Cava Obstruction Research Articles

Intracardiac Tuberculoma in the Immunocompromised Population – A review

PurposeTo understand the peculiar factors which guide the diagnosis of intracardiac tuberculoma especially in sick children with altered immunity.MethodLiterature review of scientific papers in Med‐line/Pub Med, and other popular search engines.ResultsTuberculosis (TB) is a contagious airborne disease. WHO ranks TB alongside HIV/AIDS as a leading cause of death worldwide. [1] At least one‐third of people living with HIV worldwide in 2014 were infected with TB bacteria. HIV patients are 20 to 30 times more likely to develop active TB disease. It is estimated that pediatric cases account for 10–15% of the global TB. Majority of them occur in infants and children under 5 years of age. [2] Diagnosis of TB in children is more difficult than in adults, because few signs are associated with primary infection, interferon‐gamma release assays (IGRA) and tuberculin skin test (TST) are less reliable in younger children.[3] In the pediatric age, the prodromal stage is often very short, and the risk of progression to active disease is higher (30–40% in those younger than 1 year of age) and children (24% in 1–5 years of age) compared to the subsequent ages if treatment is inadequate. Following the inhalation of mycobacteria, innate immunity controls infection in immune‐competent patients. [3] Children are prone to develop extra‐pulmonary TB; about 4% of children infected under the age of 5 years, develop tubercular meningitis or miliary TB. [4] Cardiac tuberculosis is usually found at postmortem examination. Endocardial tuberculoma is extremely rare, found in only 0.14% of autopsy cases. Studies carried out before the introduction of specific anti‐tuberculous therapy assert that the myocardium is involved in less than 0.30% of patients dying of tuberculosis. [5] The myocardium might be affected by direct spread from the mediastinal gland, by the lymphatic routes, or the bloodstream. Organ involvement with M. tuberculosis infection was classified as being miliary or nodular. Nodular myocardial tuberculosis might develop into a ventricular aneurysm. [6] A tuberculoma is a clinical manifestation of tuberculosis which conglomerates tubercles into a firm lump, and so can mimic cancer tumors of many types in medical imaging studies. [7] Since these are evolutions of primary complex, the tuberculomas may contain within caseum or calcification. They can affect any organ such as the brain, intestines, ovaries, breast, lungs, esophagus, liver, pancrease, bones, the heart and many others. As the histological and clinical indications, as well as tumor markers such as CA‐125, are similar, it is often difficult to differentiate tuberculoma from cancer. For these reasons, tuberculosis should always be considered in differential diagnosis of cancer. [8] Intracardiac tuberculomas may be localized at any part of the heart. They were found at the proximal superior vena cava and the right atrium in a 17year old male patient initially diagnosed as angiosarcoma or rhabdomyosarcoma. [9] “Clinically, tuberculomas may be asymptomatic or may present with arrhythmias, complete heart block, congestive heart failure, superior vena caval obstruction, right ventricular outflow obstruction, aortic insufficiency and sudden cardiac death”. [11]ConclusionsIntracardiac tuberculoma is a rare finding, often diagnosed in patients with miliary tuberculosis. The increased prevalence of HIV/AIDS and other immunosuppressive conditions lead to their emergence among infants and adults with tuberculosis.Support or Funding InformationAPS Minority Travel Award (applied)This abstract is from the Experimental Biology 2018 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

Abstract No. 634 Efficacy of endovascular stenting for malignant versus nonmalignant caval obstruction

To assess factors associated with symptom resolution after endovascular stenting for superior or inferior vena cava syndrome. Eighty-six consecutive vena cava stent placements in 82 patients (53 ± 14 years old) at a single institution were reviewed for patient demographics, comorbidities, and durability of stent patency (also evaluated were persistent/recurrent symptoms, stent occlusion, and need for repeat stenting). Logistic regression was used to identify independent factors associated with stent patency, and phi coefficients and analysis of variance were used to compare cases subdivided by lesion location (superior vena cava, inferior vena cava) and the presence or absence of malignancy. Clinical follow-up was available in 77/86 (90%) cases. Technical success with clinical failure (persistent symptoms) occurred in 40% of these cases with a median follow-up of 67 (interquartile range, 14-570) days and mortality rate of 63% during this period. Malignant obstructions had a significantly higher clinical failure rate of 54% compared to 15% for non-malignant obstructions (Φ = 0.34, p = 0.002). However, only metastatic disease was independently associated with clinical failure when controlling for demographics, other comorbidities, and differential follow-up: adjusted odds ratio (aOR) and 95% confidence interval of 8.27 (2.79–24.50). Vena cava stenting effectively resolves symptoms without need for repeat interventions in 85% of non-malignant obstructions compared to only 46% of malignant obstructions. Patients should be counselled accordingly, and those with malignant obstructions may require closer follow-up to evaluate the need for reintervention and goals of care.

