Recanalization and Reconstruction of a Chronically Occluded Inferior Vena Cava Through an Existing Transjugular Intrahepatic Portosystemic Shunt in the Setting of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) results from the occlusion or flow reduction in the hepatic veins or inferior vena cava and can be treated with transjugular intrahepatic portosystemic shunt when hepatic vein recanalization fails.1-3 Hypercoagulable patients with primary BCS are predisposed to development of new areas of thrombosis within the TIPS shunt or IVC. This case details a patient with BCS, pre-existing TIPS extending to the right atrium, and chronic retrohepatic IVC thrombosis who underwent sharp recanalization of the IVC with stenting into the TIPS stent bridging the patient until his subsequent hepatic transplantation.

Computational fluid dynamics simulation of time-resolved blood flow in Budd-Chiari syndrome with inferior vena cava stenosis and its implication for postoperative efficacy assessment.

BACKGROUND: This study aimed to adopt computational fluid dynamics to simulate the blood flow dynamics in inferior vena cava stenosis based on time-dependent patient-specific models of Budd-Chiari syndrome as well as a normal model. It could offer valuable references for a retrospective insight into the underlying mechanisms of Budd-Chiari syndrome pathogenesis as well as more accurate evaluation of postoperative efficacy. METHODS: Three-dimensional inferior vena cava models of Budd-Chiari syndrome patient-specific (preoperative and postoperative) and normal morphology model were reconstructed as per magnetic resonance images using Simpleware. Moreover, computational fluid dynamics of time-resolved inferior vena cava blood flow were simulated using actual patient-specific measurements to reflect time-dependent flow rates. FINDINGS: The assessment of the preoperative model revealed the dramatic variations of hemodynamic parameters of the stenotic inferior vena cava. Moreover, the comparison of the preoperative and postoperative models with the normal model as benchmark showed that postoperative hemodynamic parameters were markedly ameliorated via stenting, with the attenuation of overall velocity and wall shear stress, and the increase of pressure. However, the comparative analysis of the patient-specific simulations revealed that some postoperative hemodynamic profiles still bore some resemblance to the preoperative ones, indicating potential risks of restenosis. INTERPRETATION: Computational fluid dynamics simulation of time-resolved blood flow could reveal the tight correlation between the hemodynamic characteristics and the pathological mechanisms of inferior vena cava stenosis. Furthermore, such time-resolved hemodynamic profiles could provide a quantitative approach to diagnosis, operative regimen and postoperative evaluation of Budd-Chiari syndrome with inferior vena cava stenosis.

Long-Term Outcomes of Endovascular Interventions in More than 500 patients with Budd-Chiari Syndrome.

PURPOSE: To assess and compare the long-term outcomes of various endovascular interventions in patients with Budd-Chiari syndrome (BCS). MATERIALS AND METHODS: In this single-center retrospective study, 510 consecutive patients with BCS who had undergone a total of 618 endovascular procedures from January 2001 to December 2019 were included. Details of the type of endovascular intervention, technical success, clinical success, patency rate, complications, and survival outcomes were analyzed. RESULTS: The overall technical success rate was 96% (593 of 618 procedures; 500 in treatment-naïve patients and 93 repeat interventions for recurrent disease). Endovascular procedures included recanalization procedures (angioplasty and stent placement) in 355 patients (71%) and transjugular intrahepatic portosystemic shunt (TIPS) creation in 145 (29%). Major postprocedure complications occurred in 14 patients (2.8%). Vascular/stent restenosis occurred in 95 patients (19%), and successful repeat intervention was performed in 82 of those 95 (86.3%). An additional 11 of these 82 (13.4%) underwent a third intervention for restenosis. In the recanalization and TIPS groups, the 1- and 5-y cumulative patency rates were 87% and 74% and 95% and 68%, respectively. The 1- and 5-y survival rates were 96% and 89% and 90% and 76%, respectively. CONCLUSIONS: Endovascular interventions for BCS are feasible and safe in the majority of patients, with excellent short- and long-term patency and survival rates.

An Update on the Management of Budd-Chiari Syndrome.

Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.

Sequential interventional therapy for Budd-Chiari syndrome associated with fresh inferior vena cava thrombosis.

