A surgical method to be reminded for the treatment of symptomatic ipsilateral central venous occlusions in patients with hemodialysis access: Axillo-axillary venous bypass case report and review of the literature.

BACKGROUND: In this case report, we present two chronic hemodialysis patients with upper extremity swelling due to central venous occlusions together with their clinical presentation, surgical management and brief review of the literature. METHODS: The first patient who was a 63-year-old female patient with a history of multiple bilateral arteriovenous fistulas (AVFs) was referred to our clinic. Physical examination demonstrated a functioning right brachio-cephalic AVF, with severe edema of the right arm, dilated venous collaterals, facial edema, and unilateral breast enlargement. In her history, multiple ipsilateral subclavian venous catheterizations were present for sustaining temporary hemodialysis access. The second patient was a 47-year-old male with a history of failed renal transplant, CABG surgery, multiple AV fistula procedures from both extremities, leg amputation caused by peripheral arterial disease, and decreased myocardial functions. He was receiving 3/7 hemodialysis and admitted to our clinic with right arm edema, accompanied by pain, stiffness, and skin hyperpigmentation symptoms ipsilateral to a functioning brachio-basilic AVF. He was not able to flex his arms, elbow, or wrist due to severe edema. RESULTS: Venography revealed right subclavian vein stenosis with patent contralateral central veins in the first patient. She underwent percutaneous transluminal angioplasty (PTA) twice with subsequent re-occlusions. After failed attempts of PTA, the patient was scheduled for axillo-axillary venous bypass in order to preserve the AV access function. In second patient, venography revealed right subclavian vein occlusion caused secondary to the subclavian venous catheters. Previous attempts for percutaneously crossing the chronic subclavian lesion failed multiple times by different centers. Hence, the patient was scheduled for axillo-axillary venous bypass surgery. CONCLUSION: In case of chronic venous occlusions, endovascular procedures may be ineffective. Since preserving the vascular access function is crucial in this particular patient population, venous bypass procedures should be kept in mind as an alternative for central venous reconstruction, before deciding on ligation and relocation of the AVF.

Costoclavicular ligament as a novel cause of venous thoracic outlet syndrome: from anatomic study to clinical application.

PURPOSE: Venous thoracic outlet syndrome (VTOS) is a compressive disorder of subclavian vein (SCV); we aimed to investigate the role of costoclavicular ligament (CCL) in the pathogenesis of VTOS. METHODS: A cadaver study was carried out to investigate the presence and morphology of CCL in thoracic outlet regions, as well as its relationship with the SCV. Six formalin-fixed adult cadavers were included, generating 12 dissections of costoclavicular regions (two sides per cadaver). Once CCL was identified, observation and measurement were made of its morphology and dimensions, and its relationship with SCV was studied. To take a step further, a clinical VTOS case was reported to prove the anatomical findings. RESULTS: Two out of twelve costoclavicular regions (2/12, 16.7%) were found to possess CCLs. Both ligaments were located in the left side of two male cadavers and were closely attached to the lateral aspect of sternoclavicular joint capsules. The lateral fibers of the ligament proceed in a superolateral-to-inferomedial manner, while the medial fibers proceed more vertically. Both ligaments were tightly adherent to the SCV, causing significant compression on the vein. In the clinical case, multiple bunches of CCLs were found to compress the SCV tightly intraoperatively. After removing the ligaments, the patient's symptom kept relief during a follow-up period of 2 years. CONCLUSION: Our study demonstrated that CCL could be a novel cause of VTOS by severe compression of SCV. Patients diagnosed with this etiology could get less invasive surgical treatment by simply removing the ligament.

Novel variations in spatial relations between the facial nerve and superficial temporal and maxillary veins.

Variations in the relationship of the retromandibular vein to the facial nerve have been widely reported due to their relevance for surgical approaches in parotid, osteotomy and mandibular condyle surgery. In the context of the retromandibular retroparotid approach, remaining deep to the retromandibular vein is advised to decrease the likelihood of encountering the facial nerve during mandibular condyle surgery. In the present report, an unusual variant of the superficial temporal vein lying superficial to the facial nerve is described. This represents a variation of the venous branching pattern within the parotid gland, whereby the superficial temporal vein joins the maxillary vein inferior to its usual communication. These findings are discussed in the context of commonly used surgical approaches to the mandible for condylar trauma or osteotomy surgery.

