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Inferior vena cava (IVC) anomalies have a 0.5% incidence rate and could be associated with other congenital abnormalities. In later stage of the disease, trophic ulcers with or without deep vein thrombosis (DVT) is consistent finding.
A 29-year-old male patient presented with recurrent lower extremity ulcers. Further workup revealed an absent infra...
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Citations
... Most previous studies have reported dysplasia as the most common type of IVC anomaly causing deep vein thrombosis (DVT). Sitwala et al. (21) identified congenital IVC deficiency as a risk factor for lower limb deep vein thrombosis in young people. Kim et al. (22) researched the types of abnormal development of the inferior vena cava in the normal population and in patients with DVT, and they reported that agenesis and hypoplasia of the inferior vena cava were the most common types in patients with DVT. ...
Background
Double inferior vena cava (DIVC) is a rare vascular malformation. With advances in radiological techniques and diagnosis, more and more types of DIVC were identified and diagnosed. Recognition of the variety of DIVC seen on imaging is essential for subsequent venous interventions.
Case presentation
A 77-year-old man presented with low back pain with left lower limb pain for 1 month. Scattered petechiae above the skin surface on the left lower leg, especially on the extensor surface, with flaking and mild tingling of the skin, were noted 3 weeks ago. Ultrasound revealed deep vein thrombosis (DVT) in the left lower limb. Computed tomography pulmonary angiography (CTPA) suggested no significant thrombus in the pulmonary artery. Computed tomography venography (CTV) of bilateral lower limbs showed that iliac vein compression syndrome with formation of deep and superficial venous traffic branches in bilateral lower limbs, predominantly on the left side. CTV of the inferior vena cava (IVC) suggested that the left common iliac vein crossed the common iliac artery bifurcation from dorsal to ventral and continued to travel cranially as a ventral vessel, and connected with the ventral IVC anterior to the right common iliac artery. The right common iliac vein extended cephalad as a dorsal vessel, which was narrowed at the level of 4th lumbar vertebra by compression of the hyperplastic bone and the osteophyte. The patient was discharged after right and left common iliac vein angiography and balloon dilation of bilateral common iliac vein.
Conclusion
The formation of both ventrally and dorsally aligned DIVC is rarer. It should be clarified the effects of DIVC on DVT formation, and the importance of imaging for preoperative planning.
... 13 Accurate identification of anomalous venous channels on cross-sectional imaging is essential as a part of preoperative evaluation, in planning catheter insertion, deciding cannulation strategies, and accurate mapping for intracardiac repair. 5,13,14 The course, caliber, and drainage points of such channels must be clearly delineated and communicated to the treating surgeon and the anesthesiologist. Multidetector CT allows accurate identification of systemic venous anomalies in addition to delineation of associated conditions, thereby helping in proper surgical planning. ...
Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies.
... Most previous studies have reported dysplasia as the most common type of IVC anomaly causing deep vein thrombosis(DVT). Sitwala et al [20] identi ed congenital IVC de ciency as a risk factor for lower limb deep vein thrombosis in young people. Kim et al [21] researched the types of abnormal development of the inferior vena cava in the normal population and in patients with DVT, and they reported that agenesis and hypoplasia of the inferior vena cava were the most common types in patients with DVT. ...
Background: Double inferior vena cava（DIVC）is a rare vascular malformation. With advances in radiological techniques and diagnosis, more and more types of DIVC were identified and diagnosed. Recognition of the variety of DIVC seen on imaging is essential for subsequent venous interventions.
Case presentation: A 77-year-old man presented with low back pain with left lower limb pain for 1 month. Scattered petechiae above the skin surface on the left lower leg, especially on the extensor surface, with flaking and mild tingling of the skin, were noted 3 weeks ago. Colour Doppler ultrasound revealed deep vein thrombosis（DVT） in the left lower limb with bilateral common femoral and left saphenofemoral valve insufficiency. Computed tomography pulmonary angiography (CTPA) suggested no significant thrombus in the pulmonary artery. Computed tomography venography (CTV) of bilateral lower limbs suspected of iliac vein compression syndrome with formation of deep and superficial venous traffic branches in bilateral lower limbs, predominantly on the left side. CTV of the inferior vena cava（IVC） suggested that the left common iliac vein crossed the common iliac artery bifurcation from dorsal to ventral and continued to travel cranially as a ventral vessel, and connected with the ventral IVC anterior to the right common iliac artery. The right common iliac vein extended cephalad as a dorsal vessel, which was narrowed at the level of 4th lumbar vertebra by compression of the hyperplastic bone and the osteophyte. The patient was discharged after right and left common iliac vein angiography and balloon dilation of the left common iliac vein.
