Improvement In Ascites Research Articles

S3117 Partial Splenic Artery Embolization for Patients With Portal Hypertension Undergoing Organ Transplant Evaluation: A Case Series

Introduction: Partial splenic artery embolization (PSAE) is an infrequently utilized approach to reduce portal hypertension (PHT) by reducing splenic arterial inflow and thereby splenic vein flow into the portal vein. We describe three patients who underwent PSAE to address diverse manifestations of PHT due to contraindications to transjugular intrahepatic portosystemic shunts (TIPS). Case Description/Methods: Case 1: A 50-year-old male with CKD stage 4 and idiopathic PHT had diuretic-refractory ascites and thrombocytopenia, precluding surgery for an arteriovenous fistula (AVF) and kidney transplantation. He was not a candidate for TIPS due to extensive portal vein thrombosis but underwent PSAE leading to resolution of ascites and marked improvement in thrombocytopenia (Figure 1). The patient ultimately received an AVF and was placed on the kidney transplant waitlist. He has been followed for 30 months with sustained clinical improvement. Case 2: A 60-year-old male with prior hepatocellular carcinoma (HCC) status post right partial hepatectomy and hepatitis C cirrhosis complicated by severe thrombocytopenia and refractory ascites, had two previously aborted TIPS procedures due to unusual hepatic vein anatomy. He underwent PSAE that led to a reduction in frequency of therapeutic paracenteses and improvement of thrombocytopenia (Figure 1). Eight months later, TIPS was reattempted and resulted in complete ascites control for over six years. He is currently on the waitlist for liver transplantation. Case 3: A 60-year-old male had cryptogenic cirrhosis complicated by mild ascites, significant thrombocytopenia, and massive splenomegaly with mass effects. He underwent successful PSAE to alleviate splenic compression of the bladder. One year later, he had a repeat PSAE for diaphragmatic compression secondary to splenic hypertrophy. His thrombocytopenia also mildly improved (Figure 1). The patient was declined for liver transplant due to lack of social support. He expired from advanced HCC after two years of follow-up. Discussion: We report three cases of PSAE in patients with PHT awaiting organ transplantation. All three cases had improvement in ascites, splenomegaly, and thrombocytopenia. Although PSAE is not the primary treatment for PHT, it may have a therapeutic role in advanced cases, particularly in transplant candidates when other interventions like TIPS are not possible. Future studies should be conducted to further evaluate the role of PSAE in patients with PHT awaiting organ transplantation.Figure 1.: *2nd PSAE attempt in case 3 represented at time 0.

Tolvaptan therapy of Chinese cirrhotic patients with ascites after insufficient diuretic routine medication responses: a phase III clinical trial

BackgroundTo determine the safety and efficacy of different doses of tolvaptan for treating Chinese cirrhotic patients with or without hyponatraemia who still had ascites after routine therapy with diuretics.MethodsIn the present placebo-controlled, randomized, double-blinded, multicentre clinical trial, patients with cirrhotic ascites who failed to adequately respond to a combination of an aldosterone antagonist plus an orally administered loop diuretic were randomly placed at a 4:2:1 ratio into 3 groups [the 15 mg/day tolvaptan group (N = 301), 7.5 mg/day tolvaptan group (N = 153) and placebo group (N = 76)] for 7 days of treatment. The effects and safety were evaluated on days 4 and 7. A change in body weight from baseline on day 7 of treatment was the primary endpoint.ResultsThe administration of 7.5 or 15 mg/day tolvaptan significantly decreased body weight from baseline on day 7 of treatment compared to that with placebo treatment (P = 0.026; P = 0.001). For the secondary endpoints, changes in abdominal circumference from baseline and improvements in ascites were markedly different in the treatment groups and the placebo group on day 7 (P7.5 = 0.05, P15.0 = 0.002 and P7.5 = 0.037, P15.0 = 0.003), but there was no difference between the 7.5 mg/day and 15 mg/day dosage groups. The 24-h cumulative urine volume was higher in the 7.5 mg/day and 15 mg/day tolvaptan groups than the placebo group (P = 0.002, P < 0.001) and was greater in the 15 mg/day tolvaptan group than the 7.5 mg/day tolvaptan group (P = 0.004). Sodium serum concentrations were higher in patients with hyponatraemia after tolvaptan treatment, with no significant difference between the two dosage groups. The incidence of serious adverse drug reactions was not different between the groups (P = 0.543).ConclusionsTolvaptan treatment at 7.5 mg per day might be a good therapeutic choice for Chinese cirrhotic patients with ascites who did not achieve satisfactory clinical responses to previous treatment regimens with combination therapy with an aldosterone antagonist and an orally administered loop diuretic.Trial registrationNCT01349348. Retrospectively registered May 2011.

