right IVC. Inferiorly each IVC continues downwards as respective iliac veins. Right kidney had a lower polar artery from aorta. The RPLND was tedious for the dissection of additional major vessel and the constant look out for more anomalies. IVC, aorta, and pelvic vessels were dissected and looped during the resection [Figure 2]. All lumbar vessels were ligated and divided. Postoperative histopathology revealed mature and immature teratoma with a focus of embyonal carcinoma.
| Discussion|| |
The development of the IVC during the early embryonic period is a complex process. The entire process starts in the sixth week of gestation and continues till the tenth week. It involves formation, regression, and fusion between three longitudinal pairs of veins - postcardinal, subcardinal, and supracardinal veins. The postcardinal veins appear first and later regress to form iliac bifurcation. This is followed by the appearance of subcardinal veins of which right subcardinal vein becomes suprarenal IVC and the left regresses. The supracardinal veins appear last and the right one forms the infrarenal IVC. The left one disappears. The junction of supracardinal and subcardinal veins contributes to the development of renal veins. IVC is rightsided in nearly 98% of the population. The most common variation is duplication of the vein, with prevalence of 0.2-3%. Double IVC described in our case typically ends after receiving the left renal vein before crossing the aorta. This is due to the persistence of left supracardinal vein. A left-sided IVC anomaly has been reported with a prevalence of 0.2-0.5%.  A left IVC anomaly could cross the aorta either in front or behind the level of the renal hila, and might be misinterpreted as an lymphnode, which could alter the staging and management NSGCT. There may be changed passage of ureters in association with IVC anomalies. Enumeration of various IVC anomalies is beyond the scope of this article.
Associated anomalies reported with duplication of the abdominal vena cava include, cloacal extrophy, congenital absence of the right kidney, renal ectopia with abdominal aortic aneurysm, right retrocaval ureter, and congenital heart disease like atrial septal defect (ostium primum and secundum). ,,
The most important clinical consequences of the duplication of the vena cava are observed in retroperitoneal surgery.  Because there is an increased risk of operative complication, determination of any anatomical variation is an essential component of preoperative assessment. In order to reduce the hazards of significant venous hemorrhage, the surgeons should be aware of the embryogenesis and should be able to recognize and identify the major venous anomaly in this region. Another surgical implication observed is during whole organ transplantation and radical nephrectomy.  During the staging of testicular tumors, the radiologist should be aware of the possible anomalies in order to avoid possibly misleading reports such as para aortic lymphadenopathy.
Major venous anomalies such as double IVC can also lead to alteration in lymphatic drainage as the lymphatic drainage generally tends to follow the vascular pattern. Patient with an abnormal venous anatomy may have unusual pattern of lymphatic drainage and lymph node metastases.  In our case he had unusual lymph node site metastases for testicular cancer (inguinal and popliteal).
| Conclusion|| |
Major venous anomalies in the retroperitoneum should be borne in mind while considering retroperitoneal surgery. Preoperative identification of such anomalies enables the surgeon to avoid catastrophe during the procedure. Onus of the same is not only on the imaging modality but also on the radiologist and surgeon.
| Abbreviations|| |
IVC - Inferior vena cava, NSGCT - Nonseminomatous germ cell tumor, RPLND - Retroperitoneal lymph node dissection
| References|| |
|1.||Chaurg VP, Mena CE, Hoskins PA. Congenital anomalies of the inferior vena cava: Review of embryogenesis and presentation of a simplified classification. Br J Radiol 1974;47:206-13. |
|2.||Gayer G, Zissin R, Strauss S, Hertz M. IVC anomalies and right renal aplasia detected on CT: A possible link? Abdom Imaging 2003;28:395-9. [PUBMED] [FULLTEXT]|
|3.||Kameoka H, Matsumaoto F, Namiki M, Sonoda T, Kashiwagi T. Three dimensional imaging of left sided vena cava: A case report. Urol Int 1992;48:350-2. |
|4.||Aljabri B, MacDonald PS, Santin R, Stein LS, Obrand DI, Steinmetz OK. Incidence of major venous and renal anomalies relevant to aortoiliac surgery as demonstrated by computed tomography. Ann Vasc Surg 2001;15:615-8. |
|5.||Philips E, Embryology, normal anatomy and anomalies. In: Ferris EJ, Hipona FA, Kanh PC, Philips E, Shapiro JH, editors. Venography of inferior vena cava and its branches. Baltimore, MD: Williams and Wilkins; 1969. p. 1-32. |
|6.||Shaw MB, Cutress M, Papavassiliou V, White S, Thompson M, Sayers R. Duplicated inferior vena cava and crossed renal ectopia with abdominal aortic aneurysm: Preoperative anatomic studies facilitate surgery. Clin Anat 2003;16:355-7. [PUBMED] [FULLTEXT]|
|7.||Vigano M, Montemartini C, Setti M, Petroboni E. Unusal congenital heart disease: Association of ostium premium, ostium secondum and double vena cava inferior with abnormal left atrium drainage: Apropos of a successfully operated case. Minerva Cardioangiol 1973;21:79-88. |
|8.||Gazaigne J, Alkhouya AS, Sebe P, Mozziconacci JG. Transcaval ureter. Prog Urol 2002;12:486-9. |
|9.||Babaian RJ, Johnson DE. Major venous anomalies complicating retroperitoneal surgery. South Med J 1979;72:1254-8. [PUBMED] [FULLTEXT]|
|10.||Evans JC, Earis J, Curtis J. Thrombosed double IVC mimicking paraaortic lymphadenopathy. Br J Radiol 2001;74:192-4. [PUBMED] [FULLTEXT]|
[Figure 1], [Figure 2]