Stented CorMatrix® conduit to bypass benign superior vena caval obstruction.

Surgical venous bypass is an effective treatment option in the management of benign superior vena caval obstruction. Here, we present CorMatrix® as a novel conduit choice, supported internally by an endovascular stent in an attempt to prevent stenosis and reduce reintervention rate following surgical bypass.

The collateral venous system in late pregnancy: A systematic review of the literature.

Recent literature has reported an association between maternal supine sleep position and stillbirth during late pregnancy. In this position the gravid uterus almost completely obstructs the inferior vena cava. A small number of women experience supine hypotension, thought to be due in part to inadequate collateral venous circulation. The aim of this paper is to review the literature describing the anatomy of the collateral venous system and in particular the azygos system, the abdominal portion of which has not been well studied. A systematic review was conducted using the electronic databases: Medline, Embase, Scopus, and Google Scholar. Relevant anatomical and radiological literature concerning the azygos system in particular was reviewed. The search was limited to adult human studies only. The collateral venous system can be divided into superficial, intermediate and deep systems. The azygos system in particular provides immediate collateral venous circulation in the event of acute inferior vena caval obstruction. The abdominal portion of this pathway, including the ascending lumbar vein, has not been well studied and there are certain variations that can render it ineffective. In conclusion, the collateral venous system provides an alternative route for blood to flow back to the systemic circulation when acute occlusion of the inferior vena cava occurs in the supine position during late pregnancy. However, certain anatomical variations can render this pathway ineffective, and this could have implications for the development of supine hypotension and stillbirth in late pregnancy. Clin. Anat. 30:1087-1095, 2017. © 2017 Wiley Periodicals, Inc.

Endovascular Management of Malignant Inferior Vena Cava Syndromes.

Malignant obstructions of the inferior vena cava (IVC) are a common cause of the IVC syndrome. As reports on interventional treatment of malignant inferior caval obstructions are very sparse, the purpose of this study was to retrospectively assess the outcome of endovascular treatment of symptomatic, malignant IVC syndromes. Between 2000 and 2015, 19 patients (six women; mean age 59years±14) received endovascular treatment of malignant IVC obstruction/occlusion. Patients' demographics as well as interventional and clinical outcome data were collected. All 19 patients underwent stenting of the IVC. Technical success was 100%. Clinical success was 79% (15/19). Three patients (16%) required early re-intervention (days 1-8) due to stent compression. Three patients (16%) with initially good post-interventional results required late repeated intervention due to tumor progression, and repeated intervention could alleviate symptoms in all cases. Best results were achieved when choosing a stent diameter between 16 and 20mm and a stent length approximately 15-20mm longer than the lesion length. Too large stent diameters (>28mm) can lead to stent compression, too small stent diameters (<14mm) can lead to stent migration, and too short stent lengths can lead to a reoccurrence of symptoms with obstruction of a non-treated segment. Endovascular treatment of malignant IVC syndromes is a safe and effective approach enabling immediate relief of inferior inflow congestions. Recurrent venous obstruction is common but can be avoided when stent diameter and stent length are adapted to the degree of IVC compression as well as expected progression of the underlying malignancy.