Stent patency and outcome of TIPS through the left versus the right portal branches

BackgroundOur purpose is to compare the stent patency and clinical outcome of trans-jugular intra-hepatic porto-systemic shunt (TIPS) through the left branch portal vein (TIPS-LPV) to the standard TIPS through the right branch (TIPS-RPV). We retrospectively reviewed all patients (n = 54) with refractory portal hypertension who were subjected to TIPS-LPV at our institute (TIPS-LPV) between 2016 and 2018. These patients were matched with 56 control patients treated with the standard TIPS-RPV (TIPS-RPV). The 2 groups were compared regarding the stent patency rate, encephalopathy, and re-interventions for 1 year after the procedure.ResultsTIPS-LPV group showed 12 months higher patency rate (90.7% compared to 73.2%) (P < 0.005). The number of the encephalopathy attacks in the TIPS-LPV group was significantly lower than that of the TIPS-RPV group at 6 and 12 months of follow-up [P = 0.012 and 0.036, respectively]. Re-bleeding and improvement of ascites were the same in the two groups [P > 0.05]. Patients underwent TIPS-LPV needed less re-interventions and required less hospitalizations than those with TIPS-RPV [P = 0.039 and P = 0.03, respectively].ConclusionThe new TIPS approach is to extend the stent to LPV. This new TIPS-LPV approach showed the same clinical efficiency as the standard TIPS-RPV in treating variceal bleeding and ascites. However, it proved a better stent patency with lower rates of re-interventions, encephalopathy, and hospital admissions than TIPS through the right branch.

A Technical Look at Fetoscopic Laser Ablation for Fetal Laryngeal Surgical Recanalization in Congenital High Airway Obstruction Syndrome.

Background: Congenital high airway obstruction syndrome (CHAOS) is a rare condition characterized by complete obstruction of the upper fetal airways. Left untreated, it is uniformly fatal. Ex utero intrapartum treatment (EXIT) has been used to establish a surgical airway in affected fetuses during delivery. While this procedure benefits those fetuses that survive to delivery, high mortality in the prenatal period necessitates earlier innovative strategies. Herein, we report a novel technique for in utero intervention. Methods: A fetoscopic intervention was performed at 28 weeks on a 35-year-old G1P0 woman with fetal CHAOS from a laryngeal obstruction measuring 11 mm in length on prenatal imaging. Under ultrasound guidance, a 3.3-mm curved fetoscope was used to access the uterine cavity through a single subcentimeter maternal skin incision. The scope was driven through the fetal oral cavity and manipulated to attain a view of the vocal cords. A subglottic obstruction was observed. A 600-micron laser fiber was passed through the working channel of the scope and used to ablate the obstructed airway. Using the laser fiber and a guidewire, the ablated opening was traversed with the fetoscope to the level of the carina. Results: Postoperatively, the lungs became less hyperinflated. There was improvement in ascites and diaphragmatic eversion. At 31 1/7 weeks' gestation, the mother experienced preterm premature rupture of membranes with active labor and the fetus was delivered through EXIT to tracheostomy. The infant was managed on mechanical ventilation and is currently thriving at home with a tracheostomy at 2 years of age. Conclusion: Fetoscopy with laser ablation of the airway obstruction is an effective prenatal management strategy that offers the potential to alter the devastating natural course of CHAOS.

1631 A Rare Case of Myxedema Ascites and Ischemic Colitis

INTRODUCTION: Myxedema is a severe form of hypothyroidism with various signs and symptoms. We present a rare case of myxedema ascites complicated with ischemic colitis (IC). CASE DESCRIPTION/METHODS: A 71-year-old female with a history of coronary artery disease and congestive heart failure (Ejective fraction 40%) presented with abdominal distention. Pertinent vitals included blood pressure of 100/54 mmHg and heart rate 38 beats/min. Pertinent labs included hematocrit of 28.2 %, albumin 3.1 g/dL, transaminases mildly elevated, alkaline phosphatase (ALP) 382 IU/L, thyroid stimulating hormone (TSH) 141 IU/mL, free triiodothyronine (T3) 1.26 pg/ml, total T3 45.30 ng/dL. Rest of the electrolytes, blood counts, including coagulation parameters, were normal. Electrocardiogram showed sinus bradycardia. She was started on levothyroxine for severe hypothyroidism. Abdominal ultrasound showed a large volume of ascites and normal liver contour. Paracentesis showed total nucleated cells of 2242, total protein 3.6 g/dL, lactate dehydrogenase 229, albumin 1.8 g/dL, serum ascites albumin gradient (SAAG) &lt; 1.1, and glucose 86 mg/dL. Ascitic fluid cultures were negative. Hence, the patient was diagnosed with neutrocytic ascites and treated with empiric antibiotics. Ascitic fluid was negative for malignancy and tuberculosis. She was managed with diuretics (furosemide and spironolactone) and therapeutic paracentesis. She later developed bloody diarrhea, and flexible sigmoidoscopy showed mild to moderate IC of the rectosigmoid colon. She was treated conservatively. Her transaminases normalized, but ALP remained elevated. She was discharged after a two-week hospital stay and six weeks follow up showed significant improvement in ascites and TSH of 10 IU/mL. DISCUSSION: Myxedema ascites is seen in only 4% of the patients with uncontrolled hypothyroidism. There are no case reports on IC in the setting of myxedema ascites thus far. It is a rare complication of myxedema megacolon; however, our patient had no signs of megacolon on repeated imaging. Therefore, our case is unique as it highlights a rare (ascites) and uncommon (IC) complication of severe hypothyroidism. Ascites with SAAG &lt; 1.1 should prompt physicians to consider hypothyroidism in the differential diagnosis for ascites especially when total ascitic fluid protein is &gt; 2.5 g/dL and other causes such as portal hypertension, malignancy, and infections are ruled out. It is easy to treat, can lead to complete resolution of ascites and decrease morbidity.

