The inferior vena cava, also known as the posterior vena cava, is the large vein
that carries de-oxygenated blood from the lower half of the body into the right
atrium of the heart. Congenital anomalies of the inferior vena cava result from
the persistence of the embryonic venous system. The majority of cases are
clinically silent and are diagnosed in routine dissection studies, in retroperitoneal
surgeries, or through imaging for other reasons. Although these anomalies
are rare, they are of great importance during operations in the abdominal
area or in the treatment of thromboembolic diseases. We report two cases of
double vena cava and left vena cava, respectively, and a short review of the
relevant literature. (Folia Morphol 2010; 69, 3: 123-127

Dimitrakopoulou, G.

Fiska, A.

Galani, P.

Kapetanakis, S.

Papadopoulos, C.

English

Via Medica Journals

Folia Morphol. Vol. 69, No. 3, pp. 123–127Copyright © 2010 Via MedicaISSN 0015–5659www.fm.viamedica.plR E V I E W   A R T I C L E123Address for correspondence: S. Kapetanakis, Orthopaedic Surgeon, MD, PhD, Anatomist Department of Anatomy, Medical Schoolof Alexandroupolis, tel: 00306972707384, fax: 00302106096722, e-mail: kastegepe@yahoo.grAnomalies of the inferior vena cava:a report of two cases and a short reviewof the literatureS. Kapetanakis1, C. Papadopoulos1, P. Galani2, G. Dimitrakopoulou2, A. Fiska11Department of Anatomy, Medical School of Alexandroupolis, Greece2Department of Radiology, General Hospital Amalia Fleming, Athens, Greece[Received 13 May 2010; Accepted 15 June 2010]The inferior vena cava, also known as the posterior vena cava, is the large veinthat carries de-oxygenated blood from the lower half of the body into the rightatrium of the heart. Congenital anomalies of the inferior vena cava result fromthe persistence of the embryonic venous system. The majority of cases areclinically silent and are diagnosed in routine dissection studies, in retroperito-neal surgeries, or through imaging for other reasons. Although these anoma-lies are rare, they are of great importance during operations in the abdominalarea or in the treatment of thromboembolic diseases. We report two cases ofdouble vena cava and left vena cava, respectively, and a short review of therelevant literature. (Folia Morphol 2010; 69, 3: 123–127)Key words: inferior vena cava, anomalies, double, leftINTRODUCTIONAnomalies of the inferior vena cava are rare andresult mainly from abnormal embryological deve-lopment. Among these, the most common are dou-ble inferior vena cava (IVC), with an incidence of 0.2––3% [2, 11], and left sided IVC with an incidence of0.69% [20]. They are mostly incidentally diagnosedon imaging of the area, usually with computed to-mography (CT scanning) or magnetic resonanceimaging (MRI) done for other reasons. However, itis important to recognise these anomalies as theycan have significant clinical implications, especiallyfor surgeons and for the treatment of thromboem-bolic diseases.In this particular study we present two rare cas-es. In the first case we report an 80 year-old manwith acute renal failure and urine infection, in whoma double vena cava was noted in routine CT ima-ging of the area. In the second case we reporta 66 year-old man with acute pain in the abdomen,in whom CT imaging showed a left-sided IVC. Finally,we discuss the clinical importance of recognising theanomalies of the IVC and we review the relevant li-terature.CASE 1A case of double IVC on both sides of the aortawas observed during routine imaging with CT scan-ning (Fig. 1) in an 80 year-old man with past medi-cal history of ischaemic heart disease, atrial fibrilla-tion, and cancer of the urine bladder which appeared4 months previously. The patient presented symp-toms of urine infection, haematuria, and total oede-ma of the body. From the laboratory tests we founddeterioration of the renal function/acute renal fail-ure and low levels of albumin in the blood. Therewere positive urine cultures for Klebsiella pneumo-nia > 105 and we started medication with amoxicil-lin and garamycin. We proceeded with CT scanningof the lower abdomen, due to the poor images of124Folia Morphol., 2010, Vol. 69, No. 3the ultrasonography, to examine the kidneys andthe urine bladder. During the imaging we inciden-tally observed a double IVC, which was on both sidesof the aorta. We were not able to carry out furtherexamination of the upper abdomen to see wherethese two venae cavae joined and formed one mainIVC because the patient presented with a high feverand died five days later.CASE 2A 66 year-old, previously