Refractory Ascites Research Articles

The Role of the Transjugular Intrahepatic Porto-Systemic Shunt in an Emergency Setting.

Transjugular intrahepatic porto-systemic shunt (TIPSS) is an interventional radiology procedure whose aim is to create artificial communication between the portal and the hepatic blood flow in order to reduce the pressure gradient in portal hypertension. The indications to perform a TIPSS procedure can be framed in an elective or emergency setting: refractory ascites to diuretic therapy and secondary prophylaxis of variceal hemorrhage are the most frequent reasons for executing a TIPSS in an election context, while acute uncontrolled variceal bleeding is the principal indication that a TIPSS needs to be performed in an emergency setting. In recent years, the role of the TIPSS has been redefined for several conditions, such as ectopic varices, portal vein thrombosis, Budd-Chiari syndrome, hepatic veno-occlusive disease, and many others. This review aims to perform a deep analysis of when and why a TIPSS procedure should be carried out in an emergency, pointing out the related most common technical difficulties and complications.

Peritoneal Mesothelioma in a High Volume Peritoneal Surface Malignancies Unit.

Diffuse malignant peritoneal mesothelioma (PM) is a rare neoplasm, traditionally associated with a poor prognosis. There are other varieties of PM that are even less frequent and of uncertain malignancy. Cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) has achieved prolonged survival in selected patients. The aim of this study is to analyze the patients with PM assessed in our center. Clinicopathological characteristics, diagnostic procedures and survival results from patients with PM appraised at our unit, according to the applied treatment, were analyzed. Seventeen patients were assessed between 2007 and 2019. Three cases had multicystic PM that were treated with complete CRS + HIPEC; all patients are alive and free of disease after a long follow-up. Three other cases had biphasic PM; a curative treatment could be performed in none of them, and their survival was minimal (<6 moths). Lastly, 11 cases with epithelioid PM were treated. Two cases were considered unresectable at laparoscopy (PCI 39); one of them had a long survival (67 months) with three iterative laparoscopic palliatives HIPECs for refractory ascites. The other nine cases were treated with curative CRS + HIPEC, with a median PCI of 14 (range 4-25), and a median overall survival (OS) of 58 months, with a 5-year OS of 47.4%. In conclusion, CRS + HIPEC, when possible, appears to be the optimal treatment for patients with PM. Knowledge of this therapeutic option is crucial, both to offer it to patients and to avoid delays in their referral to appropriate centers for treatment.

The Effects of SGLT2 Inhibitors on Liver Cirrhosis Patients with Refractory Ascites: A Literature Review.

Decompensated liver cirrhosis is often complicated by refractory ascites, and intractable ascites are a predictor of poor prognosis in patients with liver cirrhosis. The treatment of ascites in patients with cirrhosis is based on the use of aldosterone blockers and loop diuretics, and occasionally vasopressin receptor antagonists are also used. Recent reports suggest that sodium-glucose cotransporter 2 (SGLT2) inhibitors may be a new treatment for refractory ascites with a different mechanism with respect to conventional agents. The main mechanisms of ascites reduction with SGLT2 inhibitors appear to be natriuresis and osmotic diuresis. However, other mechanisms, including improvements in glucose metabolism and nutritional status, hepatoprotection by ketone bodies and adiponectin, amelioration of the sympathetic nervous system, and inhibition of the renin-angiotensin-aldosterone system, may also contribute to the reduction of ascites. This literature review describes previously reported cases in which SGLT2 inhibitors were used to effectively treat ascites caused by liver cirrhosis. The discussion of the mechanisms involved is expected to contribute to establishing SGLT2 therapy for ascites in the future.