Metastatic Renal Cell Carcinoma With Hepatic Dysfibrinogenemia—A Case Report

Background and Aim: Anaemia and edema have been shown to be poor prognostic factors in advanced malignancy. Thrombocytopenia, functional factor deficiency and accelerated fibrinolysis are possible hepatic causes of abnormal bleeding. Secondary dysfibrinogenemia due to increased fibrinogen sialylation which impairs the ability to form fibrin polymers is seen in advanced liver disease and renal cell carcinoma. Intravenous albumin infusion has been used to prevent paracentesis induced circulatory dysfunction (post LVP) and provide nephroprotection by improving microcirculation. Intravenous albumin and terlipressin have been used in hepatorenal syndrome due to malignant infiltration, which is often considered the functional equivalent of cirrhosis. Case Summary: 27-year-old male, a known case of metastatic renal cell carcinoma with distant spread to the liver and spleen with history of malignant ascites (post therapeutic paracentesis) presented with complaints of haematuria (multiple episodes). Examination findings revealed anasarca, hepatosplenomegaly and inferior vena caval obstruction. Coagulation profile was suggestive of an elevated PT and INR. Component transfusion with fresh frozen plasma (FFP) or cryoprecipitate and intravenous albumin infusion was considered. He showed clinical improvement after receiving tranexamic acid and three units of FFP. Conclusion: Management of bleeding and preservation of hepatic function form an important component of supportive management. Presence of active bleeding and need for interventional procedures are indications for component transfusion. Eight to ten units of cryoprecipitate may be required in severe liver disease. Intravesical installation of tranexamic acid, endoscopic ligation of the bleeding vessel, application of local haemostyptic agents and application of adrenaline soaked gauze have been to control bleeding. Role of intravenous albumin infusion in advanced cancer need to be evaluated further. Management of hepatic encephalopathy (in the absence of well defined cirrhosis) also needs to be discussed. The author has none to declare.

Hepatic vena cava syndrome: New concept of pathogenesis.

Hepatic vena cava syndrome, also known as membranous obstruction of inferior vena cava (IVC), was considered a rare congenital disease and classified under Budd-Chiari syndrome. It is now recognized as a bacterial infection-induced disease related to poor hygiene. Localized thrombophlebitis of the IVC at the site close to hepatic vein outlets is the initial lesion which converts on resolution into stenosis or complete obstruction, the circulatory equilibrium being maintained by development of cavo-caval collateral anastomosis. These changes persist for the rest of the patient's life. The patient remains asymptomatic for a variable period until acute exacerbations occur, precipitated by bacterial infection, resulting in deposition of thrombi at the site of the lesion and endophlebitis in intrahepatic veins. Large thrombus close to hepatic vein outlets results in ascites from hepatic venous outflow obstruction, which is followed by development of venocentric cirrhosis. Endophlebitis of intrahepatic veins results in ischemic liver damage and development of segmental stenosis or membrane. Acute exacerbations are recognized clinically as intermittent jaundice and/or elevation of aminotransferase or ascites associated with neutrophil leukocytosis and elevation of C-reactive protein; sonologically, they are recognized as the presence of thrombi of different ages in IVC and thrombosis of intrahepatic veins. Development of liver cirrhosis and hepatocellular carcinoma is related to severity or frequency of acute exacerbations and not to duration or type of caval obstruction. Hepatic vena cava syndrome is a common co-morbid condition with other liver diseases in developing countries and it should be considered in differential diagnosis in patient with intermittent elevation serum bilirubin and or aminotransferase or development of ascites and cirrhosis.

The Technical Evolution and Procedural Outcomes of the Contemporary Hybrid Operative Procedure for Incapacitating Post-Thrombotic Iliofemoral and Inferior Vena Caval Obstruction

