Journal of Vascular Surgery
Volume 48, Issue 1 , Pages 218-222, July 2008

Thrombosed iliac venous aneurysm: A rare form of presentation of a congenital anomaly of the inferior vena cava

  • August Ysa, MD

      Affiliations

    • Vascular Surgery Department, Hospital de Cruces, Barakaldo, Spain
    • Corresponding Author InformationCorrespondence: August Ysa, MD, Vascular Surgery Department, Hospital de Cruces. Pza de Cruces s/n Barakaldo, 48903 Spain.
    • ,
  • Maite R. Bustabad, MD

      Affiliations

    • Vascular Surgery Department, Hospital de Cruces, Barakaldo, Spain
    • ,
  • Amaia Arruabarrena, MD

      Affiliations

    • Vascular Surgery Department, Hospital de Cruces, Barakaldo, Spain
    • ,
  • Eduardo Pérez, MD

      Affiliations

    • Vascular Surgery Department, Hospital de Cruces, Barakaldo, Spain
    • ,
  • Estepan Gainza, MD

      Affiliations

    • Radiology Department, Hospital de Cruces, Barakaldo, Spain.
    • ,
  • Juan Antonio García Alonso, MD

      Affiliations

    • Vascular Surgery Department, Hospital de Cruces, Barakaldo, Spain

Received 7 January 2008; accepted 5 February 2008.

Article Outline

Aneurysms of the iliac veins are extremely rare. We report a case of a 51-year-old male who was admitted for swelling of the lower right limb. Sonography and ascending phlebogram showed a complete occlusion of the right femoropopliteal veins, both iliacs and the inferior vena cava (IVC). A large collateral circulation throughout the paravertebral plexus and azygos system was also observed. The CT scan revealed a 5 × 9cm thrombosed aneurysm of the right external iliac vein and a congenital hypoplasia of the infrarenal IVC. Anticoagulant treatment and compression with elastic stocking was started. The 3-month follow-up showed mild residual edema of the right lower limb. The literature on this pathology is extensively reviewed.

 

The iliac vein is the least frequently reported location for aneurysms of the venous system. We present an unusual case of an iliac aneurysm secondary to a congenital anomaly in the drainage of the inferior vena cava (IVC). The literature on this pathology is extensively reviewed and analyzed.

Back to Article Outline

Case report 

A 51-year-old male patient presented with fever, pain, and edema of the lower right limb for the last 3 days. The patient did not have any past medical or surgical history of interest. Additionally, history of varicose veins or trauma and presence of respiratory symptoms suggestive of pulmonary thromboembolism (PTE) were denied. On physical examination, a global edema of the right lower limb with severe cyanosis was notable. Distal pulses were present. The rest of the examination was normal except for moderate varicocele.

A venous Doppler ultrasound of the lower limbs followed by an ascending phlebogram were performed, showing a complete occlusion of the right femoropopliteal veins, right external iliac, both common iliacs, and the IVC. A large collateral circulation throughout the paravertebral plexus and azygos system was observed (Fig 1).

An abdominal 64-slice angio-CT scan showed thrombosis of the IVC and both common iliac veins. A 5 × 9 cm aneurysm of the right external iliac vein and a 2.5 cm ectasia on the left iliac were observed. Additional finding included a hypoplasia of the infrarenal IVC at its connection to the suprarenal IVC (Fig 2) along with a large collateral circulation by the prevertebral plexus and azygos (Fig 3) No images suggestive of intra- or extravascular tumor or associated adenopathies were observed.

The lab results were normal, except for a slight increase in C-reactive protein and a D-dimer value of 15175 ng/ml (0-275). Markers of oncological, rheumatologic, or hypercoagulability disorders were negative. Serum protein electrophoresis and hormonal blood profiles were also normal. Anticoagulant treatment and compression with elastic stocking for both legs was started. The 3-month follow-up only showed mild residual edema of the right lower limb.

