Inferior Vena Cava Agenesis Research Articles

Inferior vena cava agenesis in patients with lower limb deep vein thrombosis in the RIETE registry. When and why to suspect

BackgroundLimited data exist about the clinical presentation and outcomes of patients with inferior vena cava agenesis (IVCA) who develop deep vein thrombosis (DVT). MethodsWe used the RIETE (Registro Informatizado Enfermedad Trombo Embólica) registry to compare clinical characteristics and outcomes of patients with lower limb DVT, according to the presence or absence of IVCA. Major outcomes included recurrent DVT, major bleeding and post-thrombotic syndrome (PTS). ResultsAmong 50,744 patients with lower-limb DVT recruited in October 2018, 31 (0.06%) had IVCA. On multivariable analysis, patients aged < 30 years (odds ratio [OR]: 17.9; 95%CI: 7.05–45.3), with unprovoked DVT (OR: 2.49; 95%CI: 1.17–5.29), proximal (OR: 2.81; 95%CI: 1.05–7.53) or bilateral DVT (OR: 11.5; 95%CI: 4.75–27.8) were at increased risk to have IVCA. Patients with DVT and IVCA had lower odds to present with coexisting PE (OR: 0.22; 95%CI: 0.07–0.73). During the first year of follow-up, the rates of DVT recurrences (hazard ratio [HR]: 1.30; 95%CI: 0.07–6.43), pulmonary embolism (HR: 2.30; 95%CI: 0.11–11.4) or major bleeding (HR: 1.32; 95%CI: 0.07–6.50) were not significantly different with those with versus those without IVCA. One year after the index DVT, IVCA patients had a higher rate of skin induration (OR: 3.70; 95%CI: 1.30–9.52), collateral vein circulation (OR: 3.57; 95%CI: 1.42–8.79) or venous ulcer (OR: 5.87; 95%CI: 1.36–1.87) in the lower limb than those without IVCA. ConclusionsCertain clinical features such as unprovoked and bilateral proximal DVT in young patients should raise the suspicion for IVCA. Patients with IVCA had higher odds for symptoms of post-thrombotic syndrome.

Transjugular approach for zero-fluoroscopy catheter ablation of the right anterolateral accessory pathway

We present a case of successful zero-fluoroscopy catheter ablation (CA) of the right anterolateral accessory (AP) pathway via a transjugular approach in an 18-year-old patient with a congenital agenesis of inferior vena cava (IVC). Three-dimensional (3D) electroanatomic mapping (EAM) system and intracardiac echocardiography (ICE) were used to navigate the catheters in the heart.

Embolie pulmonaire et agénésie de veine cave inférieure : une association inhabituelle

Inferior vena cava agenesis is a rare congenital anomaly, generally associated with thrombophilic conditions, and a predisposing factor for deep venous thrombosis (DVT), rarely complicated by pulmonary embolism, in a young population with atypical clinical features and frequent absence of risk factors. We report the case of a 30-year-old woman who developed a right iliac DVT, initially presenting as a low back pain and complicated by a pulmonary embolism, 8 months after a sleeve gastrectomy. Chest CT angiography revealed abnormalities that led to the diagnosis of inferior vena cava agenesis. Thrombophilic screening showed a heterozygous prothrombin gene mutation G20210A and hyperhomocysteinemia. The patient was treated with rivaroxaban with good results after 2 years of follow-up. In young patients without risk factors developing a deep venous thrombosis, an inferior vena cava anomaly should be considered. Although no therapeutic consensus has been currently established, inferior vena cava agenesis seems to be associated with a high prevalence of thrombophilic disorders. Screening could be useful, particularly in patients with a thrombotic family history.

SS16. A Novel Technique for Iliocaval Extravascular Reconstruction and Associated Outcomes in Patients With Inferior Vena Cava Agenesis

