Vitamin A toxicity: when one a day doesn't keep the doctor away.

Vitamin A toxicity has been reported to cause severe liver disease and, occasionally, liver failure. Herein we present the case of a 60-year-old male with symptoms of muscle soreness, alopecia, nail dystrophy, and ascites. He continued to deteriorate with the development of refractory ascites, renal insufficiency, encephalopathy, and failure to thrive. A liver biopsy demonstrated presence of Ito cells and vacuolated Kupffer cells without the presence of cirrhosis. His clinical history revealed ingestion of large doses of vitamin A. His worsening clinical situation ruled out the possibility of a transjugular intrahepatic portosystemic shunt. The patient underwent orthotopic liver transplantation with resolution of symptoms. Vitamin A toxicity should be considered in the differential diagnosis of noncirrhotic portal hypertension. In conclusion, liver transplantation is a valid option if no improvement occurs in spite of cessation of the medication.     

Successful Treatment of a Patient With Diffuse Portosplenomesenteric Thrombosis Using a Pericholedochal Varix for Portal Flow Reconstruction During Deceased Donor Liver Transplantation: A Case Report

Portal vein thrombosis remains a challenging issue in liver transplantation. When thrombectomy is not feasible due to diffuse portosplenomesenteric thrombosis, other modalities are adapted such as the use of a jump graft or portal tributaries or even multivisceral transplantation. For patients with diffuse thrombosis of the splanchnic venous system, a large pericholedochal varix can be a useful vessel for providing splanchnic blood flow to the graft and for relieving portal hypertension. We report our experience of successfully treating a patient with diffuse portosplenomesenteric thrombosis using a pericholedochal varix for portal flow reconstruction during deceased donor liver transplantation and eventually preventing unnecessary multivisceral transplantation. A 56-year-old man diagnosed with liver cirrhosis due to hepatitis B underwent deceased donor liver transplantation due to refractory ascites. Preoperative imaging revealed diffuse portosplenomesenteric thrombosis with large amount of ascites. During the operation, dissection of the main portal vein was not possible due to the development of multiple large pericholedochal varices and cavernous change of the main portal vein. After outflow reconstruction, portal inflow was restored by anastomosing the graft portal vein to a large pericholedochal varix. Postoperatively, although abdominal computed tomography scan showed stenosis of portal vein anastomosis site, liver function tests improved, and Doppler sonogram revealed no flow disturbance. During follow-up, the patient repeatedly developed hydrothorax and ascites. In addition, stenosis of the portal vein anastomosis and thrombosis of the portomesenteric system still remained. The patient underwent transhepatic portal vein stent insertion. After portal vein stent insertion, hydrothorax and ascites improved and the extent of thrombosis of the portomesenteric system decreased without anticoagulation therapy. In conclusion, enlarged pericholedochal varix in patients with totally obliterated splanchnic veins can be a source of useful inflow to restore portal flow and decrease the extent of thrombosis, thereby preventing unnecessary multivisceral transplantation.     

Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation

Nodular regenerative hyperplasia of the liver is an uncommon cause of portal hypertension. Patients with nodular regenerative hyperplasia have signs and symptoms of portal hypertension, without evidence of hepatocellular failure or encephalopathy. We report the case of a 44-year-old woman with recurrent esophageal bleeding and refractory ascites who had a history of hemosiderosis, hepatitis C, and chronic renal allograft rejection. Our preoperative diagnosis was cirrhotic end-stage liver disease and end-stage renal disease for which the patient underwent combined hepatic and renal transplantation. Her portal hypertension symptoms resolved, and her renal function has been normal for 18 months of follow-up. Histologic examination of the liver revealed nodular regenerative hyperplasia, and a review of the literature regarding the surgical management of patients with nodular regenerative hyperplasia revealed that various shunting procedures are generally recommended. After the failure of medical management in patients with nodular regenerative hyperplasia, portosystemic shunting may be indicated before proceeding to hepatic transplantation.     

Management of Portal Hypertension After Liver Transplantation

Introduction

Post-transplantation portal hypertension has severe complications, such as esophageal varix bleeding, therapy refractory ascites, extreme splenomegaly, and graft dysfunction. The aim of our study was to analyze the effectiveness of the therapeutic strategies and how to visualize the procedure.

