Diagnostic Considerations

Because patients with inferior vena caval (IVC) thrombosis (IVCT) may present with a variety of signs and symptoms, the diagnosis can be challenging. Using a system to classify the symptoms (eg, predominantly thrombotic in origin or predominantly embolic in nature) may aid in the diagnosis.

Thrombosis occurring at the level of the renal veins raises the possibility of renal cell carcinoma (RCC). However, more commonly, thrombosis at this level suggests a nephrotic syndrome.

The clinician must have a profound awareness of the associated elements that make up the clinical complex of congenital vena caval thrombosis in order to avoid diagnostic and treatment pitfalls. In addition, knowledge of the association of other anomalies in patients with an absent IVC, such as renal atrophy or agenesis, can highlight underlying vascular anomalies, which are in and of themselves of significant clinical importance. With physician awareness of the possibility of IVCT in various situations, the patient is more likely to receive prompt diagnosis and subsequent intervention that minimizes ensuing sequelae.

Workup

Anderson FA Jr, Wheeler HB, Goldberg RJ, Hosmer DW, Patwardhan NA, Jovanovic B, et al. A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism. The Worcester DVT Study. Arch Intern Med. 1991 May. 151 (5):933-8. [QxMD MEDLINE Link].
White RH. The epidemiology of venous thromboembolism. Circulation. 2003 Jun 17. 107 (23 Suppl 1):I4-8. [QxMD MEDLINE Link]. [Full Text].
McAree BJ, O'Donnell ME, Fitzmaurice GJ, Reid JA, Spence RA, Lee B. Inferior vena cava thrombosis: a review of current practice. Vasc Med. 2013 Feb. 18 (1):32-43. [QxMD MEDLINE Link].
Alkhouli M, Shafi I, Bashir R. Inferior vena cava filter thrombosis and suprarenal caval stenosis: a double whammy. JACC Cardiovasc Interv. 2015 Feb. 8 (2):e23-e25. [QxMD MEDLINE Link].
Alkhouli M, Morad M, Narins CR, Raza F, Bashir R. Inferior Vena Cava Thrombosis. JACC Cardiovasc Interv. 2016 Apr 11. 9 (7):629-43. [QxMD MEDLINE Link].
Kaufman LB, Yeh BM, Breiman RS, Joe BN, Qayyum A, Coakley FV. Inferior vena cava filling defects on CT and MRI. AJR Am J Roentgenol. 2005 Sep. 185 (3):717-26. [QxMD MEDLINE Link].
Marcy PY, Magné N, Frenay M, Bruneton JN. Renal failure secondary to thrombotic complications of suprarenal inferior vena cava filter in cancer patients. Cardiovasc Intervent Radiol. 2001 Jul-Aug. 24 (4):257-9. [QxMD MEDLINE Link].
Alkhouli M, Bashir R. Inferior vena cava filters in the United States: less is more. Int J Cardiol. 2014 Dec 20. 177 (3):742-3. [QxMD MEDLINE Link].
Angel LF, Tapson V, Galgon RE, Restrepo MI, Kaufman J. Systematic review of the use of retrievable inferior vena cava filters. J Vasc Interv Radiol. 2011 Nov. 22 (11):1522-1530.e3. [QxMD MEDLINE Link].
Dabbagh O, Nagam N, Chitima-Matsiga R, Bearelly S, Bearelly D. Retrievable inferior vena cava filters are not getting retrieved: where is the gap?. Thromb Res. 2010 Dec. 126 (6):493-7. [QxMD MEDLINE Link].
Ahmad I, Yeddula K, Wicky S, Kalva SP. Clinical sequelae of thrombus in an inferior vena cava filter. Cardiovasc Intervent Radiol. 2010 Apr. 33 (2):285-9. [QxMD MEDLINE Link].
Fox MA, Kahn SR. Postthrombotic syndrome in relation to vena cava filter placement: a systematic review. J Vasc Interv Radiol. 2008 Jul. 19 (7):981-985. [QxMD MEDLINE Link].
Chee YL, Culligan DJ, Watson HG. Inferior vena cava malformation as a risk factor for deep venous thrombosis in the young. Br J Haematol. 2001 Sep. 114 (4):878-80. [QxMD MEDLINE Link].