A245 A TERTIARY CENTRE REVIEW OF PORTO-SYSTEMIC SHUNTS: AN INCREASINGLY IMPORTANT TOOL IN LIVER DISEASE MANAGEMENT

Abstract Background Portal hypertensive complications arising from chronic liver disease are increasingly prevalent. Radiologically placed porto-systemic shunts are used successfully to address complications such as ascites and recurrent variceal bleeding. Purpose We sought, with approval from ethics to electronically chart review the UHN experience for TIPSS (transjugular intrahepatic porto-systemic shunt) and DIPS (direct intrahepatic porto-caval shunt) over time. Method We employed a search strategy focused on radiologic databases, to identify UHN patients who had received a porto-systemic shunt insertion over three decades between January 1, 1990, to September 14, 2021. A total of 238 UHN patients that had a shunt imaged, re-assessed, or inserted were included in this retrospective electronic chart review. Demographic information was recorded at baseline. Data pertaining to clinical outcomes of portal hypertension such as refractory ascites, recurrent gastrointestinal bleeding and ESLD progression was recorded at baseline, 1 month, 3 months and 6 months post-shunt insertion. HVPG was recorded pre-shunt and post-shunt insertion. Transplant-free survival and post-operative complications was also assessed. Patients were followed until death or liver transplant. Result(s) Out of 238 patients confirmed, 219 had a TIPSS insertion and 19 a DIPS insertion. The average age at time of shunt was 55.1 ± 8.4 years. 53.7% (n=128) of patients were male. Indications for portosystemic shunt placement included refractory ascites (n=141), recurrent gastrointestinal bleeding (n=73), hydrothorax (n=10), hepatorenal syndrome (n=8), and portal vein thrombosis (n=4). Etiology of disease was categorized as alcohol-associated liver disease (n=107), non-alcohol associated fatty liver disease (n=36), viral hepatitis (n=27), autoimmune hepatitis (n=38) and vascular liver disease (n=20). Relevant events noted post procedure included hepatic encephalopathy (n=34), hydrothorax (n=17), vascular (including stent) thrombosis (n=17) and renal failure (n=4). 12 months post-shunt insertion 2.5% (n=6) of patients had died or received liver transplant; of these 1.7% (n=4) were transplanted. HPVG fell consistently post-procedure: 15.6 ± 1.5mmHg pre-shunt to 5.9 ± 1.2mmHg post-shunt. There was no observable increase in MELD-Na score in the first month post-shunt. Temporal trends showed that more patients aged 50 to 69, and more female patients, are now receiving shunt insertion than at the initiation of the chart review. Image Conclusion(s) Portosystemic shunt placement in the form of TIPSS or DIPS insertion is an increasingly common and effective treatment for portal hypertension in patents with advanced liver disease at UHN. Disclosure of Interest None Declared

Effects of transjugular intrahepatic portosystemic shunt on abdominal muscle mass in patients with decompensated cirrhosis

Sarcopenia is a common finding in patients with decompensated cirrhosis without effective therapy. We aimed to examine whether a transjugular portosystemic shunt (TIPS) could improve the abdominal muscle mass assessed by cross-sectional images in patients with decompensated cirrhosis and to investigate the association of imaging-defined sarcopenia with the prognosis of such patients. In this retrospective observational study, we enrolled 25 Decompensated cirrhosis patients aged >20 who received TIPS for the control of variceal bleeding or refractory ascites between April 2008 and April 2021. All of them underwent preoperative computed tomography or magnetic resonance imaging, which was used to determine psoas muscle (PM) and paraspinal muscle (PS) indices at the third lumbar vertebra. First, we compared baseline muscle mass with muscle mass at 6 and 12 months after TIPS placement and analyzed PM- and PS-defined sarcopenia to predict mortality. Among 25 patients, 20 (80.0%) and 12 (48.0%) had PM- and PS-defined sarcopenia, respectively, at baseline. In total, 16 and 8 patients were followed up for 6 and 12 months, respectively. All imaging-based muscle measurements performed 12 months after TIPS placement were significantly greater than the baseline measurements (all p<0.05). Unlike patients with PS-defined sarcopenia (p=0.529), patients with PM-defined sarcopenia had poorer survival than did patients without (p=0.036). PM mass in patients with decompensated cirrhosis may increase by 6 or 12 months after TIPS placement and imply a better prognosis. Patients with preoperative PM-defined sarcopenia may suggest poorer survival.