OBJECTIVE: Our study aimed to evaluate the safety and efficacy of sequential interventional therapy for Budd-Chiari syndrome (BCS) caused by obstruction of the inferior vena cava (IVC) with fresh thrombus in the IVC. METHODS: Full medical records were obtained for 20 patients with BCS associated with fresh IVC thrombus who received sequential interventional therapy from 2014 to 2019 at our hospital. All patients underwent small-diameter percutaneous transluminal angioplasty (PTA) balloon catheter predilation combined with sequential catheter-directed thrombolysis and large-diameter PTA balloon dilation. Ultrasound examinations were performed at 1 week, 1 month, 3 months, and every 6 months thereafter. Therapeutic effects and perioperative and postoperative adverse effects were recorded to assess the safety of the treatment. RESULTS: All 20 patients were treated with small PTA balloon catheters (diameter, 10-14 mm) to predilate the occlusive segment of the IVC. Urokinase 400,000 to 600,000 (465,000 ± 93,000) units was administered to patients through the catheter for 6 to 20 (9.7 ± 4.2) consecutive days postoperatively. Ultrasound re-examination showed that the IVC thrombus disappeared completely in 14 patients (70.0%), and a small amount of the old thrombus remained in 6 patients (30.0%). After thrombolysis, all 20 patients received PTA balloon dilation (diameter, 26-30 mm) in the stenosed IVC segment, and blood flow recovered subsequently. No pulmonary embolism or death occurred in the perioperative course. The perioperative survival rate was 100.0%. CONCLUSIONS: Sequential interventional therapy for BCS associated with fresh IVC thrombus is safe and effective.

[Primitive Budd-Chiari syndrome: a diagnostic and therapeutic challenge]. / Budd-Chiari primitif: défi diagnostique et thérapeutique.

Primary Budd-Chiari syndrome is an hepatopathy characterized by obstruction of the hepatic venous flow in the vascular space between the hepatic venules and the junction between the inferior vena cava and the right atrium, thus excluding the causes of upstream (sinusoidal obstruction syndrome) and downstream (cardiac causes). This endoluminal venous obstruction is mainly due to thrombosis or its fibrotic consequences. This is a rare disease, mainly affecting young adults. Clinical manifestations are extremely variable; it can be asymptomatic, acute, subacute or chronic. The diagnosis is mainly based on Doppler ultrasound and/or magnetic resonance imaging. Several causes have been identified, in particular, myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, inherited thrombotic disorders. Symptomatic and etiologic treatments are the commonly used approaches, treatment recommendations are organized in algorithm. Recent therapeutic advances can significantly improve the prognosis.

Ectopic Balloon Device Placement to Correct the Positional Hepatic Venous Outflow Obstruction in Liver Transplantation.

OBJECTIVES: Hepatic vein outflow obstruction in liver transplantation can lead to graft or patient loss. We used an intrauterine balloon to overcome this complication in 13 liver transplant recipients. Here, we report the results of these cases; our report, as far as we know, involves the highest number of patients on this issue. MATERIALS AND METHODS: Positional hepatic vein outflow obstruction was diagnosed in 13 of 651 liver transplant recipients between January 2014 and December 2016. The grafts were repositioned by intrauterine balloon placed to the right subdiaphragmatic area. Data of donors, recipients, and grafts and postoperative courses were analyzed. RESULTS: Of the 13 patients, 9 were men, with age range of patients of 22 to 70 years. The amount of saline used to inflate the balloon was variable (200-450 cm3), and hepatic vein outflow obstruction was relieved after balloon implantation in all patients. There were no balloon-related complications. Removal was done at bedside, without any additional sedation or any additional skin incision on days 2 to 15. Doppler ultrasonography scans were performed before and after the balloon removal. There were no vascular complications after removal. CONCLUSIONS: Intrauterine balloon can be safely and efficiently used for hepatic vein outflow obstruction during liver transplant when needed.

Pediatric Hepatic Venous Outflow Tract Obstruction: Experience from a Transplant Center.

We performed a review of case records of children diagnosed with hepatic venous outflow tract obstruction at our center in last 10 years. Out of 11 cases identified, 6 had variable blocks in the hepatic venous system and 4 had combined hepatic venous and inferior vena cava (IVC) block. One child with paroxysmal nocturnal hemoglobinuria (PNH) had isolated IVC involvement. Angioplasty was attempted in 3 patients; among them 2 had successful outcome. Seven children with advanced liver disease underwent transplantation, which was successful in six. With availability of modalities like interventional radiology and transplantation, the overall prognosis of hepatic venous outflow tract obstruction seems to be good when managed in a well-equipped center.

