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Inferior Vena Cava System Anomalies: Surgical Implications

  • Kidney Diseases (G Ciancio, Section Editor)
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Abstract

Purpose of Review

The inferior vena cava (IVC) system is the major venous collecting blood network of the human body. This structure is formed in a complicated series of developmental stages between the fourth and eighth weeks of intrauterine life. Alterations in the developing process of the IVC system give rise to an array of different congenital variants or developmental anomalies.

Recent Findings

IVC anomalies are uncommon, usually of little physiological consequence, and mostly discovered incidentally during cross-sectional imaging in otherwise healthy individuals. However, they do have implications of relevance to surgeons because they may lead to significant complications during vascular interventional radiology procedures and retroperitoneal surgery when undiagnosed.

Summary

This review synthesizes the current literature pertaining the development and identification of IVC anomalies, highlighting their possible implications for surgical procedures involving this retroperitoneal vessel.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Mayo J, Gray R, St Louis E, et al. Anomalies of the inferior vena cava. AJR Am J Roentgenol. 1983;140:339–45.

    Article  CAS  PubMed  Google Scholar 

  2. •• González J, Ciancio G. Retroperitoneal venous diseases. In: PanVascular Medicine, Second Edition. Berlin Heidelberg: Springer; 2015. p. 3821–54. 10.1007/978-3-642-37078-6_148. Complete review on IVC embriology and embriological origin of caval anomalies.

  3. Eldefrawy A, Arianayagam M, Kanagarajah P, Acosta K, Manoharan M. Anomalies of the inferior vena cava and renal veins and implications for renal surgery. Cent European J Urol. 2011;64:4–8.

    Article  PubMed  PubMed Central  Google Scholar 

  4. Langman J, editor. Medical embryology. Baltimore: Williams and Wilkins; 1985. p. 212–7.

    Google Scholar 

  5. Warwick R, Williams P. Gray’s anatomy. Edinburgh, London, Melbourne, New York: Churchill Livingstone; 1989. p. 326–7.

    Google Scholar 

  6. Hamilton WJ, Mossman HW, editors. Hamilton, Boyd & Mossman’s Human Embryology. Cambridge: Heffer; 1972. p. 261.

    Google Scholar 

  7. Giordano JM, Trout HH. Anomalies of the inferior vena cava. J Vasc Surg. 1986;3:924–8.

    Article  CAS  PubMed  Google Scholar 

  8. Bass JE, Redwine MD, Kramer LA, et al. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional Imaging findings. Radiographics. 2000;20:639–52.

    Article  CAS  PubMed  Google Scholar 

  9. Moore KL, Persaud TVN. The cardiovascular system. In: Moore KL, editor. The developing human: clinically orientated embryology. London: Saunders; 2008. p. 285–336.

    Google Scholar 

  10. Meyer D-R, Huppe T, Andresen R, Freidrich M. Intra- and infrahepatic agenesis of the inferior vena cava with azygous continuation accompanied by duplication of the postrenal segment. Invest Radiol. 1998;33:113–6.

    Article  CAS  PubMed  Google Scholar 

  11. McClure CFW, Butler EG. The development of the vena cava inferior in man. Am J Anat. 1925;35:331–83.

    Article  Google Scholar 

  12. Shingleton WB, Hutton M, Resnick MI. Duplication of inferior vena cava: its importance in retroperitoneal surgery. Urology. 1994;43:113–5.

    Article  CAS  PubMed  Google Scholar 

  13. Soltes GD, Fisher RG, Whigham CJ. Placement of dual bird’s nest filters in an unusual case of duplicated inferior vena cava. J Vasc Intervent Radiol. 1992;3:709–11.

    Article  CAS  Google Scholar 

  14. Zhang L, Yang G, Shen W, et al. Spectrum of the inferior vena cava: MDCT findings. Abdom Imaging. 2007;32:495–503.

    Article  PubMed  Google Scholar 

  15. Bastide G, Lefebvre D. Anatomy and organogenesis and vascular malformations. In: Belov S, Loose DA, Weber J, editors. Vascular malformations. Reinbek: Einhorn-Presse Verlag GmbH; 1989. p. 20–2.

