scholarly journals Refractory Hepatic Hydrothorax: A Rare Complication of Systemic Sclerosis and Presinusoidal Portal Hypertension

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Gary A. Abrams ◽  
Robert Chapman ◽  
Samuel R. W. Horton
Keyword(s):  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Liver Biopsy ◽  
Rare Complication ◽  
Portal Pressure ◽  
Nodular Regenerative Hyperplasia ◽  
Hepatic Hydrothorax ◽  
Noncirrhotic Portal Hypertension ◽  
Portal Systemic Shunt ◽  
Hypertension Portal

We report on a rare case of refractory hepatic hydrothorax in an individual with Scleroderma/CREST syndrome and noncirrhotic portal hypertension. Portal pressure measurements revealed a normal transjugular hepatic venous portal pressure gradient, mild pulmonary hypertension, and an unremarkable liver biopsy except for mild sinusoidal dilation. Pulmonary hypertension, cardiac diastolic dysfunction, and chronic kidney disease were determined to be the causes of his refractory pleural effusions and ascites. Over the year, he underwent 50 thoracenteses and 20 paracenteses averaging 10–12 liters/week. Repeat pulmonary evaluation determined his pulmonary pressures to be normal and a secondary review of the “unremarkable” liver biopsy noted mild venous outflow obstruction and possibly Nodular Regenerative Hyperplasia (NRH). Repeat portal pressures indirectly and directly confirmed the existence of presinusoidal portal hypertension that has been associated with NRH. A transjugular intrahepatic portal systemic shunt (TIPS) was placed and he has not required thoracentesis or paracentesis over the past 18 months.

scholarly journals Noncirrhotic portal hypertension and nodular regenerative hyperplasia of the liver in dogs with mucopolysaccharidosis type I

Hepatology ◽  
1998 ◽  
Vol 28 (2) ◽  
pp. 385-390 ◽  
Author(s):  
Michael F. McEntee ◽  
Kathy N. Wright ◽  
Ian Wanless ◽  
Robert DeNovo ◽  
John F. Schneider ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Nodular Regenerative Hyperplasia ◽  
Type I ◽  
Mucopolysaccharidosis Type ◽  
Mucopolysaccharidosis Type I ◽  
Noncirrhotic Portal Hypertension

scholarly journals Portal Hypertension, Nodular Regenerative Hyperplasia of the Liver, and Obstructive Portal Venopathy due to Metastatic Breast Cancer

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Andrew T. Turk ◽  
Matthias J. Szabolcs ◽  
Jay H. Lefkowitch
Keyword(s):  
Breast Cancer ◽  
Portal Hypertension ◽  
Breast Carcinoma ◽  
Portal Vein ◽  
Metastatic Breast ◽  
Nodular Regenerative Hyperplasia ◽  
Metastatic Breast Carcinoma ◽  
Noncirrhotic Portal Hypertension ◽  
Diagnostic Difficulties ◽  
Recurrent Breast

Nodular regenerative hyperplasia (NRH) of the liver is associated with noncirrhotic portal hypertension, rheumatologic and hematologic disorders, administration of certain drugs, and other underlying conditions. This report describes a 64-year-old man with clinically presumed cirrhosis who presented to our institution with coffee-ground emesis, esophageal varices, ascites, and encephalopathy. Eleven years earlier he had been treated for breast cancer with mastectomy and chemo-radiotherapy. He died suddenly, and the autopsy showed no evidence of cirrhosis but instead demonstrated NRH with extensive emboli of recurrent breast carcinoma within the portal vein and its intrahepatic branches. Neoplastic occlusion of the portal vein as a cause of presinusoidal noncirrhotic portal hypertension has not previously been reported for metastatic breast carcinoma. This case highlights the importance of obstructive portal venopathy in the pathogenesis of NRH as well as the diagnostic difficulties that may be encountered in determining the cause of portal hypertension.

scholarly journals Portal hypertension after prolonged use of trastuzumab emtansine: nodular regenerative hyperplasia with autoimmune features

