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Acute iliofemoral deep vein thrombosis may present with pain and swelling or phlegmasia cerulea dolens. When thrombosis occurs in the setting of an underlying venous obstruction, stent reconstruction should be performed after thrombus clearance to prevent rethrombosis. Stent reconstruction after thrombus clearance is associated with high technical success rates and durable patency. This report describes transient lower extremity arterial insufficiency and neurologic deficit after external iliac vein stent expansion and reconstruction within a confined space resulting from a malignant obstruction. It serves as a cautionary tale that, in rare cases, aggressive venous stenting within a confined space can transfer clinically significant forces to adjacent arteries and nerves.

Varicose veins of the neck are far less common than lower extremity varicosities. Often, neck varicosities can be a sign of a more central venous obstruction. Here, we describe a patient with no risk factors for central venous obstruction who presented with a recurrent left subclavian vein (LSV) varicosity causing significant pain and discomfort that was recalcitrant to repeated phlebectomy. Venography revealed a dilated LSV with no significant venographic stenosis in the LSV or brachiocephalic vein. Intravascular ultrasound subsequently revealed a culprit hypertrophied valve that was successfully treated with valvuloplasty, resulting in durable resolution of the patient's symptoms, suggesting that intravascular ultrasound was essential in the diagnosis and treatment of this hypertrophied valve. Keywords: Intravascular ultrasound (IVUS), Varicose veins, Hypertrophied valve, Valvuloplasty

Phlegmasia cerulea dolens is an uncommon entity. We present a case of phlegmasia cerulea dolens secondary to an aortoiliac aneurysm that compressed the common iliac vein. Catheter-directed thrombolysis was not considered to be a suitable option, because the patient needed an urgent fasciotomy. The aneurysm was treated with a bifurcated stent graft and the thrombosed veins were opened with pharmacomechanical thrombectomy and recombinant tissue plasminogen activator. The reopened iliac veins, including an aneurysmal external iliac vein, were stented and fasciotomy was performed. Pharmacomechanical thrombectomy can be performed with a low dose of recombinant tissue plasminogen activator and allows for subsequent surgery. Keywords: Phlegmasia cerulea dolens, Pharmacomechanical thrombectomy, Abdominal aortic aneurysm, Venous obstruction, Compartment syndrome

Background Proximal splenorenal shunt (PSRS) is one of the most commonly performed portosystemic shunt (PSS) in extrahepatic portal venous obstruction (EHPVO) for portal decompression. Sometimes various anatomical and surgical factors related to the splenic vein and/or left renal vein may make the construction of a PSRS difficult or impossible. Unconventional shunts are required to tide over such conditions. Methods From January 2008 to December 2018, 189 patients with EHPVO underwent PSS, of which, the 10 patients who underwent unconventional shunts form the study group of this paper. Results The ten unconventional shunts included 8 proximal splenoadrenal shunts, one collateral-renal shunt, and one inferior mesenteric vein to inferior vena cava (IMV-Caval) shunt. The mean percentage drop in omental pressure was 34.2% post-shunt with a mean anastomotic diameter of 13.7 ± 3.1 mm. Three patients experienced some form of postoperative complication. With a mean follow-up period of 32.3 months (maximum of 111 months) all patients had patent shunts on follow-up Doppler. None of the patients had variceal bleed, or features of biliopathy and hepatic encephalopathy in follow-up. Conclusion Unconventional shunts can be used safely and effectively with good postoperative outcomes in EHPVO.

