Transjugular intrahepatic portosystemic shunts for refractory ascites after liver transplantation

PURPOSE: To study the efficacy of transjugular intrahepatic portosystemic shunts (TIPS) in the management of refractory ascites after liver transplantation. PATIENTS AND METHODS: Between January 1995 and December 2003, 309 primary adult liver transplants were performed. Refractory ascites was defined as active interventions (salt restriction, diuretic use, repeated paracentesis) needed beyond 30 days after transplantation. These patients were managed with TIPS placement. RESULTS: Eight TIPS were placed in 8 patients at a mean of 11.5 months after transplantation (range, 2-36 months). There were 5 males and 3 females, age 54 +/- 8.2 years. Hepatitis C was the primary diagnosis in 7 patients and primary biliary cirrhosis in 1. Indications for TIPS included refractory ascites (8), associated variceal bleeding (2), and various degrees of hepatic vein outflow stenosis (3). Seven patients had resolution of ascites and associated findings of portal hypertension, and 1 patient with persistent ascites had severe hepatic vein outflow stenosis and associated hepatitis C in the allograft. Two patients required retransplantation for recurrent hepatitis C. There were 3 deaths: liver failure (1), organ failure after retransplantation (1), and lung cancer 5 months after TIPS (1). Currently, 5 patients are alive without clinical evidence of ascites 9, 13, 15, 24, and 70 months after TIPS. CONCLUSIONS: The TIPS device can be used safely and effectively to control refractory ascites after liver transplantation. In the setting of organ dysfunction, these patients should be considered sooner for retransplantation.     

Management of medically refractory ascites

BACKGROUND: Medically refractory ascites is a clinical entity for which there exists few effective therapeutic options. Available treatment modalities include diuresis and sodium restriction, peritoneovenous shunt, liver transplant, transjugular intrahepatic portosystemic shunts and surgical shunts, and large-volume paracentesis. Herein we review the current therapeutic options for medically refractory ascites focusing on indications, benefits, and drawbacks of each specific therapy. DATA SOURCES: Data and recommendations are based on the authors' cumulative experience with complicated cirrhotic and cancer patients and on past and current literature addressing intractable ascites. CONCLUSIONS: The absence of a single, effective therapy in the management of refractory ascites speaks to the complex nature of this complication. Although most patients will respond to medical management, thoughtful application of available therapeutic options in patients who fail, as described herein, not only makes decisions regarding their care easier but also provides the best palliation in a vexing clinical scenario.     

Management of umbilical hernia in cirrhotic patients

The treatment of umbilical hernia in the setting of cirrhosis poses unique and specific management problems due to the pathophysiology of cirrhotic ascites. The high intra-abdominal pressures generated by ascites when applied to areas of parietal weakness are the cause of hernia formation and enlargement. Successful surgical treatment depends on minimization or elimination of ascites. Umbilical rupture and hernia strangulation are the most life-threatening complications of umbilical hernia with ascites and they demand urgent surgical intervention. In non-emergency situations, medical therapy to control ascites should precede hernia repair. When ascites is refractory to medical therapy, treatment will vary depending on whether transplantation is an option. In liver transplantation candidates, hernia repair can be performed at the end of the transplantation procedure. If transplanation is not envisaged, concomitant treatment of both ascites and hernia is best achieved by placement of a peritoneo-venous shunt at the time of the parietal repair.     

Use of splenic artery embolization to relieve tense ascites following liver transplantation in a patient with paroxysmal nocturnal hemoglobinuria.

Recurrent venous thrombosis following liver transplantation for Budd-Chiari syndrome is common, particularly in the setting of an underlying myeloproliferative disorder. We describe a patient who developed refractory ascites due to portal vein thrombosis following liver transplantation for Budd-Chiari syndrome in the setting of paroxysmal nocturnal hemoglobinuria. Extensive portal vein thrombosis, dense abdominal adhesions, and portosystemic collaterals precluded the use of a transjugular intrahepatic portosystemic shunt or surgical portosystemic shunt to manage the patient's ascites. Splenic artery embolization to decrease portal hypertension was performed, and this resulted in complete resolution of ascites. This case demonstrates the successful use of splenic artery embolization to manage ascites due to portal vein thrombosis following liver transplantation. Splenic artery embolization may be considered as an alternative option for the management of refractory ascites due to portal hypertension in patients who are unable to undergo safe transjugular intrahepatic portosystemic shunt or surgical shunt placement.     

肝移植时代的门静脉高压治疗

胃食管静脉曲张出血是门静脉高压的常见并发症。药物和内窥镜治疗是静脉曲张的基础治疗。经颈静脉肝内门体静脉分流被推荐用于处理难治性或复发性胃食管静脉曲张出血。当患者存在危及生命的出血风险,而传统治疗风险较高、存在禁忌或效果不理想时,应选择肝移植治疗。传统治疗可以获得短期疗效,甚至可以较长时间稳定病情,但如果这些治疗导致门静...     