2021 ◽  
Vol 10 (8) ◽  
pp. e10810817005
Author(s):  
Gustavo Vargas Borgongino Monteiro ◽  
Marcela Chagas Lima Mussi ◽  
Mateus Jorge Nardelli ◽  
Bruno Campos Santos ◽  
Cláudia Alves Couto
Keyword(s):  
Portal Hypertension ◽  
Esophageal Varices ◽  
Ductal Carcinoma ◽  
Rare Complication ◽  
Nodular Regenerative Hyperplasia ◽  
Trastuzumab Emtansine ◽  
Her2 Breast Cancer ◽  
History Of ◽  
The Right

We report a rare complication associated with prolonged use of trastuzumab emtansine (T-DM1) – a composed therapy for HER2+ breast cancer – presenting with laboratorial autoimmune features that could have delayed the diagnosis or led to misdiagnosis. A 55-year-old female was referred to the hepatologist with a computed tomography suggestive of portal hypertension for etiological investigation. History of invasive ductal carcinoma in the right breast undergoing treatment for 5 years. She had already undergone neoadjuvant chemotherapy, mastectomy, radiotherapy and adjuvant chemotherapy. By the time of metastatic diagnosis, she was in monotherapy with T-DM1 for 2.5 years. Upper endoscopy showed esophageal varices and portal hypertension gastropathy. Laboratorial tests revealed increased transaminases, hypergammaglobulinemia and positive antinuclear antibody. Liver biopsy was performed for autoimmune hepatitis differential diagnosis but revealed nodular regenerative hyperplasia. T-DM1 was discontinued. After a 2-year follow-up, the patient did not present any complications of portal hypertension, although persisted with esophageal varices.

scholarly journals Noncirrhotic Portal Hypertension due to Nodular Regenerative Hyperplasia Treated with Surgical Portacaval Shunt

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Lisa M. Louwers ◽  
Jared Bortman ◽  
Alan Koffron ◽  
Veslav Stecevic ◽  
Steven Cohn ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Lupus Erythematosus ◽  
Beta Blockers ◽  
Portacaval Shunt ◽  
Nodular Regenerative Hyperplasia ◽  
Noncirrhotic Portal Hypertension ◽  
Parenchymal Liver ◽  
Abdominal Mri ◽  
Synthetic Function ◽  
History Of

Nodular regenerative hyperplasia (NRH) is an uncommon condition, but an important cause of noncirrhotic intrahepatic portal hypertension (NCIPH), characterized by micronodules of regenerative hepatocytes throughout the liver without intervening fibrous septae. Herein, we present a case of a thirty-seven-year-old female with systemic lupus erythematosus (SLE) who was discovered to have significant esophageal varices on endoscopy for dyspepsia. Her labs revealed a slight elevation in the alkaline phosphatase and mild thrombocytopenia. Abdominal MRI revealed seven focal hepatic masses, splenomegaly, no ascites, and a patent portal vein. Ultrasound-guided core biopsy was reported as focal nodular hyperplasia. However, her varices persisted despite treatment with beta-blockers and four additional upper endoscopies with banding. She was subsequently referred for a surgical opinion. At that time, given her history of SLE, azathioprine use, and portal hypertension, suspicion for NRH was raised. Given her normal synthetic function and lack of parenchymal liver disease, the patient was offered surgical shunting. During shunt surgery, a liver wedge biopsy was also performed and this confirmed NRH. An upper endoscopy six weeks after shunting verified complete resolution of varices. Currently, fifteen months after surgery duplex ultrasonography demonstrates shunt patency and the patient is without recurrence of her portal hypertension.

Mild increases in portal pressure upregulate vascular endothelial growth factor and endothelial nitric oxide synthase in the intestinal microcirculatory bed, leading to a hyperdynamic state

2006 ◽  
Vol 290 (5) ◽  
pp. G980-G987 ◽  
Author(s):  
Juan G. Abraldes ◽  
Yasuko Iwakiri ◽  
Mauricio Loureiro-Silva ◽  
Omar Haq ◽  
William C. Sessa ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Portal Hypertension ◽  
Portal Pressure ◽  
No Production ◽  
Portal Vein Ligation ◽  
Vegf Expression ◽  
Enos Expression ◽  
Hyperdynamic Circulation ◽  
Intestinal Microcirculation ◽  
Hypertension Portal