    Google Scholar 

  16. Lee BB, Laredo J, Lee SJ, Huh SH, Joe JH, Neville R. Congenital vascular malformations: general diagnostic principles. Phlebology. 2007;22:253–7.

    Article  CAS  PubMed  Google Scholar 

  17. Lee BB, Laredo J, Kim YW, Neville R. Congenital vascular malformations: general treatment principles. Phlebology. 2007;22:258–63.

    Article  CAS  PubMed  Google Scholar 

  18. Lee BB. Arteriovenous malformation. In: Zelenock, Huber, Messina, Lumsden, Moneta, editors. Mastery of vascular and endovascular surgery. Philadelphia: Lippincott, Williams and Wilkins publishers; 2006. p. 597–607.

    Google Scholar 

  19. Vaket L, Poppelier G, Vermeire P. Sur un cas d’anomalie combinée de la veine cave supérieure et du système azygos. Acta Anat. 1958;32:235–9.

    Article  Google Scholar 

  20. Zamboni P, Cossu A, Carpanese L, Simonetti G, Massarelli G, Liboni A. The so-called venous aneurysms. Phlebology. 1990;5:45–50.

    Article  Google Scholar 

  21. Rao KS, Gupta BK, Banerjee A, Srivastava KK. Chronic Budd-Chiari syndrome due to congenital membranous obstruction of the inferior vena cava: clinical experience. Aust N Z J Surg. 1989;59:335–8.

    Article  CAS  PubMed  Google Scholar 

  22. Croquet V, Aube C, Pilette C, et al. Syndrome due to membranous obstruction of the inferior vena cava of congenital origin. Ten-year follow-up after radiologic treatment. Gastroenterol Clin Biol. 1999;23:259–63.

    CAS  PubMed  Google Scholar 

  23. Mathews R, Smith PA, Fishman EK, Marshall FF. Anomalies of the inferior vena cava and renal veins: embryologic and surgical considerations. Urology. 1999;53:873–80.

    Article  CAS  PubMed  Google Scholar 

  24. Siegfried MS, Rochester D, Bernstein JR, et al. Diagnosis of inferior vena cava anomalies by computerized tomography. Comput Radiol. 1983;7:119–23.

    Article  CAS  PubMed  Google Scholar 

  25. Arisawa C, Kihara K, Fujii Y, et al. Possible misinterpretation on computed tomography of left inferior vena cava as retroperitoneal lymph node metastasis: a report of two cases. Int J Urol. 1999;6:215–8.

    Article  CAS  PubMed  Google Scholar 

  26. Byler TK, Disick GI, Sawczuk IS, Munver R. Vascular anomalies during laparoscopic renal surgery: incidence and management of left-sided inferior vena cava. JSLS. 2009;13:77–9.

    PubMed  PubMed Central  Google Scholar 

  27. Downey RS, Sicard GA, Anderson CB. Major retroperitoneal venous anomalies: surgical considerations. Surgery. 1990;107:359–65.

    CAS  PubMed  Google Scholar 

  28. Davachi AA, Thomas J, Dale WA, et al. Acute spontaneous rupture of an arteriosclerotic aneurysm into an isolated left sided inferior vena cava. Am J Cardiol. 1965;15:416–8.

    Article  CAS  PubMed  Google Scholar 

  29. Wartmann CT, Kinsella Jr CR, Tubbs RS, et al. A rare case of a complete left inferior vena cava associated with the symptoms of Dunbar syndrome. Clin Anat. 2011;24:262–5.

    Article  PubMed  Google Scholar 

  30. Savader SJ, Ronsivalle JA. Permanent inferior vena cava filters. In: Savader SJ, editor. Venous interventional radiology with clinical perspectives. New York: Thieme; 2000. p. 511–27.

    Google Scholar 

  31. Hirsch Jr DM, Chan KF. Bilateral inferior vena cava. JAMA. 1963;185:729–30.

    Article  PubMed  Google Scholar 

  32. Nagashima T, Lee J, Andoh K, et al. Right double inferior vena cava: report of 5 cases and literature review. J Comput Assist Tomogr. 2006;30:642–5.

    Article  PubMed  Google Scholar 

  33. Gomez CS, Arianayagam M, Casillas VJ, Ciancio G. Laparoscopic radical nephrectomy in the presence of a duplicated inferior vena cava. CEJ Urol. 2010;4:196–7.

