Polysplenia with situs inversus totalis, azygos continuation of the inferior vena cava, and duplication of the superior vena cava in a healthy adult: A case report.

Polysplenia syndrome is an embryological disorder whereby the usual left-right asymmetry of thoracic and abdominal viscera fails to develop. It is a rare entity, estimated to occur at a frequency of 1 in 40,000, and is often associated with cardiac and biliary abnormalities. More than 75% of patients die before the age of 5 years, and even in the absence of cardiac anomalies, only 5%-10% of patients are expected to survive into adulthood without complications. Although polysplenia syndrome encompasses a wide range of anatomic abnormalities, there is no single pathognomonic feature. Hence, the prognosis of patients with polysplenia depends on their anatomy, thus necessitating radiology in their management. Here we present a case of a 56-year-old man with polysplenia syndrome and situs inversus totalis. This presentation is atypical because polysplenia is usually considered a form of situs ambiguus, and cases with situs inversus totalis are exceedingly rare. Also noted in our patient are variations in the great vessels, including aortic arch branches and the venae cavae which are features not typically associated with either polysplenia syndrome or situs inversus totalis. The patient is healthy and asymptomatic at baseline, with his diagnosis being made incidentally. Our case report is the first to describe this unique combination of cardiothoracic and cardiovascular anatomy. It also emphasizes the importance of radiologists in caring for patients with laterality defects. As these disorders are uncommon, more data on their anatomic variations may help provide better medical care to this patient population.

Clinical Implications of Circulating Tumor Cells in Patients with Esophageal Squamous Cell Carcinoma: Cancer-Draining Blood Versus Peripheral Blood.

Circulating tumor cells (CTCs) in cancer-draining veins have diagnostic and prognostic value. However, studies on esophageal squamous cell carcinoma (ESCC) are limited. This study aimed to compare CTCs obtained from different sampling sites (peripheral vein vs. cancer-draining azygos vein) and to investigate their association with the clinicopathological characteristics of ESCC patients. Blood samples were collected preoperatively from both veins in 40 ESCC patients at Pusan National University Hospital from June 2020 to April 2022. CTCs were detected using a centrifugal microfluidic method with fluid-assisted separation. CTCs and TWIST (+) CTCs were detected more frequently in the azygos vein blood than in the peripheral vein blood; however, the difference was not statistically significant (85.0% [34/40] vs. 77.5% [31/40], p = 0.250 and 82.5% [33/40] vs. 75.0% [30/40], p = 0.586, respectively). CTC and TWIST (+) CTC counts were significantly higher in the azygos vein blood than in the peripheral vein blood (7 vs. 3, p < 0.001, and 6 vs. 2, p < 0.001, respectively). CTCs and TWIST (+) CTCs from peripheral and azygos veins showed no association with clinicopathological characteristics. Further large-scale studies are needed to clarify their role as predictive biomarkers for prognosis and chemotherapy responses in ESCC patients.

[Thoracoscopic resection of azygos vein aneurysm]. / Torakoskopicheskoe udalenie anevrizmy dugi neparnoi veny. Redkoe klinicheskoe nablyudenie.

Azygos vein aneurysm is a rare thoracic disease that often mimics posterior mediastinum tumors. Pathogenesis of azygos vein aneurysm is unclear. Discussions about possible causes are still ongoing. Most aneurysms are asymptomatic and diagnosed incidentally during standard examinations. Severe complications of azygos vein aneurysm include rupture with massive bleeding and pulmonary embolism. Contrast-enhanced chest CT and MRI are essential. Differential diagnosis includes tumors, cysts and rare neoplasms of the mediastinum. Treatment algorithm is not established. There are traditional surgical and endovascular methods. We present minimally invasive surgical treatment of azygos vein aneurysm in a 53-year-old woman. The patient underwent thoracoscopic resection of aneurysm. Histological examination revealed a partially thrombosed azygos vein aneurysm.

Radiological features and diagnostic pitfalls of idiopathic azygos vein aneurysm: A case report.