Conclusion: The formation of both ventrally and dorsally aligned DIVC is rarer, which we consider being a newly discovered type V. Venous thrombosis of the lower limbs seems likely to be more closely associated with compression of the left common iliac vein by the right common iliac artery. A definitive diagnosis of the type of DIVC malformation plays a crucial role in guiding interventional treatment.
... In addition, patients with double IVC have a high tendency for thromboembolic events (6). The IVC malformations are associated with 5% of deep vein thrombosis cases due to slow blood flow (30). It is crucial to confirm whether double IVC is present for patients requiring the placement of an IVC filter, and venography should be performed to rule out vascular variation for patient with planned IVC filter placement (31). ...
Cases of a retroperitoneal tumor with double inferior vena cava (IVC) are rarely reported. The present report documents a case of a retroperitoneal lymphoma with double IVC, and discusses its embryological, clinical and radiological significance. In addition, previous cases of a double IVC are reviewed. In the present report, a 52-year-old male patient was hospitalized for a retroperitoneal lymphoma tumor and double IVC. CT urography was performed, whilst a three-dimensional visualization model was also established based on CT data, to reveal a retroperitoneal tumor with double IVC. The present case involved a double IVC with interiliac vein, which was type 2b from the left IVC. The retroperitoneal tumor was identified to be a lymphoma measuring 116x83 mm by percutaneous puncture biopsy. Surgical treatment is generally not recommended for lymphoma. Therefore, this patient was transferred to the Hematology Department for treatment according to the lymphoma management guidelines. The size of the tumor was reduced after chemotherapy during the patient's follow-up. In conclusion, the three-dimensional visualization model can directly and accurately present the anatomical features of the double IVC and its surrounding tissue structure. In addition, variations in the features of IVC can have important clinical significance. It is also important for surgeons, interventional radiologists and clinicians to understand such abnormalities in anatomical features to avoid misdiagnosis and reduce the occurrence of serious intraoperative complications.
... However, those with congenital IVC anomalies have a 60% to 80% lifetime risk of thrombotic events. 5,6 Thrombosis of the congenitally abnormal IVC, a prominent feeding vessel, or collateral channel can lead to acute or subacute proximal deep vein thrombosis, with associated acute and chronic sequelae, including phlegmasia cerulea dolens, pulmonary embolus, renal vein thrombosis, venous claudication, post-thrombotic syndrome, and ulceration. 7,8 The initial presentation will often be underwhelming with back pain and/or symptoms that mimic musculoskeletal complaints. ...
Inferior vena cava (IVC) anomalies remain silent until collateralized venous drainage is lost. Initial signs may be subtle including back pain and are often missed initially with progressive changes toward motor weakness, phlegmasia cerulea dolens, or renal impairment. We present a case of acute occlusion of an atretic IVC and infrarenal collateral drainage in an adolescent patient, treated with successful thrombolysis, thrombectomy and endovascular revascularization for IVC stenting and reconstruction.
... Cuando el inicio sintomático se produce a una edad más tardía, se caracteriza por edema, pesadez o úlceras venosas recurrentes. Sin embargo, la presentación más común es asintomática y suele ser un hallazgo imagenológico 1,4 . ...
La agenesia de la vena cava inferior infrarrenal es una malformación infrecuente. Habitualmente es un hallazgo imagenológico, aunque puede manifestarse con disnea, falla cardíaca, trombosis o úlceras. La asociación de agenesia de la vena cava inferior con una trombofilia es extremadamente infrecuente. Es probable que exista una relación causal entre ambas entidades, que excede la mera coexistencia. Se presenta el caso de un paciente con úlceras debido a agenesia de la vena cava inferior infrarrenal y deficiencia de proteína S, que evolucionó favorablemente con curaciones avanzadas y elastocompresión.
... The thrombosis preferentially involves the iliac and femoral veins, often bilaterally, and usually occurs anticoagulation varied. Considering the irreversible risk for future thrombosis, lifelong anticoagulation may be necessary (10,4) Elastic stockings support and leg elevation should be also suggested, as well as avoidance of additional risk factors such as prolonged immobilization and oral contraceptive use (9) . In some cases, venous recanalization by thrombolysis can be a good alternative particularly in the younger patients because of their higher risk of long lasting disability. ...