Fluoropyrimidine with or without platinum as first-line chemotherapy in patients with advanced gastric cancer and severe peritoneal metastasis: a multicenter retrospective study

BackgroundThere is no standard first-line chemotherapy for advanced gastric cancer with severe peritoneal metastasis. Although fluoropyrimidine is often used, its efficacy is limited, and it remains unclear whether combination therapy with platinum improves clinical outcomes.MethodsThis retrospective study involved patients at six Japanese academic hospitals between 2010 and 2016. Patients with advanced gastric cancer and severe peritoneal metastasis were included if they had massive ascites and/or inadequate oral intake requiring intravenous nutritional support. We then compared the efficacy and safety of fluoropyrimidine monotherapy with those of fluoropyrimidine/platinum combination therapy.ResultsCompared with the combination therapy group (n = 64), the monotherapy group (n = 65) had worse general health (more patients with elderly age, performance status > 2, and having both massive ascites and inadequate oral intake). Both overall survival (9.0 vs 5.0 months, p < 0.01) and progression-free survival (4.3 vs 2.3 months, p < 0.01) were significantly longer in the combination group, and the significance remained after adjusting for prognostic variables (hazard ratios of 0.47 and 0.41, respectively; p < 0.01). Improvements in ascites and oral intake were also greater in the combination group. Although neutropenia (grade ≥ 3) occurred more frequently with combination therapy, both treatments in this study were tolerable.ConclusionsCombination therapy with fluoropyrimidine and platinum might be more effective than monotherapy with fluoropyrimidine and was tolerable for patients with advanced gastric cancer and severe peritoneal metastasis.

Encapsulating peritoneal sclerosis in a patient after allogeneic hematopoietic stem cell transplantation: a case report

BackgroundEncapsulating peritoneal sclerosis (EPS) is a chronic clinical syndrome of acute or subacute gastrointestinal obstruction seen mainly in patients undergoing peritoneal dialysis. Although there are a few reports on EPS developing in non-peritoneal dialysis patients, it has not been reported in patients undergoing allogeneic haematopoietic stem cell transplantation (HSCT). Here, we report a case of EPS after a second HSCT.Case presentationA 46-year-old man with myelodysplastic syndrome showed relapse after HSCT and received a second HSCT. The patient was diagnosed with chronic graft-versus-host disease (cGVHD)-associated serositis because of persistent ascites. His ascites improved gradually and disappeared without immunosuppressive therapy. He presented with nausea, weight loss, and constipation 1 year after improvement of ascites. Computed tomography revealed no organic obstruction, but did reveal dilated, thickened, and adhered small bowel loops with a mass-like appearance. He was diagnosed with EPS on the basis of clinical symptoms and image findings. He received corticosteroid therapy (20 mg/body) without any improvement in symptoms. He developed recurrence of myelodysplastic syndrome at 1 month after initiation of corticosteroid therapy. This progressed into acute myeloid leukaemia after 3 months. He died 31 months after the second HSCT. At autopsy, the small and large intestines had formed extensive adhesions and showed signs of progressive fibrosis with peritoneal sclerosis, fibroblast swelling, fibrin deposition, and inflammatory cell infiltration, which confirmed the diagnosis of EPS.ConclusionThis case suggests that EPS may complicate patients with cGVHD-associated serositis. Although the mechanism of EPS development is not clear, clinicians should be aware of this eventuality.

Modified FOLFOX6 as a first-line treatment for patients with advanced gastric cancer with massive ascites or inadequate oral intake.

BackgroundOral fluoropyrimidine plus platinum is a standard first-line treatment for advanced gastric cancer (AGC). However, this treatment is problematic for AGC patients with massive ascites or inadequate oral intake. This study aimed at evaluating the efficacy and safety of modified oxaliplatin (L-OHP) with l-leucovorin (l-LV) and bolus/continuous infusion of 5-fluorouracil (5-FU) (mFOLFOX6) regimen for patients with massive ascites or inadequate oral intake.MethodsThis retrospective study was conducted at a single Japanese institute from November 2015 to May 2018. The mFOLFOX6 regimen consisted of 85 mg/m2 L-OHP, 400 mg/m2 bolus of 5-FU, and 400 mg/m2 1-LV on the first day, followed by 2,400 mg/m2 of 5-FU as a continuous infusion in 46 hours for first-line treatment. The definition of inadequate oral intake was the need for total parenteral nutrition (TPN). Massive ascites was defined as continuous ascites from the pelvic cavity to the upper abdomen. Improvement in oral intake was defined as no TPN for more than 7 days, and improvement in ascites was defined as a decrease in ascites of more than one grade defined by the Japan Clinical Oncology Study Group trial (JCOG0106).ResultsAmong the 364 patients with AGC who received first-line chemotherapy, 17 patients (13 [76.5%] had inadequate oral intake, and four [23.5%] had massive ascites) were enrolled in this study. Median time to treatment failure and overall survival were 4.8 (95% CI=1.5–7.5) and 8.8 months (95% CI=2.3–not available), respectively. Objective improvements in oral intake and ascites were seen in 11 of 13 patients (84.6%) and 6 of 12 patients (50%), respectively. The major grade 3 or 4 adverse events were neutropenia (35.3%), febrile neutropenia (5.9%), fatigue (5.9%), anorexia (5.9%), and infection (5.9%). No treatment-related deaths occurred.ConclusionWe found that mFOLFOX6 can be a novel treatment option as the first-line treatment for AGC patients with massive ascites or inadequate oral intake.