Congenital absence of the inferior vena cava is a rare deep venous malformation associated with venous stasis ulceration. Endovascular recanalization is limited as patients lack normal anatomic pathways required for endovascular repair. The purpose of this study was to report outcomes of a novel retroperitoneal technique for iliocaval extravascular reconstruction (TIER) using re-entry techniques for the treatment of C5 to C6 disease in patients with iliocaval agenesis. A prospectively maintained single-institution database was used to observe patients who underwent TIER for management of C5 to C6 venous disease. Reconstruction was performed by sharply exiting the deep venous system and entering the retroperitoneum at or above the renal confluence and the bilateral external iliac veins. Cone beam computed tomography was used to exclude potential injury to adjacent structures during retroperitoneal transcatheter dissection. Reconstruction was performed using self-expanding Elgiloy stents. The Villalta clinical scoring system for post-thrombotic syndrome was used to assess symptomatic improvement preoperatively and postoperatively. Postoperatively, patients with C6 venous disease underwent continued compression therapy and were monitored for wound healing. Twelve patients underwent TIER for complete absence of the inferior vena cava resulting in C5 to C6 disease during the study period (Fig 1). Technical success was 100%, with primary patency achieved in all patients (Fig 2). There were no significant intraoperative complications, and the 30-day mortality was 0%. Mean contrast agent volume used was 87 mL, mean fluoroscopy time was 143 minutes, and mean reference air kerma was 6239 mGy. A minimum of six stents were used in all cases. At follow-up intervals of 1, 3, and 6 months, primary patency was 100%. Symptomatic and sustained improvement was seen in all patients with an average decrease in Villalta score of 13. Nine patients had C6 venous disease with ulcers on 15 limbs due to the presence of bilateral disease. Thirteen limbs had complete ulcer resolution during the follow-up period. The remaining limbs demonstrated >75% ulceration size reduction. Iliocaval extravascular reconstruction using novel re-entry techniques appears to be a safe and effective endovascular approach for the treatment of symptomatic congenital absence of the inferior vena cava. The TIER procedure can be performed with excellent patency, minimal morbidity, improved Villalta scores, and improved healing.Fig 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Deep vein thrombosis and inferior vena cava agenesis

Abstract We report the case of a 13-year old male patient who presented with a right swollen limb. A magnetic resonance angiography (MRA) revealed inferior vena cava agenesis (IVCA) which was regarded as the etiologic factor for the deep vein thrombosis (DVT) of the patient. IVCA is a rare vascular abnormality which presents in 5% of young people aged under 30 years with lower limb DVT. It is usually underestimated but should be carefully considered in patients with no other apparent risk factors for DVT.

A rare case of inferior vena cava agenesis associated with deep venous thrombosis

A rare case of inferior vena cava agenesis associated with deep venous thrombosis

Inferior Vena Cava Agenesis: An Unusual Cause of Deep Vein Thrombosis and Pulmonary Embolism in Young Adult Patients

IntroductionInferior vena cava agenesis (IVCA) is one of the many anomalies of this vessel. It is one of the most uncommon anomalies, with an estimated prevalence of 0.0005–1% in the general population. Around 5% of the patients younger than 30 years with a diagnosis of deep vein thrombosis (DVT) have a total or segmental IVCA.ReportHere two unique cases of young and previously healthy male patients are reported: one with bilateral lower extremity DVT, the second with lower extremity DVT and pulmonary embolism. Both patients were found to have segmental agenesis of the inferior vena cava on computed tomography angiography (CTA). Treatment consisted of ultrasound enhanced thrombolysis (EKOS + alteplase) and venous angioplasty. Both patients were discharged with long-term (up to 24 months) oral anticoagulation and compression stockings. Follow up at 3 and 12 months revealed no new thrombotic episode.DiscussionIVCA can be asymptomatic but the majority of the symptomatic patients present with DVT. IVCA confers a risk factor for DVT. IVCA should be considered and ruled out as a rare but important risk factor and cause of DVT in previously young healthy patients. Once diagnosed, aggressive treatment must be started because of the high risk of post-thrombotic syndrome.

Inferior Vena Cava Agenesis and Deep Vein Thrombosis in the Young: A Review of the Literature and Local Experience.