Chronic, post-thrombotic iliofemoral and inferior vena cava (IVC) obstruction is associated with debilitating post-thrombotic morbidity. Whereas balloon venoplasty and stenting of the post-thrombotic iliac veins are often successful, these procedures performed in the presence of a diseased or occluded common femoral vein (CFV) are frequently associated with incomplete recanalization and failure. Open surgical CFV endovenectomy is designed to provide venous drainage from the involved limb, to restore venous inflow to a recanalized iliocaval segment, and to provide a nondiseased landing zone for the iliac vein stent, thereby improving procedural success and reducing the severity of the post-thrombotic syndrome. During the past 8 years, the procedure has evolved to one in which success can be anticipated and complications and failure minimized. Thirty-one patients undergoing CFV endovenectomy and proximal endoluminal reconstruction (iliac, IVC) have been analyzed. The initial techniques of patient management were compared with the present techniques, evaluating procedural complications and failures. The initial procedure consisted of preoperative venography, CFV endovenectomy, patch venoplasty, and intraoperative passage of a guidewire into the patent IVC followed by venoplasty and stenting, with postoperative systemic anticoagulation. Iliac vein rupture was treated with a stent graft. The contemporary procedure begins with preoperative venography with guidewire/catheter advancement into the IVC the day before the operative procedure. Patients receive preoperative combined platelet inhibition. The following day, the patient is operated on. This procedure consists of a complete CFV endovenectomy, patch venoplasty, intraoperative balloon venoplasty and stenting of the IVC (if necessary) and iliac veins, landing of the stent into the endovenectomized CFV above the saphenofemoral junction, completion intravascular ultrasound, construction of a small (4-mm) distal CFV-arteriovenous fistula, low-dose regional anticoagulation with unfractionated heparin through a popliteal vein sheath, conversion to oral anticoagulation with warfarin, early ambulation, and indefinite oral anticoagulation. Ruptured iliac veins are now treated with a second stent relining the first. Of the 17 patients, 9 (53%) treated with the early technique had major complications: 4 iliofemoral thromboses, 3 major wound bleeds, and 2 wound infections. One iliac vein rupture treated with a stent graft thrombosed. Of the 24 patients treated with the contemporary techniques, 2 (8%) had procedural complications, 1 seroma and 1 wound infection. One iliac vein rupture, treated with a second stent relining the first, remains patent. The contemporary hybrid operative procedure for incapacitating post-thrombotic iliofemoral/venacaval obstruction increases procedural success and reduces complications compared with the initial technique. The contemporary technique is preferred for all patients undergoing hybrid operative management of iliofemoral/venacaval post-thrombotic occlusion involving the CFV.

Superior mediastinal masses in children – two cases of lymphoma

&lt;div&gt;&lt;p&gt;The exact incidence of superior mediastinal masses in children is largely unknown. They present as a spectrum of disease ranging from an incidental finding on a Chest X-Ray to being markedly symptomatic with superior vena caval syndrome or obstruction of the upper airways. Lymphomas seem to be the most common causes of superior mediastinal masses in children. We present two cases of confirmed T cell lymphoma in children with superior mediastinal masses. In doing so, we explore a diagnostic approach and visit the complications the physician needs to be aware of when confronted with a child with a superior mediastinal mass.&lt;/p&gt;&lt;/div&gt;

Hernia of the diaphragmatic caval foramen causing right atrial “mass”, caval obstruction and pulmonary embolism

Hernia of the diaphragmatic caval foramen causing right atrial “mass”, caval obstruction and pulmonary embolism

Mediastinal Mass with Hyper- eosinophilia in a Young Boy -A Diagnostic Dilemma

Mediastinal masses in children comprises of a heterogeneous group of tumours. In such cases, biopsy and histological analysis are mandatory for planning of treatment. We have reported an unusual aetiology for a mediastinal mass in a young boy presenting with features of Superior Vena Caval Obstruction (SVCO) who also had marked blood and marrow eosinophilia mimicking Chronic Eosinophilic Leukaemia (CEL). We have also discussed the differential diagnoses of mediastinal tumours with hyper-eosinophilia and possible therapeutic implications.

0168: Occlusion of superior vena cava due to a pacing lead after an electrification

Transvenous pacing is a relatively safe treatment with a low complication rate, but serious thromboembolic complications have been reported to occur in 0.6% to 3.5% of cases. Superior vena cava obstruction syndrome is generally an uncommon but serious complication occurring in <0.1% of patients. A 28-year-old lady with history of DDD permanent pacemaker implantation secondary to a cardiac surgery (mitral and aortic valves replacements). She was unfortunately electrified occasioning a threshold elevation. An attempt to implant a new endocavitary lead failed, the venography showed a partial occlusion of the right subclavicular vein and a total occlusion of superior vena cava with supleance, confirmed by the angioscanner. Her ancient leads were already functional; we replace only the pulse generator. General examination doesn’t revealed features suggestive of superior vena caval obstruction which was later confirmed by imaging. She was treated by continuing her long term oral anticoagulation. A review of the literature suggests that neither thrombotic nor fibrotic obstruction in patients with pacemaker leads is strictly related to the number of abandoned leads, the presence of severed leads, or the time elapsing from pacemaker implant. In our case despite anticoagulation for mechanic valve replacement the occlusion of the VCS occurred. Cardiac surgery, traumatic placement of ancient leads and latest electrocution probably contributed to this fact. Superior vena cava obstruction in patients with transvenous pacing leads, although rare, is a well recognized complication. One should carefully look for thromboembolic complications during follow-up in patients with transvenous pacemaker leads, as it has implications for future management and carries significant morbidity and mortality.

