Situs inversus with interrupted inferior vena cava is an uncommon anatomic
variant found in the abdominal and thoracic viscera. In this report, we present
a 59-year-old woman with this variation, found during gross anatomical dissection.
While this type of variation has been variable, in the present case the
hepatic veins drained directly into a very short (2.2 cm) inferior vena cava. The
infrarenal component of the inferior vena cava was present and drained into
the azygos and hemiazygos veins. Clinical considerations of this variant anatomy
are of interest, as they may present in patients as pathology on cross sectional
imaging

Loukas, M.

Shoja, M.M.

Strike, D.R.G.

Tarneja, N.

Tubbs, R.S.

English

Via Medica Journals

Folia Morphol. Vol. 68, No. 3, pp. 184–187Copyright © 2009 Via MedicaISSN 0015–5659www.fm.viamedica.plC A S E   R E P O R T184Address for correspondence: M. Loukas, MD, PhD, Professor, Department of Anatomical Sciences, St George’s University, School ofMedicine, Grenada, West Indies, tel: 473 444 4175 x2005, fax: 473 444 2887, e-mail: edsg2000@yahoo.com, mloukas@sgu.eduAn interrupted inferior vena cava ina situs inversus: a case report andreview of the literatureM. Loukas1, D.R.G. Strike1, R.S. Tubbs2, N. Tarneja1, M.M. Shoja31Department of Anatomical Sciences, School of Medicine, St George’s University, Grenada, West Indies2Section of Paediatric Neurosurgery, Children’s Hospital, Birmingham, AL, USA3Tuberculosis and Lung Disease Research Centre, Tabriz University of Medical Sciences, Tabriz, Iran[Received 9 March 2009; Accepted 11 May 2009]Situs inversus with interrupted inferior vena cava is an uncommon anatomicvariant found in the abdominal and thoracic viscera. In this report, we presenta 59-year-old woman with this variation, found during gross anatomical dis-section. While this type of variation has been variable, in the present case thehepatic veins drained directly into a very short (2.2 cm) inferior vena cava. Theinfrarenal component of the inferior vena cava was present and drained intothe azygos and hemiazygos veins. Clinical considerations of this variant anato-my are of interest, as they may present in patients as pathology on cross sec-tional imaging. (Folia Morphol 2009; 68, 3: 184–187)Key words: situs inversus, inferior vena cava, azygos vein,hemiazygos veinsINTRODUCTIONDuring early human development, totipotentcells migrate to desired locations. Once in their de-sired location they begin to divide and mature intoa terminally differentiate state, they then grow anddivide and eventually become tissues and organs.It has been postulated that signalling pathways arecritically important in the drive towards normal an-atomical positioning of organs. Asymmetric expres-sion of TGF-b, Homeobox genes, Sonic hedgehogand Fibroblast growth factor have all demonstrat-ed their importance in normal left-right develop-ment [2]. It is postulated that when improper sig-nalling occurs the result is some level of visceralabnormality, such as situs inversus. The normal po-sitioning of the major organs, i.e. the liver, spleen,stomach, and heart, are referred to as situs solitus.Additionally, the apex of the heart must be definedin which the possible positioning is to the right,left, or centre and is referred to as dextrocardia,levocardia, and mesocardia, respectively. Situs in-versus is a congenital abnormality that occurs inapproximately 0.01% of the population [5]. It re-sults in abnormal folding of organs and ultimatelycauses the major visceral organs to be the mirrorimage of their normal positions, including a rightsided spleen, stomach, and the apex of the heartpointed to the right (dextrocardia). Situs inversuswith levocardia has also been described; the resultis abnormal positioning of all major organs withthe exception of the heart, which remains in itsnormal position with the apex to the left. This ab-normality occurs in 1/22,000 of the normal popu-lation, although it occurs more frequently in pa-tients with congenital cardiac abnormalities, 0.4––1.2/100 [6]. An additional