A Rare Case of Recurrent Lower Extremity Ulcer.

Venous leg ulcers are a manifestation of lower extremity chronic venous disorder. Venous hypertension caused by abnormal venous blood flow is considered to be the primary mechanism of venous ulcers. The etiology of lower extremity venous ulcer is complicated, and it is difficult to be treated. At present, it has achieved a certain effect for venous leg ulcers through the combination of drugs, pressure therapy, and surgical treatment. Budd-Chiari syndrome is a rare syndrome characterized by portal hypertension and/or inferior vena cava syndrome. Treatment of Budd-Chiari syndrome includes anticoagulation, thrombolysis, angioplasty, stenting, transjugular intrahepatic portosystemic shunt, and liver transplantation. This article reports an elderly female patient with recurrent ulceration of both lower extremities that healed poorly after long-term dressing and skin grafting. During further examination, she was found to have Budd-Chiari syndrome. Through multidisciplinary treatment, which includes removing the inferior vena cava stenosis and implanting the ulcer wound, the ulcer wounds then healed.

Effectiveness and Postoperative Prognosis of Using Preopening and Staged Percutaneous Transluminal Angioplasty of the Inferior Vena Cava in Treating Budd-Chiari Syndrome Accompanied with Inferior Vena Cava Thrombosis.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disorder that has relatively high prevalence in the Huang-Huai area of China. Effective treatment of BCS accompanied with inferior vena cava thrombosis is challenging. OBJECTIVE: This study retrospectively analyzed the clinical effectiveness and safety of traditional open operations versus preopening and staged percutaneous transluminal angioplasty (PTA) of the inferior vena cava in treating BCS accompanied with inferior vena cava thrombosis. METHODS: Data from patients hospitalized and treated for BCS accompanied with inferior vena cava thrombosis between January 1997 and December 2017 were retrospectively analyzed. Thirty-two patients received traditional open operation (open group). Fifty-six patients received preopening and staged PTA of the inferior vena cava (PTA group). Baseline and clinical data were compared between groups. The patients were followed for up to 60 months. Postoperative recurrence rates and restenosis degree were recorded. RESULTS: Eighty-eight patients were included (47 males and 41 females), aged 41.82 ± 10.12 years (range 29-65). In the open group, no pulmonary arterial embolism was found during and after the operation, and the technique success rate was 100%. One patient died of intrathoracic bleeding. In the PTA group, 2 patients had shifting of thrombus in the inferior vena cava that blocked the blood flow restored by the preopening, one resulted in treatment failure, while the other had blood flow restored by dilation with a 12-mm balloon; the success rate was 55/56 (98.21%). The median follow-up time was 32 months (range 3-60). Two patients in the open group developed restenosis 2 years after operation (recurrence rate: 6.25%), and were successfully treated by balloon PTA. Seven patients in the PTA group had severe restenosis 18-42 months after operation (recurrence rate: 12.96%). No thrombosis was found in these 7 patients, and normal blood flow was restored in the inferior vena cava after balloon PTA. CONCLUSIONS: Preopening and staged PTA of the inferior vena cava is a safe and simple method for the treatment of BCS accompanied with inferior vena cava thrombosis, with satisfactory treatment effectiveness that could be applied in clinical practice.

Usefulness of Venous Pressure Measurement in Endovascular Treatment of Budd-Chiari Syndrome: A Retrospective Cohort Study.

Objective Therapeutic predictors derived from the venous pressure before therapy have not been identified for Budd-Chiari syndrome (BCS). The aim of this study was to determine whether or not measuring the distal pressure or pressure gradient was useful for predicting treatment efficacy in BCS. Methods We retrospectively analyzed seven consecutive patients diagnosed with symptomatic BCS at our hospital between 2008 and 2017. Distal and proximal venous pressures at occlusion sites of BCS were measured before treatment in all cases. The pressure gradient was defined as the difference between distal and proximal venous pressures. A receiver operating characteristics (ROC) analysis was performed for venous pressures. Results Percutaneous old balloon angioplasty (POBA) was performed in seven cases, with technical success achieved in all cases (100%). No complications were encountered. The median primary patency was 574 (interquartile range, 439.5-1,056.5) days. The 1-year primary patency rate was 71.73%. Six cases (85.7%) showed resolution of symptoms, representing clinical success. The ROC analysis revealed a high distal pressure (area under the ROC curve = 0.83, cut-off=12 mmHg) as a predictor of treatment efficacy of POBA for symptomatic BCS. In addition, the pressure gradient was considered significant from a clinical perspective, because the 6 successful cases with resolution of symptoms showed a large pressure gradient (range, 8-21 mmHg) before treatment, whereas the failed case showed a relatively small pressure gradient (7 mmHg). Conclusion High distal pressure and a large pressure gradient might predict the treatment efficacy of balloon angioplasty for symptomatic BCS.