Ascites after liver transplantation--a mystery.

Ascites after liver transplantation, although uncommon, presents a serious clinical dilemma. The hemodynamic changes that support the development of ascites before liver transplantation are resolved after transplant; therefore, persistent ascites (PA) after liver transplantation is unexpected and poorly characterized. The aim of this study was to define the clinical factors associated with PA after liver transplantation. This was a retrospective case-control analysis of patients who underwent liver transplantation at the University of Pennsylvania. PA occurring for more than 3 months after liver transplantation was confirmed by imaging studies. PA was correlated with multiple recipient and donor variables, including etiology of liver disease, preoperative ascites, prior portosystemic shunt (PS), donor age, and cold ischemic (CI) time. There were 2 groups: group 1, cases with PA transplanted from November 1990 to July 2001, and group 2, consecutive, control subjects who underwent liver transplantation between September 1999 and December 2001. Both groups were followed to censoring, May 2002, or death. Twenty-five from group 1 had ascites after liver transplantation after a median follow-up of 2.6 years. In group 1 vs group 2 (n = 106), there was a male predominance 80% vs 61% (P =.10) with similar age 52 years; chronic hepatitis C virus (HCV) was diagnosed in 88% vs 44% (P <.0001); preoperative ascites and ascites refractory to treatment were more prevalent in group 1 (P =.0004 and P =.02, respectively), and CI was higher in group 1, (8.5 hours vs 6.3 hours, P =.002). Eight of the 25 (group 1) had portal hypertension with median portosystemic gradient 16.5 mm Hg (range, 16-24). PS was performed in 7 of 25 cases, which resulted in partial resolution of ascites. The development of PA after liver transplantation is multifactorial; HCV, refractory ascites before liver transplantation, and prolonged CI contribute to PA after liver transplantation.     

Portal hypertension and ascites

Portal hypertension is secondary to increased resistance to blood flow and increased blood flow through the portal system. The most common cause is liver cirrhosis. The most severe and life-threatening presentation of portal hypertension is acute variceal bleeding. Pharmacotherapy with vasoactive agents (terlipressin or somatostatin), endoscopic band ligation and radiological treatment with transjugular intrahepatic portosystemic shunt (TIPSS) are the most common treatment options for variceal bleeding. However, where surgical expertise exists, portosystemic shunts can be considered for refractory bleeding in patients without significant liver failure, especially when TIPSS is unavailable or contraindicated. Diuretic therapy with spironolactone and furosemide are the basis for the management of ascites. If ascites becomes refractory, repeat large volume paracentesis and TIPSS are potential treatment options. Liver transplantation offers the definitive treatment for portal hypertension secondary to cirrhosis as it cures the underlying liver disease.     

Portal hypertension and ascites

Portal hypertension is secondary to increased resistance to blood flow and increased blood flow through the portal system. The commonest cause is liver cirrhosis. The most severe and life-threatening presentation of portal hypertension is acute variceal bleeding. Pharmacotherapy with vasoactive agents (terlipressin or somatostatin), endoscopic band ligation and radiological treatment with transjugular intra-hepatic portosystemic shunt (TIPSS) are the commonest treatment options for variceal bleeding. However, where surgical expertise exists, portosystemic shunts can be considered for refractory bleeding in patients without significant liver failure, especially when TIPSS is unavailable or contraindicated. Diuretic therapy with spironolactone and furosemide are the basis for the management of ascites. If ascites becomes refractory, repeat large volume paracentesis and TIPSS are potential treatment options. Liver transplantation offers the definitive treatment for portal hypertension secondary to cirrhosis as it cures the underlying liver disease.     

Orthotopic liver transplantation in case of TIPS stent dislocation

Transjugular intrahepatic portosystemic shunts (TIPS) are indicated in patients with liver cirrhosis and portal hypertension for treatment of variceal bleeding or refractory ascites. Additionally implantation of stents may lead to stent dislocation or thrombosis in up to 20 % of cases. Detailed information about stent dislocation and its impact on subsequent orthotopic liver transplantation (OLT) is rare regarding the literature. We report on a patient suffering from ethyltoxic liver cirrhosis in which OLT was technically complicated by a thrombosed TIPS stent, dislocated in the portal vein. This stent was implanted prior to OLT due to refractory ascites and partial portal vein thrombosis. We conclude that TIPS stent insertion, especially in liver transplant candidates, should only be performed by radiologists in centers with expertise and experience.     