No Differences in Rotational Thromboelastometry Measurements between Portal and Peripheral Circulation in Cirrhotic Patients Undergoing TIPS

In patients with liver cirrhosis, transjugular intrahepatic portosystemic shunt (TIPS) is considered a standardized treatment of refractory ascites or variceal bleeding. TIPS thrombosis (TT) and/or portal vein thrombosis (PVT) are possible complications during/after TIPS placement. Previous studies suggested increased clotting activity in portal circulation (PORC). This pilot study aimed to evaluate alterations and differences of coagulation function in PORC and in peripheral circulation (PERC) via rotational thromboelastometry during TIPS. Blood samples were collected from cirrhotic patients (n = 13; median Model of End Stage Liver Disease, MELD Score: 12; median age: 60 years) undergoing TIPS (10/13 TIPSs were elective procedures due to refractory ascites) as follows: median cubital vein (MCV; PERC)-confluence of the three hepatic veins to the inferior cava vein (HV/ICV; PORC)-portal vein (PV; PORC)-TIPS (PORC). This research utilized four variables of the extrinsic test EXTEM, i.e., clotting time (CT), clot formation time (CFT), maximum clot firmness (MCF), and maximum lysis (ML). EXTEM results [mean, M (range) ± standard deviation, SD (range)] showed no significant differences for CT [M (70-73) ± SD (9-13); p = 0.93] or CFT [M (137-155) ± SD (75-112); p = 0.97] or MCF [M (51-54) ± SD (9-10); p = 0.90] or ML [M (9-10) ± SD (4-5); p = 0.89] between the compartments, i.e., MCV vs. HV/ICV vs. PV vs. TIPS. Overall, we detected no differences in coagulation function between PERC and PORC. These results are in contrast to previous reports suggesting increased clotting activity in PORC vs. PERC in association with liver cirrhosis. Rotational thromboelastometry-based evaluation of coagulation function in PERC appears to reliably reflect coagulation function in PORC with respect to risk estimation for TT and/or PVT in cirrhotic patients undergoing TIPS.

Vitamin D supply and its relationship with nutritional status and survival in patients with liver cirrhosis

Annotation. Prognosis assessment is critical for liver cirrhosis (LC). Vitamin D deficiency is considered a possible unfavorable factor. The aim of the study was to assess the level of vitamin D in LC patients, to establish the prevalence of its deficiency, and to determine the relationship with the severity of LC, nutritional status, and patient survival. 95 LC patients, 61 men, and 34 women (age 50.1±1.15 years) were enrolled in the prospective study. The nutritional status was assessed using Onodera’s Prognostic Nutritional Index (Onodera’s PNI). The serum 25(OH)D3 was determined by enzyme immunoassay. Statistical analysis was performed in SPSS v 21.0. It was established that vitamin D insufficient supply was present in 9% of LC patients, deficiency – in 74%, and severe deficiency – in 15% of patients. LC decompensation was accompanied by a worsening of vitamin D deficiency. The serum 25(OH)D3 was correlated with Child-Turcotte-Pugh and Model For End-Stage Liver Disease (r= -0.481, -0.487, respectively, p˂0.001). The lowest 25(OH)D3 levels were in patients with severe and refractory ascites, hepatic encephalopathy, and significant hypoalbuminemia. Malnutrition was found in 34.7% of decompensated patients and was associated with severe vitamin D deficiency. The 25(OH)D3 was correlated with Onodera’s PNI (r=0.679, p˂0.001). The 25(OH)D3 had a good predictive value for the 1-year mortality (AUC 0.708, p=0.004). The 25(OH)D3 less than 12.5 ng/ml should be considered a marker of a poor prognosis. Thus, vitamin D deficiency is highly prevalent in LC patients. It is associated with the severity of liver disease, malnutrition, and poor prognosis. Further studies are needed to determine whether vitamin D supplementation can improve survival in LC patients.

Proximal Splenic Artery Embolization for Refractory Ascites and Hydrothorax Post-Liver Transplant.

Proximal splenic artery embolization (pSAE) has been advocated as a valuable tool to ameliorate portal hyper-perfusion (PHP). The purpose of this study was to determine the safety and efficacy of pSAE to treat refractory ascites (RA) and/or refractory hydrothorax (RH) in the setting of PHP post-liver transplant. A total of 30 patients who underwent pSAE for RA and/or RH after liver transplantation (LT) between January 2007 and December 2017 were analyzed retrospectively. The patients were divided into groups according to the time frame from pSAE to clinical resolution in order to identify predictors of RA/RH response to the procedure. Twenty-four (80%) patients responded to pSAE within three months, whereas 6 (20%) still required additional treatments for RA/RH at three months post-pSAE. In all cases clinical symptoms resolved within six months. Complications after pSAE were as follows: 2 cases of splenic infarction (6.6%), one case of post-splenic embolization syndrome (3.3%), one case of hepatic artery thrombosis (3.3%) and one case of portal vein (PV) thrombosis (3.3%). Increased intraoperative PV flow volume and increased pre-pSAE PV velocity, as well as higher estimated glomerular filtration rate were associated with early RA/RH resolution. pSAE is safe and effective in treating RA and RH due to PHP after LT. This study suggests that clinical parameters indicating more severe PHP and better kidney function are possible predictors for early response to pSAE.