Hemodynamic aspects of the Budd-Chiari syndrome of the liver: A computational model study.

Budd-Chiari syndrome (BCS) is a rare liver disease characterised by the obstruction of draining hepatic veins, and subsequent reduced blood return to the heart. Although many clinical BCS studies have been reported, few studies have quantified the associated changes that occur in the entire hepatic circulation. In this communication, we present an electrical analog model of the hepatic circulation that incorporates the Hepatic Arterial Buffer Response (HABR) mechanism in both the left and right lobes. Using this model we can simulate the hepatic flow under both normal and acute BCS conditions. The model can capture subtle features in the hepatic circulation, such as reduced total portal flow but increased arterial flow under BCS. This observation was previously reported in literature and may have clinical implications. As such, we suggest the presented model could be used for the analysis of systematic haemodynamic changes of BCS and therefore may be useful in supporting clinical interventions.

Liver disease in pregnancy: Medical aspects and their implications for mother and child.

Liver disease during pregnancy is more common than expected and may require specialized intervention. It is important to determine if changes in liver physiology may develop into liver disease, to assure early diagnosis. For adequate surveillance of mother-fetus health outcome, liver disease during pregnancy might require intervention from a hepatologist. Liver diseases have a prevalence of at least 3% of all pregnancies in developed countries, and they are classified into two main categories: related to pregnancy; and those non- related that are present de novo or are preexisting chronic liver diseases. In this review we describe and discuss the main characteristics of those liver diseases associated with pregnancy and only some frequent pre-existing and co-incidental in pregnancy are considered. In addition to the literature review, we compiled the data of liver disease occurring during pregnancies attended at the National Institute of Perinatology in Mexico City in a three-year period. In our tertiary referral women hospital, liver disease was present in 11.24 % of all pregnancies. Associated liver disease was found in 10.8% of all pregnancies, mainly those related to pre-eclampsia (9.9% of pregnancies). Only 0.56% was due to liver disease that was co-incidental or preexisting; the acute or chronic hepatitis C virus was the most frequent in this group (0.12%). When managing pregnancy in referral hospitals in Latin America, it is important to discard liver alterations early for adequate follow up of the disease and to prevent adverse consequences for the mother and child.

Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome.

BACKGROUND: Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. AIM: To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. METHODS: Analysis of recent literature by using Medline, PubMed and EMBASE databases. RESULTS: Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. CONCLUSIONS: With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.

Current knowledge in pathophysiology and management of Budd-Chiari syndrome and non-cirrhotic non-tumoral splanchnic vein thrombosis.

Budd-Chiari syndrome and non-cirrhotic non-tumoral portal vein thrombosis are 2 rare disorders, with several similarities that are categorized under the term splanchnic vein thrombosis. Both disorders are frequently associated with an underlying prothrombotic disorder. They can cause severe portal hypertension and usually affect young patients, negatively influencing life expectancy when the diagnosis and treatment are not performed at an early stage. Yet, they have specific features that require individual consideration. The current review will focus on the available knowledge on pathophysiology, diagnosis and management of both entities.

Liver and Spleen Stiffness Measurements for Assessment of Portal Hypertension Severity in Patients with Budd Chiari Syndrome.