Back to Article Outline

Discussion 

Iliac venous aneurysms are extremely rarely observed abnormalities. Following a systematic search in MEDLINE [1850-Nov 2007], EMBASE [1980-Nov 2007], and OVID with an unrestricted search strategy and with exploded MeSH terms (iliac veins/iliac venous, hypogastric, inferior cava vein, and aneurysms), only 21 reported articles were retrieved.1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21

In 1964, Abbot22 classified these aneurysms as primary or secondary, according to their cause. The latter being considered a consequence of arteriovenous fistulas (AVF), proximal obstruction, or cardiovascular anomalies that increase the flow or pressure within the venous system.

In our review, the AVFs, commonly due to prior trauma, were the most common cause (41%). This figure could be underestimated accounting that Mansfeld's review included at least 14 cases of aneurysms secondary to AVF between 1867 and 1984.5

The drainage impairment due to congenital anomalies represents 9% of our review. Our own case consisted of an anomaly due to hypoplasia of the infrarrenal vena cava probably secondary to an atresia or failure in the embryological development of the junction between the hepatic segment and the right subcardial veins. The presence of a varicocele, a large collateral venous drainage throughout the prevertebral and azygos system, and the near complete flow obstruction of the IVC immediately below the renal veins support this diagnosis.

The iliac compression syndrome was clearly identified as the cause of the aneurysm secondary to flow obstruction in only one case.8 However, it is worth noting that in the overall analysis of the series, we found three cases corresponding to patients with high level of physical activity.8, 9, 15 Hurwitz suggests that an obstruction of the venous return associated with an increased flow during exercise can generate sufficient venous hypertension to eventually dilate the vein over the time. Also of note, is the presence of three pregnant women in the review.4, 13, 20 Uterine pressure, hyperperfusion, and hormonal changes related to gestation have been indicated as possible predisposing factors.4

Primary aneurysms are very infrequent with only seven reported cases.9, 14, 15, 17, 19, 20, 21

Finally, there is one case of venous aneurysm that developed after thermal lesion of the vein wall, which the author describes as the venous equivalent to an arterial pseudoaneurysm.16 The most frequent clinical presentation among the reviewed cases was venous thrombosis (41%), followed by chronic venous insufficiency (32%). In our case, the patient presented with unilateral edema in spite of having a bilateral iliac thrombosis. We believe that this difference is due to the absence of thrombosis and preservation of the left femoropopliteal territory. Additionally, the large intrapelvic collateral circulation could also minimize the symptoms due to a common left iliac vein thrombosis.

Other initial symptoms previously reported have been abdominal pain or mass, hip pain, and pulmonary thromboembolism (PTE). Only four patients were diagnosed with absence of symptoms. Rupture of the aneurysm has also been described as a possible form of presentation.23

The analysis of different subgroups revealed that most aneurysms secondary to posttraumatic AVF were associated with symptoms of long-term chronic venous stasis (7 of 8). Patients with flow obstruction (congenital or acquired) remained asymptomatic until the onset of a thrombosis of the venous system. This is probably explained by the development of a compensatory collateral circulation over time. Similarly, patients with primary aneurysms did not present with long-term history of symptoms.

The use of ultrasounds, phlebogram, or computed tomography (CT) scan/angio magnetic resonance imaging (MRI) helps making the diagnosis in many occasions. In our case, the 64-slice angio-CT provided the most reliable information for diagnosis, showing a congenital anomaly of the vena cava, presence of a right venous aneurysm, and a thrombotic left iliac system probably secondary to the retrograde progression of the infrarrenal vena cava thrombosis.

There appears to be a general agreement that abdominal and lower limb vein aneurysms should be corrected surgically once diagnosed, given their emboligenic potential, the possibility of compression of adjacent structures, or even rupture.24, 25 There is also agreement over the need to re-establish venous continuity whenever possible to prevent the appearance of post-thrombotic syndrome.20 When the flow is restored via bypass, a proximal AVF has also been used to prevent graft thrombosis.8

In our review, the surgical treatment was heterogeneous and frequently combined various techniques (Table I). Table II summarizes the treatments according to the cause of the aneurysm, the intrinsic characteristics of the group, and the permeability of the venous axis following the treatment. In the case of a diagnosed aneurysm following a symptomatic thrombosis, the latest recommendation seems to support medical treatment with anticoagulation and elastic compression measures.21