AbstractCongenital agenesis of the inferior vena cava (AIVC) is a rare vascular abnormality with a prevalence of 0.0005 to 1% in the general population. This condition is found in almost 5% of young patients (younger than 30 years) with proximal, typically bilateral, deep venous thrombosis (DVT) of the lower limbs, often in the absence of apparent risk factors. However, AIVC in young patients with DVT is probably underestimated because AIVC cannot be detected by the standard DVT diagnostic workup. Inherited thrombophilia has been reported in patients with AIVC-associated DVT, as an additional risk factor, but its role is poorly investigated. The best therapeutic strategy and the optimal duration of anticoagulant treatment of DVT in patients with AIVC are still unclear. Here, we describe 14 young patients (2 females and 12 males, mean age at first DVT: 27.8 ± 10.1 years), with color Doppler ultrasound (US) and abdominal computed tomography scan confirmed AIVC-associated DVT and discuss their characteristics in the framework of a literature review on this topic. Our patients were mainly males (10/12) and experienced proximal DVT events, not complicated by pulmonary embolism. DVT-precipitating factors were detected only in five cases, the two female patients (oral contraceptives and puerperium), and three male patients (one after leg fracture and two after abdominal surgery). Thrombophilic abnormalities were found in eight patients (heterozygous factor V Leiden mutation, n = 3; mild/moderate hyperhomocysteinemia, n = 3; reduced protein C activity, n = 1; antiphospholipid syndrome, n = 1). The majority of patients (13/14) were treated with long-term oral anticoagulant therapy and elastic stockings (ES), with a very low rate of DVT recurrence (1/14, mean follow-up 7 years). These clinical characteristics were largely consistent with those of 161 patients identified by review of the literature. On the whole, although many aspects are still poorly known, our case series and literature review suggest that a search should be undertaken for AIVC in young patients with proximal idiopathic, particularly when bilateral, DVT, which should then be treated with prolonged anticoagulation and ES.

CRT-300.07 Total Endovascular Caval Reconstruction for the Treatment of Inferior Vena Cava Agenesis

To report the technical success, complications, and long-term patency of iliocaval reconstruction for the treatment of inferior vena cava (IVC) agenesis. 30 (3%) patients with suspected IVC agenesis were identified from a venous registry of 994 patients from 2000 until 2016. Patients included 23

Combined thrombolytic and endovascular treatment of proximal deep vein thrombosis in inferior vena cava agenesis

Combined thrombolytic and endovascular treatment of proximal deep vein thrombosis in inferior vena cava agenesis

Congenital agenesis of inferior vena cava as a rare cause of deep vein thrombosis: Case report and review of the literature

Inferior vena cava agenesis (IVCA) is an uncommon cause of deep vein thrombosis (DVT). We report the case of a 26-year-old Caucasian female with unilateral iliofemoral DVT. On further evaluation, CT abdomen revealed diminutive inferior vena cava consistent with IVCA. We have used systemic anticoagulation and catheter mediated thrombolysis with success in this case. Physicians should suspect IVCA in patients with extensive DVT especially in younger individuals.

Agenesis of Inferior Vena Cava with Cavernous Transformation and Hypoplastic Infrarenal Part of IVC, Rare Congenital Vascular Anomalies: A Case Series

Congenital vascular anomalies are although rare but may cause serious complications. Vascular pathologies are best demonstrated by contrast enhanced computed tomography, with the advent of multidetector computed tomography and applying multiplanar reformation techniques. However, ultrasound color Doppler and catheter angiography are accessory imaging modalities utilized to rule out vascular anomalies. We present here a case series of 2 patients aged 19 years and 54 years, diagnosed with congenital vascular anomalies (agenesis of inferior vena cava and hypo plastic infra renal segment of inferior vena cava). The development of inferior vena cava (IVC) includes early embryogenesis stages including formation, confluence and involution of three primary longitudinal venous systems. Agenesis of inferior vena cava or hypoplasia of segment of inferior vena cava leads to formation of collaterals to maintain the venous flow. Contrast enhanced computed tomography with multiplanar reformation provides ulterior spatial and contrast resolution in cases of recognized vascular abnormalities. Inferior vena cava obstruction due to thrombosis or congenital vascular anomaly leads to extensive collateralization accompanied by varicose veins. In our case series, we found contrast enhanced computed tomography as an ulterior modality in setting ultimate diagnosis with accuracy and maximum detail.

Epidural venous plexus engorgement: what lies beneath?

Epidural venous plexus engorgement may occur due to several conditions that prevent the normal venous circulation. Inferior vena cava agenesis is a very rare cause of epidural venous enlargement. We present a case with a very thin inferior vena cava and left iliac vein agenesis who presented with back pain due to epidural vein engorgement and lacked other venous problems such as deep vein thrombosis.

Deep Vein Thrombosis Provoked by Inferior Vena Cava Agenesis.

Inferior vena cava agenesis (IVCA) is a rare congenital anomaly that can be asymptomatic or present with vague, nonspecific symptoms, such as abdominal or lower back pain, or deep vein thrombosis (DVT). Here, we present a 55-year-old male who came with painless swelling and redness of his left lower limb. On examination, swelling and redness were noted extending from the left foot to the upper thigh; it was also warm compared to his right lower limb. Venous Doppler ultrasound was done which showed DVT extending up to the common femoral vein. Subsequently, computed-tomography (CT) of the chest and abdomen was done to exclude malignancy or venous flow obstruction; it revealed congenital absence (agenesis) of the infrarenal inferior vena cava (IVC).