[Síndrome de Paget-Schrötter: presentación de cuatro casos]. / Paget-Schrötter syndrome: description of four cases.

Deep vein thrombosis (DVT) of the upper limb is a rare entity, estimated to account for 10% of all cases of DVT. Classically, they are classified into primary (idiopathic, due to subclavian vein compression or exercise related) and secondary (cancer, thrombophilia, trauma, shoulder surgery, associated to venous catheters or due to hormonal causes). The Paget- Schrötter syndrome is a primary thrombosis of the subclavian vein in the subclavian-axillary junction, related either to repetitive movements or to exercise; leading to microtrauma in the endothelium with consequent activation of the coagulation cascade. Clinically, it presents abruptly with pain, edema and feeling of heaviness in the affected limb. The treatment varies from thrombolytics and anticoagulation to surgical intervention, depending on the time of evolution. We present four cases of exercise-related subclavian vein thrombosis.

Superior vena cava syndrome due to central port catheter thrombosis: a real life-threatening condition.

Superior vena cava syndrome (SVCS) represents undoubtedly a rare life-threatening condition. Herein, we present a rare case of a 69-year-old woman, with a history of hepatic flexure tumor and an indwelling central venous port, presenting with acute signs and symptoms of SVCS due to thrombosis of the catheter. The patient was treated with intravenous anticoagulation and fibrinolytic therapy and showed regression of symptoms. It is reported that central venous catheters are routinely used in clinical practice mainly in oncological cases for chemotherapy, parenteral nutrition or dialysis. However, complications related to implantation technique, care, or maintenance of these catheters may arise. High index of suspicion for SVCS should always arise when a patient presents with common symptoms and long-term central catheters, in order to avoid unfavorable outcomes. Local thrombolysis appears to be a safe and effective therapy for port catheter-associated thrombosis.

Spontaneous Subclavian Vein Thrombosis in a Healthy Adolescent Cheerleader: A Case of Paget-Schroetter Syndrome.

We present the case of a healthy 13-year-old female adolescent who developed acute progressive swelling and pain in her right upper extremity that was secondary to an acute deep venous thrombosis of her right subclavian vein. Dynamic imaging revealed subclavian vein compression at the junction of the first rib and proximal third of the clavicle consistent with Paget-Schroetter syndrome, also known as effort-related thrombosis. The compressive etiology of her thrombus was most likely related to her cheerleading activity, in which she served as the pyramid base. The patient received multimodal therapy including anticoagulation, mechanical and site-directed thrombolysis, and a first rib resection. This case illustrates that frontline providers should have a high index of suspicion for an upper extremity thrombosis in pediatric patients who present with unilateral arm swelling.

[Venous thromboembolic complications in patients with cardiovascular implantable electronic devices]. / Venoznye tromboémbolicheskie oslozhneniia u patsientov s serdechno-sosudistymi implantiruemymi élektronnymi ustroistvami.

The problem of venous thromboembolic complications (VTECs) in patients with cardiovascular implantable electronic devices (CIEDs) is extremely important today because of an annually increasing number of surgical interventions for life-threatening arrhythmias and chronic heart failure. There are hitherto no clearly defined reliable risk factors for VTECs due to heterogeneity of the available literature data. Some sources point to elevated thrombus formation in patients with a large number of electrodes, in repeat operative interventions, in the presence of a temporary pacemaker, in implantation on the left side, silicon cover of an electrode, others refute these facts. Still undetermined remains the choice of antithrombotic therapy for prevention and treatment of VTECs in this cohort of patients. Implantation of a VTEC may be accompanied by thrombosis of deep veins of the upper extremities up to the development of total occlusion of veins. In rare cases, thrombosis extends proximally, there appears superior vena cava syndrome which may require surgical treatment. Diagnosis of these diseases is complicated by their symptom-free course in the majority of cases. The most dangerous VTEC is pulmonary thromboembolism very commonly not accompanied by clinical symptomatology or taking its course under the mask of other more frequent diseases. Despite the fact that pulmonary thromboembolism with a source in the system of the superior vena cava is rarely massive, it may lead to the development of chronic postembolic pulmonary hypertension and to decreased quality of life. The article contains a review of the present-day literature and a clinical case report concerning the development of VTECs in a patient with a CIED in the form of thrombosis of the right internal jugular, subclavian and brachiocephalic veins, pulmonary embolism of small branches of the right pulmonary artery, suppuration of the pacemaker's bed and sepsis. Therapy with antibiotics, low-molecular-weight heparins, antiplatelet drugs and anti-inflammatory agents with regular sanitation of the pacemaker's bed turned out effective.