INTRODUCTION AND IMPORTANCE: We present a case of idiopathic azygos vein aneurysm (AVA) and describe its radiological features. Clinicians should be aware of the imaging findings to avoid the risk of bleeding caused by biopsy. CASE PRESENTATION: An asymptomatic 46-year-old woman was found to have an abnormal shadow on a chest computed tomography (CT) scan during a medical checkup. Plain CT revealed a well-defined, homogeneous mass on the posterior side of the right main bronchus. Contrast-enhanced CT revealed a mass with marked enhancement pointing to a vascular structure. Three-dimensional reconstruction showed that the mass was connected to the azygos arch, and inflow to and outflow from the azygos vein was detected. Subsequently, video-assisted thoracic surgery was performed. CLINICAL DISCUSSION: Patients with idiopathic AVA often present with accidental findings of a mediastinal or lung mass on a chest radiographs or CT scans, which can be mistakenly diagnosed as a paratracheal tumor, bronchial cyst, or posterior mediastinal tumor. Needle biopsy should be avoided due to the risk of massive bleeding. During the evaluation of thoracic malignancies, AVAs should be considered in the differential diagnosis, especially in area of the right tracheobronchial angle or right upper or posterior mediastinum. Three-dimensional reconstruction can help surgeons to clarify the disease diagnosis. CONCLUSION: Imaging findings combined with enhanced CT and three-dimensional reconstruction are useful for diagnosing AVAs.

[Surgery for complicated accessory lobe of the azygos vein]. / Khirurgiya oslozhnennoi dobavochnoi doli neparnoi veny.

Congenital anomalies of respiratory system are quite diverse and not all of them are subject to surgical treatment. One example is accessory lobe of the azygos vein. This anomaly usually has no clinical manifestations and requires only follow-up, as well as attention in surgery on the right half of the chest for some other disease. This situation changes when complications occur, for example, purulent-inflammatory process. Therapy is not always effective, and lung tissue destruction requires surgical treatment. Progressive destruction complicates diagnosis and choosing surgical tactics. We present a rare case of severe purulent-inflammatory complication with abscess in accessory lobe of v. azygos. Anatomical abnormalities following this congenital pulmonary anomaly can cause difficulties in surgeries for other intra-thoracic diseases. The situation is especially relevant for thoracoscopic access. This report will be useful for radiologists, pulmonologists and thoracic surgeons.

Peripherally inserted central catheter malposition into the azygos vein: A case series report and review of the literature.

BACKGROUND: Peripherally inserted central catheters (PICCs) are frequently utilized for extended intravenous treatments. However, complications such as incorrect placement into the azygos vein can result in significant health issues. A thorough understanding of these aspects is crucial to enhance the safety and effectiveness of PICC procedures, thereby improving patient care outcomes. METHODS: The research was conducted on a cohort of 12 patients who experienced PICC malposition into the azygos vein. Systematically reviewed information on classification, imaging manifestations, potential influencing factors, and methods for identification, prevention, and management. RESULTS: The analysis uncovered two PICC malposition classifications within the azygos vein, each with unique imaging characteristics. It also pinpointed several factors influencing malposition, offering valuable insight into potential risks. Moreover, it established effective detection methods, underscoring the significance of imaging techniques. Additionally, it outlined strategies for preventing and managing PICC malposition in the azygos vein, enhancing overall comprehension of the issue. CONCLUSIONS: The findings emphasize the importance of taking a proactive stance to avoid malposition and stress the necessity of prompt and precise intervention when malposition does happen, thereby enhancing the safety and effectiveness of PICC processes.

On the way to the azygos vein: a road of return rather than ruined.