Background An increase in endovenous interventions for deep venous pathologies has been observed. This article aims to provide an overview of the role of venous stenting in the management of chronic conditions affecting the deep venous system of the lower limbs, with a focus on intervention relating to the vena cava and iliofemoral venous segments. Methods An overview of the literature on the minimally invasive venous stenting procedures that are being increasingly used in the management of chronic conditions affecting the deep venous system of the lower limbs. Results We discuss key areas of interest to a venous specialist practicing in this area, including diagnostic imaging in chronic deep venous disease, with a focus on the use of intravascular ultrasound examination in this context; the treatment of chronic venous outflow obstruction, including the rationale and structural indications for stenting, current guidance regarding stent placement, and fundamental points to consider during decision-making (endophlebectomy and stenting, stenting across the inguinal ligament, optimal sizing of venous stents, extension of venous stenting to beyond the common femoral vein confluence, the role of thrombolysis useful in chronic venous disease, and arteriovenous fistulae); outcomes and initial reports of stenting; and the future of venous stents. Conclusions Deep venous stenting has become a key treatment option for chronic (thrombotic or nonthrombotic) obstructive venous disease. Dedicated venous stents and intravascular ultrasound examination represent important technological advances in the minimally invasive treatment of symptomatic chronic deep venous obstruction, which previously required open surgical reconstruction.

A 67-year-old woman presented to our vein clinic for chronic left lower extremity edema, pain, and varicosities. After failed conservative management, a computed tomography scan revealed central venous stenosis secondary to compression of the left common iliac vein by a large osteophyte along the anterolateral aspect of the L5-S1 disk space. An anterior osteophytectomy was performed, followed by iliac venous stenting at a 1-month interval. The patient had resolution of symptoms and remains symptom free at 15 months of follow-up. This report describes a spinal exostosis causing symptomatic venous compression successfully relieved by surgical decompression. Keywords: Iliac vein, Exostosis, Limb swelling, Venous obstruction

Highlights • Prognosis for patients with HCC and PVTT is historically poor. • Definitive RT may improve OS by prolonging the time to hepatic failure. • Our data suggest dose escalation may further improve OS. • Patients who received a BED >75 Gy had significantly better OS. • RT was well tolerated with no confirmed cases of radiation-induced liver disease., Background The purpose of this study is to review the results of radiation therapy (RT) for hepatocellular carcinoma (HCC) with portal venous tumor thrombus (PVTT) in a Western patient population. Methods Thirty-four patients with HCC PVTT treated from 2007 to 2014 with RT were identified. Biologically effective dose (BED) was calculated for each patient, and greater than the median dose delivered (75 Gray (Gy)) was evaluated as a potential prognostic factor. Survival was compared and independent prognostic variables were evaluated by a Cox proportional hazards regression model. Results Twenty-six patients (76.5%) exhibited a radiographic response to RT, and 10 patients (29.4%) ultimately developed local failure. Local control, liver control, distant control and OS at one year were 57.1%, 36.4%, 55.2% and 57.4%, respectively. Patients who received a BED >75 Gy had a significantly better local control at 1 year (93.3% vs 45.6%; Log Rank p = 0.0184). Patients who received a BED >75 Gy also had significantly better median survival (24.7mo vs 6.1mo) and 1-year overall survival (76.5% vs 30.0%) when compared with BED ≤75 Gy (Log-Rank p = 0.002). Conclusion Our data suggest that RT should be considered for well-selected patients with HCC and PVTT for the purpose of improving local control and potentially prolonging the time to worsening venous obstruction and liver failure. When feasible, dose-escalation should be considered with a target BED of >75 Gy if normal organ dose constraints can be safely met.

Chronic venous disease is a common condition affecting a large proportion of patients and leads to heaviness, aching, swelling, throbbing, and itching in the legs. On the extreme end, this condition can lead to lipodermatosclerosis and ulceration. Appropriate assessment of these patients with Doppler ultrasound is necessary to assess for superficial venous insufficiency and/or deep venous obstruction. Saphenous vein ablation utilizing thermal techniques such as endovenous laser ablation or radiofrequency ablation has proved effective for venous closure and reduction of venous hypertension with a low incidence of complications. Nonthermal, nontumescent options including cyanoacrylate closure (VenaSeal), mechanochemical ablation (ClariVein), and chemical ablation (Varithena) for saphenous vein closure are becoming more frequently used with high rates of effectiveness. This chapter reviews indications for treatment, technical procedural details, and potential complications of varicose vein management.