Spontaneous Upper Extremity Venous Thrombosis in a Collegiate Soccer Player: A Case Report.

Spontaneous effort-induced thrombosis is a rare but reported phenomena that was originally described over 100 years ago. The pathogenesis of this thrombosis arises from an abnormality of the thoracic outlet usually combined with a history of physical activity that includes repetitive arm motions, usually of the dominant hand. We present the case of an adolescent patient who presented to a pediatric emergency department with progressive pain, discoloration, and swelling of the shoulder of his nondominant hand. The pain became acutely worse with graying appearance of his arm. The patient was diagnosed with spontaneous thrombosis of the upper extremity extending from the left subclavian vein extending to the axillary vein. Treatment of this patient included aggressive anticoagulation, thrombolysis, and costectomy.

Bilateral Subclavian Vein Occlusion in a SAPHO Syndrome Patient Who Needed an Implantable Cardioverter Defibrillator.

A 79-year-old Asian man was hospitalized because of progressive exertional dyspnea with decreasing left ventricular ejection fraction and frequent non-sustained ventricular tachycardia. Pre-procedure venography for implantable cardioverter defibrillator (ICD) implantation showed occlusion of the bilateral subclavian veins. In consideration of subcutaneous humps in the sterno-clavicular area and palmoplantar pustulosis, we diagnosed him as having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and speculated that it induced peri-osteal chronic inflammation in the sterno-clavicular area, resulting in occlusion of the adjacent bilateral subclavian veins. An automatic external defibrillator (AED) was installed in the patient's house and total subcutaneous ICD was considered. Venous thrombosis in SAPHO syndrome is not frequent but has been reported. To the best of our knowledge, this is the first case of bilateral subclavian vein occlusion in a SAPHO syndrome patient who needs ICD implantation.

Respiratory-gated noncontrast SPACE MR angiography sequence at 3T for evaluation of the central veins of the chest: a feasibility study.

PURPOSE: To evaluate the feasibility of a respiratory-gated noncontrast magnetic resonance angiography (MRA) sequence for imaging the central veins of the chest. MATERIALS AND METHODS: Eleven healthy subjects underwent MRA of the central veins of the chest with a respiratory-gated noncontrast (SPACE) sequence. Qualitative visualization and signal homogeneity of each central venous segment were scored by two radiologists on a scale of 1-4. Signal-to-noise and contrast-to-noise ratios (SNR and CNR) were also calculated. Retrospective review of our imaging database revealed 13 patients with suspected pathology of the central veins who underwent a clinical MRA examination using the SPACE sequence as well as reference standard central venous imaging with contrast-enhanced MRA or conventional venography. RESULTS: In healthy subjects, all central venous segments demonstrated good to excellent venous visualization and homogeneity scores with the noncontrast SPACE sequence. The mean SNR and CNR of the central venous system were 192.7 and 175.0, respectively. In the 13 clinical examinations, the sensitivity and specificity for detection of stenosis or occlusions was 100% and 100% for reader 1 and 95% and 91% for reader 2, respectively. CONCLUSION: The respiratory-gated noncontrast SPACE sequence provided excellent imaging characteristics of the central veins in healthy subjects with promising diagnostic accuracy in patients with central venous pathology.

Venous thoracic outlet syndrome.