BACKGROUND: The malposition of central venous catheters (CVCs) may lead to vascular damage, perforation, and even mediastinal injury. The malposition of CVC from the right subclavian vein into the azygos vein is extremely rare. Here, we report a patient with CVC malposition into the azygos vein via the right subclavian vein. We conduct a comprehensive review of the anatomical structure of the azygos vein and the manifestations associated with azygos vein malposition. Additionally, we explore the resolution of repositioning the catheter into the superior vena cava by carefully withdrawing a specific length of the catheter. CASE PRESENTATION: A 79-year-old female presented to our department with symptoms of complete intestinal obstruction. A double-lumen CVC was inserted via the right subclavian vein to facilitate total parenteral nutrition. Due to the slow onset of sedative medications during surgery, the anesthetist erroneously believed that the CVC had penetrated the superior vena cava, leading to the premature removal of the CVC. Postoperative contrast-enhanced computed tomography of the chest confirmed that the central venous catheter had not penetrated the superior vena cava but malpositioned into the azygos vein. The patient was discharged 15 days after surgery without any complications. CONCLUSIONS: CVC malposition into the azygos vein is extremely rare. Clinical practitioners should be vigilant regarding this form of catheter misplacement. Ensuring the accurate positioning of the CVC before each infusion is crucial. Utilizing chest X-rays in both frontal and lateral views, as well as chest computed tomography, can aid in confirming the presence of catheter misplacement.

Surgical resection of an intraluminal tumor in the azygos vein with an unknown primary site causing superior vena cava syndrome.

Intraluminal tumor in the azygos vein is a rare disease that can cause superior vena cava (SVC) syndrome. Radiotherapy and endovascular stenting with or without chemotherapy are reported to have a high clinical success rate for the management of SVC syndrome with malignancy, but a poor survival rate. Here, we report a 69-year-old man who presented with swelling of the face and upper extremities, who was diagnosed with SVC syndrome caused by an intraluminal tumor in the azygos vein. Enhanced chest computed tomography revealed an intraluminal mass with a filling defect from the azygos vein to the SVC, with no extravascular extension or dissemination of the primary tumor. Surgical resection of the mass en bloc with the azygos vein and SVC reconstruction was performed. A poorly differentiated carcinoma was diagnosed on postoperative pathological evaluation. Twelve months after resection, the patient was well with no signs of recurrent disease. This case highlights that surgical resection should be considered as a treatment of choice for the management of SVC syndrome caused by an intraluminal malignancy in the azygos vein.

Successful resection of a primary angiosarcoma of the azygos vein: A case report.

A 63-year-old woman was admitted to our department for the investigation of superior vena cava (SVC) syndrome. Computed tomography revealed an azygos tumor extending into the SVC. Video-assisted thoracic surgery (VATS) was performed to remove the distal end of the azygos vein in the left lateral position, followed by complete resection of the entire tumor under median sternotomy in the supine position. The histological diagnosis was a primary angiosarcoma of the azygos vein. The patient was discharged without any complications and is now alive and tumor-free 24 months after surgery. In addition, contrast-enhanced computed tomography revealed no graft occlusion in the two reconstructed brachiocephalic veins. Thoracoscopic surgery in the lateral position is useful for safe and reliable complete resection of a tumor arising from the azygos vein.

Azygos Vein Pseudoaneurysm After Trauma: A Case Report.

High-speed motor vehicle collisions (MVCs) often result in severe musculoskeletal, neurological, and vascular injuries. Among these, azygos vein pseudoaneurysms (AVPs) are a rare and potentially life-threatening vascular complication. Our case study highlights an instance of an AVP arising from a high-velocity MVC, underscoring their critical significance in trauma scenarios. Additionally, this report delves into the complexities of managing AVPs, both traumatic and idiopathic, emphasizing the urgent need for intervention and the intricacies of their treatment.

CT Findings of Azygos Venous System: Congenital Variants and Acquired Structural Changes.

The azygos venous system is a crucial conduit of the posterior thorax and potentially vital collateral pathway. However, it is often overlooked clinically and radiologically. This pictorial essay reviews the normal azygos venous anatomy and CT findings of congenital variations and structural changes associated with acquired pathologies.

A 4-year tunneled hemodialysis catheter malpositioned into the azygos vein and how to remedy the hemodialysis circuit.