anomaly is situs ambi-guous, or heterotaxy syndrome. In heterotaxy syn-drome there is neither a normal arrangement of185M. Loukas et al., Situs inversusthe internal organs nor a mirroring of the normalarrangement. It is considered present when themajor internal organs have no clear lateralization,and could be considered a combination of bothsitus inversus and situs solitus [9]. Heterotaxy syn-drome is typically linked with cardiac malforma-tions, and often occurs in association with otherpathologies, including polysplenia, asplenia, andcardiac malformations [5]. The inferior vena cava(IVC) may be absent with azygos continuation orduplicated in any of the aforementioned abnor-malities. With the exception of situs solitus, theseterms describe anomalous formation of the vis-ceral organs, which may or may not include dex-trocardia and/or vascular abnormalities [10]. Thecomplex development of IVC is a result of the pos-terior cardinal, subcardinal and supracardinalveins, of which components of each are believedto regress and eventually form IVC [17]. However,if the supracardinal channel on the left and rightFigure 2. This is a schematic representation of this variation.Figure 1. A total interruption of the single left-sided inferior venacava (IVC) superior to the hepatic veins. Notice the right and leftcommon iliac veins ascending to form a right IVC at the level ofthe right renal vein. At this level, the IVC split into two vesselsand finally drained into dilated azygos and hemiazygos veins.Notice also the multiple spleens which are present as nineisolated smaller spleens characteristic of polysplenia syndrome.side persists, a double IVC will remain, which isestimated to occur in ~0.03% of individuals [4, 7].We report a case of situs inversus with double IVCdiscovered upon gross dissection.CASE REPORTWe present a case of situs inversus with inter-rupted IVC in a 59-year-old female cadaver (Figs. 1, 2).The case was discovered during a routine anatomi-cal dissection of the thorax and abdomen at theUniversity of Alabama in Birmingham during 2008.The cadaver did not show any other gross abnor-malities or evidence of procedures involving the tho-rax and the abdomen.The dissection revealed a double IVC; one onthe left side superior to the hepatic veins and oneon the right draining to the azygos vein. The he-patic veins were draining into a short IVC (2.2 cmin length) and then into the right atrium. The rightand left common iliac veins ascended and joinedto form a right inferior vena cava at the level ofthe right renal vein. At this level, the IVC split intotwo vessels and finally drained into dilated azy-gos and hemiazygos veins. The superior vena cava186Folia Morphol., 2009, Vol. 68, No. 3is derived from two sources, the right suprasubcar-dinal and postsubcardinal anastomosis, and finallythe infrarenal segment is derived from the right su-pracardinal vein [1].It has been reported that vascular abnormalitiesof the IVC in association with situs inversus are ex-tremely rare [14]. IVC duplications occur mainly in-ferior to the kidneys and have and incidence of 0.3––3% [16]. While it has been reported that witha left-sided IVC, which has an incidence rate of 0.2–0.5%,the statistics are as follows: a retro-aortal renal veinoccurs in 1–4% of cases, and a circumaortic venousring occurs in 1.5–16% of cases [16]. An additionalvisceral abnormality commonly seen in cases of in-testinal malrotation, IVC agenesis, and situs inver-sus is polysplenia, which is associated with impro-per lateralization and/or tissue segmentation of thespleen in embryonic development [14]. Furthermore,it has been demonstrated that different visceral ab-normalities are associated with asplenia and polys-plenia with situs inversus. Cardiac defects are morecommonly seen with asplenia while vascular abnor-malities are seen in polysplenia [14]. In a study pre-sented by Fulcher and Turner [5], in which 19 adultcases with situs anomalies were examined, 7 caseshad multiple spleens and 7 cases had an interrup-tion of the IVC with azygos or hemiazygos