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.     

Successful Living-Donor Liver Transplantation With Intraoperative Endovascular Recanalization via Transsplenic Access in a Recipient With Grade III Portal Vein Thrombosis: A Case Report

Extensive portosplenomesenteric thrombosis is regarded as a relative contraindication to liver transplantation because of the complexity of the surgical procedure. This report describes a case of living-donor liver transplantation (LDLT) for a patient with extensive portosplenomesenteric thrombosis, in whom portal flow was successfully restored by intraoperative transplenic portal vein and superior mesenteric vein stenting after surgical thrombectomy. The patient’s liver function remained normal with a patent portal vein stent 6 months after LDLT, and Doppler ultrasonography demonstrated a normal wave form for portal flow. To the best of our knowledge, this is the world’s first case of endovascular management of the portal vein via percutaneous transsplenic access during LDLT, demonstrating that transsplenic access can be an alternative approach without liver graft injury when the superior mesenteric vein branch and inferior mesenteric vein cannot be used as access routes.     

Orthotopic liver transplantation for portosystemic encephalopathy in an adult with congenital absence of the portal vein.

Congenital absence of the portal vein (CAPV) is a very rare venous malformation in which mesenteric venous blood drains directly into the systemic circulation. There is no portal perfusion of the liver and no portal hypertension. This abnormality is usually coincidentally discovered in children, the majority of whom have no signs of encephalopathy and only slightly abnormal liver function tests. Additional anomalies common in CAPV are cardiovascular abnormalities and hepatic tumors. To date, only 5 adult patients (>18 years) with CAPV have been described, none of whom underwent liver transplantation. We describe a 45-year-old man with CAPV and end-stage renal insufficiency due to focal segmental glomerulopathy, who developed therapy-resistant encephalopathy with intermittently high ammonia levels. The patient underwent a combined liver and kidney transplantation and is doing well at 2.5 years of follow-up. Histopathological examination of the native liver showed no portal vein branches in the portal tracts. In conclusion, our experience suggests that, although children with CAPV usually have no symptoms of encephalopathy, this may still develop at a later stage in adult life. When encephalopathy becomes refractory to medical therapy, orthotopic liver transplantation (OLT) can be successfully performed with restoration of normal cerebral function.     

Pancreas Transplantation After Liver Transplantation: A Case Report

Our aim was to describe the clinical indications, surgical technique, and clinical outcomes of a pancreas transplantation, performed 4 years after liver transplantation, as treatment for new-onset, uncontrolled diabetes mellitus in a 53-year-old man. Liver transplantation was performed for end-stage liver disease secondary to hepatitis B virus infection and hepatocellular carcinoma. The patient had no history of diabetes prior to the liver transplantation. The decision to proceed with a pancreas transplantation was made when the patient's blood sugar levels could not be normalized despite insulin doses >100 IU/d. A modified cadaveric transplantation technique was used, with the recipient's inferior vena cava dissected for anastomosis with the portal vein of the graft, using a diamond-shaped patch procedure. Moreover, the right common iliac artery was anastomosed with a Y-graft in the pancreas graft, and the duodenum remnant of the graft was anastomosed to the recipient's duodenum using a side-to-side procedure. The 6-month postoperative follow-up included repeated endoscopic biopsy of the graft duodenum, with no evidence of thrombosis or rejection of the graft, with glucose level within normal limits without requirement for diabetic drugs. To our knowledge, this is the first reported case of pancreas transplantation after liver transplantation.     

Morbidly Obese Patients Awaiting Liver Transplantation—Sleeve Gastrectomy: Safety and Efficacy From a Liver Transplant Unit Experience

Background

The prevalence of obesity has increased dramatically, even in the population awaiting a liver transplantation. Despite their associated complications, we cannot consider morbid obesity any longer as an absolute contraindication to liver transplantation. Dietary approaches alone are usually completely ineffective. Bariatric surgery is the gold-standard treatment for morbid obesity and can be performed before, during, or after transplantation.