Table I. Review of the literature
Sex/ageSymptomsThromboembolic complicationLocationDiameter (cm)Related anomaliesTreatmentOutcomeFollow up
Cornet (1969)♂30Inflammatory injury and limb swellingNoneRight CIVOrange likePosttraumatic AVF below adductor hiatusUnsuccessful surgical reviewParetic post-thrombotic limb12 mo
♂50Abdominal massNoneLeft EIV20Posttraumatic AVF (SFA)AVF ligation+ vein ligation+ subsequent AR (6m)No complications3 mo
Raitherl (1972)♂48Intermittent claudication and swellingNoneLeft EIVNo dataPosttraumatic AVF (SFA)AVF ligationNo complications17 d
Vaccaro (1975)♂65Edema, leg ulcer and popliteal swellingNoneRight EIV25Posttraumatic AVF (POP)NONEDeath (stroke)2 mo
Parer (1984)♀23AsymptomaticNoneLeft EIV10Dialysis femoral-saphenous AV shuntAVF ligation+anticoagulationAneurysm diameter reduction6 wk
Mansfeld (1985)♂56Intermittent claudication and swellingDVT lower limbRight EIV15Posttraumatic AVF (SFA)AVF ligation + subsequent ARPTE + post-thrombotic limbno data
Valdes (1986)♂58Abdominal pain and constipationNoneLeft hypogastric vein10.6 × 8Congenital AVMEmbolization + ARAsymptomatic12 mo
Tisnado (1988)♂57Chronic venous stasisNoneRight EIV8 × 10Posttraumatic AVF (SFA)No dataNo dataNo data
Hurwitz (1989)♂69Painful limb swellingThrombosed aneurysmLeft CIV and EIV8.8 × 4.4Iliac compression syndromeAR with graft reconstruction+ AVF+ anticoagulationThrombolysis of the graft 6m, thereafter patent22 mo
Postma (1989)♂33Exercise intolerant + hemoptysisRecent PTELeft hypogastric vein3-4 approximatelyNoneSimple ligation at originAsymptomatic12 mo
Gade (1991)♂13Limb swellingThrombosed right CFVLeft EIV and hypogastric junction5 × 5Congenital IVC hypoplasiaUnsuccessful thrombolysis + right femoral thrombectomy +ARAsymptomatic1.5 mo
Salman (1994)♂53Chronic venous stasis, leg ulcer, and abdominal massThrombosed distal SFVLeft CIV25Posttraumatic AVF (CFA) Iliac artery aneurysmAVF ligation AR + end to end anastomosis Arterial aneurysm correctionImprovement of venous chronic insufficiency9 mo
Saito (1995)♂19Abdominal painThrombosed IVC and left iliac veinsRight CIV and IVC junctionNo dataDouble IVC, hypoplastic right IVC, pre-renal stenosis of IVCAnticoagulationAsymptomatic1 mo
Labropoulos (1996)♀34Limb swellingThrombosed aneurysmRight EIV and CFVNo dataDouble EIV estenosisVein ligation + anticoagulationPost-thrombotic limb24 mo
Petrunic (1997)♂19Abdominal painThrombosed aneurysmRight CIV4.5 × 8.9NoneAR + lateral venorrhaphy + anticoagulationAsymptomatic12 mo
Alatri (1997)♂39AsymptomaticNoneBilateral CIV3.9 × 6.0 4.3 × 7.3IVC, bilateral FEM-POP ectasiaNoneNo dataNo data
Jalaluddin (1998)♀63Pain in the hip and pulsatile swelling at right iliac fossaNoneRight EIV3.5Vein wall thermal injuryNoneNo complications12 mo
Fourneau (1998)♀21AsymptomaticNoneLeft EIV5 × 10NoneAR with contralateral SFV reconstruction + anticoagulationSlight irritation saphenous nerve18 mo
Frikha (1999)♂30Chronic venous stasis and leg ulcerNoneRight EIV5 approximatelyPosttraumatic AVF (SFA)AVF ligationNo complicationsno data
Alonso (2002)♂67Limb swellingThrombosed aneurysmBilateral CIVNo data5 cm IVC aneurysmAR + anticoagulationPost-thrombotic limb6 mo
Banno (2004)♀20AsymptomaticNoneLeft EIV8NoneAR+ lateral venorrhaphy + AnticoagulationAsymptomatic16 mo
Cañibano (2007)♂69Back pain and limb swellingThrombosed aneurysmLeft CIV and EIV11 × 4.2NoneAnticoagulationNo data1 mo
Ysa (2008)♂51Fever and limb swellingThrombosed aneurysmRight EIV5 × 9Pre-renal IVC hypoplasiaAnticoagulationresidual edema3 mo