Abstract No. 620 Incidence and pathophysiology of transjugular intrahepatic portsystemic shunt (TIPS) complicated with lower extremity edema

TIPS is consistently proven to benefit patients with ascites, hydrothorax and gastrointestinal bleeding, however adverse events do occur after TIPS. Lower extremity edema (LEE), an under reported complication of TIPS, can cause significant morbidity. This study aims to measure the incidence and pathophysiology of LEE after TIPS. Between 2006-2016, a total of 220 patients underwent TIPS at our institution. LEE was defined as new onset or worsened edema up to one year after TIPS. Changes in diuretic use, clinical outcome of LEE, etiology of cirrhosis and effectiveness of TIPS in reducing ascites/hydrothorax were documented. Cardiac ventricular function was evaluated by echocardiography. Patients without close follow-up were excluded. Improvement in ascites was defined 25% fewer paracenteses. 205 patients were eligible for inclusion. 132 (64%) were male. Mean (SD) age was 54 (11) years old. Mean portosystemic shunt gradient changed from 18 to 7 mm Hg with mean (SD) post TIPS gradient of 7 (3). 80 (39%) patients had LEE at baseline. 101 patients (49%) had new onset or worsened LEE with median onset at one month follow-up. 62 out of 101 patients with LEE (61%) subsequently improved their edema (9 with conservative measures, 53 with diuretics). LEE was persistent despite diuretics in 36 patients (35%) at median follow-up of 21 months. Of note 30 out of 80 (37%) with baseline LEE had worsening edema. Multiple logistic regression analysis showed no significant impact from age, gender, ejection fraction, portosystemic gradient change and improvement in ascites on LEE. Presence of baseline edema prior to TIPS was protective in multivariate analysis (OR = -2.7, p = 0.007). LEE can complicate TIPS in almost half of patients, independent of left ventricular function. Close clinical follow-up after TIPS, without initially changing diuretics is recommended. Baseline edema does not necessarily lead to worsening edema after TIPS. Further studies are needed to better clarify the pathophysiology of LEE after TIPS.

Long Term Survival of Patients Undergoing TIPS in Budd-Chiari Syndrome

There has been significant improvement in understanding the etiology and management of Budd-Chiari Syndrome (BCS). Patients with chronic or acute-on-chronic BCS need radiological interventions in the form of angioplasty, hepatic vein/inferior vena cava stenting or Transjugular Intrahepatic Portosystemic Shunt (TIPS). Data regarding the long term follow up of patients undergoing TIPS is limited. We thus prospectively followed-up BCS patients who underwent TIPS at our center. This study included 42 patients with BCS who underwent TIPS with a covered stent between 2004 and 2014. We analyzed the etiology, symptoms, severity, laboratory parameters and imaging pre and post TIPS. All patients underwent surveillance for hepatocellular carcinoma. Patients demographics included 26 males and 16 females with a mean age of 40.5 years (19-68 years). The mean Model for End-Stage Liver Disease score of the entire cohort was 15.38 (range: 9-25). Thirty-four patients were grouped into Rotterdam Class 2 and remaining into Class 3. There was significant improvement in ascites, gastrointestinal bleed, renal function and transaminase levels post TIPS. There were 11 deaths over the follow-up period - 4 within one month, 2 within six months and the rest after 3 years following TIPS. Median duration from clinical presentation to TIPS was 2.1 weeks and median survival till follow-up was 45.5 months (0-130 months). 33/42 patients underwent TIPS prior to 2013, and their median survival till follow-up was 55 months. Six out of eleven deaths that occurred within six months post-TIPS were before 2006; when the technique of TIPS creation was evolving. The cumulative 1 year, 5 years and 10 years OLT-free survival was 86%, 81% and 76%, respectively. Two patients underwent a liver transplant at 4 and 7 years after TIPS. Our results validate the role of TIPS in the management of patients with BCS. With the accessibility of TIPS, the requirement for liver transplantation has become rare.

The Curious Case of a Swollen Belly: Myxedema Ascites

Introduction: Less than 4% of patients with hypothyroidism develop ascites. Ascites as the only presenting feature of hypothyroidism is uncommon, hence diagnosis is often delayed. Once it is diagnosed, treatment of hypothyroidism leads to quick clinical improvement in ascites. We report a case of ascites secondary to severe hypothyroidism.Figure 1Case: A 56 year old female with long standing history of Hashimoto's thyroiditis and non-compliance to thyroid supplementation presented with a four week history of progressive painless abdominal distension. She had recently undergone extensive work-up at a regional hospital. Review of records revealed high ascitic fluid protein content (3.9 g/dl) and serum-ascites-albumin gradient (SAAG) of 1. Ultrasound and computed tomography of the abdomen and pelvis showed massive ascites. Morphology of liver and spleen was unremarkable without any evidence of increased portal pressure. Viral hepatitis serology was negative and tumor markers (CA19-9, CA125) were within normal limits. Hepatic Doppler ultrasound showed normal portal pressure without evidence of portal or hepatic vein thrombosis. We ordered thyroid stimulating hormone level that resulted as >100 units/ml, with low free T4 (0.2 ng/dl). Further evaluation revealed dyslipidemia and mildly elevated alanine and aspartate transaminases and creatine kinase, consistent with thyroid myopathy. The diagnosis of myxedema ascites was made on this diagnostic work-up and the patient was started on thyroid replacement. On a follow-up visit at 4 weeks, there was no clinical evidence of ascites and TSH decreased to 56 units/ml. Discussion: Severe uncontrolled hypothyroidism is a rare but reversible cause of ascites. Various theories have been hypothesized to explain the pathophysiology. These include: 1) an increase in capillary permeability leading to the extravasation of plasma proteins into the extravascular compartment; 2) direct hygroscopic effect caused by hyaluronic acid accumulating in the skin and producing edema; 3) diminished free water clearance due to excess ADH production. Myxedema ascites responds well to thyroid replacement therapy and is completely reversible. Conclusion: Our case illustrates that the diagnosis of myxedema ascites should be borne in mind in a patient with uncontrolled hypothyroidism who presents with ascites. It also signifies the need for testing TSH level as part of the workup for ascites of unknown etiology.