continu-ation. In a different study by Mayo and Rice [13],7/76 patients with situs inversus had abnormalitiesof the biliary tract or gallbladder, while in the studyof Fulcher et al. [5], 10 cases presented with chole-cystitis. The failure of the aforementioned organsand vasculature to attain their normal anatomicallocation could be the result of aberrant signallingpathways [2]; this should be a point of further workto elucidate the mechanism involved. The findingsin our cadaver support the association of vascularabnormalities with situs inversus.It is clinically important to be able to identifypatients with vascular abnormalities such as an in-terrupted IVC in cases of situs inversus. The preciselocation of the abnormal vasculature is required inorder to prevent any unnecessary injury to the pa-tient or damage to the organs. These variations havebeen examined in particular for surgical proceduressuch as aortic bifurcational replacements, inferiorcaval shunt, and portal caval shunts [15]. In addi-tion, an interrupted IVC can pose problems duringretroperitoneal and thoracic surgeries where thereis an issue of venous haemorrhage, while also com-plicating abdominal aortic aneurysm surgeries [3].Reports have described the requirement of a secondand its tributaries were normal. The spleen waspresent to the left as nine isolated small spleenscharacteristic of polysplenia syndrome. The splenicvein was formed from tributaries of all nine splenicsegments to drain in the portal system. The restof the organs appeared to be normal during grossexamination, and the rest of the cadaver was un-remarkable.DISCUSSIONThe literature demonstrates a clear associationbetween situs inversus and other congenital condi-tions such as tetralogy of Fallot, Kartagener syn-drome, duodenal atresia biliary atresia, and gas-troschisis [11, 12]. Since this information can beuseful for diagnosticians and surgeons, situs inver-sus is a clinically relevant anomaly, which should bereported as often as it is observed.A review of the embryogenesis is helpful for anunderstanding of the etiology of the venous ano-malies. The IVC develops between the 6th and 8th weeksof gestation, as a structure comprised of three pairedembryonic veins: the posterior cardinal, subcardinal,and the supracardinal veins. There is a cyclical pro-cess of development and regression, which eventu-ally culminates into the permanent asymmetric IVC[16]. Briefly, the posterior cardinal veins return allof the blood from the body wall inferior to heart,while blood from the viscera is returned by the vi-telline veins. Eventually an intersubcardinal anasto-mosis forms between the paired subcardinal veinswhile an anastomosis forms between the subcardi-nal veins and the posterior cardinal veins. Duringthis step, the right subcardinal vein joins the hepa-tic segment of the IVC forming the vitelline vein. Even-tually the pre-renal division of the IVC is formed bythe progressive degeneration of the posterior cardi-nal veins, therefore shunting blood through the he-patic segment of the IVC [1]. Next, there is forma-tion of the paired supracardinal veins, which even-tually separate into the superior and inferior por-tion of the azygos. The posterior cardinal veins con-tinue to atrophy, and blood from the lower bodypasses through the suprasubcardinal anastomosisand onto the prerenal IVC, while blood from the leftbody is sent across the interpostcardinal anastomo-sis [1]. In general, the normal IVC can be segmentedinto four main components; suprarenal, renal, in-frarenal, and hepatic. Each portion develops froma distinct source. The right subcardinal vein devel-ops into the suprarenal portion while the vitelline veingives rise to the hepatic portion. The renal segment187M. Loukas et al., Situs inversusGreenfield filter to be implanted when a previouslyundetected double IVC was recognized in order toprotect against pulmonary embolus [1]. However, incases in which an IVC filter is required, it has beenreported that it is very difficult to identify left IVC andinterrupted IVC with