CIV, Common iliac vein; EIV, external iliac vein; CFV, common femoral vein; SFV, superficial femoral vein; IVC, inferior vena cava; CFA, common femoral artery; SFA, superficial femoral artery; POP, popliteal artery; AVF, arteriovenous fistula; AVM, arteriovenous malformation; AR, aneurysm resection; DVT, deep venous thrombosis; PTE, pulmonary thrombo embolism.

Table II. Summary of the different treatments according to the cause of the aneurysm and the permeability of the venous axis
nCharacteristicsAE without VFRAE with VFRNo surgical treatment
Primary (n = 7)3Thrombosed111
4Nonthrombosed031
Secondary (n = 15)
Flow obstruction4Thrombosed121
Thermal injury1Nonthrombosed001
AVFa7AVF ligation313
3No AVF ligation003

AE, Aneurysm exclusion; VFR, venous flow reconstruction; AVF, arteriovenous fistula.

aNone thrombosed.

Although there are no reports of endovascular management of iliac venous aneurysms, posttraumatic and stenotic lesions of the ilio-caval segment have been corrected successfully with endoprosthesis.26

During an average follow-up of 9 months (15 days to 24 months), there was only one case of death secondary to a stroke and only one PTE. The most frequently reported complain was postphlebitic syndrome. In 32% of cases, there was persistence or worsening of the previous symptomatology. Among the 15 patients in whom continuity of venous flow was preserved upon admission (postrevascularisation or absence of prior thrombosis), only 7% showed residual edema of the lower limb. In the group where venous flow was not preserved, 50% of the patients showed residual edema (Fisher exact test, P = .053).

This is the largest reported review in the literature with regard to iliac venous aneurysms. Despite the extensive descriptive analysis carried out, the low number of cases identified and the inter-group heterogeneity makes it difficult to draw possible conclusions with regard to its natural history and prognosis.

Back to Article Outline

 

The authors acknowledge Dr J. Perez for his editing help.