    Google Scholar 

  34. Sartori MT, Zampieri P, Andres AL, et al. Double vena cava filter insertion in congenital duplicated inferior vena cava: a case report and literature review. Haematologica. 2006;91:ECR30.

    PubMed  Google Scholar 

  35. Kouroukis C, Leclerc JR. Pulmonary embolism with duplicated inferior vena cava. Chest. 1996;109:1111–3.

    Article  CAS  PubMed  Google Scholar 

  36. Mano A, Tatsumi T, Sakai H, et al. A case of deep venous thrombosis with a double inferior vena cava effectively treated by suprarenal filter implantation. Jpn Heart J. 2004;45:1063–9.

    Article  PubMed  Google Scholar 

  37. Smith DC, Kohne RE, Taylor FC. Steel coil embolization supplementing filter placement in a patient with a duplicated inferior vena cava. J Vasc Interv Radiol. 1992;3:577–80.

    Article  CAS  PubMed  Google Scholar 

  38. Anderson RC, Adams Jr P, Burke B. Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava). Report of 15 new cases. J Pediatr. 1961;59:370–83.

    Article  CAS  PubMed  Google Scholar 

  39. Schultz CL, Morrison S, Bryan PJ. Azygos continuation of the inferior vena cava: demonstration by NMR imaging. J Comput Assist Tomogr. 1984;8:774–6.

    Article  CAS  PubMed  Google Scholar 

  40. Mazzucco A, Bortolotti U, Stellin G, Gallucci V. Anomalies of the systemic venous return: a review. J Card Surg. 1990;5:122–33.

    Article  CAS  PubMed  Google Scholar 

  41. Wijdicks CA, Roseman DA. The clinical consequences of a circumaortic renal vein. Clin Anat. 2007;20:986–7.

    Article  PubMed  Google Scholar 

  42. Aljabri B, MacDonald PS, Satin R, et al. Incidence of major venous and renal anomalies relevant to aortoiliac surgery as demonstrated by computed tomography. Ann Vasc Surg. 2001;15:615–8.

    Article  CAS  PubMed  Google Scholar 

  43. Trigaux JP, Vandroogenbroek S, De Wispelaere JF. Congenital anomalies of the inferior vena cava and left renal vein: evaluation with spiral CT. J Vasc Interv Radiol. 1998;9:339–45.

    Article  CAS  PubMed  Google Scholar 

  44. Walker TG, Geller SC, Delmonico FL, et al. Donor renal angiography: its influence on the decision to use the right or left kidney. AJR Am J Roentgenol. 1988;151:1149–51.

    Article  CAS  PubMed  Google Scholar 

  45. Gibo M, Onitsuka H. Retroaortic left renal vein with renal vein hypertension causing hematuria. Clin Imaging. 1998;22:422–4.

    Article  CAS  PubMed  Google Scholar 

  46. Karaman B, Koplay M, Ozturk E, et al. Retroaortic left renal vein: multidetector computed tomography angiography findings and its clinical importance. Acta Radiol. 2007;48:355–60.

    Article  CAS  PubMed  Google Scholar 

  47. Nam JK, Park SW, Lee SD, Chung MK. The clinical significance of a retroaortic left renal vein. Korean J Urol. 2010;51:276–80.

    Article  PubMed  PubMed Central  Google Scholar 

  48. Lin CH, Steinberg AP, Ramani AP, et al. Laparoscopic live donor nephrectomy in the presence of circumaortic or retroaortic left renal vein. J Urol. 2004;171:44–6.

    Article  PubMed  Google Scholar 

  49. • Watson CJE, Harper SJF. Anatomical variation and its management in transplantation. Am J Transplant. 2015;15:1459–71. Review containing the most commonly found anatomic variants including those pertaining the IVC system, and their implications for retrieval and implantation surgery.

    Article  CAS  PubMed  Google Scholar 

  50. Kanojia RP, Bawa M, Handu AT, et al. Retrocaval ureter with stone in the retrocaval segment of the ureter. Pediatr Surg Int. 2010;26:863–5.