Budd-Chiari syndrome--case presentation

The Budd-Chiari syndrome represents the obstruction of hepatic veins usually due to a hepatocarcinoma. We present the case of a 68 year old patient, in medical evidence for ten years with a Child A ethanolic liver cirrhosis, who was admitted in emergency for hematemesis and melena. Clinical examination and the laboratory findings at the admittance revealed signs of decompensated cirrhosis and severe anemia. Ultrasound examination showed a cirrhotic liver with portal hypertension signs and a multinodular mass in the right lobe of the liver with portal, biliary and right hepatic vein invasions extended to inferior caval vein. In upper digestive endoscopy stage IV esophageal varices were evidenced with signs of recent bleeding (sclerotherapy was performed) along with gastric varices and portal gastropathy. The particularity of the case consists in the invasive complications of the hepatocarcinoma regarding hepatic and inferior caval veins wich defines the Budd-Chiari syndrome (posthepatic portal hypertension added to the intrahepatic and prehepatic ones), the invasions of the biliary tract and portal vein being more frequent.     

Portal vein stenosis: a rare yet clinically important cause of delayed-onset ascites after adult deceased donor liver transplantation: two case reports

Vascular complications following liver transplantation are well documented. While complications involving the portal vein are less common than the hepatic artery, portal vein complications can lead to potentially life-threatening sequelae including graft loss. Portal vein stenosis is an infrequent complication following liver transplant. The majority of these complications are seen in living donor liver transplants and pediatric liver transplants. We present 2 cases of delayed onset portal vein stenosis in adult deceased donor liver transplantation (ADDLT). The first patient presented with refractory ascites twelve months after ADDLT. He was diagnosed and successfully treated with percutaneous transhepatic portovenography and venoplasty. The second patient had a history of irradiation to his portal bed in the setting of cholangiocarcinoma. He developed refractory ascites and esophageal variceal bleeding >2 years after ADDLT. He underwent percutaneous transhepatic portovenoplasty, but eventually required placement of a portal stent due to continued problems with recurrent ascites. These 2 cases highlight the importance of considering portal vein stenosis in the differential diagnosis of late-onset ascites following liver transplantation, especially if there have been any predisposing risk factors such as portal bed irradiation or prior splenectomy.     

Liver transplantation for sinusoidal obstructive syndrome (veno-occlusive disease): case report with review of the literature and the UNOS database

Abstract: Background:  Severe sinusoidal obstructive syndrome (SOS) is a life-threatening complication of stem cell transplantation. We report the case of a young man transplanted for SOS.
Method:  A single chart review with query of the United Network of Organ Sharing database and review of the medical literature.
Case:  A 23-yr-old male diagnosed with chronic myeloid leukemia underwent a matched unrelated stem cell transplant. The conditioning regimen included high-dose cyclophosphamide and busulfan. Within one month, he developed painful hepatomegaly, jaundice, ascites, and weight gain, and was diagnosed with biopsy-proven SOS. Despite therapy with defibrotide, he continued to deteriorate with the development of progressive renal failure and encephalopathy. The patient underwent orthotopic liver transplantation. After surgery, he developed cytomegalovirus infection and six wk later presented with a bile leak, hepatic artery thrombosis, and a liver abscess. A repeat bone marrow biopsy showed no evidence of recurrent disease. Although the patient was listed for re-transplantation, he succumbed prior to an organ becoming available.
Conclusion:  Severe SOS in the setting of bone marrow transplantation portends a poor prognosis. Careful patient selection, timing, and perhaps less immunosuppression should be considered when performing a liver transplantation in the setting of severe SOS.     