Strict volume control in the treatment of nephrogenic ascites.

BACKGROUND: Nephrogenic ascites refers to the condition of refractory ascites of unknown aetiology and occurs mainly in patients with end-stage renal disease who are undergoing haemodialysis. Despite many treatment modalities, nephrogenic ascites remains difficult to control and has a poor prognosis. METHODS: We investigated six such patients who had developed severe, apparently refractory ascites during haemodialysis. They all had seriously disturbed cardiac dimensions and function. They were treated with repeated isolated ultrafiltration and severe salt restriction, while their cardiac functions were monitored with echocardiography. RESULTS: After a mean of 18+/-4 l of fluid per patient was removed in 27+/-8 days, ascites disappeared in all patients. Blood pressure and cardiothoracic indices were decreased from 130+/-20/83+/-10 to 95+/-11/60+/-6 mmHg (P<0.02) and from 0.61+/-7 to 0.47+/-5 (P<0.02), respectively. At the end of treatment, heart rates had decreased from 102+/-10 to 85+/-6 beats/min. Previously increased left atrial diameters, end-systolic and end-diastolic dimensions of the left ventricles, and right ventricular diameters reached normal values. Ejection fractions initially decreased in all patients, and then increased slightly to markedly after treatment. CONCLUSION: Nephrogenic ascites is a component of right-sided cardiac congestion mediated by volume overload, and it should be treated with severe salt restriction and frequent ultrafiltration with haemodialysis and, if that fails, with daily isolated ultrafiltration.     

Portal hypertension and ascites

Portal hypertension occurs secondary to increased resistance to portal blood flow. It is a principle consequence of liver cirrhosis and leads to severe life-threatening complications, such as variceal bleeding, ascites and hepatic encephalopathy. Acute variceal bleeding is a medical and surgical emergency requiring a multidisciplinary management approach. Prompt resuscitation along with pharmacotherapy agents (terlipressin or somatostatin analogues) followed by early endoscopic variceal banding is the cornerstone of effective treatment. Refractory bleeding despite endoscopic band ligation requires emergency trans-jugular intrahepatic portosystemic shunt (TIPSS). Diuretic therapy with spironolactone and furosemide are the first line of management of ascites. If ascites becomes refractory, repeat large volume paracentesis (LVP) and TIPSS are potential treatment options. Liver transplantation remains the only curative option for all patients with portal hypertension, but a careful selection policy and assessment is mandatory when considering transplantation.     

Management in patients with liver cirrhosis and an umbilical hernia

BACKGROUND: Optimal management in patients with umbilical hernias and liver cirrhosis with ascites is still under debate. The objective of this study was to compare the outcome in our series of operative versus conservative treatment of these patients. METHODS: In the period between 1990 and 2004, 34 patients with an umbilical hernia combined with liver cirrhosis and ascites were identified from our hospital database. In 17 patients, treatment consisted of elective hernia repair, and 13 were managed conservatively. Four patients underwent hernia repair during liver transplantation. RESULTS: Elective hernia repair was successful without complications and recurrence in 12 out of 17 patients. Complications occurred in 3 of these 17 patients, consisting of wound-related problems and recurrence in 4 out 17. Success rate of the initial conservative management was only 23%; hospital admittance for incarcerations occurred in 10 of 13 patients, of which 6 required hernia repair in an emergency setting. Two patients of the initially conservative managed group died from complications of the umbilical hernia. In the 4 patients that underwent hernia correction during liver transplantation, no complications occurred and 1 patient had a recurrence. CONCLUSIONS: Conservative management of umbilical hernias in patients with liver cirrhosis and ascites leads to a high rate of incarcerations with subsequent hernia repair in an emergency setting, whereas elective repair can be performed with less morbidity and is therefore advocated.     

活体肝移植后小肝综合征4例

目的:探讨活体肝移植后小肝综合征的病因及其诊治。方法:结合文献,回顾性分析4例小肝综合征的临床特点及治疗经验。结果:4例均有高胆红素血症,2例出现顽固性腹水,最终2例死亡,1例经保守治疗治愈,1例经急诊再次肝移植后治愈。结论:小肝综合征是活体肝移植术后严重并发症,诊治较困难;术前CT评估体积并不能绝对避免小肝综合征的发生;严重者需行再次肝移植。     

Chylous ascites after radical nephrectomy and inferior vena cava thrombectomy. Successful conservative management with somatostatin analogue

Postoperative chylous ascites is a rare complication of retroperitoneal surgery. The treatment of postoperative chylous ascites is primarily conservative, consisting of repeated paraceteses, medium chain triglyceride (MCT) diet, salt restriction, diuretics and bowel rest with total parenteral nutrition. Occasionally, chylous ascites may take a protracted course which may necessitate insertion of peritoneo-venous shunts or direct surgical lymphostasis. Recently, Somatostatin was shown to be highly effective in closure of refractory lymphatic fistulas. We present a case of refractory chylous ascites following radical nephrectomy with inferior vena caval thrombectomy that failed to respond to conventional conservative measures and resolved rapidly following the administration of Somatostatin.     