    Article  PubMed  Google Scholar 

  51. Hadzi-Djokic J, Basic D, Dzamic Z, et al. Retrocaval ureter based on a series of 16 cases. Prog Urol. 2009;19:33–8.

    Article  CAS  PubMed  Google Scholar 

  52. Brooks Jr RE. Left retrocaval ureter associated with situs inversus. J Urol. 1962;88:484–7.

    PubMed  Google Scholar 

  53. Pierro JA, Soleimanpour M, Bory JL. Left retrocaval ureter associated with left inferior vena cava. AJR Am J Roentgenol. 1990;155:545–6.

    Article  CAS  PubMed  Google Scholar 

  54. Chou CT, Yang AD, Hong YC, et al. Bilateral retrocaval ureters with IVC duplication. Abdom Imaging. 2006;31:596–7.

    Article  CAS  PubMed  Google Scholar 

  55. Hemal AK, Rao R, Sharma S, Clement RG. Pure robotic retrocaval ureter repair. Int Braz J Urol. 2008;34:734–8.

    Article  PubMed  Google Scholar 

  56. Dogan HS, Oktay B, Vuruskan H, Yavascaoglu I. Treatment of retrocaval ureter by pure laparoscopic pyelopyelostomy: experience on 4 patients. Urology. 2010;75:1343–7.

    Article  PubMed  Google Scholar 

  57. Chung BI, Gill IS. Laparoscopic dismembered pyeloplasty of a retrocaval ureter: case report and review of the literature. Eur Urol. 2008;54:1433–6.

    Article  PubMed  Google Scholar 

  58. Ramanathan T, Hughes TM, Richardson AJ. Perinatal inferior vena cava thrombosis and absence of the infrarenal inferior vena cava. J Vasc Surg. 2001;33:1097–9.

    Article  CAS  PubMed  Google Scholar 

  59. Wax JR, Pinette MG, Fife J, Blackstone J, Cartin A. Absent infrarenal inferior vena cava: an unusual cause of pelvic varices. J Ultrasound Med. 2007;26:699–701.

    Article  PubMed  Google Scholar 

  60. Dougherty MJ, Calligaro KD, DeLaurentis DA. Congenitally absent inferior vena cava presenting in adulthood with venous stasis and ulceration: a surgically treated case. J Vasc Surg. 1996;23:141–6.

    Article  CAS  PubMed  Google Scholar 

  61. 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.

    Article  CAS  PubMed  Google Scholar 

  62. Van Veen J, Hampton KK, Makris M. KILT syndrome? Br J Haematol. 2002;118:1199–200.

    Article  PubMed  Google Scholar 

  63. Milner LB, Marchan R. Complete absence of the inferior vena cava presenting as a paraspinous mass. Thorax. 1980;35:798–800.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  64. Benbow EW. Idiopathic obstruction of the inferior vena cava: a review. J Royal Soc Med. 1986;79:105–8.

    Article  CAS  Google Scholar 

  65. Koc Z, Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings. Eur J Radiol. 2007;62:257–66.

    Article  PubMed  Google Scholar 

  66. Kang SK, Kim D, Chandarana H. Contemporary imaging of the renal mass. Curr Urol Rep. 2011;12:11–7.

    Article  PubMed  Google Scholar 

  67. Gorin MA, Shirodkar SP, Young E, Ciancio G. En bloc mobilization of the spleen, pancreas, and colon during hand-assisted laparoscopic left donor nephrectomy. Eur Urol. 2011;60:601–2.

    Article  PubMed  Google Scholar 

  68. González J, Ciancio G. Caval thrombus in conjunction with renal tumors: indication for surgery and technical details. Curr Urol Rep. 2014;15:451. Technical review containing a detailed description of the maneuvers derived from transplant surgery that may be of help in gaining medial exposure before hilar dissection in nephrectomy.

    Article  PubMed  Google Scholar 

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Correspondence to Javier González.

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Javier González, Jeffrey J. Gaynor, and Luis F. Albéniz each declare no potential conflicts of interest.

Gaetano Ciancio is a section editor for Current Urology Reports.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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González, J., Gaynor, J.J., Albéniz, L.F. et al. Inferior Vena Cava System Anomalies: Surgical Implications. Curr Urol Rep 18, 10 (2017). https://doi.org/10.1007/s11934-017-0658-y

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