Hemoptysis may occur in patients with pulmonary venous obstruction and prominent decompressing vessels in the airways adjacent to the affected pulmonary veins. The options for treatment of hemoptysis are limited, particularly when efforts to alleviate pulmonary venous obstruction have failed. Here we describe a patient with hemoptysis associated with stenosis of the central left upper pulmonary vein and occlusion of the central left lower pulmonary vein. The left upper pulmonary vein was dilated with balloon catheters and a vascular plug was placed in the left lower pulmonary artery. Vascular engorgement regressed in the left bronchus and hemoptysis has not recurred for 4 years despite recurrence of left upper pulmonary vein stenosis. Selective occlusion of branch pulmonary arteries may be an effective option for the treatment of hemoptysis from bleeding in lung segments with inoperable pulmonary venous obstruction.

Central venous obstruction following pacemaker implantation is not uncommon, and can prove challenging in the case of system upgrade. We report a case of DDDR to CRT-P (with multi-site pacing) upgrade, where a subclavian occlusion was overcome resorting to an atrial lead extraction (using only a locking stylet). This allowed regaining of the venous access with subsequent implantation of not just one, but two new leads and subsequent successful upgrade.

Venous angioplasty with stenting of iliac veins is an important treatment option for patients suffering from post-thrombotic syndrome due to chronic venous obstruction. Interventional treatment of a chronically occluded vena cava, however, is challenging and often associated with failure. We describe a case of a chronic total occlusion of the entire inferior vena cava that was successfully recanalized using bidirectional wire access and a balloon puncture by a re-entry catheter to establish patency of the inferior vena cava.

Objective: The Pemberton sign is related to venous obstruction in patients with goiters. It has been mainly reported in patients with euthyroid and hyperthyroid goiters and is rarely seen in hypothyroidism. Methods: We present the case of a 52-year-old male with hypothyroidism due to Hashimoto thyroiditis who developed cervical compression with an impressive Pemberton sign. Results: A 52-year-old male was diagnosed with primary hypothyroidism due to Hashimoto disease based on positive peroxidase antibodies, high thyroid-stimulating hormone (TSH) and low free thyroxine (T4). His initial complaints were fatigue and neck discomfort. He was initially treated medically with levothyroxine without improvement and referred to endocrinology for further management. Physical examination at the initial endocrine visit was pertinent for an impressive Pemberton sign. Because the patient was interested in medical therapy, levothyroxine was increased and liothyronine was added. He eventually underwent total thyroidectomy due to worsening neck discomfort and breathing difficulties, which alleviated all his symptoms. Conclusions: The Pemberton sign is elicited by a simple maneuver and is very useful for identifying patients who may require thyroidectomy for compressive symptoms. Abbreviation: TSH thyroid-stimulating hormone

Because of the presence of portal cavernoma, paracholedochal and pericholedochal varices, portal cavernoma cholangiopathy (PCC) has become an entity unique to patients with extrahepatic portal venous obstruction (EHPVO). Majority of patients with these abnormalities are asymptomatic and are incidentally detected to have the presence of biliary abnormalities on cholangiography. Minority of patients present with symptoms of chronic cholestasis with or without biliary pain or acute cholangitis related most often to the presence of biliary strictures or stones. Other than the age of the patient and duration of EHPVO, presence of gall stones and common bile duct stones are other risk factors for the causation of symptoms in patients with PCC. This review summarizes the clinical characteristics of asymptomatic and symptomatic patients with PCC giving details of the prevalence of symptoms, their risk factors and overall burden of symptomatic PCC.

The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5–38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure.

