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 ◽  
...  

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.

2017 ◽  
Vol 5 (1) ◽  
pp. 232470961769074
Author(s):  
Mindy Lee ◽  
Manhal Izzy ◽  
Ashwin Akki ◽  
Kathryn Tanaka ◽  
Harmit Kalia

Introduction: Nodular regenerative hyperplasia (NRH) is a known etiology of noncirrhotic portal hypertension. Cases of biopsy-proven NRH in human immunodeficiency virus (HIV)–positive patients have been described. While these patients often have normal synthetic liver function, several reports described disease progression to liver failure. Case: We here present a 26-year-old woman with history of congenital HIV on antiretroviral therapy complicated by Pneumocystis carinii pneumonia at age 14. CD4 counts have been >300 with undetectable viral load. She was referred to our Hepatology service for evaluation of splenomegaly, elevated liver tests, and thrombocytopenia. On initial presentation, she reported easy bruising and gingival bleeding, and abdominal imaging showed evidence of portal hypertension without associated cirrhosis. Upper endoscopy was significant for large esophageal varices without bleeding stigmata. Liver biopsy showed minimal fibrosis around the portal areas without significant inflammation. The lobules showed focal zones of thin hepatocyte plates on reticulin stain with adjacent areas showing mild regenerative changes. The diagnosis of NRH was made and patient was placed on propranolol for variceal bleeding prophylaxis. Two years later, the patient presented with bleeding gastric varices warranting transjugular intrahepatic portosystemic shunt. Postprocedure course was complicated by mild encephalopathy. Subsequent magnetic resonance imaging showed a 1.7 × 1.3 cm lesion suggestive of hepatocellular carcinoma (HCC). The patient was deemed to be a candidate for liver transplantation, and she is now delisted due to ongoing pregnancy. Conclusion: This report describes the first case of HCC in an HIV patient with NRH. The possible association of NRH with HCC warrants further investigation.


2021 ◽  
Author(s):  
Rafael Fusaro Aguiar Oliveira ◽  
Anna Carolina Faria Moreira Gomes Tavares ◽  
Lucas Moyses Carvalho de Oliveira ◽  
Matheus Fonseca Cardoso ◽  
Ana Paula Bernardes Real

Hepatology ◽  
1998 ◽  
Vol 28 (2) ◽  
pp. 385-390 ◽  
Author(s):  
Michael F. McEntee ◽  
Kathy N. Wright ◽  
Ian Wanless ◽  
Robert DeNovo ◽  
John F. Schneider ◽  
...  

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Andrew T. Turk ◽  
Matthias J. Szabolcs ◽  
Jay H. Lefkowitch

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.


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

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.


Kanzo ◽  
1984 ◽  
Vol 25 (10) ◽  
pp. 1312-1317 ◽  
Author(s):  
Shinsuke OHURA ◽  
Isamu WATANABE ◽  
Shunji FUTAGAWA ◽  
Yoshihiro YAGI ◽  
Mitsuo SUGIURA ◽  
...  

2019 ◽  
Vol 6 (12) ◽  
pp. e00257 ◽  
Author(s):  
Cindy Piao ◽  
Abhinav Koul ◽  
Dorina Gui ◽  
Ling-xin Chen ◽  
Souvik Sarkar

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