Mispositioning in the azygos vein is a rare but hazardous complication of central venous catheterization. A patient was admitted for a dysfunctional hemodialysis tunneled cuffed catheter (TCC) placed in the azygos vein for 4 years. Computed tomography angiography revealed multiple sites of occlusion, including the superior vena cava (SVC), right and left innominate veins (IVs), and right femoral vein. Percutaneous transluminal angioplasty and a TCC replacement based on a segment-by-segment recanalizing strategy were performed. First, an 8-Fr sheath was inserted through the left femoral vein approach to retrogradely traverse the occlusive SVC followed by a guidewire extending to the occlusive left IV. A left transjugular 15-cm snare was inserted to capture the transfemoral guidewire and achieve recanalization from the left IV to the SVC. Second, a transjugular guidewire was advanced through the dysfunctional TCC yet shunted into the left IV due to the inability to cross the SVC. A left transfemoral 15-cm snare was inserted to capture the guidewire and achieve complete recanalization from the right internal jugular vein to the SVC. Balloons were passed over the guidewires to dilate the obstructive lesions sequentially, and a new TCC was inserted successfully with the tip positioned in the right atrium.

Azygos vein stenting as an alternate route for inferior vena cava obstruction.

Chronic venous insufficiency, caused by inferior vena cava occlusion, can lead to thromboembolic complications and tissue loss. We present two cases of azygos vein stenting (AVS) in which vena cava recanalization techniques were exhausted. In the first case, the left iliac vein and vena cava were recanalized and stented; however, the right iliac vein had been previously resected and required AVS. Conventional recanalization attempts from the right and left iliac systems failed in the second patient with congenital inferior vena cava occlusion; therefore, AVS was chosen to establish in-line drainage.

Azygos vein preservation revisited: impact on early outcomes after repair of esophageal atresia/tracheoesophageal fistula in newborns.

Since the first successful repair of esophageal atresia/tracheoesophageal fistula (EA-TEF) was performed approximately 8 decades ago, surgeons have made considerable technical advances in solving intraoperative surgical challenges and reducing postoperative complications. According to some surgeons, preserving the Azygos vein makes this modification attractive. This study aimed to evaluate the benefits of preserving the Azygos vein during surgery for esophageal atresia with tracheoesophageal fistula and to highlight its advantages in reducing anastomotic leak, stricture, and other postoperative outcomes. This prospective comparative series was conducted between April 2020 and April 2023. The study included all newborns with EA-TEF eligible for primary repair. Patients were randomized to either Group A or B. Group A underwent Azygos vein preservation, whereas the remaining patients (Group B) underwent Azygos vein disconnection. Sixty-four patients were included in this study. Thirty-two patients (Group A) underwent Azygos vein preservation during EA-TEF repair, and the remaining thirty-two patients (Group B) underwent Azygos vein ligation and disconnection. Both groups were comparable in terms of demographics, clinical data, and operative findings (P > 0.05). Pneumonitis occurred in 4 patients in Group A and 16 patients in Group B. Anastomotic leaks occurred in two (6.2%) patients in Group A and six (18.7%) patients in Group B. There were two deaths in Group A and six deaths in Group B, with a significant difference between the two groups (P = 0.0485). Preserving the Azygos vein during esophageal atresia repair reduces the occurrence of postoperative pneumonia, leakage, and stenosis, and decreases postoperative mortality. Therefore, we declare that this modification is a significant and valuable addition to the current surgical procedures.

Successful left ventricular lead placement by way of an azygos vein for a patient performing cardiac resynchronization therapy implantation with an occluded left subclavian vein: A case report.

Central venous obstruction following pacemaker implantation is not uncommon and can prove challenging in the case of a system upgrade to a cardiac resynchronization therapy pacemaker (CRT-P). We describe the case of a patient who underwent a successful upgrading procedure of a pacemaker to a CRT-P in the presence of an occluded left subclavian vein and superior vena cava, using collateral veins that drained into right atrium.

Variations of azygos vein: a cadaveric study with clinical relevance.