Clinical Characteristics and Outcomes of Transjugular Intrahepatic Portosystemic Shunt Procedure for Portal Hypertension Secondary to Myeloproliferative Neoplasms

▪Portal hypertension is a common complication of myeloproliferative neoplasms (MPN) and portends a poor prognosis. Arising in 7-18% of MPN patients, portal hypertension develops through several distinct mechanisms, including abdominal vein thrombosis, extramedullary hematopoiesis (EMH), and nodular regenerative hyperplasia (NRH). Similar to cirrhotic patients, MPN-associated portal hypertension presents with refractory ascites, gastrointestinal bleeding, hepatic encephalopathy, and susceptibility to infections. Transjugular intrahepatic portosystemic shunt (TIPS) procedure has been used empirically to treat refractory ascites and variceal bleeding in MPN-related portal hypertension; however, there is limited data in regards to TIPS effectiveness in MPN-associated portal hypertension. To assess the safety and efficacy of TIPS for treatment of portal hypertension in MPN, we performed a retrospective analysis of outcomes of TIPS procedure in patients with MPN in our institution.Using the University of Pennsylvania electronic medical record database, we identified nine patients with MPN who underwent TIPS procedure for treatment of refractory portal hypertension between 2005 and 2015. Patients with portal hypertension from causes other than MPN were excluded from the study. Clinical characteristics and long-term outcomes were analyzed.The mean age at time of TIPS was 47 years (range 30-67 years). Seven of nine patients were female (78%) and all seven carried a diagnosis of polycythemia vera (PV) or post-PV myelofibrosis (PV-MF); both male patients had a diagnosis of primary myelofibrosis (PMF) (Table 1). All nine patients were positive for JAK2V617F mutation, and one patient had a concurrent diagnosis of Philadelphia-chromosome positive CML. The most common etiology of portal hypertension was Budd-Chiari Syndrome (BCS) in six patients (67%), followed by NRH in five patients (56%), and EMH in two patients (22%); one patient had several distinct causes of MPN-related portal hypertension. Indications for TIPS included refractory ascites in five patients (63%), ascites and esophageal varices in three patients (33%), and ascites and hydrothorax in one patient. All patients demonstrated immediate normalization of portal pressures following TIPS without any reported periprocedural complications (Table 2). Additionally, all patients received indefinite anticoagulation (low molecular weight heparin, 3 patients; vitamin K antagonist, 2 patients; fondaparinux, 2 patients). TIPS intervention had 1-year patency rate of 89%. However, one third of patients required subsequent shunt revision at a median interval of 22.3 months (range 10-34 months) due to stent stenosis or thrombosis despite anticoagulation; one patient had radiographic evidence of TIPS dysfunction without clinical symptoms. Of note, all three patients who developed TIPS stenosis/thrombosis had BCS and NRH. The majority of patients (89%) experienced complete resolution of ascites, while the remaining patient had partial improvement in ascites but no longer required routine paracentesis. Grade 1 and 2 hepatic encephalopathy was the most common complication post-TIPS (67%), occurring in half of the patients with BCS and in all three patients with portal hypertension due to EMH. One patient had variceal bleeding in the setting of TIPS thrombosis. All patients were alive 2 years post-TIPS (excluding one patient with recent TIPS; range 2-5 years).Our study represents the first systematic analysis of outcomes of TIPS procedure for management of portal hypertension in MPN using data from a single-institution over a ten-year period. Our results suggest that TIPS procedure can be performed safely in this high-risk population, and can effectively mitigate the clinical consequences of portal hypertension in patients with MPN. However, the prevalence of TIPS complications, particularly of TIPS stenosis/thrombosis and hepatic encephalopathy, remains significant and must be balanced against the desired clinical benefits. Future studies with a larger number of patients are needed to delineate prognostic factors that identify MPN patients most likely to benefit from TIPS. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Chylous Ascites Following Severe Acute Necrotizing Pancreatitis