healthy man present-ed to our hospital with acute pain in the abdomen.All laboratory tests and ultrasonography of the areawere negative for pathologic findings. Nevertheless,we proceeded with CT imaging of the abdomenbecause of the persistence of the symptoms. Thisexamination was also normal, so we pinned thesymptoms on a possible colic of the large intestine.Apart from other findings, the CT imaging inciden-tally showed two common iliac veins that joined atthe left side of the abdominal aorta (Fig. 2) andformed a left sided IVC (Fig. 3) that crossed anterio-rly to the right side at the level of the renal arteries(Fig. 4). The patient took the appropriate medica-tion with analgesics and went home with dietaryinstructions.DISCUSSIONThe IVC is formed by the right and left commoniliac veins on the right anterior surface of the fifthlumbar vertebra, 2.5 cm to the right of the medianplane, inferior to the bifurcation of the abdominalaorta, and brings blood to the right atrium of theheart from all parts of the body below the dia-phragm [10]. The IVC is right-sided in nearly 98% ofthe population [15]. The abdominal part of the IVCFigure 4. The left-sided inferior vena cava crosses anteriorly tothe aorta at the level of the renal arteries.Figure 1. Double inferior vena cava, bilateral of the aorta.Figure 2. Bifurcation of the left-sided inferior vena cava to thecommon iliac veins.Figure 3. Left-sided inferior vena cava.125S. Kapetanakis et al., Anomalies of the inferior vena cavais 20 cm long and ascends on the right side of thebodies of the lumbar vertebrae and to the right ofthe aorta. It enters the thorax through the caval fo-ramina in the central tendon of the diaphragm,which is at the level of the eighth thoracic vertebra.Unlike the superior vena cava, it has a substantialnumber of tributaries. It also anastomoses with theazygos vein system (which runs on the right side ofthe vertebral column). The tributaries include theveins that collect blood from the muscles and co-verings of the loins, and from the walls of the abdo-men, the reproductive organs, the kidneys, and theliver. The specific levels of the tributaries are as fol-lows: the common iliac vein is at L5, lumbar veinsare at the level between L1–L5, the gonadal vein isat L2, the renal and suprarenal veins are at L1, andfinally the hepatic and inferior phrenic veins are atT8. Because the IVC is not centrally located, thereare some asymmetries. The testicular (gonadal) veinsand suprarenal veins drain into the IVC on the rightside but into the renal vein on the left side, which inturn drains into the IVC. By contrast, all the lumbarveins and hepatic veins usually drain directly intothe IVC.The IVC develops in a complex process which be-gins on the fourth week of gestation and is com-pleted approximately on the eighth week [21]. Itinvolves the development, regression, anastomosis,and replacement of three pairs of embryonic veins(posterior cardinal, subcardinal, and supracardinalveins) [4]. The posterior cardinal veins appear firstand regress, except for the distal aspects which be-come the iliac bifurcation. The subcardinal veinsappear on the fifth week. They form the stem of theleft renal vein, the suprarenal (adrenal) veins, andthe prerenal segment of the IVC. The left subcardi-nal vein completely regresses. Subsequently, thesupracardinal veins develop. The left regresses andthe right forms the infrarenal IVC. The persistenceof the left supracardinal vein is the cause of most ofthe anomalies of the IVC.In this study we report two cases of anomaliesof the IVC. In the first case a double vena cava wasincidentally observed during imaging for other rea-sons. The IVC was situated bilaterally to the abdomi-nal aorta and, according to the embryology, thisresulted from the failure of regression of the leftsupracardinal vein. The occurrence of this variationof the IVC is about 0.2–3% in the population [11].The duplicated left IVC usually drains into the leftrenal vein that crosses anterior to the aorta and joinsthe IVC. Unfortunately, we could not run furtherimaging on our patient because of his bad medicalcondition and his eventual death. Thus, we can onlyassume the course of the duplicated IVC of our pa-tient.In the second case, a 66 year-old man presentedabdominal pain, and the imaging of the area withCT scanning showed a left-sided IVC. It is one of themost common variations of the IVC with a preva-lence of 0.69% [20]. A left sided IVC usually ascendsto the left side of the abdominal aorta that crossesanteriorly to the right side at the level of the