Increased nitric oxide (NO) is the main factor leading to the hyperdynamic circulation associated with advanced portal hypertension (PHT), but the initial mechanisms and the magnitude of increase in portal pressure required to trigger NO production are not known. We addressed these issues by studying systemic and splanchnic hemodynamics and endothelial NO synthase (eNOS) and VEGF expression in rats with different degrees of portal hypertension. Portal vein ligation (PVL) performed over needles of three different calibers (16-, 18-, and 20-gauge) yielded different degrees of PHT and portosystemic shunting. Compared with sham rats, all three groups of PVL rats exhibited features of hyperdynamic circulation. Rats with minimal portal hypertension (PVL with a 16-gauge needle) showed an early increase in VEGF and eNOS expression selectively at the jejunum. Immunofluorescence showed that VEGF expression was located in highly vascularized areas of the mucosa. Inhibition of VEGF signaling markedly attenuated the increase in eNOS expression. In conclusion, mild increases in portal pressure are enough to upregulate eNOS at the intestinal microcirculation, and this occurs, at least in part, through VEGF upregulation.

Multidisciplinary approach to the complex treatment for non-cirrhotic portal hypertension – case-report-based discussion

2021 ◽  
Vol 59 (01) ◽  
pp. 43-49
Author(s):  
Simon J. Gairing ◽  
Roman Kloeckner ◽  
Michael B. Pitton ◽  
Janine Baumgart ◽  
Charis von Auer-Wegener ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Esophageal Varices ◽  
Rare Complication ◽  
Treatment Options ◽  
Portal Pressure ◽  
Recurrent Bleeding ◽  
Factor V ◽  
Factor X ◽  
Warren Shunt ◽  
Shunt Occlusion

AbstractNon-cirrhotic portal vein thrombosis (PVT) in patients with antiphospholipid syndrome (APS) is a rare complication, and the management has to be determined individually based on the extent and severity of the presentation. We report on a 37-year-old male patient with non-cirrhotic chronic PVT related to a severe thrombophilia, comprising APS, antithrombin-, factor V- and factor X-deficiency. Three years after the initial diagnosis of non-cirrhotic PVT, the patient presented with severe hemorrhagic shock related to acute bleeding from esophageal varices, requiring an emergency transjugular intrahepatic portosystemic stent shunt (TIPSS). TIPSS was revised after a recurrent bleeding episode due to insufficient reduction of the portal pressure. Additionally, embolization of the dilated V. coronaria ventriculi led to the regression of esophageal varices but resulted simultaneously in a left-sided portal hypertension (LSPH) with development of stomach wall and perisplenic varices. After a third episode of acute esophageal varices bleeding, a surgical distal splenorenal shunt (Warren shunt) was performed to reduce the LSPH. Despite anticoagulation with low molecular weight heparin and antithrombin substitution, endoluminal thrombosis led to a complete Warren shunt occlusion, aggravating the severe splenomegaly and pancytopenia. Finally, a partial spleen embolization (PSE) was performed. In the postinterventional course, leukocyte and platelet counts increased rapidly and the patient showed no further bleeding episodes. Overall, this complex course demonstrates the need for individual assessment of multimodal treatment options in non-cirrhotic portal hypertension. This young patient required triple modality porto-systemic pressure reduction (TIPSS, Warren shunt, PSE) and involved finely balanced anticoagulation and bleeding control.

scholarly journals An Unexpected Hepatic Hydrothorax After a Successful Kasai Portoenterostomy: A Case Report

2021 ◽  
Vol 9 ◽  
Author(s):  
Giulia Ranucci ◽  
Fabiola Di Dato ◽  
Daniela Liccardo ◽  
Marco Spada ◽  
Giuseppe Maggiore ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Portal Hypertension ◽  
Bile Flow ◽  
Rare Complication ◽  
Kasai Portoenterostomy ◽  
Hepatic Hydrothorax ◽  
Pediatric Case ◽  
End Stage Liver Disease ◽  
Cirrhotic Patients ◽  
End Stage

Hepatic hydrothorax (HH) represents a rare complication of portal hypertension among adult cirrhotic patients. Here, we describe a pediatric case of HH, observed in a biliary atresia infant. The child presented with recurrent right-sided pleural effusion, after a successful Kasai portoenterostomy with restoration of bile flow and without overt signs of hepatic failure. Recurrence of HH led the patient to liver transplant despite a low pediatric end-stage liver disease value. Although rare, HH can also occur in children and should be suspected in patients with portal hypertension and respiratory distress. HH may be an indication for liver transplantation.

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