Aims: Budd-Chiari Syndrome (BCS) is a rare vascular disease of the liver caused by the obstruction of the hepatic venous outflow located from the small hepatic venules up to the entrance of the inferior vena cava (IVC) into the right atrium. Current prognostic indexes are suboptimal for an individual prognostic assessment and subsequent management of patients with BCS. Liver (LSM) and spleen (SSM) stiffness measurements are widely validated prognostic tools in hepatology, but the evidence in patients with BCS is limited. This paper describes LSM and SSM in patients with BCS and their correlation with clinical, biochemical, and ultrasound findings from the same patients. Methods: We investigated a case series of seven patients with BCS diagnosis and available LSM and SSM evaluated by transient elastography (TE). Biochemical, imaging, and endoscopic findings nearest to the TE evaluation were recorded. Clinical outcomes and BCS evolution were described for each patient. When available, repeated TE assessments were also recorded. Results: Patients with acute nonfulminant manifestation of BCS presented near-the-upper-limit values (75 kPa) of LSM and SSM, which often persist until the placement of a transjugular intrahepatic portosystemic shunt (TIPS). On the other hand, TE values were markedly lower in patients with compensated BCS. In some patients with repeated TE measurement years after TIPS placement, LSM had decreased to values of <10 kPa years. SSM changes in these patients were, however, less evident. Conclusions: Extremely elevated values of LSM and SSM are suggestive of BCS. The evaluation of both LSM and SSM by TE could help clinicians in the initial evaluation, risk stratification, and therapy response monitoring of patients with BCS.

Effect of Transjugular Intrahepatic Portosystemic Shunt Creation on Pulmonary Gas Exchange in Patients with Hepatopulmonary Syndrome: A Prospective Study.

PURPOSE: To evaluate effect of transjugular intrahepatic portosystemic shunt (TIPS) creation on pulmonary gas exchange in patients with hepatopulmonary syndrome (HPS). MATERIALS AND METHODS: All patients with cirrhosis or Budd-Chiari syndrome undergoing elective TIPS creation at a single institution between June 2014 and June 2015 were eligible for inclusion. Twenty-three patients with HPS (age 55.0 y ± 14.4; 11 men; Model for End-Stage Liver Disease score 10.2 ± 2.7) who achieved technical success were included in the analysis. Diagnosis of HPS was established by contrast-enhanced echocardiography demonstrating intrapulmonary vascular dilatation and arterial blood gas analysis demonstrating arterial oxygenation defects. RESULTS: Mean portosystemic gradient was reduced from 21.7 mm Hg ± 8.3 before TIPS creation to 10.8 mm Hg ± 5.1 after TIPS creation. Among the 5 (21.7%) patients who experienced dyspnea, 4 (80.0%) reported improvement after TIPS creation. This improvement was not maintained at 3 months after TIPS creation in 2 (50.0%) patients. Compared with before TIPS creation, mean change in alveolar-arterial oxygen gradient for patients with HPS was statistically significant at 1 month (-9.2 mm Hg ± 8.0; P < .001) after TIPS creation, but not at 2-3 days (-0.9 mm Hg ± 10.5; P = .678) or 3 months (-3.4 mm Hg ± 11.8; P = .179) after TIPS creation. CONCLUSIONS: TIPS creation can transiently improve pulmonary gas exchange in patients with HPS.

Clinical Efficacy of Spectral Computed Tomography for Evaluating Liver Function in Patients with Budd-Chiari Syndrome.

RATIONALE AND OBJECTIVES: To analyze the clinical relevance of quantitative spectral parameters in evaluating the treatment of patients with Budd-Chiari syndrome (BCS) with different classes of liver function by comparing normalized iodine concentration (NIC) before and after BCS treatment. MATERIALS AND METHODS: Angiographic data were obtained from 41 patients with confirmed BCS between December 2015 and March 2017. All patients underwent spectral computed tomography (CT) before and after BCS treatment; the average interval between scans was 2-4 months. Iodine concentration and NIC were measured and calculated during the portal venous phase in liver segments I-VIII. Clinical liver function parameters including prothrombin time (PT), albumin (ALB), total bilirubin (TBIL), aspartate aminotransferase, and alanineaminotransferase were recorded. Liver function was classified according to the Child-Pugh grading standard (before treatment). Liver NIC and liver function-related parameters before and after treatment were compared using the paired t-test; Pearson correlation analysiswas performed to analyze the aforementioned parameters among different liver function classes before BCS treatment. p < 0.05 was to be statistically considered significant. RESULTS: PT and TBIL were negatively correlated with liver NIC (p < 0.05), whereas ALB and liver NIC exhibited a positive correlation (p < 0.05). Comparison of NIC before and after treatment revealed that NIC in liver segments I-VIII was higher after treatment. The p values for segments II-VIII were 0.041, 0.046, 0.041, 0.038, 0.039, 0.042, and 0.040, respectively; the differences were statistically significant (p < 0.05). The increase in NIC in liver segments I-VIII before and after treatment was more significant in patients with class C liver function than in their class B and A counterparts. Comparison of liver function-related parameters revealed that PT was shortened after treatment; ALB levels were increased; and TBIL, alanine aminotransferase, and aspartate aminotransferase levels were decreased. CONCLUSION: NIC determined through spectral CT was beneficial for evaluating liver function in patients with BCS, and can provide imaging data for reexamination, prognostic evaluation, and follow-up of patients with BCS after treatment. Liver parenchyma NIC values in BCS patients with varying liver function may provide a degree of diagnostic value. Multi-locus and multi-parameter studies of spectral CT can help to further evaluate liver function and assess prognosis in patients with BCS.