Background Gastric varices (GV) are found in patients with portal hypertension. Incidence of bleeding from GV is relatively low, but is severe, and associated with higher mortality. Aims and objectives To compare the types of GV in cirrhosis vs. extrahepatic portal venous obstruction (EHPVO) and the results of endoscopic N-butyl-2-cyanoacrylate (NBC, glue) injection. Methods Four hundred and fifty-four patients undergone glue injection of GV for primary prophylaxis or control of bleeding for first episode of bleeding from GV between August 2010 and August 2015 were analyzed. Results Of 454 patients, 64% (n = 292) were cirrhotics and 36% (n = 162) had EHPVO. Types of GV were GOV1 in 16.4% (n = 48) of cirrhotics vs. 7.4% (n = 12) of EHPVO, GOV2 in 76.7% (n = 224) of cirrhotics vs. 53.1% (n = 86) of EHPVO, Isolated gastric Varices (IGV1) in 39.5% (n = 64) of patients with EHPVO vs. 6.8% (n = 20) cirrhotics. The patients were treated with NBC injections. The mean volume of glue injected was 2.89 ± 1.59 ml over a median of 1 session (range: 1–7). The total volume of glue required was lower in cirrhotics (2.44 ± 1.17 ml vs. 3.69 ± 1.91 ml, P 1 sessions of glue injection as compared to 102 (63%) of EHPVO patients. Over mean follow up of 14.7 ± 6.46 months, rebleeding (10% vs. 13%) was similar in patients with cirrhosis and EHPVO and mortality (15.4% vs. 2.5%) was higher in cirrhotics than EHPVO. Conclusion In patients with bleeding from GV, GOV2 are more common in cirrhotics and IGV1 in patients with EHPVO. Patients with EHPVO required higher total volume of glue and more glue sessions for GV obturation.

Publisher Summary This chapter discusses the venous ulcer and arterial insufficiency. Venous stasis ulceration (VSU) results from chronic venous disease. VSU in combination with peripheral arterial disease (PAD) represents a morbid condition requiring keen diagnostic and treatment skills of the health care provider. Although somewhat conflicting in its prevalence, the overall ubiquity of leg ulcers requires a methodical approach to identify all factors leading to lack of healing and recalcitrance. Patients with VSU should undergo vascular laboratory testing to confirm diagnosis of CVD and appropriately ascribe the cause of pathophysiologic dysfunction to reflux, obstruction, or both etiologies. Each patient's treatment plan must be tailored to the distribution of arterial and venous insufficiency that exists. Therefore, the health care team should be equipped to offer the full spectrum of treatment techniques in arterial revascularization, venous insufficiency, and venous obstruction. Primary recommendation of amputation should not be considered a failure in selected patients. Freedom from pain, infection, and wounds is often the quickest way to alleviate prolonged suffering.

Publisher Summary This chapter discusses the lower extremity ultrasound evaluation and mapping for evaluation of chronic venous disease. Duplex ultrasound scanning is considered the test of choice among different diagnostic tools for detailed evaluation of chronic venous insufficiency. It is very well documented that duplex ultrasound scanning is the most reliable, cost-effective, quick, and noninvasive method to identify venous reflux. This type of scanning is also able to detect venous obstruction and assess recurrence of both reflux and thrombosis. Several reports document that the type and duration of treatment and the rate of recurrence of venous ulcers are significantly different when the deep venous system is involved. Knowledge of the venous anatomy, pathology, and their variations is an important prerequisite for the person who performs the duplex ultrasound in order to identify precisely the etiology and the anatomic distribution of the venous malfunction in each patient. In patients with significant edema, dorsi/plantar flexion is performed to determine reflux in these veins. This is important because manual compression alone may not show reflux in dilated perforator veins. The common femoral vein is tested from the inguinal ligament to its union with the femoral and the deep femoral veins.

Publisher Summary Venous balloon angioplasty and stenting is a safe, relatively simple, and efficient method to treat iliocaval vein obstruction. The introduction of endovascular treatment with percutaneous stenting drastically changed the treatment and view on venous outflow obstruction. Iliac venous stenting has largely replaced surgery as the “method of choice” for treatment of venous blockage. The relative simplicity, safety, and efficacy of the intervention has refocused the interest on the role of venous outflow obstruction in patients with chronic venous disorders, and has renewed interest in the nature and pathophysiology of venous obstruction in itself and in tests for detection of hemodynamically significant lesions. However, venous stenting is still under development, and there are several issues regarding diagnosis, assessment of outcome, and selection of patients, which needs to be resolved. Venous stenting must maintain the clinical improvement and high stent patency over a longer period. Longer-term results are now appearing and the initial favorable result appears to be maintained. The long-term effects of stents in the venous system are still not fully known. Several more years of monitoring is required to assess the efficacy and safety of this therapeutic modality in venous disease. In addition, further research on understanding the nature of venous obstruction and development of reliable methods to test hemodynamic consequences are needed.