Introduction: The accumulation of milk-like fluid rich in triglycerides within the peritoneal cavity is termed chylous ascites. It results from extravasation of lymph due to disruption of the lymphatic system from trauma or obstruction. Abdominal malignancy and cirrhosis are responsible for the majority of cases in the developed world. Infectious etiologies, such as tuberculosis and filariasis, are most commonly implicated in developing countries. Definitive diagnosis is made by paracentesis, which demonstrates a fluid triglyceride level of greater than 200 mg/dL. Chylous ascites is a rare occurrence and presents a therapeutic dilemma in cases where the underlying disease process is not readily reversible. Very few case reports exist of chylous ascites following severe necrotizing pancreatitis. There is anecdotal evidence in the literature describing improvement with the use of parenteral nutrition and octreotide. Herein, we describe a case of chylous ascites following an episode of severe necrotizing pancreatitis successfully treated with parental nutrition and octreotide. A 70-year-old man with a history of asthma and dyslipidemia suffered a severe episode of acute necrotizing pancreatitis. He presented 3 months after his initial episode of pancreatitis with worsening abdominal distention and early satiety. Imaging revealed ascites in addition to a large pancreatic pseudocyst. A paracentesis was performed, yielding white turbid ascitic fluid with a triglyceride level of 652 mg/dL, consistent with chylous ascites. Furthermore, the amylase level was normal and bacterial culture, fungal culture, AFB, cytology, and flow cytometry were negative. Investigation of underlying etiologies ruled out tuberculosis, malignancy, cirrhosis, and cardiac causes. He was started on parental nutrition and octreotide 150 mcg subcutaneously every 8 hours in an attempt to reduce lymph production. Therapy was continued until a noticeable improvement in ascites was appreciated, a total of 10 days. Repeat paracentesis confirmed resolution of the chylous ascites, with a triglyceride level of 55 mg/dL. It was postulated that chylous ascites ensued after thoracic duct disruption from severe inflammatory changes related to pancreatitis. This hypothesis was further supported by the presence of a coinciding chylothorax at the time of diagnosis. The patient’s hospital course was complicated by infection of the pancreatic necrosis requiring open necrosectomy, which did not reveal any other underlying abdominal pathology to account for the chylous ascites. Ultimately, after a prolonged hospital course and extended recovery, the patient returned home. Subsequent outpatient follow-up failed to reveal a cause of his necrotizing pancreatitis.

Abdominal Swelling in a Newborn With Trisomy 21

A newborn with trisomy 21 presents with abdominal swelling (Figure 1). Figure 1. Abdominal Swelling 19 days after birth ### Prenatal and Birth Histories The spontaneous vaginal delivery in the hospital had no complications. ### Physical Examination at Birth ### Later Findings

Impact of Nutritional Supplementation in Patients with Alcoholic Cirrhosis and Alcoholic Hepatitis: A Meta-Analysis of Randomized Placebo Controlled Trials

Purpose: Whether nutritional supplementation (NS) is beneficial in patients with alcoholic liver disease (ALD) is controversial. We performed a meta-analysis of randomized studies to examine benefits of NS in ALD. Methods: Electronic databases (1980- 2010) were searched to include randomized controlled trials (RCT) on NS in alcoholic cirrhosis (AC) and/or alcoholic hepatitis (AH), and extract data on short-term mortality (STM) at 28 days, long term mortality (LTM) at 1 year, proportion of patients with resolution of encephalopathy or ascites, improvement in serum albumin, AST, or total bilirubin. Pooled data on categorical variables was reported as odds ratios (OR) with 95% confidence interval (CI), and continuous variables as differences in means (pretreatment - post treatment) with 95% CI. Data were considered signifi cant if the 95% CI on OR did not cross one, and on mean difference, did not cross 0. Random effects model was used for analyzing the pooled data. I2 and Egger's tests were used for assessing heterogeneity and publication bias, respectively. Results: A total of 19 RCT were included; 13 in AH patients (n=804), and six in AC (n=818). Seven RCT examined parenteral, and 12 enteral nutrition, of which five used branched chain amino acids (BCAA). RCTs differed with respect to patient cohorts, intervention details, and treatment duration. Pooled data showed trend for lower STM with NS for all studies: 0.87 [0.69-1.09], but not when analyzed separately for AH: 0.85 [0.52-1.38], or AC: 0.87 [0.67-1.13], or with BCAA supplementation: 0.93 [0.71-1.21]. However, STM improved significantly in nine RCTs, with difference between treatment and placebo groups of >1000 calories and/or 40 g protein/d: 0.64 [0.41-0.99]. There were trends for improved LTM: 0.69 [0.46-1.05] and encephalopathy 0.75 [0.51-1.11], without improvement in ascites 1.06 [0.44-2.56] in favor of supplementation. NS was associated with increase in mean serum albumin 2.08 [1.05 to 3.1] and decrease in AST 1.02 [0.18-1.86] and serum bilirubin 1.5 [0.49 - 2.5]. There were no adverse effects of NS. Conclusion: Nutritional supplementation in ALD patients is associated with improved liver tests and a trend for improvement in encephalopathy. A subgroup of patients with anorexia and markedly reduced intake derive potential survival benefit with supplementation. Because of strong medical rationale and good safety profile of nutritional supplementation, it should be considered a standard of care for ALD patients, especially in those with poor appetite and intake. Further large prospective studies are suggested to examine benefits of nutritional supplementation in patients with alcoholic liver disease.