Radical surgical treatment of Budd-Chiari syndrome through entire exposure of hepatic inferior vena cava.

OBJECTIVE: Therapies for Budd-Chiari syndrome (BCS) can be divided into three main categories: medical, endovascular, and surgical. Surgery is applicable to the disease when other therapeutic options have failed. We introduce a surgical method of recanalization through exposure of the entire hepatic inferior vena cava (IVC) and hepatic vein (HV) outflow tract for BCS and investigate the long-term outcomes. METHODS: From July 2002 to December 2015 in our center, 83 consecutive symptomatic BCS patients with failure of endovascular therapy were treated by radical surgical recanalization. IVC recanalization was the first goal for all patients, and recanalization of at least one HV was the second goal for selected patients at the same surgical operation. Patients were followed up, and data on technical and clinical success, survival, and patency of target vessels were analyzed. RESULTS: Technical success of surgical recanalization was achieved in 80 patients (96.4%), with relief of clinical symptoms and improvement of liver function. During a mean follow-up of 84 ± 25.9 months, the cumulative 1-, 3-, and 5-year primary patency rates of the HV were 96.7%, 90.0%, and 83.3%, respectively. The cumulative 1-, 3-, and 5-year primary patency of the IVC was 86.7%, 71.7%, and 68.3%, respectively. No factor demonstrated significant association with recurrence of obstruction. During follow-up, 10 patients died, 8 of end-stage hepatic disease and 2 of unknown causes. The cumulative 1-, 3-, and 5-year all-cause survival rates were 91%, 90%, and 87%, respectively. Female sex, encephalopathy, severe ascites, and hypersplenism had an impact on survival in univariate analysis. With Cox regression, encephalopathy was the only independent determining factor for surgical survival. CONCLUSIONS: Surgical recanalization through exposure of the entire hepatic IVC for BCS is suitable for most primary BCS patients after failure of endovascular therapies.

Investigation of sensory thresholds in Cavalier King Charles Spaniels with and without Chiari-like malformations and syringomyelia.

BACKGROUND: Cavalier King Charles Spaniels (CKCS) suffer pain associated with Chiari-like malformation and syringomyelia (CMSM). People suffer from a similar condition and describe numerous sensory abnormalities. Sensory changes have not been quantified in affected CKCS. OBJECTIVES: To use quantitative sensory testing (QST) to quantify thermal and mechanical thresholds in CKCS and to compare QST in dogs with and without syringomyelia (SM). ANIMALS: Forty-four CKCS. METHODS: Prospective study. Dogs underwent neurological examinations and craniocervical magnetic resonance imaging (MRI). Thermal testing was performed over the humerus and thorax (n = 32); mechanical testing was performed on the paw and neck (n = 44). Latencies, thresholds, and response rates were compared with presence and severity of SM on MRI, presence of pain reported by the owner and pain identified on examination. RESULTS: Thirty dogs had SM, 30 were painful on examination, 29 were owner-reported symptomatic. Thermal and mechanical variables were not significantly different based on presence or severity of SM. Dogs with pain on examination had decreased mechanical thresholds on the paw (0.38 kg, SD = 0.18) and neck (2.05 kg, SD = 0.74) compared to thresholds of dogs without pain on examination on the paw (0.60 kg, SD = 0.30) and neck (2.72 kg, SD = 0.57; P = .021). CONCLUSIONS AND CLINICAL IMPORTANCE: Mechanical and thermal sensitivity does not appear to be related to the presence of SM, but mechanical sensitivity appears to be related to the presence of pain and clinical signs. Mechanical testing may be useful for assessing sensory abnormalities during clinical trials.