azygos continuation on veno-graphic examination, especially with situs inversus. Inthis case, computed tomography or ultra sound shouldbe used for proper identification and diagnosis [14].However, caution should still be used when usingimaging techniques, it has been reported that a dou-ble IVC may be incorrectly identified as a lymphaden-opathy [7, 8], which is of particular importance in can-cer patients who are being monitored for metastasis.Other potential complications can occur during remov-al of renal cell carcinoma, lumbar sympathectomy, ure-teral surgery, and finally in the dissection of lymphnodes because the lymph drainage flow follows thecourse of the vasculature [3]. There have been veryfew reports of double IVC in association with Situsinversus. Kulesza et al. [10] previously described whatwas believed to be the first case.CONCLUSIONSWe have demonstrated a patient with situs in-versus, polysplenia, and a total interruption of thesingle left-sided IVC superior to the hepatic veins.The abnormal vasculature is to be expected withpolysplenia [14].REFERENCES1. 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: 639–652.2. Casey B, Hackett B (2000) Left-right axis malformationsin man and mouse. Cur Opin Gen Dev, 10: 257–261.3. Dougherty MJ, Calligaro KD, DeLaurentis DA (1996)Congenitally absent inferior vena cava presenting inadulthood with venous stasis and ulceration: a surgi-cally treated case. J Vasc Surg, 23: 141–146.4. Friedland GW, deVries PA, Nino-Murcia M, King BF,Leder RA, Stevens S (1992) Congenital anomalies ofthe inferior vena cava: Embryogenesis and MR features.Urol Radiol, 13:237–248.5. Fulcher A, Turner M (2002) Abdominal manifestationsof situs anomalies in adults. Radiographics, 22: 1439––1456.6. Gindes L, Hegesh J, Barkai G, Jacobson JM, Achiron R(2007) Isolated levocardia prenatal diagnosis, clinicalImportance and literature review. J Ultrasound Med,26: 361–365.7. Giordano JM, Trout HH (1986) Anomalies of the inferiorcava. J Vasc Surg, 3: 924–928.8. Hunter JD, Callaway MP, Wakely CJ (1998) Double andnothing. Br J Radiol, 71: 99–100.9. Jacobs JP, Anderson RH, Weinberg PM, Walters HL 3rd,Tchervenkov CI, Del Duca D, Franklin RC, Aiello VD,Béland MJ, Colan SD, Gaynor JW, Krogmann ON,Kurosawa H, Maruszewski B, Stellin G, Elliott MJ (2007)The nomenclature, definition and classification of car-diac structures in the setting of heterotaxy. CardiolYoung, 17: 1–28.10. Kulesza RJ, Kalmey B, Dudas B, Buck WR (2007) Vascu-lar anomalies in a case of situs inversus. Folia Morphol,66: 69–73.11. Lee SE, KimWK, Jung SE, Lee SC, Park KW, Kim WK(2006) Situs anomalies and gastrointestinal abnorma-lities. J Pediatr Surg, 41: 1237–1242.12. Marta MJ, Falcão LM, Saavedra JA, Ravara L (2003)A case of complete situs inversus. Rev Port Cardiol, 22:91–104.13. Mayo CW, Rice RG (1949) Situs inversus totalis: a sta-tistical review of data on seventy six cases with specialreference to disease of the biliary tract. Arch Surg, 58:724–730.14. Mihmanli I, Bulakbasi N, Kantarci F, Adaletli I, Pabuscu Y(2001) The value of ultrasonography in interruptedinferior vena cava with azygos continuation. Eur JUltrasound, 14: 179–182.15. Milloy F, Anson BJ, Cauldwell EW (1962) Variations inthe inferior caval veins and in their renal and lumbarcommunications. Surg Gynecol Obstet, 115: 2–10.16. Meyer R, Hüppe T, Andresen R, Friedrich M (1998)Intra- and infrahepatic agenesis of the inferior vena cavawith azygos continuation accompanied by duplicationof the postrenal segment. Inrvest Radiol, 33: 113–116.17. Oguzkurt L, Ozkan U, Tercan F, Koc Z (2007) Catheter--directed thrombolysis of acute deep vein thrombosis inthe lower extremity of a child with interrupted inferiorvena cava. Cardiovasc Intervent Radiol, 30: 332–334.

An interrupted inferior vena cava in a situs inversus: a case report and review of the literature

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An interrupted inferior vena cava in a situs inversus: a case report and review of the literature

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