Gayer G, Luboshitz J, Hertz M, Zissin R, Thaler M, Lubetsky A, et al. Congenital anomalies of the inferior vena cava revealed on CT in patients with deep vein thrombosis. AJR Am J Roentgenol. 2003 Mar. 180 (3):729-32. [QxMD MEDLINE Link].
Sitwala PS, Ladia VM, Brahmbhatt PB, Jain V, Bajaj K. Inferior vena cava anomaly: a risk for deep vein thrombosis. N Am J Med Sci. 2014 Nov. 6 (11):601-3. [QxMD MEDLINE Link].
Chuang 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 Apr. 47 (556):206-13. [QxMD MEDLINE Link].
Spentzouris G, Zandian A, Cesmebasi A, Kinsella CR, Muhleman M, Mirzayan N, et al. The clinical anatomy of the inferior vena cava: a review of common congenital anomalies and considerations for clinicians. Clin Anat. 2014 Nov. 27 (8):1234-43. [QxMD MEDLINE Link].
Truty MJ, Bower TC. Congenital anomalies of the inferior vena cava and left renal vein: implications during open abdominal aortic aneurysm reconstruction. Ann Vasc Surg. 2007 Mar. 21 (2):186-97. [QxMD MEDLINE Link].
Kraft C, Schuettfort G, Weil Y, Tirneci V, Kasper A, Haberichter B, et al. Thrombosis of the inferior vena cava and malignant disease. Thromb Res. 2014 Sep. 134 (3):668-73. [QxMD MEDLINE Link].
Takayama H, Kinouchi T, Meguro N, Maeda O, Saiki S, Kuroda M, et al. Renal vein thrombosis misdiagnosed as a renal cell carcinoma with a tumor thrombus in the inferior vena cava. Int J Urol. 1998 Jan. 5 (1):94-5. [QxMD MEDLINE Link].
Hartman DS, Hayes WS, Choyke PL, Tibbetts GP. From the archives of the AFIP. Leiomyosarcoma of the retroperitoneum and inferior vena cava: radiologic-pathologic correlation. Radiographics. 1992 Nov. 12 (6):1203-20. [QxMD MEDLINE Link].
Figueroa AJ, Stein JP, Lieskovsky G, Skinner DG. Adrenal cortical carcinoma associated with venous tumour thrombus extension. Br J Urol. 1997 Sep. 80 (3):397-400. [QxMD MEDLINE Link].
Gotoh A, Gohji K, Fujisawa M, Okada H, Arakawa S, Hanioka K, et al. Renal angiomyolipoma associated with inferior vena caval tumour thrombus. Br J Urol. 1998 May. 81 (5):773-4. [QxMD MEDLINE Link].
Paolillo V, Sicuro M, Nejrotti A, Rizzetto M, Casaccia M. Pulmonary embolism due to compression of the inferior vena cava by a hepatic hemangioma. Tex Heart Inst J. 1993. 20 (1):66-8. [QxMD MEDLINE Link]. [Full Text].
Simpson AH, Kilby JO. Inferior vena cava thrombosis following a cycle ride. J R Soc Med. 1988 Dec. 81 (12):738-9. [QxMD MEDLINE Link]. [Full Text].
Flynn P, Zammit-Maempel I. Case report: computed tomography demonstration of inferior vena caval thrombosis and incorporation into an abdominal aortic aneurysm. Clin Radiol. 1998 Apr. 53 (4):306-7. [QxMD MEDLINE Link].
O'Sullivan DA, Torres VE, Heit JA, Liggett S, King BF. Compression of the inferior vena cava by right renal cysts: an unusual cause of IVC and/or iliofemoral thrombosis with pulmonary embolism in autosomal dominant polycystic kidney disease. Clin Nephrol. 1998 May. 49 (5):332-4. [QxMD MEDLINE Link].
Peillon C, Manouvrier JL, Testart J. Inferior vena cava thrombosis secondary to chronic pancreatitis with pseudocyst. Am J Gastroenterol. 1991 Dec. 86 (12):1854-6. [QxMD MEDLINE Link].
Jones AL, Ojar D, Redhead D, Proudfoot AT. Case report: Use of an IVC filter in the management of IVC thrombosis occurring as a complication of acute pancreatitis. Clin Radiol. 1998 Jun. 53 (6):462-4. [QxMD MEDLINE Link].
Stringer MD, Michell M, McIrvine AJ. Inferior vena caval thrombosis complicating acute pancreatitis. Case report. Acta Chir Scand. 1988 Feb. 154 (2):161-3. [QxMD MEDLINE Link].
Campbell DN, Liechty RD, Rutherford RB. Traumatic thrombosis of the inferior vena cava. J Trauma. 1981 May. 21 (5):413-5. [QxMD MEDLINE Link].
Nagy KK, Duarte B. Post-traumatic inferior vena caval thrombosis: case report. J Trauma. 1990 Feb. 30 (2):218-21. [QxMD MEDLINE Link].
