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Schematic drawing of embryologic development of the inferior vena cava (IVC). Three pairs of veins—posterior cardinal (blue), supracardinal (purple), and subcardinal (orange) and anastomoses between them (black) give rise to the development of IVC in succession on both sides of aorta (red). Some portions of these veins regress (dashed colored lines), whereas others persist (solid colored lines). The hepatic segment develops from the vitelline vein (green). The suprarenal segment develops from the right subcardinal vein. The right suprasubcardinal and postsubcardinal anastomoses give rise to the renal segment. The infrarenal segment develops from the right supracardinal veins. In the pelvis, caudal portions of the postcardinal veins form the common iliac veins, whereas other parts regress progressively. In the thorax, the azygos and hemiazygos veins (lilac) develop from the supracardinal veins.
Source publication
The duplication of the inferior vena cava (IVC) is a rare congenital anomaly, which also has some variations regarding the complex embryological development of the IVC. In the typical form, infrarenal IVC segments are duplicated and the left IVC joins the left renal vein, which crosses anterior to the aorta in the normal fashion to join the right I...
Contexts in source publication
Context 1
... congenital anomalies of the inferior vena cava (IVC) can be recognized in a computed tomography (CT) or magnetic resonance imaging examination in an asymptomatic patient. These anomalies reflect the complex and multisegmental nature of the development of the IVC during embryogenesis. 1 Three paired embryological veins, which are termed as poster- ior cardinal, subcardinal, and supracardinal veins, contribute to the development of the IVC between the sixth and eighth weeks of embryological life ( Figure 1). 2 Any abnormality in the regression or persistence of those embryological veins may cause different anatomical variations, which are classified by Huntington and McLure up to 14 theoretical variations. ...
Context 2
... IVC has 4 segments that are derived from different veins. 1,7 The hepatic, suprarenal, and infrarenal segments develop from the vitelline vein, right subcardinal vein, and right supracardinal vein, respectively ( Figure 1). The right suprasubcardinal and postsubcardinal anastomoses give rise to the renal segment. ...
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Background
Fetal thrombotic vasculopathy is a described placental diagnosis associated with adverse perinatal outcomes. It may also predispose children to somatic thromboembolic events. As far as we know, this is the first case of inferior vena cava thrombosis associated with fetal thrombotic vasculopathy in a completely asymptomatic newborn.
Case...
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 f...
Leiomyosarcoma of the Inferior Vena Cava (IVC) is a rare soft tissue tumour which accounts for only 0.5% of all soft tissue sarcomas. The patients usually presented with non specific clinical signs and often diagnosed at advanced stage. We reported a case of a 58-year-old lady who presented with right sided abdominal pain for 6 months. CT scan show...
Purpose
Our aim was to study the anatomy of the left and right main adrenal veins (LAV and RAV) and to identify their anatomical variations in order to see the practical application of these findings to adrenal venous sampling (AVS).
Methods
Our work is based on dissection of 80 adrenal glands from fresh corpses in the forensic medicine department...
Citations
... The prevalence of bilateral DIVC globally ranges from 0.2% to 3%, as observed incidentally from intraoperative, radiologic, dissection, and electrophysiology studies (Adachi, 1940;Reis and Esenther, 1959;Sarma, 1966;Chuang et al., 1974;Mj et al., 1979;Cohen et al., 1982;Doyle et al., 1992;Soltes et al., 1992;Shingleton et al., 1994;Nagashima et al., 2006;Grubb et al., 2007;Milani et al., 2008;Bergman et al., 2009;Ng and Ng, 2009;Sahin et al., 2017;Cheung and Wong, 2020). Multiple classification systems for bilateral DIVC exist within prior literature and have vastly improved general understanding (Huntington and McClure, 1920;Edwards, 1951;Sarma, 1966;Chuang et al., 1974;Meyer et al., 1998;Morita et al., 2007;Natsis et al., 2010). ...
... While the implications for the presence of bilateral DIVC during reconstructive surgery of the abdominal aorta are widely described in the literature (Shindo et al., 2000;Aljabri et al., 2001;Karkos et al., 2001;Dimic et al., 2017), there is limited knowledge for the implications of its presence during the exposure phase of the anterior lumbosacral spine. More detailed anatomical knowledge of the bilateral DIVC may be crucial to spine and vascular (access) surgeons as well as general and visceral surgeons for a multitude of reasons (Sahin et al., 2017). The anterior approach to the retroperitoneal space is also the preferred route for oncological resections in cases of retroperitoneal sarcomas, pancreatic tumors with multivisceral infiltration, liver tumors with retroperitoneal extension, or adrenal tumors (Watson and Harper, 2015;Yamaguchi et al., 2021). ...
... Embryological development of the IVC involves the formation, anastomosis, regression, and substitution of the paired embryological veins (posterior cardinal, supracardinal, and subcardinal veins) (Fig. 3A-D) (Patten, 1909;Augier, 1910;Johnston, 1913;Huntington and McClure, 1920;McClure and Butler, 1925;Reagan, 1927;Gladstone, 1929;Sahin et al., 2017;Tankruad et al., 2017;Karaosmanoglu et al., 2021). The IVC develops around the 6th to 10th week of gestation. ...