Transjugular intrahepatic portosystemic shunt for the treatment of medically refractory ascites

This study was performed to assess the safety, efficacy, and clinical outcomes of transjugular intrahepatic portosystemic shunt (TIPS) creation for treatment of medically refractory ascites and to identify prognostic factors for clinical response, morbidity, and mortality. In this retrospective study, 80 patients (male:female, 52:28; mean age, 56 years; mean Model for End-Stage Liver Disease [MELD] score, 15.1) who underwent elective TIPS creation for refractory ascites between 1999-2012 were studied. A medical record review was performed to identify data on demographics, liver disease, procedures, and outcome. The influence of these parameters on 30-day, 90-day, and one-year mortality was assessed using binary logistic regression. Overall survival was analyzed with Kaplan-Meier statistics. TIPS was successfully created using covered (n=70) or bare metal (n=10) stents. Hemodynamic success was achieved in all cases. The mean final portosystemic pressure gradient (PSG) was 6.8 mmHg. Thirty-day complications included mild encephalopathy in 35% of patients. Clinical improvement in ascites occurred in 78% of patients, with complete resolution or a ≥50% decrease in 66% of patients. No predictors of response or optimal PSG threshold were identified. The 30-day, 90-day, and one-year mortality rates were 14%, 23%, and 33%, respectively. Patient age (P = 0.026) was associated with 30-day mortality, while final PSG was associated with 90-day (P = 0.020) and one year (P = 0.032) mortality. No predictors of overall survival were identified. TIPS creation effectively treats medically refractory ascites with nearly 80% efficacy. The incidence of mild encephalopathy is nontrivial. Older age and final PSG are associated with mortality, and these factors should be considered in patient selection and procedure performance.

8:16 AM Abstract No. 119 - TIPS for treatment of refractory ascites: A single-center review of safety, efficacy, and clinical outcomes

Purpose To assess the safety, efficacy, and clinical outcomes of transjugular intrahepatic portosystemic shunt (TIPS) creation for treatment of medically refractory ascites. Materials and Methods In this single institution retrospective study, 65 patients (M:F=43:22, mean age 55 years, mean Child-Pugh score 9.9, mean Model for End Stage Liver Disease or MELD score 15.2) who underwent elective TIPS creation for refractory ascites between 1999-2012 were selected for analysis. Medical record review was used to identify demographic (age, gender), liver disease (Child-Pugh, MELD score), procedure (indication, urgency, stent type, portosystemic gradient or PSG reduction), and outcome (3-month pre- and post- procedure frequency of paracenteses, complications) data, and the influence of these parameters on 30- and 90-day mortality was assessed using multivariate analysis of variance. Results TIPS were successfully created using Wallstents (n=8) or Viatorr covered stent-grafts (n=57). Hemodynamic success was achieved in all cases. Mean PSG reduction was 13.5 mm Hg, and mean final PSG was 7.1 mm Hg. Complications at 30-days included encephalopathy (22/65, 33.9%) and liver failure (2/65, 3.1%). 30- and 90-day mortality rates were 7.7% (5/65) and 15.4% (10/65). Clinical improvement in ascites occurred in 78.5% (51/65) of patients. At 3-month follow-up, the need for paracentesis was completely eliminated in 32.3% (21/65), while the frequency of paracenteses was reduced by ≥50% in 32.3% (21/65) and by ≤50% in 13.9% (9/65). No change in ascites occurred in 9.2% (6/65), and 13.9% (9/65) required more frequent paracenteses. No PSG threshold was identified as most optimal for ascites reduction. Child-Pugh and MELD scores were significantly associated with 30- (P=0.001, 0.036) and 90-day (P=0.001, 0.004) mortality. No other parameters showed a statistically significant relationship with survival. Conclusion TIPS is a safe and effective procedure to treat medically refractory ascites, eliminating or reducing the need for paracenteses in 80% of cases within 3-months. While the rate of hepatic encephalopathy is non-trivial, these results further validate TIPS creation in this setting.

Raloxifene and Bevacizumab for severe complications of hereditary haemorrhagic telangiectasia in a haemodialysis patient.

Hereditary haemorrhagic telangiectasia (HHT), or Osler–Weber–Rendu syndrome, is inherited in an autosomal-dominant fashion. There are two different forms of HHT: HHT type 1, caused by mutations in the endoglin gene, located in chromosome 9 (which encodes endoglin), and HHT type 2, caused by mutations in the gene encoding activin A receptor type II-like 1 (ALK1) on chromosome 12. Mucocutaneous telangiectasias, arteriovenous malformations, epistaxis, gastrointestinal bleeding and iron-deficiency anaemia are the commonest clinical manifestations of HHT [1]. Hepatic involvement, characterized by different arteriovenous malformations within the liver parenchyma, occurs in up to 74% of patients with HHT [2]. As far as we know, no information has been published on therapeutic alternatives for haemodialysis patients suffering from severe complications of HHT. We report on a 59-year-old woman with chronic renal failure secondary to bilateral renal hypoplasia and undergoing haemodialysis, who received the diagnosis of HHT. She had presented relapsing epistaxis during the last year, with severe anaemia and hypotension. Due to the need for weekly transfusions and the poor tolerance to haemodialysis sessions, we consider treatment with raloxifene (60 mg/day), a selective estradiol receptor modulator. Raloxifene increases the expression of endoglin and ALK1, whose synthesis is defective in HHT patients, at the surface of endothelial cells [3]. A remarkable improvement of HHT symptoms along with a significant decrease in the number and severity of epistaxis was observed after raloxifene treatment. However, 6 months after the onset of raloxifene treatment, the patient presented with signs and symptoms of high-output cardiac failure, with severe ascites and a left pleural effusion. Increase of haemodialysis ultrafiltration along with large-volume paracentesis were started, but the patient showed a poor tolerance to these measures, owing to the presence of anaemia, hypotension and chronic liver disease. Serum biochemistry demonstrated a progressive cholestasis. A computed tomography (CT) scan showed liver cirrhosis with splenomegaly and massive arteriovenous malformations (Figure 1). Prompted by the progressive clinical deterioration of the patient, we decided to start treatment with bevacizumab, a humanized recombinant monoclonal antibody against the vascular endothelial growth factor (VEFG). The VEGF is a key regulator of angiogenesis and is upregulated in a variety of diseases. On the other hand, endoglin and ALK1 are involved in the transforming growth factor-β signalling pathway, which is a potent stimulator of VEFG production. Bevacizumab has been shown to be effective in diseases of abnormal angiogenesis, improving survival and symptomatology [4, 5]. Bevacizumab has been also used for the treatment of liver complications of HHT, inducing a clear improvement [6]. After obtaining the patient's informed consent, six courses of bevacizumab (5 mg/kg) were administered during a 3-month period and raloxifene was maintained without changes. A further reduction in the frequency of epistaxis was observed besides a remarkable improvement of ascites and liver enzymes. As shown in Figure 1, serial CT scans demonstrated a clear diminution of hepatic vascularity and a smooth contour of the liver. Fig. 1. CT scan of the liver with intravenous contrast before and 6 months after bevacizumab treatment, showing a dramatic reduction of vascularity and improvement of liver surface. Many different therapies have been proposed for epistaxis and hepatic involvement in HHT, but none of them have shown conclusive results. Hormonal therapy with estradiol for epistaxis and gastrointestinal management of HHT has shown a variable degree of efficacy depending on the patient. According to our experience, both raloxifene and bevacizumab are very effective alternatives for the treatment of severe HHT in haemodialysis patients.