Severe Vascular Complications Due to Liver Hydatid Cyst Relapse: A Case Report and Review of the Literature

Vascular complications of liver hydatidosis are infrequent but severe. We present a case with severe vascular problems due to a recurrence of a liver hydatid cyst. A 39 year-old male consulted the Emergency Department after frank hematemesis due to esophagealvarices. He had undergone surgery at the age of seven in his native country for liver and lung hydatid cysts. Abdominal CT showed a 20 cm multivesicular cyst, occupying segments IV, V and VIII of the liver, displacing and partially compressing a dilated portal vein and causing portal hypertension and obstruction of the inferior cava vein. A subtotal cystectomy was performed. After surgery, portal vein flow was normal and esophagealvarices disappeared, but caval obstruction remained. We review the vascular complications due to liver hydatidosis, focusing on therapeutic management. performed a subtotal cystectomy and left a lar ge drain in the cyst cavity. Postoperatively, the patient presented low output biliary fistula that did not require any treatment. The microbiological study of the liquid of the cyst confirmed Echinococcusgranulosus infestation.

Usefulness of Vascular Stenting With and Without Transvenous Pacing Leads for Vena Caval Obstruction Among Children and Adults With Repaired Congenital Heart Disease

Vena caval obstruction (VCO) is a common complication after vascular manipulation for congenital heart disease. Long-term efficacy of stent therapy for relief of VCO and long-term stent patency with and without intrastent transvenous pacing leads (TPLs) is not well described. This was a retrospective review of patients treated for VCO, including those who received intrastent TPLs, between 1995 and 2012. Patient demographics, diagnoses, vascular pressure gradients, and vessel diameters were analyzed. Forty-one patients (mean age 23.5 ± 10.3years) with and without congenital heart disease underwent stent implantation, 26 of whom also received intrastent TPLs. Short-term stent implantation success in relieving obstructions was 93%. Poststent vascular pressure gradients and percentage vascular narrowing significantly improved (from 6.2 ± 4.5 to 1.1 ± 1.6mm Hg and from 63.1 ± 19.5% to 18.0 ± 17.1%, respectively, p <0.05). On follow-up in 38 of 41 patients from 0.2 to 18years (median 6.0), all survived; 6 (14%) required stent reintervention. Freedom from reintervention was 87% at 15years. Patients with short-term procedural failure were at higher risk for stent reintervention. Among 27 patients with intrastent TPLs, freedom from reintervention was 96%. In 26 patients with follow-up catheterization, intrastent intimal proliferation was not significantly associated with TPL but was higher in the superior vena cava-innominate vein junction compared with other stent locations (p<0.05). In conclusion, stent therapy for VCO can be successfully and safely performed with good long-term results. Pre-pacing lead stent placement for VCO is effective in allowing TPL placement with encouraging long-term patency.

WITHDRAWN: Steroids, radiotherapy, chemotherapy and stents for superior vena caval obstruction in carcinoma of the bronchus.