Venous thoracic outlet syndrome is a complex but rare disease that often can have excellent outcomes if quickly recognized and treated. The syndrome results from compression of the subclavian vein along its exit from the thoracic cavity and frequently affects young otherwise healthy patients. Modern diagnosis is made with a combination of clinical exam, appropriate non-invasive imaging, and, finally, contrast venography, which can be both diagnostic and therapeutic. Treatments have evolved over time to the point where patients can undergo less extensive procedures than previously performed and still maintain excellent outcomes. One of the most important predictors of outcome is the initiation of treatment within 14 days of symptoms. Hence, the importance of the accurate and prompt diagnosis of this syndrome in patients with an upper-extremity deep vein thrombotic episode cannot be further underscored. This review is a concise summary of the background and treatment algorithm for this patient population.

A Primary Primitive Neuroectodermal Tumor Arising from Left Subclavian Vein and Extending along Left Brachiocephalic Vein and Superior Vena Cava into Right Atrium.

Primitive neuroectodermal tumor (PNET) is an extremely rare malignancy thought to be derived from fetal neuroectodermal precursor cells. It usually occurs in central and peripheral nervous system or soft tissue and bone, while intravenous or intracavitary PNET is considered as an extremely rare tumor. We reported a case of a 44-year-old woman who presented with the left unilateral facial and neck swelling. Magnetic resonance imaging revealed a tape-shaped solid mass within left subclavian vein, left brachiocephalic vein, superior vena cava, and right atrium; the proximal end proportion occupied almost the entire right atrium with a pedicle flip protruded into the right ventricle. Ultrasonography revealed an irregular hypoechnoic mass arising from the left subclavian vein, which extended along the left brachiocephalic vein and superior vena cava into the right atrium and up to the right ventricle. Positron emission tomography-computed tomography revealed several hypermetabolic thyroid nodules with no evidence of intravenous hyperactive lesion. The patient underwent tumor resection under cardiopulmonary bypass. At 15 days postoperatively, total thyroidectomy and resection of the left subclavian vein were simultaneously performed. The patient received chemotherapy and radiotherapy later. Histologically, the neoplasm displayed small, round, blue cells with hyperchromatic nuclei and scant cytoplasm. The neoplastic cells showed a strong immunopositivity for CD99, synaptophysin, CD56, CD57, and friend leukemia integration 1, thus confirming a diagnosis of the PNET. Histopathological examination of the thyroid showed papillary carcinoma. Thus, this PNET had no definitive organ or tissue of origin, which primarily originated from the left subclavian vein with tumor extension along the superior vena cava to the right ventricle.

Pulmonary Embolism and Subclavian Vein Thrombosis in a Patient with Parathyroid Carcinoma: Case Report and Review of Literature.

Parathyroid carcinoma is a rare etiology of primary hyperparathyroidism responsible for 0.4 to 5.2% of all primary hyperparathyroidism cases. The overt hyperparathyroid bone or renal disease with palpable neck mass, as well as severe hypercalcemia with extremely high parathyroid hormone, are clinical parameters raising the suspicionforparathyroid carcinoma. However a definite diagnosis can be confirmed only by examining the histopathology of the tumor. The curative treatment solely depends on an en bloc surgical approach. Therefore, preoperative clinical diagnosis of carcinoma is essentialfor optimal surgical planning. Thepresent study reported asymptomatic subclavian vein thrombosis andpulmonary embolism in parathyroid carcinoma, suggesting paraneoplastic syndrome of hypercoagulability in this cancer type. The presence of this paraneoplastic syndrome in a case of overt clinical hyperparathyroidism in addition to a palpable neck mass indicated the diagnosis of carcinoma preoperatively in the present patient, which led to an en bloc surgical plan. Since this paraneoplastic syndrome can be asymptomatic, the exploration ofthis syndrome by a commonly used imaging technique for parathyroid tumor localization, computerized tomography, would enable a preoperative diagnosis of cancer especially in an equivocal situation.

Abdominal collateral vein as an unconventional vascular access for hemodialysis in patient with central vein occlusion.