... Especially, angiography is important to recognize the exact anatomy of the drainage for surgical purposes. Therefore, detection of these venous anomalies is significant because it would hamper right heart catheterization via the femoral vein approach as well as cardiopulmonary bypass surgery and pacemaker placement (7). ...
... drainage system. All conditions are rare and unusually to find simultaneously (7). ...
Inferior vena cava (IVC) interruption with azygos/hemiazygos continuation is an extremely uncommon congenital vascular anomaly, which may present with multiple variants. As a result, it is challenging to find in the literature the same anatomical variant. We report a unique case of an interrupted IVC with hemiazygos and transhepatic continuation in an 83-year-old female patient. The case was evaluated by performing Computed Tomography (CT) as imaging modality, with a multiphase protocol, able to detect accurately this complex vascular anomaly. The purpose of this case report is not only to present this remarkable case but also to briefly show the types of interrupted IVC, starting from the anatomy and the embryology of the IVC and the azygos system, and to discuss the value of imaging in detecting the vascular anomaly.
... This developmental anomaly appears to be a relatively benign finding; nearly all cases were discovered incidentally in asymptomatic individuals, and to our knowledge there has been no reported association with significant comorbidity [ 2 ,6 ,7 ]. One case described by Sahin et al. 2017 was associated with pelvic congestion syndrome, but the patient had several additional venous anomalies including a duplicated IVC, which is known to be associated with an increased incidence of venous congestion and thrombosis [8] . ...
... This developmental anomaly appears to be a relatively benign finding; nearly all cases were discovered incidentally in asymptomatic individuals, and to our knowledge there has been no reported association with significant comorbidity [ 2 ,6 ,7 ]. One case described by Sahin et al. 2017 was associated with pelvic congestion syndrome, but the patient had several additional venous anomalies including a duplicated IVC, which is known to be associated with an increased incidence of venous congestion and thrombosis [8] . ...
We report a case of intrahepatic inferior vena cava interruption with azygos and transhepatic venous continuation discovered incidentally on CT angiography for acute aortic syndrome. The lesion was initially misdiagnosed as a congenital portosystemic shunt on multiphase CT of the liver but subsequent fluoroscopic venogram revealed no evidence of portosystemic shunting. While intrahepatic IVC interruption with azygos continuation is an uncommon but well-known anatomical variant, transhepatic venous continuation is extremely rare and only a few cases have been published. Excluding portosystemic shunting is important for determining management as persistent congenital portosystemic shunts can be associated with significant morbidity.
... The most common anomalies of the IVC include the circumaortic left renal vein (1.5%-8.7%), retroaortic left renal vein (2.1%), double IVC (0.2%-3%), azygos, hemi-azygos continuation of IVC (0.6%), and isolated left-sided IVC (0.2%-0.5%) [Sahin 2017]. The double IVC, azygos and hemiazygos continuation, intrahepatic IVC interruption, and transhepatic venous shunt are rare. ...
... Most patients with IVC malformation are asymptomatic. The prevalence of thromboembolic disease in patients with a duplicated IVC is unknown, yet there are case reports of pulmonary embolism in those patients [Sahin 2017]. ...
A 57-year-old female with paroxysmal atrial fibrillation and an interrupted inferior vena cava (IVC) was referred to our hospital for radiofrequency ablation. Transseptal puncture and left atrium ablation failed through a standard IVC approach via the femoral vein due to intrahepatic interruption of IVC. We performed a modified mini-maze procedure in this patient through the left thoracic cavity under video-assisted thoracoscopic surgery (VATS). We can successfully complete pulmonary vein (PV) isolation, left atrium box isolation, cardiac ganglia ablation, Marshall ligament ablation, and coronary sinus epicardium ablation using this technique.
... Chentanez et al [1] reported a DIVC in a male cadaver. Sahin et al [14] reported a female case of DIVC incidentally discovered by CT. Ito et al [15] reported the DIVC in a female cadaver. ...
... This accuracy is important, as historically, radiological imaging through axial CT has led to the misinterpretation of DIVC as either lymphadenopathy or ureteric dilation. Currently, the widespread use of multi-detector CT systems has rendered this issue of less importance [14]. ...
Patient: Male, 36-year-old
Final Diagnosis: Double inferior vena cava
Symptoms: Asymptomatic
Medication:—
Clinical Procedure: —
Specialty: Genetics • Radiology • Surgery
Objective
Congenital defects/diseases
Background
With a prevalence of about 2% to 3%, duplication is the most common anomaly associated with the inferior vena cava (IVC). In general, systemic venous anomalies are being more frequently diagnosed in asymptomatic patients. We report the case of a young man with an incidental finding of an asymptomatic duplicated IVC, along with a literature review.
Case Report
A 36-year-old man was brought to our Emergency Department (ED) following a high-speed motor vehicle collision (MVC), reporting right flank and hip pain. Upon examination, the “seatbelt sign” was noticed, along with abrasions over his right side. He sustained a small-bowel mesenteric injury, for which he was admitted and was treated conservatively. A CT scan incidentally revealed a duplicate IVC (DIVC). He later underwent a laparotomy with limited right hemi-colectomy and was discharged home in good condition.