Background Superior vena caval obstruction (SVCO) is an uncommon manifestation of carcinoma of the bronchus characterised by neck swelling and distended veins over the chest. In recent years, the majority of patients with small cell lung cancer (SCLC) with SVCO at diagnosis have tended to receive chemotherapy whilst the majority of patients presenting with non-small cell lung cancer (NSCLC) and SVCO have tended to receive radiotherapy. Steroids may also be prescribed. Stenting now provides a further treatment option which may be combined with radiotherapy and chemotherapy or used on its own. The optimal timing of stenting at present is unclear. Objectives To determine the relative effectiveness of treatments currently employed in the management of SVCO. Search strategy Electronic searching of Cochrane Clinical Trials register, Medline and Embase. Identification of further studies from references cited in trials identified by electronic searching. Selection criteria Both randomised and non-randomised controlled trials in which patients with carcinoma of the bronchus and a diagnosis of SVCO had been treated with any combination of the following treatment modalities: steroids, chemotherapy, radiotherapy or insertion of an expandable metal stent. Data collection and analysis There were 3 randomised and 98 non-randomised studies of which 2 and 44 respectively met the inclusion criteria. Main results SVCO was present at diagnosis in 10.0% of patients with SCLC and 1.7% of patients with NSCLC. In one randomised trial in SCLC, the rate of SVCO relapse was not significantly reduced by giving radiotherapy on completion of chemotherapy. In the other, in NSCLC, the addition of induction chemotherapy to a course of synchronous chemo-radiotherapy did not increase the rates of relief of SVCO. In SCLC, chemotherapy and/or radiotherapy relieved SVCO in 77%; 17% of those treated had a recurrence of SVCO. In NSCLC, 60% had relief of SVCO following chemotherapy and/or radiotherapy; 19% of those treated had a recurrence of SVCO. Insertion of an SVC stent relieved SVCO in 95%; 11% of those treated had further SVCO but recanalisation was possible in the majority resulting in a long-term patency rate of 92%. Morbidity following stent insertion was greatest if thrombolytics were administered. No study described the effectiveness of steroids in SVCO. Reviewer's conclusions Chemotherapy and radiotherapy are effective in relieving SVCO in a proportion of patients whilst stent insertion appears to provide relief in a higher proportion and more rapidly. The optimal timing of stent insertion (whether at diagnosis or following failure of other modalities) is currently uncertain. The effectiveness of steroids in SVCO remains uncertain.

Unusual Presentation of Anterior Mediastinal Mass

Approximately half of all mediastinal lesions are asymptomatic and are detected on chest radiographs taken for unrelated reasons. The absence of symptoms suggests that a lesion is (maybe) benign, whereas the presence of symptoms suggests malignancy. In adults, 48-62% of lesions are symptomatic, whereas the percentage of symptomatic lesions is higher in children (58 to 78%). The most common symptoms are cardiorespiratory in particular, chest pain and cough. Other manifestations are heaviness in the chest, dyspnea, signs of superior vena caval obstruction with facial swelling, and cyanosis. Recurrent respiratory infections are also common.

Chylous Pericardial Effusion and Tamponade Due to Catheter Induced Superior Vena Caval Obstruction in a Premature Infant

Chylous Pericardial Effusion and Tamponade Due to Catheter Induced Superior Vena Caval Obstruction in a Premature Infant

Liver cirrhosis in hepatic vena cava syndrome (or membranous obstruction of inferior vena cava).

Hepatic vena cava syndrome (HVCS) also known as membranous obstruction of inferior vena cava reported mainly from Asia and Africa is an important cause of hepatic venous outflow obstruction (HVOO) that is complicated by high incidence of liver cirrhosis (LC) and moderate to high incidence of hepatocellular carcinoma (HCC). In the past the disease was considered congenital and was included under Budd-Chiari syndrome (BCS). HVCS is a chronic disease common in developing countries, the onset of which is related to poor hygienic living condition. The initial lesion in the disease is a bacterial infection induced localized thrombophlebitis in hepatic portion of inferior vena cava at the site where hepatic veins open which on resolution transforms into stenosis, membrane or thick obstruction, and is followed by development of cavo-caval collateral anastomosis. The disease is characterized by long asymptomatic period and recurrent acute exacerbations (AE) precipitated by clinical or subclinical bacterial infection. AE is managed with prolonged oral antibiotic. Development of LC and HCC in HVCS is related to the severity and frequency of AEs and not to the duration of the disease or the type or severity of the caval obstruction. HVOO that develops during severe acute stage or AE is a pre-cirrhotic condition. Primary BCS on the other hand is a rare disease related to prothrombotic disorders reported mainly among Caucasians that clinically manifest as acute, subacute disease or as fulminant hepatic failure; and is managed with life-long anticoagulation, porto-systemic shunt/endovascular angioplasty and stent or liver transplantation. As epidemiology, etiology and natural history of HVCS are different from classical BCS, it is here, recognized as a separate disease entity, a third primary cause of HVOO after sinusoidal obstruction syndrome and BCS. Understanding of the natural history has made early diagnosis of HVCS possible. This paper describes epidemiology, natural history and diagnosis of HVCS and discusses the pathogenesis of LC in the disease and mentions distinctive clinical features of HVCS related LC.