... If medical treatment fails venous bypass graft/ stenting should be considered. Venous bypass graft from the common iliac vein to the azygous vein or proximal cava segment is considered to prevent DVT or to heal the ulcers if conservative treatments fails (10) . ...
Introduction:
Inferior vena cava agenesis is one of the most uncommon anomalies of this vessel, with an estimated prevalence of 0.0005-1% in the general population. However, around 5% of the patients younger than 30 years with a diagnosis of deep vein thrombosis have this anomaly.
Methods and material:
Report of two clinical cases of inferior vena cava agenesis with different clinical presentations.
Clinical case 1: A 40-year-old man was admitted with a 3 days history of unilateral lower limb swelling and pain, gradually progressing to the inability to walk. On physical examination he had swelling, bruising and tenderness involving leg and thigh and prominent engorged superficial abdominal collateral veins.
Venous Doppler Ultrasound showed left deep venous thrombosis extending from popliteal vein to common iliac vein. A computed tomography angiogram showed agenesis of the infrarenal vena cava and patent renal veins draining
in to the azygous system and hemiazygous systems.
The patient as discharged with rivaroxaban and compression stockings. At 2 months follow up he was asymptomatic and still anticoagulated.
Clinical case 2: A 35 year- old woman, with a previous history of recurrent lower limb varicose veins surgery and left internal malleolar ulcer at 30 years presented at medical department 3 years later with complains of ulcer recurrence.
A computed tomography angiogram revealed an absent infrahepatic vena cava, prominently dilated azygos and hemiazygos veins with enlarged retroperitoneal collaterals.
The patient initiated dressing care with oxide zinc and oral rivaroxaban.
Discussion/conclusion:
The majority of cases remain asymptomatic. However, when symptomatic, the majority present as proximal DVT involving the iliac and femoral veins. CT or MRI should be the imagiological methods used to diagnose this anomaly. No clear consensus has been reached on therapeutic strategy, other than long-term anticoagulation and elastic stockings.
... As a result, the most encountered presentation is unprovoked DVTs, usually bilateral proximal (iliofemoral) DVTs in young patients. 6 Much more rare than that, intraabdominal particularly renal vein thrombosis in these patients has also been reported in the literature. 7 In patients with IVCA, pulmonary embolism and post-thrombotic syndrome (PTS) are the main concerns after venous thrombosis. ...
Abstract
Inferior vena cava agenesis (IVCA) is a rare entitiy wity many patients remain clinically silent and found incidentally on
radiological imaging studiesas a result of the compensatory enlargements of many collateral veins to maintain venous return.
However, IVCA may also be diagnosed following serious acute complications, particularly unprovoked proximal iliofemoral
deep vein thromboses (DVT) in young individuals. Herein, we report a 25-year old postpartum female patient presented
with acute swelling on the right lower extremity and diagnosed with IVCA following acute occlusive iliofemoral DVTs.We
aimed to emphasize the importance of radiological evaluation and management of these patients with some considerations
out of routine practice.
... 2,16 Our patient had the added complexity of having a duplicated IVC. Whereas this congenital anomaly has been associated with complications such as deep venous thrombosis, [26][27][28] its connection with NCS has been observed only once. In a case report by Yoshida et al, 14 a female patient with a duplicated IVC was found to have NCS after presenting with left lower extremity deep venous thrombosis; she was successfully treated with LRV pharmacomechanical thrombolysis and stenting. ...
Nutcracker syndrome is a rare vascular disorder that involves compression of the left renal vein (LRV), most often at the level of the aortomesenteric angle. In some cases, this compression syndrome arises in the setting of unusual vascular anatomic variants. We describe the case of a 43-year-old woman with a duplicated inferior vena cava who was found to have LRV compression on magnetic resonance angiography and venography with intravascular ultrasound. The patient was successfully treated with concurrent transposition of the LRV and left-sided inferior vena cava, with complete resolution of symptoms.
... There is no data currently available regarding prophylactic anticoagulation for these patients. This particular patient population is susceptible to clot formation at baseline but their risk rises exponentially if they are exposed to factors that can contribute to hypercoagulable state [3,4]. Treatment is with anticoagulants/thrombolysis when appropriate. ...