Publisher Summary The anatomic components contributing to ambulatory venous hypertension are venous valvular incompetence and luminal obstruction. It has been consistently shown that the most severe postthrombotic sequelae and the highest ambulatory venous pressures occur in patients with valvular incompetence accompanied by luminal venous obstruction. Venous obstruction is not synonymous with occlusion. Occlusion is complete obliteration whereas obstruction (for the most part) is relative narrowing of the lumen. Although relative degrees of obstruction are reliably quantitated on the arterial side of the circulation, technology has not advanced to the point that allows this degree of accuracy on the venous side. Furthermore, physicians often cannot put venous obstruction into proper perspective pathophysiologically, in terms of its contribution to postthrombotic discomfort or distal leg soft-tissue damage. Unfortunately, physiologic testing on the venous side of the circulation has not kept pace with similar advances on the arterial side of the vascular tree. Vascular laboratories have traditionally (and paradoxically) tested the hemodynamics of venous obstruction with patients in the resting, supine position with their legs elevated, which is the standard position for measuring maximum venous outflow, the commonly accepted test for venous obstruction.

Intracardiac repair for complex congenital heart defects in premature neonates with very low birth weight (VLBW) is still a challenge to pediatric cardiac surgeons. We report the successful rerouting of cardiac type total anomalous pulmonary venous return (TAPVR) in a premature newborn (36 th gestational week) with VLBW (1250 g). She had severe hypoxemia and low cardiac output despite medical treatment. Rerouting surgery of TAPVR was performed under deep hypothermia circulatory arrest at the age of 20 days. The sternum was left open and approximated 2 days later. Follow-up echocardiography showed good ventricular function without pulmonary venous obstruction. The endotracheal tube was removed 7 days postoperatively. She was then discharged without complication. In conclusion, with improved cardiopulmonary bypass technique and perioperative care, open heart surgery can be performed in premature newborns with VLBW. [ J Formos Med Assoc 2007;106(2 Suppl):S23-S26]

Publisher Summary This chapter discusses the diagnosis and treatment of major venous obstruction in chronic venous insufficiency. Endovenous therapy represents a significant advance in the treatment of chronic venous insufficiency. The clinical options for treatment of residual obstruction, following DVT, now include reconstruction of the native axial veins with stents. The evolution of the tools and techniques is ongoing because the majority of the procedures have been adapted from arterial interventions. But experience and careful observation of clinical outcomes constantly help us refine technique and patient selection. However, there are far too many patients who need treatment given the relatively few physicians who safely and willfully perform endovenous procedures. Chronic venous insufficiency is that “something old” that seems to be in a Renaissance in the midst of new pharmaceutical discoveries and the emerging field of endovenous surgery. To better prevent and treat post-thrombotic chronic venous insufficiency continued innovation, multidisciplinary efforts, and clinical evidence are needed.

Publisher Summary Symptoms of proximal chronic venous obstruction may vary greatly, ranging from moderate swelling and pain to discoloration and stasis ulcers. Symptoms are also influenced by any concomitant deep or superficial reflux. Obstruction plays an important role in the clinical expression of chronic venous disease, especially as pain. Less common causes of chronic blockage of the iliaccaval vein include benign or malignant tumors, retroperitoneal fibrosis, iatrogenic injury, irradiation, cysts, and aneurysms. Relief of symptoms is immediate, following successful stenting of malignant obstructions. The long-term outcome appears to depend largely on the progress of the tumor. Iliocaval stenosis due to retroperitoneal fibrosis has been treated successfully by stenting. Ultrasound investigation and outflow fraction determinations by plethysmographic methods have been shown to be unreliable and play only a limited role. Although abnormal plethysmography findings may indicate obstruction to the venous outflow, significant blockage may exist in the presence of normal findings. Even the invasive pressures (i.e., hand/foot pressure differential and reactive hyperemia pressure) increase and indirect resistance calculations appear insensitive, and do not define the level of obstruction.