Outcomes of Liver Transplantation, Partial Hepatectomy, and Transcatheter Arterial Embolization for Massive Polycystic Liver in Autosomal Dominant Polycystic Kidney Disease Patients.

Background: Polycystic liver disease(PLD) is the most common extra-renal manifestation in autosomal dominant polycystic kidney disease(ADPKD). Patients with PLD suffer from hepatomegaly leading to pain, dyspnea and caval obstruction. Interventions for symptomatic PLD include transcatheter arterial embolization(TAE), partial hepatic resection and liver transplantation(LT). We compared outcomes of each treatment modality. Methods: We retrospectively analyzed 29 patients who had undergone TAE, partial hepatectomy, or LT in ADPKD patients in a single center. Results: Six and seven patients underwent LT and partial hepatectomy, respectively, while sixteen patients underwent TAE. Living donor LT was performed for intractable hepatomegaly-related symptoms despite TAE in 2 patients, and for uncontrolled ascites in a patient. Deceased donor(DD) LT was performed in 2 patients for hepatic failure or uncontrolled ascites after TAE. Another patient underwent DDLT for hepatic failure after partial hepatectomy. The most common complications were ascites and pleural effusion. Two patients were under hemodialysis at LT, and acute kidney injury resulted in kidney failure in 1 DDLT patient. All patients had maintained good liver function at a median follow-up of 15.5 months, although subclinical acute cellular rejection was found in 2 patients. Causes of partial hepatectomy were recurrent cyst infection and hepatomegaly-related symptoms. One patient underwent partial hepatectomy, because multiple TAE had failed to control symptoms. Preoperative CTP score was lower in the partial hepatectomy group than in the LT group. A few serious infectious complications occurred, and antibiotic treatment cured them without mortality. Eleven patients received single TAE, and five patients underwent TAE twice. Two patients died of obstructive jaundice and cyst infection. Five patients (31.3%) in the TAE group needed LT or partial hepatectomy to relieve symptoms or rescue hepatic failure. Conclusion: LT is more effective and tolerable treatment option for symptomatic PLD in ADPKD patients compared to partial hepatectomy and TAE.

An unusual cause of respiratory distress in an infant.

Partial anomalous venous connection (PAPVC) is a congenital cardiovascular defect where one or more (but not all) of the pulmonary veins return anomalously back to the right atrium, either via a direct or indirect connection. It often occurs with other cardiac defects, most commonly a secundum atrial septal defect. Individuals with a large degree of shunting will present with dyspnea, fatigue, and, in some cases, heart failure. Clinical associations and variants of PAPVC include scimitar syndrome, pseudo-scimitar or meandering right pulmonary vein, sinous venosus defects, malposition of the septum primum, and Turner syndrome. The patient in this case, a previously healthy, 6-month-old, full-term male, presented to the emergency department for evaluation of respiratory distress and wheezing. The infant was first seen in his pediatrician's office, where he was noted to be tachypneic and wheezing. He was feeding without difficulty, voiding well, and was active and playful. The patient had passed critical congenital heart disease screening after his birth and prior to discharge, and the family history was negative for any respiratory or cardiac conditions. Cardiac magnetic resonance imaging is becoming the mode of choice for diagnosis of PAPVC. The definitive treatment is surgical correction, but surgery is not indicated in all cases, especially if the patient is asymptomatic and the degree of shunting is small. Patients with isolated PAPVC who undergo surgical correction have good long-term outcomes. In this case, the patient underwent the Warden procedure, which causes an increased risk of superior vena cava stenosis or obstruction to the right atrium. This patient will require lifelong follow-up to assess for new onset pulmonary venous obstruction.