Conclusions
Undiscovered and asymptomatic DIVCs pose a potential risk to patients during clinical interventions. Advancements in diagnostic imaging contribute greatly to the incidental discoveries of inferior vena cava duplication.
... During embryogenesis, the normal IVC is made up of four segments including the hepatic, prerenal, renal and post-renal segments. The IVC collects the blood from the lower limbs, pelvic area and abdomen [3,4] . Failure of the union between the hepatic and prerenal segments during embryological development results in the so-called 'infrahepatic interruption of the inferior vena cava (IVC) with azygos continuation' (Fig. 4). ...
Background:
The azygos venous system is an accessory venous pathway supplying an important collateral circulation between the superior and inferior vena cava (IVC).
Case summary:
We report a case of complicated community-acquired pneumonia with interruption of the IVC with azygos continuation that was misdiagnosed as lung neoplasm.
Discussion:
The aim of this case report is to emphasize the importance of recognizing an enlarged azygos vein at the confluence with the superior vena cava and in the retrocrural space to avoid misdiagnosis as a right-sided para-tracheal mass. The angiographic features and clinical importance of this condition are discussed.
Learning points:
An enlarged azygos vein at the confluence with the superior vena cava and in the retrocrural space can be misdiagnosed as a right-sided para-tracheal mass/neoplasm or retrocrural adenopathy.Biopsy is the most important tool to confirm the diagnosis of malignancy but could be catastrophic in this situation.Re-imaging after treatment for infection and pneumonia could potentially avoid this error.
... The IVC collects the blood from the lower limbs, pelvic area, and abdomen. [6,7] Various anomalies of the IVC can be seen depending on abnormal regression or abnormal persistence of embryonic veins. Infrahepatic interruption of the IVC with azygos and hemiazygos continuation is a rare finding especially when it is not associated with congenital heart disease. ...
Rationale:
Azygos and hemiazygos continuation of the inferior vena cava (IVC) is uncommon. It is rare especially when it is not associated with congenital heart disease or deep venous thrombosis.
Patient concerns:
We report an interesting case of an interstitial lung disease with an interrupted IVC with azygous and hemiazygos continuation. A 67-year-old man suffered from cough and shortness of breath.
Diagnoses:
Computed tomography revealed absence of the hepatic segment of the IVC with azygos and hemiazygos continuation. The patient did not have congenital anomalies of the remaining thoracoabdominal vasculature and viscera. The diagnosis of azygos and hemiazygos continuation of IVC was made by inferior venacavography.
Interventions:
There was no significant association between interstitial lung disease and expanded azygos and hemiazygos veins. The patient was treated with corticosteroids.
Outcomes:
After 6 months of follow-up, the patient was asymptomatic.
Lessons:
It is important to recognize the enlarged azygos vein at the confluence with the superior vena cava and in the retrocrural space to avoid misdiagnosis as a right-sided paratracheal mass or retrocrural adenopathy.
The embryology of the inferior vena cava (IVC) is complex, involving the sequential appearance and regression of multiple segments that ultimately form the IVC. Any alteration in this process during embryogenesis can result in congenital anomalies of the IVC. This study aimed to recognise common as well as rare anomalies of the IVC and associated veins, and their clinical implications. The anomalies tend to have diverse appearances based on the timing and segments involved. The development of the IVC is intertwined with the development of other veins like the renal vein, azygos vein and portal vein, and these veins may also be anomalous. Additionally, IVC anomalies are associated with various other congenital anomalies including cardiac anomalies, the recognition of which may be important for patient care. The IVC tends to have multiple normal variants and anomalies because of a complex process involving multiple segments contributing to the adult IVC. Knowledge of these variants is crucial for preoperative planning of procedures.
Contribution
This study would help in understanding the embryogenesis of the IVC and correlation with the imaging appearances and the clinical implications of each of these common as well as rare types of congenital anomalies.
Introduction. Developmental variations of the inferior cava vein (IVC) rarely cause symptoms, and they were detected during routine examinations performed for other reasons. The prevalence in the general population is between 0.07% and 8.7%. Various anomalies of the IVC can be seen depending on abnormal regression or abnormal persistence of embryonic veins. They are usually associated with more complex intracardiac and atrioventricular septal defects, partial anomalous pulmonary venous connection, and pulmonary atresia. Case Outline. We presented the 18th-month-old patient with double IVC, IVC interruption, azygos, and hemiazygos continuation associated with aortic coarctation. The vein malformation was discovered during percutaneous balloon angioplasty of the aortic re-coarctation and confirmed by using cross-sectional imaging modalities. Our patient had no symptoms of IVC malformation. Conclusion. In clinical practice, double IVC should be suspected in patients with recurrent pulmonary emboli. Another important point in practice is the identification of those anomalies to avoid potential complications of retroperitoneal surgery and cannulation during cardiac surgery
The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of .3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left-sided venous return with hemiazygos continuation in presence of a right-sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition.