Publisher Summary This chapter deals with subclavian vein obstruction. Subclavian vein obstruction, also called venous thoracic outlet syndrome (venous TOS), can be partial or complete. Partial obstruction is due to stenosis; complete obstruction is usually due to thrombosis. Swelling of the entire arm is the primary symptom. Swelling limited to the fingers and hand can occur with neurogenic thoracic outlet syndrome, and is not indicative of venous obstruction unless the whole arm is involved. Other symptoms are cyanosis, a feeling of fullness, aching, or pain. Some patients have mild paresthesia. Physical examination confirms swelling and cyanosis. The only other significant finding is dilated subcutaneous veins over the shoulder and upper chest wall of the affected side. The two diagnostic tools in use today are duplex scanning and venography. Venous pressure measurements can also be used, but these are cumbersome and unreliable unless there is obvious arm swelling. Finally, the approach to managing subclavian vein obstruction is three-fold—removing the thrombus, removing the extrinsic cause, and removing the intrinsic obstruction.

Many options are available to diagnose and treat patients with the Budd-Chiari syndrome who present with either thrombotic or non-thrombotic occlusion of the major hepatic veins and or vena cava. The goal of therapy is to alleviate venous obstruction and to preserve hepatic function. Low-sodium diets, diuretics, and therapeutic paracentesis are generally ineffective, except for the rare patient who presents with volume overload and incomplete hepatic venous occlusion. Anticoagulants and thrombolytics may be appropriate for selected patients with acute thrombotic venous obstruction. Percutaneous transluminal angioplasty (PTA) of hepatic venous stenoses or caval webs with or without placement of intraluminal stents yield excellent short-term results, but additional studies are warranted to assess long-term efficacy. Transjugular intrahepatic portosystemic shunts (TIPS) may be effective for patients with subacute or chronic disease and ascites refractory to sodium restriction and diuretics. Intrahepatic stents may also serve as a bridge to transplantation for selected patients presenting with fulminant hepatic failure consequent to hepatic venous occlusion. Additional studies will be necessary to assess the role of TIPS in the armamentarium of therapies for patients with the Budd-Chiari syndrome. Decompressive shunts, reconstruction of the vena cava and hepatic venous ostia, transatrial membranotomy, and dorsocranial resection of the liver with hepatoatrial anastomosis are appropriate options for patients with acute or subacute disease who are not candidates for, or fail less invasive therapies. The majority of patients benefit with improvement in liver function tests, ascites, and liver histology; however, hepatic function may deteriorate in patients with marginal reserve. Liver transplantation is reserved for patients with Budd-Chiari syndrome who present with fulminant hepatic failure or end-stage liver disease with portal hypertensive complications. Transplantation is also appropriate for patients who deteriorate after failed attempts at surgical shunting.

Publisher Summary This chapter focuses on the diagnosis of varicose thoracic veins. The veins on the chest wall may merely be unduly visible, but if they are really distended, there is probably obstruction to one or other innominate vein or to the superior vena cava; the suspicion that this is so becomes a certainty if the blood current in the distended veins can be shown to be from above downward instead of from below upward. If the distension is bilateral, and associated with edema of both arms and both sides of the neck, face, and head, it is the vena cava that is obstructed; if the distension is unilateral, with edema of the corresponding arm, but little if any of the neck or face, the obstructed vessel is probably one innominate vein. The superficial varicosity may be only slight but is sometimes extreme. In arriving at a diagnosis of the cause of the venous obstruction, malignant disease within the thorax will be uppermost in observer's mind—carcinoma of a bronchus with involvement of mediastinal glands is much the most common, though lymphoma, lymphosarcoma, and other tumors may be responsible.