Takeuchi M, Maruyama K, Nakamura M, Chikusa H, Yoshida T, Muneyuki M, et al. Posttraumatic inferior vena caval thrombosis: case report and review of the literature. J Trauma. 1995 Sep. 39 (3):605-8. [QxMD MEDLINE Link].
Peck KE, Bonoan JT, Cunha BA. Postlaparoscopic traumatic inferior vena caval thrombosis. Heart Lung. 1998 Jul-Aug. 27 (4):279-82. [QxMD MEDLINE Link].
ANDERSON RC, ADAMS P Jr, BURKE B. Anomalous inferior vena cava with azygos continuation (infrahepatic interruption of the inferior vena cava). Report of 15 new cases. J Pediatr. 1961 Sep. 59:370-83. [QxMD MEDLINE Link].
Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics. 2000 May-Jun. 20 (3):639-52. [QxMD MEDLINE Link].
Ramanathan T, Hughes TM, Richardson AJ. Perinatal inferior vena cava thrombosis and absence of the infrarenal inferior vena cava. J Vasc Surg. 2001 May. 33 (5):1097-9. [QxMD MEDLINE Link].
Van Veen J, Hampton KK, Makris M. Kilt syndrome?. Br J Haematol. 2002 Sep. 118 (4):1199-200. [QxMD MEDLINE Link].
Gayer G, Zissin R, Strauss S, Hertz M. IVC anomalies and right renal aplasia detected on CT: a possible link?. Abdom Imaging. 2003 May-Jun. 28 (3):395-9. [QxMD MEDLINE Link].
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 Jan. 23 (1):141-6. [QxMD MEDLINE Link].
White RH. The epidemiology of venous thromboembolism. Circulation. 2003 Jun 17. 107 (23 Suppl 1):I4-8. [QxMD MEDLINE Link].
Ruggeri M, Tosetto A, Castaman G, Rodeghiero F. Congenital absence of the inferior vena cava: a rare risk factor for idiopathic deep-vein thrombosis. Lancet. 2001 Feb 10. 357 (9254):441. [QxMD MEDLINE Link].
Perhoniemi V, Salmenkivi K, Vorne M. Venous haemodynamics in the legs after ligation of the inferior vena cava. Acta Chir Scand. 1986 Jan. 152:23-7. [QxMD MEDLINE Link].
Koc Z, Oguzkurt L. Interruption or congenital stenosis of the inferior vena cava: prevalence, imaging, and clinical findings. Eur J Radiol. 2007 May. 62 (2):257-66. [QxMD MEDLINE Link].
Ueda J, Hara K, Kobayashi Y, Ohue S, Uchida H. Anomaly of the inferior vena cava observed by CT. Comput Radiol. 1983 May-Jun. 7 (3):145-54. [QxMD MEDLINE Link].
Tsuji Y, Inoue T, Murakami H, Hino Y, Matsuda H, Okita Y. Deep vein thrombosis caused by congenial interruption of the inferior vena cava--a case report. Angiology. 2001 Oct. 52 (10):721-5. [QxMD MEDLINE Link].
Giordano P, Weber K, Davis M, Carter E. Acute thrombosis of the inferior vena cava. Am J Emerg Med. 2006 Sep. 24 (5):640-2. [QxMD MEDLINE Link].
Cho BC, Choi HJ, Kang SM, Chang J, Lee SM, Yang DG, et al. Congenital absence of inferior vena cava as a rare cause of pulmonary thromboembolism. Yonsei Med J. 2004 Oct 31. 45 (5):947-51. [QxMD MEDLINE Link].
D'Aloia A, Faggiano P, Fiorina C, Vizzardi E, Bontempi L, Grazioli L, et al. Absence of inferior vena cava as a rare cause of deep venous thrombosis complicated by liver and lung embolism. Int J Cardiol. 2003 Apr. 88 (2-3):327-9. [QxMD MEDLINE Link].
Takehara N, Hasebe N, Enomoto S, Takeuchi T, Takahashi F, Ota T, et al. Multiple and recurrent systemic thrombotic events associated with congenital anomaly of inferior vena cava. J Thromb Thrombolysis. 2005 Apr. 19 (2):101-3. [QxMD MEDLINE Link].
Iqbal J, Nagaraju E. Congenital absence of inferior vena cava and thrombosis: a case report. J Med Case Rep. 2008 Feb 12. 2:46. [QxMD MEDLINE Link]. [Full Text].
Lei F, Li X, Qian A, Yu X, Rong J, Sang H, et al. [Therapy of catheter-directed thrombolysis for inferior vena cava thrombosis after filter implantation]. Zhonghua Yi Xue Za Zhi. 2014 Jul 22. 94 (28):2197-200. [QxMD MEDLINE Link].