Corrigendum to “Safety, Tolerability, Pharmacokinetics, and Efficacy of Terlipressin Delivered by Continuous Intravenous Infusion in Patients with Cirrhosis and Refractory Ascites”

Corrigendum to “Safety, Tolerability, Pharmacokinetics, and Efficacy of Terlipressin Delivered by Continuous Intravenous Infusion in Patients with Cirrhosis and Refractory Ascites”

Advanced Techniques for Accessing the Portal Vein during Transjugular Intrahepatic Portosystemic Shunt Creation.

Transjugular portosystemic shunt (TIPS) placement is a widely accepted method for reducing portal pressures in conditions including symptomatic varices, refractory ascites, hepatic hydrothorax, portomesenteric thrombosis, and Budd-Chiari syndrome. The traditional method for performing TIPS involves "blind" access into a portal vein branch from a hepatic vein, typically right portal vein to right hepatic vein, using preoperative imaging and intraoperative wedged portography for guidance. However, standard access technique may not always be feasible due to occluded portal or hepatic veins, distorted anatomy, or prior TIPS. In this article we discuss alternative techniques for accessing the portal vein for TIPS placement when standard methods are not sufficient.

Hepatic Encephalopathy after Transjugular Intrahepatic Portosystemic Shunt Creation.

Transjugular intrahepatic portosystemic shunt (TIPS) creation is effective in treating the sequelae of decompensated liver cirrhosis-including medically refractory ascites and variceal bleeding-by decompressing the portal venous system through a manmade portosystemic conduit within the liver. However, the altered physiology in which splenomesenteric blood bypasses intrahepatic portal venous perfusion can precipitate varying degrees of hepatic encephalopathy (HE). While the majority of post-TIPS HE cases can be treated medically, some require escalated management strategies, including endovascular interventions to modify the indwelling TIPS and/or occlude competitive physiologic spontaneous portosystemic shunts. This review article details the epidemiology, risk factors, diagnosis, classification, and treatment of post-TIPS HE.

Review article: controversies surrounding the use of carvedilol and other beta blockers in the management of portal hypertension and cirrhosis.

Advanced chronic liver disease is an increasing cause of premature morbidity and mortality in the UK. Portal hypertension is the primary driver of decompensation, including the development of ascites, hepatic encephalopathy and variceal haemorrhage. Non-selective beta blockers (NSBB) reduce portal pressure and are well established in the prevention of variceal haemorrhage. Carvedilol, a newer NSBB, is more effective at reducing portal pressure due to additional α-adrenergic blockade and has additional anti-oxidant, anti-inflammatory and anti-fibrotic effects. To summarise the available evidence on the use of beta blockers, specifically carvedilol, in cirrhosis, focussing on when and why to start METHODS: We performed a comprehensive literature search of PubMed for relevant publications. International guidelines advise the use of NSBB in primary prophylaxis against variceal haemorrhage in those with high-risk varices, with substantial evidence of efficacy comparable with endoscopic band ligation (EBL). NSBB are also well established in secondary prophylaxis, in combination with EBL. More controversial is their use in patients without large varices, but with clinically significant portal hypertension. However, there is gathering evidence that NSBB, particularly carvedilol, reduce the risk of decompensation and improve survival. While caution is advised in patients with advanced cirrhosis and refractory ascites, recent evidence suggests that NSBB can continue to be used safely, and that premature discontinuation may be detrimental. With increasing evidence of benefit independent of variceal bleeding, namely retardation of decompensation and improvement in survival, it is time to consider whether carvedilol should be offered to all patients with advanced chronic liver disease.