A 65-year-old female patient with chronic kidney disease stage 5 and a history of spleen neoplasm with dissemination within peritoneum is presented. During 5 years of hemodialysis therapy, bilateral occlusion of brachiocephalic and iliac vein developed as a consequence of vein catheterization. An attempt to cannulate inferior vena cava was unsuccessful. A cannulation of dilated collateral abdominal veins with dialysis needles allowed to perform several hemodialysis sessions in the patient.

High-dose chemotherapy followed by autologous peripheral blood stem cell transplantation for recurrent primary mediastinal malignant germ cell tumor: a case report.

A 15-yr-old boy presented with an anterior mediastinal mass, multiple lung metastases and obstruction of the left brachiocephalic vein, the superior vena cava and the subclavian vein. Tumor biopsy by CT guidance confirmed a diagnosis of GCT. Five courses of BEP therapy were performed, and CT of the chest revealed reduction in the anterior mediastinal mass and disappearance of the multiple lung metastases. We performed the anterior mediastinal mass extraction followed by adjuvant chemotherapy consisting of ICE and TIP. However, the AFP levels became elevated soon after. Abnormal accumulation was observed in the right upper lung by DW-MRI. After the operation, two courses of TI chemotherapy and two courses of HDCT followed by auto-PBSCT were performed. He was complicated with auditory disorder and renal dysfunction. Although HDCT followed by auto-PBSCT was effective for the relapsed primary mediastinal GCT, a treatment strategy avoiding late complications is warranted.

Extravascular lipoma over the subclavian vein.

A rare instance of extravascular lipomas located over the subclavian vein in a woman in her early 60s presented unique diagnostic and therapeutic challenges due to their close proximity to critical structures. Lipomas, which are the most commonly occurring benign soft tissue tumours, are typically composed of mature adipocytes. Although lipomas can develop in any region of the body, they are most commonly found in the subcutaneous tissues of the trunk and proximal limbs. Extravascular lipomas, especially those near major blood vessels like the subclavian vein, are extremely uncommon.In this particular case, the tumour was extensively in contact with the right subclavian vein, though there was no intravascular involvement. The lipomatous tumour was successfully excised during surgery, with the subclavian vein and surrounding structures being preserved. Histopathological analysis confirmed the diagnosis of a benign lipoma. This case highlights the significance of early diagnosis, meticulous surgical planning and multidisciplinary collaboration to achieve the best possible outcomes.

Excellent response to combined radioiodine and enoxaparin in the setting of tumor venous thrombosis from differentiated thyroid carcinoma involving internal jugular and subclavian veins.

A striking clinical, radiological and tumor marker response obtained with a combined approach of radioiodine (I-131; single fraction) coupled with subcutaneous enoxaparin (a low-molecular-weight heparin) in the setting of differentiated thyroid carcinoma with jugular vein tumor thrombosis and metastatic pulmonary disease is described in this article. Pulmonary metastases, a frequent accompaniment in this group of patients, also demonstrated remarkable response with a single fraction of I-131. The proposed approach of combining targeted I-131 therapy with the antithrombotic effect of low-molecular-weight heparin in patients with thyroid cancer with documented tumor venous thrombosis is likely to produce a synergistic effect, with a dramatic clinical response that requires a minimal I-131 dose. The noninvasive nature and ease of the approach, which has an impressive response and findings, calls for administering this therapeutic option more in such patients in the future. This could lead to the development of an effective treatment strategy in these patients based upon generated evidence.

Permanent transvenous pacemaker implantation following percutaneous transluminal angioplasty in a patient with asymptomatic bilateral subclavian vein stenosis.

Subclavian venous obstruction is a disorder that arises more frequently today, due to the increased frequency of vascular interventions. It may affect one or both of the subclavian veins. When bilateral, it complicates the implantation of several devices that are preferably installed via the upper-extremity veins. Among these are pacemakers, cardiac defibrillators, catheters for haemodialysis, and even port catheters. In this study, we present a patient with symptomatic Mobitz type II AV block, who was planned to undergo a pacemaker implantation. Previously the patient had undergone two coronary bypass operations. Probably due to the interventions made at that time, he was now diagnosed with bilateral subclavian vein obstruction. Following the diagnosis, the obstruction in the right subclavian vein was successfully relieved through balloon angioplasty, after which a permanent atrioventricular pacemaker was installed.