Media Gallery

Virchow triad/venous thromboembolism (VTE) risk factors.
Photo showing dilated superficial abdominal veins (upper quadrant), with bruising and thrombosed large abdominal veins (lower quadrant).
Abdominal CT scan shows absent inferior vena cava with thrombosis of very prominent collateral veins in the abdominal wall, corresponding to right side of abdomen as seen in earlier photo.
Veins of abdomen and thorax. Unless stated otherwise, lithograph plate is from Gray's Anatomy (online edition of the 20th US edition of Gray's Anatomy of the Human Body, originally published in 1918).

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Author

Luis G Fernández, MD, KHS, KCOEG, FACS, FASAS, FCCP, FCCM, FICS Professor of Surgery, Department of Surgery, Division of Trauma Surgery/Surgical Critical Care, University of Texas Health Science Center; Clinical Assistant Professor of Surgery, Department of Surgery, University of Texas Medical Branch; Adjunct Clinical Professor of Medicine and Nursing, University of Texas; Adjunct Clinical Assistant Professor, Department of Medical Education Health Science Center, Adjunct Clinical Assistant Professor, Department of Physician Assistant Studies, School of Health Professions, University of North Texas; Medical Director, Trauma Wound Care, UT Health East; Member, ACS National Committee on Trauma; Vice Chairman, State Guard Association of the United States (SGAUS) Medical Academy; Commander Emeritus, Texas Commandery, MOFW; Brigadier General (Ret/HR), Past Commanding General, TXSG Medical Brigade/Medical Rangers

Luis G Fernández, MD, KHS, KCOEG, FACS, FASAS, FCCP, FCCM, FICS is a member of the following medical societies: American Association for the Surgery of Trauma, American College of Chest Physicians, American College of Legal Medicine, American College of Surgeons, American Society of Abdominal Surgeons, American Society of General Surgeons, American Society of Law, Medicine & Ethics, American Trauma Society, Association for Surgical Education, Association of Military Surgeons of the US, Chicago Medical Society, Illinois State Medical Society, International College of Surgeons, New York Academy of Sciences, Pan-American Trauma Society, Society of Critical Care Medicine, Society of Laparoendoscopic Surgeons, Southeastern Surgical Congress, Texas Medical Association, Undersea and Hyperbaric Medical Society

Disclosure: Received honoraria from KCI for speaking and teaching; Received honoraria from PACIRA for speaking and teaching; Speaker / teaching honoraria for URGO -Stedmed North America.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

John Geibel, MD, MSc, DSc, AGAF Vice Chair and Professor, Department of Surgery, Section of Gastrointestinal Medicine, Professor, Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director of Surgical Research, Department of Surgery, Yale-New Haven Hospital; American Gastroenterological Association Fellow; Fellow of the Royal Society of Medicine

John Geibel, MD, MSc, DSc, AGAF is a member of the following medical societies: American Gastroenterological Association, American Physiological Society, American Society of Nephrology, Association for Academic Surgery, International Society of Nephrology, New York Academy of Sciences, Society for Surgery of the Alimentary Tract

Disclosure: Nothing to disclose.

Acknowledgements

Douglas M Geehan, MD Associate Professor, Department of Surgery, University of Missouri-Kansas City School of Medicine

Disclosure: Nothing to disclose.

Michael A Grosso, MD Consulting Staff, Department of Cardiothoracic Surgery, St Francis Hospital

Michael A Grosso, MD is a member of the following medical societies: American College of Surgeons, Society of Thoracic Surgeons, and Society of University Surgeons

Disclosure: Nothing to disclose.