Alternative forms of portal vein revascularization in liver transplant recipients with complex portal vein thrombosis

Complex portal vein thrombosis (PVT) is a challenge in liver transplantation (LT). Extra-anatomical approaches to portal revascularization, including renoportal (RPA), left gastric vein (LGA), pericholedochal vein (PCA), and cavoportal (CPA) anastomoses, have been described in case reports and series. The RP4LT Collaborative was created to record cases of alternative portal revascularization performed for complex PVT. An international, observational web registry was launched in 2020. Cases of complex PVT undergoing first LT performed with RPA, LGA, PCA, or CPA were recorded and updated through 12/2021. A total of 140 cases were available for analysis: 74 RPA, 18 LGA, 20 PCA, and 28 CPA. Transplants were primarily performed with whole livers (98%) in recipients with median (IQR) age 58 (49-63) years, model for end-stage liver disease score 17 (14-24), and cold ischemia 431 (360-505) minutes. Post-operatively, 49% of recipients developed acute kidney injury, 16% diuretic-responsive ascites, 9% refractory ascites (29% with CPA, p <0.001), and 10% variceal hemorrhage (25% with CPA, p= 0.002). After a median follow-up of 22 (4-67) months, patient and graft 1-/3-/5-year survival rates were 71/67/61% and 69/63/57%, respectively. On multivariate Cox proportional hazards analysis, the only factor significantly and independently associated with all-cause graft loss was non-physiological portal vein reconstruction in which all graft portal inflow arose from recipient systemic circulation (hazard ratio 6.639, 95% CI 2.159-20.422, p= 0.001). Alternative forms of portal vein anastomosis achieving physiological portal inflow (i.e., at least some recipient splanchnic blood flow reaching transplant graft) offer acceptable post-transplant results in LT candidates with complex PVT. On the contrary, non-physiological portal vein anastomoses fail to resolve portal hypertension and should not be performed. Complex portal vein thrombosis (PVT) is a challenge in liver transplantation. Results of this international, multicenter analysis may be used to guide clinical decisions in transplant candidates with complex PVT. Extra-anatomical portal vein anastomoses that allow for at least some recipient splanchnic blood flow to the transplant allograft offer acceptable results. On the other hand, anastomoses that deliver only systemic blood flow to the allograft fail to resolve portal hypertension and should not be performed.

A preoperative risk score based on multifrequency bioelectrical impedance analysis in patients undergoing liver resection.

To investigate if body composition parameters measured by bioelectrical impedance analysis (BIA) and are reportedly correlated with clinical outcomes of patients undergoing digestive tract surgery could be useful for reliably evaluating the perioperative risk in patients undergoing hepatectomy. Consecutive 200 patients who underwent BIA before hepatectomy were retrospectively reviewed. A risk prediction model for postoperative morbidity was created using the initial 100 patients, and its performance was validated using the remaining 100 patients. Based on the correlation with postoperative morbidity, a novel risk prediction model, the protein-edema score, was created using net protein weight and extracellular water/total body water ratio measured through BIA. The protein-edema score (score 0 vs. ≥ 1) showed a reproducible correlation with Clavien-Dindo 2 or greater postoperative morbidity in the validation set (17.7% vs. 46.4%, P = 0.002) as observed in the training set (18.8% vs. 49.0%, P = 0.002) after statistical adjustment. Similar tendency was also confirmed in Clavien-Dindo 3a or greater postoperative morbidity (5.9% vs. 18.2%, P = 0.037) and postoperative refractory ascites (5.5% vs. 17.4%, P = 0.037) in the validation set. The protein-edema score created based on BIA is significantly correlated with postoperative morbidity in patients undergoing liver resection.

Lymphangiomatosis presented with melena and chylous ascites: A case report.

Lymphangioma, a rare benign tumor of the lymphatic system, is called lymphangiomatosis when it involves >1 organ, which is more rarely complicated with thoracic obstruction, the relationship between them remains unclear. With the development of enteroscopy, clinicians know more about small intestinal lymphangioma and attempt to treat it through enteroscopic injection sclerotherapy(EIS). A 59-year-old male firstly manifested with gastrointestinal bleeding after a gastric perforation, who was diagnosed with lymphangiomatosis by balloon-assisted enteroscopy and abdomen CT showing >1 organ with multiple cysts besides the small intestine. The patient received an EIS, then the melena disappeared. Surprisingly he came back because of refractory ascites confirmed to be chylous by chemical tests 7 months later. Lymphangiography could not determine the location of lymphatic leakage, Ultrasonography showed stenosis of the left cervical part of the thoracic duct. On the condition that medical treatment is ineffective, thoracic duct exploration and lysis of fibrous adhesion were performed. Ascites significantly reduced at last. Lymphangiomatosis is the malformation of the lymphatic system involving multiple organs, it has a possibility to be associated with thoracic obstruction. Capsule endoscopy and enteroscopy are effective methods to diagnose small intestinal lymphangioma, and EIS is an effective therapy.