renalarteries [9].Except for the anomalies described above, thereare many other mentioned. The four most commonanomalies that also have clinical importance are thedouble IVC (0.2–3%) [11, 15], the isolated left-sid-ed IVC (0.2–0.5%) [11, 15], the circumaortic left re-nal vein (1.5–8.7%), and the retro-aortic left renalvein (2.1%) [11]. The first two variations may be re-vealed by imaging with ultrasonography of the ab-domen [9]. All other rare anomalies are only observedon CT imaging [9] of the area or in dissection stu-dies and retroperitoneal surgeries. Rare anomaliesof the IVC include the absence of a part of the IVC,azygos, and hemiazygos continuation of a duplicat-ed IVC, double superior and double IVC, hypopla-sia, or agenesis and interruption of the IVC [21]. Inparticular, all anomalies and anatomic variations aredescribed in Table 1 where we report the relationbetween nationality and these anomalies. We shouldalso note that there is a similar incidence of theanomalies of the IVC in Asiatic populations (mostlyin India, China, and Japan) and in European and oth-er Western countries. Anomalies of the IVC are ob-served mostly in males at a percentage of 62%. Ad-ditionally, we came to the conclusion that interrupt-ed IVC is observed mostly in women, in a high per-centage, while aplasia/agenesis of the IVC is ob-served in males and particularly in European andother Western countries. This is a fact that is proba-bly due to gene mutations that appear in these po-pulations. Further research and understanding of themolecular mechanisms may help us predict suchanomalies in future in individuals and avoid manyof the mistakes made during operations in the ab-domen by surgeons who are unaware of the ana-tomic variations of the area.The majority of cases of anomalies of the IVCare diagnosed incidentally during imaging or sur-geries. Its occurrence is rare but has important clini-cal consequences. During retroperitoneal surgeries[1, 16] it is very useful to know the anatomy of the126Folia Morphol., 2010, Vol. 69, No. 3area and recognise the venous anomalies so as toreduce the hazards of significant venous haemor-rhage. Additionally, the surgeon must be aware ofsuch variations during organ transplantation [12],radical nephrectomy [1], sympathectomy, or urete-ral surgery [3, 17]. It could be fatal to mistakenlyinjure the IVC during an operation of the abdomen.Another implication of recognising the anomaliesof the IVC is in patients with thromboembolic di-seases in whom it is planned to place a filter in thevena cava. If there is an unrecognised double IVC,there may be reoccurrence of embolisms by throm-bus from the left IVC [13, 14]. A higher occurrenceof thromboembolic disease [7] and pulmonary em-bolism has also been observed in patients with ano-malies of the IVC [9]. Radiologically, the presence ofIVC anomalies such as a double IVC can be mistakenfor lymphadenopathy [6, 8, 11] and metastatic di-sease of the lymph nodes in cases of testicular carci-noma [18] or as a retroperitoneal mass [5, 16]. Thus,Table 1. Major anomalies of the inferior vena cava (IVC) and the relation with the populations in Asiatic and Westerncountries from a short review of literatureA/A Anomalies Populations No. of cases1 Double IVC 23 Asiatic, 26 Western 49 cases2 Transposition (left IVC) 8 Asiatic, 11 Western 19 cases3 Circumaortic  renal vein 1 Asiatic 1 case4 Retro-aortic (left) renal vein 1 Asiatic, 1 Western 2 cases5 Absence of the hepatic portion of the IVC 1 Western 1 case6 Right double IVC with azygos continuation of the posterior medial vein 1 Western 1 case7 Double IVC with hemiazygos and azygos continuation of the left VC 1 Western 1 case8 Azygos continuation of the IVC 5 Western 5 cases9 Double IVC with recurrent right renal vein and hemiazygos continuation of the IVC 1 Western 1 case10 Double IVC with retro-aortic left renal vein and azygos continuation of the IVC 1 Western 1 case11 Absent infrarenal IVC with preservation of the suprarenal segment 1 Asiatic, 4 Western 5 cases12 Duplication anomaly of the IVC with normal drainage of the right IVC and 1 Western 1 casehemiazygos continuation of the left13 Double superior and double IVC 1 Asiatic, 1 Western 2 cases14 Double right VC forming a periureteric venous ring 1 Asian, 2 Western 3 cases15 Agenesis/aplasia of the IVC 9 Asiatic, 21 Western 30 cases16 Interrupted IVC 5 Asiatic, 9 Western 14 cases17 Double IVC with circumaortic renal vein 1 Asiatic 1 case18 Interrupted IVC with azygos continuation 5 Asiatic, 3 Western 8 cases19 Hypoplasy of the IVC 1 Asiatic, 3 Western 4 cases20 