The azygos vein can be formed as a single root, two roots, and three roots, namely lateral, intermediate and the medial roots respectively. The hemiazygos vein and the accessory hemiazygos vein are the tributaries of azygos vein rather than its left side equivalents. Its variations, especially in young persons without any relevant risk factors, may result in thromboembolic illness. This study aimed to describe the morphological and morphometric variations of azygos system of veins. The present study was conducted on thirty formalin fixed adult human cadavers by dissecting azygos vein from formation to termination and variations were noted. The azygos vein was formed by a single root in 56.7%, by two roots: the lateral root and intermediate root in 36.7% cases and by the lateral root and medial root in 6.6%. The vertebral level of termination of azygos vein was seen at the level of T4 vertebrae in 70% cases, at the level of T3 vertebrae in 20% of cases and at the level of T5 vertebrae in 10% cases. The course of azygos vein was varying in 13.3%. These morphological variations can be useful while performing mediastinal surgery, mediastinoscopy, surgery of the deformations of the vertebral column, neurovascular surgeries of the retroperitoneal organs, disc herniation and fracture of thoracic vertebrae.

Morphologic variations of the superior intercostal vein: An anatomical study with clinical applications.

This study aimed to validate and compare the anatomical variations of the superior intercostal veins, focusing on their origin, course, anastomoses, and destination. In addition, the results were compared with findings from other relevant studies. Fifty Korean and 16 Chinese adult cadavers were dissected for this study. The superior intercostal veins were dissected and measured. In our study of 66 specimens, the right superior intercostal vein was observed in 92.3% of cases, while the left superior intercostal vein was observed in 50%. The right superior intercostal vein was subdivided into six types based on its composition, which mainly drained the second and third right posterior intercostal veins. Similarly, the left superior intercostal vein was subdivided into eight types, primarily involving the second to fourth left posterior intercostal veins. This detailed anatomical study successfully identified and classified the various morphologic types of the superior intercostal vein and reviewed the clinical significance of this vein. The findings of this study can offer valuable anatomical evidence to physicians, aiding in their understanding and utilization of the superior intercostal vein.

Implantation of an arteriovenous graft through the peripheral vascular access in the presence of superior vena cava stenosis but a patent azygos vein: A case report.

Long-term indwelling central venous catheters (CVCs) may cause complications, such as central venous disease (CVD). CVD eliminates the possibility of establishing new arteriovenous access. We herein describe a case in which an arteriovenous graft (AVG) is implanted through the peripheral veins and blood flows back to the patent azygos vein, secondary to superior vena cava stenosis. This shows that if the CVD is correctable or the collateral circulation is an alternative vascular pathway that allows blood to return back to the right atrium, the internal fistula flow is well-balanced with the collateral circulation and there are no clinical symptoms of CVD, an arteriovenous access can be established for the operation. This provides a viable, long-term vascular access option for hemodialysis patients with central venous resource depletion.

Cadaveric dissection of connection between accessory hemiazygos vein and left brachiocephalic vein.

PURPOSE: The azygos system of veins has many anatomical variations that can impact mediastinal and vascular interventions. While radiological reports on these are of great clinical value, this study is among the first to present a high-quality cadaveric dissection of a rare anatomical variant to supplement previously published radiologic studies. The azygos venous system consists of the azygos vein (AV), hemiazygos vein (HAV), and the accessory hemiazygos vein (AHAV), which develop from the last portion of the posterior cardinal veins. The normal anatomical configuration includes drainage of the posterior intercostal veins, vertebral vein, esophageal veins, HAV, and AHAV to an unpaired right-side AV at the level of the 8th/9th thoracic vertebra. The reported incidence of AHAV draining directly into the left brachiocephalic vein is 1-2%. METHODS: An adult formalin-fixed 70-year-old female cadaver was dissected as part of a medical gross anatomy elective course. RESULTS: Gross documentation of a direct connection of the HAV to the AHAV with the AHAV draining into the left brachiocephalic vein. CONCLUSION: It is important to note the variations of the azygos system to avoid confusion with a potential pathology such as mediastinal masses. Understanding of the rare variant reported here could be useful in the prevention of iatrogenic bleeding from the misplacement of venous catheters and help facilitate radiological diagnosis in the incidence of venous clot formation.