Extensive Inferior Vena Cava Thrombosis in a Liver Transplant Patient: A Case Report

Thrombosis of the inferior vena cava is a clinical condition with very diverse presentations, ranging from asymptomatic patients to others with severe edema in the legs and lower torso. We report the case of a 27-year-old female patient, previously diagnosed with autoimmune hepatitis, with asymptomatic extensive thrombosis of the inferior vena cava. The thrombus extended from the renal veins up to the emergence of the hepatic veins, causing post-sinusoidal portal hypertension (Budd-Chiari syndrome). The patient underwent an orthotopic cadaveric liver transplant with removal of the retrohepatic vena cava and thrombectomy of blood clots from the infrahepatic vena cava. She initially recovered well from surgery, but on the 8 postoperative day she had a significant increase in hepatic injury markers and was diagnosed with rethrombosis of the inferior vena cava and hepatic veins. A surgical thrombectomy was performed, with an intraoperative finding of chronic thrombus in both renal veins, previously undiagnosed. The thrombectomy was successful, but the patient's hepatic function continued to worsen and a second liver transplant was performed. After the second transplant she underwent several imaging exams that showed no signs of rethrombosis. She was kept on postoperative anticoagulation indefinitely, first with intravenous heparin then with rivaroxaban. An extensive investigation failed to identify any causes of thrombophilia associated with this vast thrombosis. She is currently alive and with good graft function 1 year and 4 months after the second transplant.     

Ten-Year Experience in Porto-Caval Hemitransposition for Liver Transplantation in the Presence of Portal Vein Thrombosis

Porto-caval hemitransposition (PCH) in liver transplantation allows revascularization of the liver when the porto-mesenteric axis is thrombosed. We, here, review our experience over an 11-year period. A total of 23 patients underwent liver transplantation using PCH. Immunosuppression was based on tacrolimus, with sirolimus used in case of renal insufficiency. Most common diagnoses were hepatitis C, Laennec's, Budd-Chiari and cryptogenic cirrhosis. Six patients needed splenectomy prior to transplant, 5 during transplant, 1 post-transplant, 11 had no splenectomy. Overall survival was 60% at 1 year and 38% at 3 years, with 10 of 23 patients currently alive and the longest survivor at 9.3 years. Most common cause of death was sepsis/multisystem organ failure, followed by pulmonary embolism. A total of 7/23 patients experienced post-operative gastrointestinal bleeding episodes, 6/23 patients developed thrombosis of the vena cava (median 162 days post-op). Post-operative ascites was noted in almost all patients. Renal dysfunction was commonly seen even after the first month post-transplant. PCH offers a feasible option for liver transplantation in those patients with complex thrombosis of the mesenteric and portal circulation.     

Biliary liver cirrhosis secondary to cystic fibrosis: a rare indication for liver transplantation

As more effective therapies prolong the lives of patients with cystic fibrosis, there are now more patients in this population diagnosed with liver diseases. Secondary biliary cirrhosis is not a rare complication of mucoviscidosis. It is diagnosed in 20% of patients with mucoviscidosis; in 2% it is accompanied by portal hypertension. On average patients with portal hypertension and its complications are 12 years old. Liver transplantation is an accepted method of treatment for children with cystic fibrosis and portal hypertension. It eliminates the cause of the portal hypertension, decreases life-threatening medical conditions, and improves their nutritional status and quality of life. Despite immunosuppressive treatment they do not seem to beat increased risk of upper respiratory tract infections. On the contrary improved respiratory function and status are generally observed. We present our first case of orthotopic liver transplantation performed in a 29-year-old man with cystic fibrosis. The donor was a 42-year-old woman who died of a ruptured cerebral aneurysm. The surgery was performed in September 2004. The patient received immunosuppression based on steroids, basiliximab, tacrolimus, and mycophenolic acid due to renal insufficiency. Antibiotic (meropenem) and antiviral prophylaxis (gancyclovir) were used. A 6-month period of observation confirmed the clinical data from the pediatric population-a good prognosis with improved nutritional status, respiratory function, and quality of life.     

Effective early treatment of hepatic venoocclusive disease with a central splenorenal shunt in an infant.

Venoocclusive disease of the liver (VOD) is a well-described complication following chemotherapy. It is manifested by jaundice and signs of portal hypertension and carries a mortality rate approaching 50%. There is no known treatment for the disease itself, although several recent reports suggest portacaval diversion may be effective in treating its sequelae. A 6.75-kg 8-month-old boy with VOD following bone marrow ablation and bone marrow transplantation (BMT) for juvenile chronic myelogenous leukemia (JCML) is presented. Over a 6-week period following bone marrow ablation he developed ascites refractory to diuretics, jaundice, and hematemasis with normal hepatocellular function. Splenectomy with a central splenorenal shunt was performed, which resulted in a significant reduction in portal pressures and complete resolution of his ascites and hematemasis without resultant encephalopathy. We propose that central end-to-side splenorenal shunting is an acceptable treatment for portal hypertension due to VOD and can be successfully performed in infants.     