Deep Venous Thrombosis of the Leg, Associated with Agenesis of the Infrarenal Inferior Vena Cava and Hypoplastic Left Kidney (KILT Syndrome) in a 14-Year-Old Child.

Agenesis of the inferior vena cava (IVC) is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT). No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

The clinical anatomy of the inferior vena cava: a review of common congenital anomalies and considerations for clinicians.

Anomalies in the course and drainage of the Inferior Vena Cava (IVC) may complicate normal functioning, correct diagnosis, and therapeutic interventions within the abdomen. Development of the IVC occurs during the 4th to 8th week of gestation, and due to its developmental complexity, there are many opportunities for malformations to occur. Although most IVC anomalies are clinically silent and are usually discovered incidentally on abdominal imaging, aberrations may be responsible for formation of thrombosis, back pain, and anomalous circulation of blood to the heart. In this review, we will discuss the most common variations and abnormalities of the IVC, which include the posterior cardinal veins, the subcardinal veins, the supracardinal veins, persistent left IVC, IVC duplication, situs inversus, left retroaortic renal vein, left circumaortic renal collar, scimitar syndrome, and IVC agenesis. For each abnormality outlined above, we aim to discuss relevant embryology and potential clinical significance with regards to presentation, diagnosis, and treatment as is important for radiologists, surgeons, and clinicians in current clinical practice.

A rare case: Vena cava inferior agenesis with aberrant hepatic vein in an asymptomatic patient

SummaryInferior vena cava agenesis is a rare anomaly in an asymptomatic patient. A 58-year-old female patient with complete absence of the inferior vena cava and aberrant right hepatic vein is presented with radiologic findings. To our best knowledge, such a combination of venous anomalies has not previously been documented in the literature and this is the first case in the literature due to the coincidence of total inferior vena cava agenesis with aberrant hepatic vein.

Compressive Symptoms Due to Thrombosed or Hypertrophic Collateral Circulation in Infrarenal Inferior Vena Cava Agenesis

Agenesis of the infrarenal segment of inferior vena cava is an uncommon and often asymptomatic congenital abnormality with an approximate incidence of 0.005-1% in the general population. Presentation of this condition as deep venous thrombosis associated with symptoms secondary to thrombosis or hypertrophy of collateral vessels is a rare clinical form. Two cases of this rare form are described, and an analysis of the related literature is presented. The first case was that of a 35-year-old man with symptoms of acute obstructive pyelonephritis with no apparent cause and swelling of the lower limb. The second case involved a 30-year-old man with lower limb swelling associated with paresthesia and motor deficit. Both patients were found to have infrarenal inferior vena cava agenesis associated with iliofemoral deep venous thrombosis and compression of anatomic structures by collateral vessels (ureteral compression due to thrombosis of an ectatic gonadal vein and L5 nerve root compression due to a hypertrophic epidural venous plexus). Patients were conservatively managed with anticoagulation therapy, with a favorable outcome and no recurrence in either case. In a young patient with iliac deep venous thrombosis of uncertain etiology, and particularly in those with atypical associated symptoms (mainly abdominal or neurologic), it is important to investigate congenital vena cava anomalies and carry out diagnostic imaging studies. Anticoagulation therapy is currently considered the best treatment option for this condition, but it should be individualized. The need for long-term anticoagulation should also be assessed in each case.

Agenesia da veia cava inferior

Agenesia da veia cava inferior é uma malformação rara. Sua causa mais comum é a disgenesia durante a embriogênese, mas também pode estar relacionada a trombose intrauterina ou perinatal. Normalmente é assintomática, em associação, ou não, com outras malformações congênitas, e pode cursar com maior risco de insuficiência venosa crônica e trombose venosa profunda, especialmente em jovens. Seu diagnóstico frequentemente é acidental, durante cirurgias abdominais ou procedimentos radiológicos. Relatamos cinco casos de agenesia da veia cava inferior detectada durante procedimentos eletrofisiológicos.

Cutaneous Aspects of Congenital Absence of the Inferior Vena Cava in a Newborn

We present the case of a 9-day-old girl with venous dilatations in lower abdomen and legs associated to cyanosis. Radiological studies revealed a congenital agenesis of the entire inferior vena cava.