Variceal recurrence, rebleeding rates and alterations in clinical and laboratory parameters following post‐variceal obliteration using endoscopic sclerotherapy

To study the rates of variceal recurrence and rebleeding following sclerotherapy and its effect on clinical and laboratory parameters in patients with portal hypertension. A total of 237 patients with portal hypertension together with esophageal variceal bleeding were included in the study. There were 138 patients with cirrhosis (group I), 42 with non-cirrhotic portal fibrosis (group II), and 57 with extrahepatic portal vein obstruction (group III). Baseline data and post-obliteration follow-up for rebleeding rates and changes in clinical and laboratory parameters were recorded. In all, 106 patients in group I, 31 in group II and 43 in group III experienced obliteration of varices. The recurrence of grade II varices occurred in 17 patients (9.4%) during a mean period of 9 months. Rebleeding from varices was observed in 4 patients (3.8%) in group I and 1 (3.2%) in group II, while none in group III experienced rebleeding. There was a significant improvement in ascites, jaundice, liver status, international normalized ratio and platelet count in group I patients after variceal eradication (P < 0.05). The main cause of death in the cirrhotic patients was active liver disease but not rebleeding. Following obliterative endoscopic sclerotherapy, rates of recurrence and rebleeding were significantly low when patients are kept under close observation. Disappearance of varices or reduction of variceal size improves the liver status in surviving cirrhotic patients.

Transjugular Intrahepatic Portosystemic Shunt (TIPS) is a Feasible Option for Management of Portal Hypertension in Pediatric Liver Transplant Recipients

Introduction: Recurrence of portal hypertension is a well-recognized clinical condition in both adult and pediatric liver transplant recipients. While retransplantation remains an option, the role of transjugular intrahepatic portosystemic shunt as a bridge to retransplantation or as a definitive therapy is still not well defined. In addition, creation of TIPS in liver transplant patients presents a significant challenge, more so in children. Data on feasibility and efficacy of TIPS in this patient population is scant. We present a case of successful placement of TIPS for management of refractory ascites in a 17-year-old female. Case Presentation: A 17-year-old female underwent a cadaveric split liver transplantation at the age of 13 years for Byler's disease. Post transplantation course was complicated by development of an anastomotic biliary stricture 1 year after the transplant requiring multiple endoscopic interventions followed by definitive surgical biliary reconstruction. Patient also had persistent elevation of liver enzymes (AST, ALT˜100 U/L) after the transplant. Serial liver biopsies done over the 3-year course after her transplant showed evidence of steatohepatitis and bridging fibrosis with no rejection. Patient remained asymptomatic until she presented with new onset of ascites 3 years after the transplantation, requiring repeated paracentesis despite maximal diuretic therapy. A transjugular liver biopsy along with portal pressure measurements demonstrated the presence of portal hypertension (corrected sinusoidal gradient 7 mm Hg) and cirrhosis. Decision was taken to place TIPS. Examination was remarkable for marked ascites, palpable liver, and peripheral edema. Pre-TIPS labs were bilirubin 0.5 mg/dl, AST 71 U/L, ALT 88 U/L, ALP 176 U/L, creatinine 0.94 mg/dl, INR 1.2. MELD score was 8 and Child-Pugh score was 7. Patient underwent successful creation of TIPS with post procedure portoatrial gradient of 3 mm Hg. Subsequent to TIPS creation, patient had clinical improvement in ascites and did not require further paracentesis. 3 months after TIPS, patient had recurrence of ascites associated with stenosis at the hepatic venous end of the stent on vascular ultrasound of the liver. Patient underwent successful revision of TIPS with reduction of portoatrial gradient from 15 mm Hg to 5 mm Hg with no immediate complications. Conclusion: TIPS can be created safely and successfully in children with split liver transplant. TIPS should be considered as a treatment modality in the management of portal hypertension in pediatric liver transplant recipients.