Back to Article Outline

References 

  1. Cornet L, Angate YA, Serres JJ. External iliac pelvic venous aneurysm at a distance from a low femoral arteriovenous fistula (Two cases). Memoires de l Academie de Chirurgie. 1969;95:740–743
  2. Raithel D. Aneurysm of the iliac vein in a 25-year existing fistula between the femoral artery and vein. Medizinische Klinik. 1972;67:13–15
  3. Vaccaro E, Lucantoni A, Solitro A, Peverini M. Case of popliteal arteriovenous fistula with secondary aneurysm of the external iliac vein. Minerva Cardioangiologica. 1975;23:784–795
  4. Parer JT, Lichtenberg ES, Callen PW, Feduska N. Iliac venous aneurysm in a pregnant patient with a renal transplant (A case report). J Reprod Med. 1984;29:869–871
  5. Mansfeld L, Poehls C, Hubner R. Secondary aneurysm of the iliac vein as a complication of traumatic arteriovenous fistulae of the lower extremity. Rofo: Fortschritte auf dem Gebiete der Rontgenstrahlen und der Nuklearmedizin. 1985;142:222–224
  6. Valdes F, Kramer A, Fava M, Cruz F, Croxatto H. Giant venous aneurysm associated with hypogastric arteriovenous malformation. Ann Vasc Surg. 1986;1:143–146
  7. Tisnado J, Cook DE, Cho SR, Bezirdjian D. General case of the day (Traumatic arteriovenous fistula between the right superficial femoral artery and the deep femoral vein with a large external iliac venous aneurysm). Radiographics. 1988;8:181–187
  8. Hurwitz RL, Gelabert H. Thrombosed iliac venous aneurysm: a rare cause of left lower extremity venous obstruction. J Vasc Surg. 1989;9:822–824
  9. Postma MP, McLellan GL, Northup HM, Smith R. Aneurysm of the internal iliac vein as a rare source of pulmonary thromboembolism. South Med J. 1989;82:390–392
  10. Gade A. Hypoplasia of the infrarenal inferior vena cava with compensatory left retroperitoneal venous aneurysm. Vasa. 1991;20:283–287
  11. Salman E, Ozeren M, Mavioglu I, Yucel E, Yorukoglu Y. Giant iliac venous aneurysm caused by acquired femoral arteriovenous fistula: a case report. Vasc Surg. 1994;28:295–300
  12. Saito H, Sano N, Kaneda I, Arakawa M, Ishida S, Takahashi S, et al. Multisegmental anomaly of the inferior vena cava with thrombosis of the left inferior vena cava. Cardiovasc Intervent Radiol. 1995;18:410–413
  13. Labropoulos N, Volteas SK, Al Kutoubi A, Nicolaides AN, Mansfield AO. A rare case of an aneurysm of the external iliac and the common femoral vein. Phlebology. 1996;11:34–38
  14. Petruni M, Kruzi Z, Tonkovi I, Augustin V, Fioli Z, Protrka N. Large iliac venous aneurysm simulating a retroperitoneal soft tissue tumour. Eur J Vasc Endovasc Surg. 1997;13:221–222
  15. Alatri A, Radicchia S. Bilateral aneurysm of the common iliac vein: a case report. Annali Italiani di Medicina Interna. 1997;12:92–93
  16. Jalaluddin M, Parnell EJ, Corbett CRR. Venous aneurysm occurring after revisional hip surgery: a case report. Phlebology. 1998;13:171–173
  17. Fourneau I, Reynders-Frederix V, Lacroix H, Nevelsteen A, Suy R. Aneurysm of the iliofemoral vein. Ann Vasc Surg. 1998;12:605–608
  18. Frikha I, Abdennadher M, Masmoudi S. Iliac venous aneurysm: complications of arteriovenous fistula. Sang Thrombose Vaisseaux. 1999;11:776–777
  19. Alonso-Perez M, Segura RJ, Vidal ED. Thrombosed aneurysm of the infrarenal vena cava: diagnosis and treatment. J Cardiovasc Surg. 2002;43:507–510
  20. Banno H, Yamanouchi D, Fujita H, Nagata J, Kobayashi M, Matsushita M, et al. External iliac venous aneurysm in a pregnant woman: a case report. J Vasc Surg. 2004;40:174–178
  21. Cañibano C, Acín F, Martinez E, Medina FJ, Bueno A, Lopez A. Primary iliac venous aneurysm: a case report and review of the literature. Angiología. 2007;59:277–282
  22. Abbott OA, Leigh TF. Aneurysmal dilatations of the superior vena caval system. Ann Surg. 1964;159:858–872
  23. Forsberg JO, Bark T, Lindholmer C. Nontraumatic rupture of the iliac vein. Eur J Vasc Surg. 1988;2:267–268
  24. Volteas SK, Labrapoulos N, Nicolaides AN. The management of venous aneurysms of the coger extremities. Panminerva Med. 1998;40:89–93
  25. Calligaro KD, Ahmad S, Dandora R, Dougherty MJ, Savarese RP, Doerr KJ, et al. Venous aneurysms: surgical indications and review of the literature. Surgery. 1995;117:1–6
  26. Sam AD, Frusha JD, McNeil JW, Olinde AJ. Repair of a blunt traumatic inferior vena cava laceration with commercially available endografts. J Vasc Surg. 2006;43:841–843

 Competition of interest: none.

PII: S0741-5214(08)00258-9

doi:10.1016/j.jvs.2008.02.008

Journal of Vascular Surgery
Volume 48, Issue 1 , Pages 218-222, July 2008

Article Tools

* Image Usage & Resolution