Anterior IVC 1 Western 1 casewhen an anomaly is suspected on CT or MRI, it isnecessary to perform an inferior venogram in orderto outline precisely the vena cava system [3, 19].CONCLUSIONSIn conclusion, although the anatomical variationsof the IVC are rare, they should be recognised byradiologists and surgeons in order to avoid mistakesduring imaging of the area or surgeries and in caseswith venous thromboembolic disease. Consequent-ly, these anomalies would not be interpreted aspathological findings and the best treatment wouldbe possible for each patient.ACKNOWLEDGEMENTSThis study has been carried out in collabora-tion with the Department of Radiology in AmaliaFleming in Athens and the Department of Anato-my in the University Hospital of Alexandroupolis,Greece.127S. Kapetanakis et al., Anomalies of the inferior vena cavaREFERENCES1. Babaian RJ, Johnson DE (1979) Major venous anoma-lies complicating retroperitoneal surgery. South Med J,72: 1254–1258.2. Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr(2000) Spectrum of congenital anomalies of the infe-rior vena cava: cross-sectional imaging findings. Radio-graphics, 20: 639–6523. Berkow AE, Henkin RE (1978) Double IVC or iliac veinocclusion? A diagnostic problem in radionuclide veno-grams. Am J Roentgenol, 130: 529–531.4. Esposito S, Mansueto G, Amodio F, Meccariello R,Galasso L, Salzano A, Vallone G (1999) Duplication ofthe vena cava inferior with a continuation into the venaazygos. A report of a rare case. Minerva Chir, 54: 261–265.5. Evans JC, Earis J, Curtis J (2001) Thrombosed doubleIVC mimicking para-aortic lymphadenopathy. Br J Ra-diol, 74: 192–194.6. Faer MJ, Lynch RD, Evans HO, Chin FK (1979) Inferiorvena cava duplication: demonstration by computedtomography. Radiology, 130: 707–709.7. Gayer G, Luboshitz J, Hertz M, Zissin R, Thaler M,Lubetsky A, Bass A, Korat A, Apter S (2003) Congenitalanomalies of the inferior vena cava revealed on CT inpatients with deep vein thrombosis Am J Roentgenol,180: 729–732.8. Klimberg I, Wajsman Z (1986) Duplicated inferior venacava simulating retroperitoneal lymphadenopathy ina patient with embryonal cell carcinoma of the testicle.J Urol, 136: 678–679.9. Kouroukis Ch, Leclerc JR (1996) Pulmonary embolismwith duplicated inferior vena cava. Chest, 109: 1111–1113.10. Kumar S (2006) An anomaly of inferior vena cava:a rare case report. Kathmandu University Med J, 4: 253––255.11. Ng WT, Ng SS (2009) Double inferior vena cava: a re-port of three cases. Singapore Med J, 50: e211–e213.12. Palit S, Deb S (2002) A rare presentation of double in-ferior vena cava with anomalous pattern of azygos andhemiazygos venous systems: a case report. J Anat SocIndia, 51: 65–67.13. Reinus WR, Gutierrez FR (1986) Duplication of the in-ferior vena cava in thromboembolic disease. Chest, 90:916–918.14. Saito H, Sano N, Kaneda I, Arakawa M, Ishida S, Taka-hashi S, Sakamoto K (1995) Multisegmental anomalyof the IVC with thrombosis of the left IVC. CardiovascIntervent Radiol, 18: 410–413.15. Satheesan B, Suresh Kumar D, Kathiresan N (2009) Dou-ble inferior vena cava in a patient with bilateral testi-cular tumor: a case report with review of literature.Urology Ann, 1: 27–29.16. Shaw MB, Cutress M, Papavassiliou V, White S, Thomp-son M, Sayers R (2003) Duplicated inferior vena cavaand crossed renal ectopia with abdominal aortic aneu-rysm: preoperative anatomic studies facilitate surgery.Clin Anat, 16: 355–357.17. Shingleton WB, Hutton M, Resnick MI (1994) Duplica-tion of inferior vena cava: its importance in retroperi-toneal surgery. Urology, 43: 113–115.18. Tatar I, Tore HG, Celik HH, Karcaaltincaba M (2005)Magnetic resonance venography of double inferior venacava. Saudi Med J, 26: 101–103.19. Tisnado J, Amendola MA, Vines FS, Beachley MC (1979)Computed tomography of a double IVC: the “doublecava” sign. Comput Tomogr, 3: 195–199.20. Tohno Y, Tohno S, Azuma Ch, Moriwake Y, Nagai R,Nakaatsuji Y, Nakayama T, Nishi R (2006) One case ofthe double inferior venae cavae found in a Japaneseman. J Nara Med Assoc, 57; 6:.193–197.21. Tore HG, Tatar I, Celik HH, Oto A, Aldur MM, Denk CC(2005) Two cases of inferior vena cava duplication withtheir CT findings and a review of the literature. FoliaMorphol, 64: 55–58.

Anomalies of the inferior vena cava: a report of two cases and a short review of the literature

https://journals.viamedica.pl/folia_morphologica/article/download/15865/12503

Anomalies of the inferior vena cava: a report of two cases and a short review of the literature

Abstract

Similar works

Full text

Available Versions

Via Medica Journals