Budd-Chiari syndrome. A report of 3 cases

Three patients with the Budd-Chiari syndrome are presented. This is a rare condition characterized by hepatomegaly, progressive and refractory ascites, distension of the abdominal wall veins, abdominal pain and leg oedema. These features are attributed to congestion of the liver and portal hypertension. The condition has been notoriously difficult to treat medically. Surgical measures are directed towards relieving the liver congestion and lowering pressure in the portal system by portal-systemic shunting operations. In some cases refractory ascites may be treated by peritoneovenous shunting with a Le Veen shunt. In a select group of patients orthotopic liver transplantation has proved to be worth while.     

Liver transplantation with simultaneous removal of an intracardiac transjugular intrahepatic portosystemic shunt and a vena cava filter without the utilization of cardiopulmonary bypass.

Transjugular intrahepatic shunts (TIPSs) are widely used in the management of portal hypertension complications including variceal bleeding, refractory ascites, and hepatic hydrothorax. Vena cava filters (VCFs) are an important therapeutic modality in the prevention of pulmonary emboli in patients suffering deep venous thrombosis and clinical contraindications for anticoagulation. Stent and filter misplacement or migration may occur, complicating liver transplantation (LT) surgery. We describe the intraoperative management of a patient with cirrhosis, who had a TIPS extending into the right atrium (RA) and a retrohepatic VCF. Stent and filter removals were deferred until the time of LT. Both procedures were performed successfully by complete cava and portal reconstruction. In conclusion, careful assessment and surgical management of patients with stent and filters permits successful LT.     

Mesocaval shunt as an alternative treatment for persistent ascites after liver transplantation: case reports

Refractory ascites after liver transplantation is a relatively rare complication. If the initial medical treatment fails, more invasive techniques may be required. The TIPS procedure has emerged as a major treatment option for decompression of the portal venous system. Mesocaval shunt can be an alternative to TIPS in selected cases. We describe two patients who underwent mesocaval shunt construction for refractory ascites.     

Lymphedema tarda after liver transplantation: a case report and review of the literature

We present a patient with lymphedema that developed after orthotopic liver transplantation. The cause of the posttransplant lymphedema was likely related to a developmental abnormality of the lymphatic system that was exaggerated by refractory chylous ascites. A peritoneal fluid with a milky appearance, chylous ascites is rich in triglyceride and is caused by the obstruction or disruption of abdominal lymphatic channels. It is a rare complication that may develop after trauma or abdominal surgery or as a result of a malignant disease, and it is even more uncommon after liver transplantation. Therapy for chylous ascites involves treating its underlying cause. In the patient we describe, lymphedema tarda, which was diagnosed 6 months after liver transplantation, was likely caused by chylous ascites and a developmental abnormality of the lymphatic system.     

Preoperative natriuretic peptide-B values and ascites in male liver transplant recipients

Plasma preoperative values of natriuretic B peptide (pro-BNP) were correlated with ascites in men experiencing hepatic cirrhosis due to different etiologies on the active waiting list for liver transplantation. The study was performed in 54 male recipients of a liver transplant. Written informed consent was obtained from the patients or their relatives, and the study protocol was approved by our local Clinical Research (Ethics) Committee. Male patients were classified into two groups: group 1 included patients with alcoholic hepatic cirrhosis (n = 30) distributed as 19 men with no ascites, four with nonrefractory ascites, and seven with refractory ascites; group 2 included cases of viral hepatitis cirrhosis (n = 24) distributed as 13 men with no ascites, nine with non-refractory ascites, and two with refractory ascites. A group of six healthy male volunteers was used to establish normal (basal) values of pro-BNP and left auricular diameter (LAD). Pro-BNP values were determined in plasma samples by an electrochemiluminiscence immunoassay. Pro-BNP plasma levels in patients with alcoholic cirrhosis were threefold greater among patients with no ascites or no refractory ascites compared with healthy men, whereas pro-BNP values were fivefold enhanced among alcoholic patients with refractory ascites. The viral hepatitis cirrhosis group showed pro-BNP plasma values 1.5-fold enhanced in men with no ascites, whereas pro-BNP reached fivefold with either nonrefractory or refractory ascites. The enhanced pro-BNP plasma levels indicated advanced hepatic degradation, seemingly related to the presence of refractory ascites associated with cirrhosis.