Predicting dry weight in patients with cirrhotic ascites undergoing large-volume paracentesis

Ascites impairs the correct diagnosis and nutritional management in patients with cirrhosis, because the body weight, which is needed for nutritional assessment and calculation of nutritional needs, is overestimated. To adjust the weight in patients with ascites, dietetic guidances indicate substracting 2.2-14kg or 5-15% of the measured body weight according to the degree of ascites, however, there is a lack of evidence to substantiate these values. The aim of this study was to develop new prediction equations to estimate the dry weight, comparing them with the currently used weight adjustments in patients with refractory cirrhotic ascites. Cross-sectional study, that included patients with decompensated cirrhosis undergoing large-volume paracentesis. Patients were submitted to nutritional risk screening, nutritional assessment, and anthropometric measurements that included body weight, abdominal circumference (both measured before and after paracentesis) height, and upper mid-arm circumference. The volume of ascitic fluid drained was also registered. For the predictions of dry weight, linear regression models were performed using as predictor variables: height, pre-paracentesis weight, pre-paracentesis abdominal circumference, or mid-upper arm circumference, and as response variable: post-paracentesis weight. The capacity of these models to predict the post-paracentesis weight was evaluated by comparing it with the currently used predictions through the intraclass correlation coefficient (ICC) and the mean squared error (MSE). Nineteen patients were included, 15 male, and 18 with high nutritional risk and malnutrition. The difference between post-paracentesis weight and pre-paracentesis weight was-5.0 (-3.6 to-9.9) kg, similar to ascitic fluid volume drained. Two equations were developed to predict post-paracentesis weight. ICC values showed that both prediction equations were strongly correlated (r>0.94) with post-paracentesis weight. Our models also showed lower MSEs (<17.97), compared with the current predictions (MSEs <64.19, when the pre-paracentesis weight is adjusted from absolute values and MSEs <33.24 when adjusted from percentage values), indicating a more accurate prediction. The predictive equations from this study may be better options for dry weight estimation in patients with refractory cirrhotic ascites since they showed higher reliability compared to the currently used weight adjustment. External validation in a larger sample is still needed to confirm the clinical applicability of these equations.

Transjugular Intrahepatic Portosystemic Shunt in Liver Transplant Recipients: Outcomes in Six Adult Patients.

Transjugular intrahepatic portosystemic shunt (TIPS) is regularly used in treatment of clinically significant portal hypertension. Liver transplant recipients are, however, rarely indicated for the procedure. The study retrospectively examines the results of TIPS placement in 6 patients after OLT. 4 males and 2 females (aged 36 to 62years), treated with TIPS between 2007 a 2018, were included in the study. 5 patients had previously undergone liver transplantation for liver graft cirrhosis, 1 patient for Budd-Chiari syndrome. The piggyback caval reconstruction technique was selected in 4/6 cases. PH developed after OLT due to the recurrence of underlying liver condition and sinusoidal obstruction syndrome in half of the cases, respectively. Indications for TIPS were refractory ascites in 4 cases and variceal bleeding in 2 cases. Standard TIPS technique was used and technical success was achieved in all cases with a procedure-related complication in 1 patient. One patient died shortly after TIPS placement. The remaining patients all reported regression of clinically significant PH. Late complications appeared in 2 patients. Liver retransplantation after TIPS creation was performed in 1 case. Median TIPS patency was 55months. 2/6 patient continue to thrive with a patent shunt. Transjugular intrahepatic portosystemic shunt in OLT recipients is technically feasible. Favorable clinical outcomes were reported particularly in patients treated for sinusoidal obstruction syndrome who were indicated to TIPS for refractory ascites.