Portal Vein Stent Placement in Anastomotic Stenosis After Deceased Donor Liver Transplantation: A Case Report

Vascular complications (VCs) after liver transplantation (LT) frequently result in graft and patient loss. The smaller vessels and the insufficient length for reconstruction in living donor LT and pediatric transplantation predispose patients to a higher incidence of VCs. Herein we present a case of portal vein stenosis (PVS) in an adult deceased donor LT recipient with portal vein thrombosis requiring extended thrombectomy at the time of LT. He presented with ascites 4 months after LT, was diagnosed with PVS, and was successfully treated with percutaneous transhepatic venoplasty and placement of a portal stent. This case highlights the importance of Doppler ultrasound as a screening modality for detection of VCs after LT and the pivotal role of endovascular repair as a first-line treatment for PVS.     

Liver Adenomatosis: A Rare Indication for Living Donor Liver Transplantation

Liver adenomatosis (LA) is a rare benign disease of the liver with unclear pathogenesis, which is characterized by multiple hepatic adenomas. The management of LA remains controversial. Herein we have reported a case of LA treated by living donor liver transplantation (LDLT). A 48-year-old woman developed multiple liver adenomas. In view of the sizes and localizations of the lesions, the patient underwent right hepatic resection and segment II nodulectomy. Thirty-four months later, she developed recurrence of multiple hepatic adenomas and 2 nodules were highly suspect for hepatocellular carcinoma. Re-resection was not indicated due to the whole liver being involved with adenomas. The patient underwent LDLT. At 45 months thereafter she is alive and disease-free. In conclusion, LDLT is indicated in cases of nonresectability; it may offer optimal results in view of the absence of portal hypertension and the elimination of waiting list time.     

Liver transplantation for PNH with Budd-Chiari syndrome

A 54-year-old male patient with end-stage liver failure from Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria (PNH) underwent liver transplantation (OLT) in 1989. Retransplantation became necessary 1 year later when thrombotic occlusion of the portal vein and common hepatic artery led to graft loss after withdrawal of anticoagulation therapy because of several gastrointestinal bleeding episodes. The patient is now alive 3 years after the first OLT. To the best of our knowledge and according to the literature, this is, to date, the longest that any PNH patient has survived after liver transplantation. Although the course of this patient was complicated in a way similar to that reported for other cases in the literature, patients with PNH should not, in principle, be excluded from liver transplantation. Lifelong anticoagulation with coumarin and the use of steroids together with cyclosporin reduce the risk of recurrent thrombosis and PNH crises.     

Portal vein aneurysm as late complication of liver transplantation: a case report

OBJECTIVE: A case of intrahepatic portal vein aneurysm in the late postoperative period after liver transplantation, as well its complications, is reported. CASE REPORT: A 59-year-old man underwent orthotopic liver transplantation in 1996 for treatment of hepatitis C virus cirrhosis. The patient received a graft from a 10-year-old child. During the follow-up from 1996 to 1998, the patient did not show any alterations. In 1999, during an annual routine exam, a portal vein aneurysm was identified; however, it had no impact on graft function. In November 2002, the patient developed jaundice and serious graft dysfunction requiring hospital admission. Helicoidal CT scan showed an intrahepatic image compatible with a portal vein aneurysm without biliary tract dilatation. During the same hospitalization, he developed upper gastrointestinal bleeding due to variceal rupture as well as kidney and liver failure, and expired on December 31, 2002. The necropsy demonstrated an intrahepatic portal vein aneurysm with portal vein thrombosis and chronic liver disease. The evolution in this case suggests that if there is an intrahepatic portal vein aneurysm after liver transplantation, the patient is likely to experience an eventual recurrence of portal hypertension; retransplant may be an alternative.