Transjugular intrahepatic portosystemic shunt in decompensated cirrhotic patients in a tertiary hospital in southern Brazil

The aim of the present study was to evaluate the outcomes of cirrhotic patients undergoing transjugular intrahepatic portosystemic shunt. A retrospective longitudinal observational study was carried out evaluating 38 cirrhotic patients undergoing transjugular intrahepatic portosystemic shunt. The outcomes were evaluated in an outpatient follow-up period of 3 months. The assumed significance level was 5%. The indications for transjugular intrahepatic portosystemic shunt were refractory ascites in 21 (55.3%), variceal hemorrhage in 13 (34.2%), and hydrothorax in 4 (10.5%) patients. There was development of hepatic encephalopathy in 10 (35.7%) patients after transjugular intrahepatic portosystemic shunt. From the 21 patients with refractory ascites, resolution was observed in 1 (3.1%) patient, and in 16 (50.0%) patients, there was ascites control. Regarding transjugular intrahepatic portosystemic shunt after variceal bleeding, 10 (76.9%) patients remained without new bleeding or hospitalizations in the follow-up period. The global survival in the follow-up period in patients with and without hepatic encephalopathy was 60 vs. 82%, respectively (p=0.032). Transjugular intrahepatic portosystemic shunt can be considered in decompensated cirrhotic patients; however, the development of hepatic encephalopathy which can shorten survival should be focused.

A Neonate with Mucopolysaccharidosis Type VII with Intractable Ascites

We report a case of a patient with severe fetal hydrops and refractory ascites, diagnosed as mucopolysaccharidosis type VII (MPS VII) by whole-exome sequencing, and discharged at 5 months of age after long-term ventilatory management. A male neonate was born by emergency cesarean section due to fetal distress at 30 1/7 weeks' gestation. Physical examination and X-rays revealed pleural effusion, ascites, and generalized edema, indicating severe fetal hydrops. He underwent tracheal intubation because of respiratory distress that was attributed to massive ascites, pulmonary hypoplasia, and pulmonary hypertension. He received mechanical ventilation and inhaled nitric oxide therapy. Prednisone, octreotide, and a factor XIII preparation were used as the treatment for ascites, and the ascites gradually decreased. He was extubated within 2 months of age. At 4 months of age, the results of whole-exome sequencing of the cord blood showed a compound heterozygous mutation in the GUSB gene, the gene responsible for MPS VII. Enzyme replacement therapy was initiated, and the ascites was resolved. Careful systemic management, including lung-protective respiratory management and the early establishment of nutrition, is important for the long-term survival of infants with fetal hydrops, and early aggressive workup, including whole-genome sequencing for the cause, should be performed in the case of refractory ascites.

Esophagogastric varix caused by extrahepatic portal vein obstruction with essential thrombocythemia: A case report.

Extrahepatic portal vein obstruction (EHPVO) is a very rare disease-causing portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is reported as a risk factor for EHPVO due to underlying persistent thrombophilia.A Japanese woman in her 40s was referred to our hospital with a one-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation demonstrated thrombocytosis despite portal hypertension. She had a mutation of clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist. Bone marrow biopsy revealed megakaryocyte lineage proliferation, leading to a diagnosis of ET.Esophagogastroduodenoscopy indicated esophagogastric varices (LsF2CbRC2, Lg-cF1RC1). Abdominal Computed Tomography and angiography revealed splenomegaly and portal vein thrombosis with cavernous transformation. These radiologic findings suggested EHPVO.The patient had a history of ruptured esophagogastric varices and required prophylaxis against further variceal bleeding prior to anti-thrombotic therapy for EHPVO with ET. We performed laparoscopic Hassab's operation followed by endoscopic variceal ligation (EVL) and hematological cytoreduction therapy.Laparoscopic Hassab's operation and three bi-monthly EVL improved the esophagogastric varix (LmF0RC0, Lg-f F0RC0) at 6 months after surgery. Platelet count decreased to 60.1 x104 /uL by cytoreduction therapy. She was very healthy at 7 months after surgery.Patients with EHPVO are traditionally referred to the gastroenterologist for abdominal pain, intestinal bleeding, or refractory ascites; however, hypercoagulative disease may be occult in such patients and require the attention of a hematologist. When encountering the patients with splanchnic thrombosis caused by EHPVO, the gastroenterologists should screen for hematological disease, including MPN.