Pathology of idiopathic noncirrhotic portal hypertension. Idiopathic noncirrhotic portal hypertension incph is characterized by the elevation of portal venous pressure with no. Histologic features of idiopathic noncirrhotic portal. Introduction as early as the 17th century, it was realized that structural changes in the portal circulation could cause gastrointestinal bleeding. In 1902, gilbert and carnot introduced the term portal hypertension to describe this condition. Idiopathic noncirrhotic portal hypertension schouten. Natural history of noncirrhotic portal hypertension full. Pdf ncph is a heterogeneous group of liver disorders of vascular origin, leading to pht with near normal hvpg. Ncph causes can be classified according to the site of por. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic. Pdf noncirrhotic portal hypertension diagnosis and. Authors juan carlos garciapagan, md, phd senior consultant in hepatology, head hepatic hemodynamic lab, liver unit, hospital clinic, idibaps and ciberehd associate professor university of barcelona jason chang pik eu, mbbs, mrcp.
Noncirrhotic portal hypertension is the main feature in a subset of these patients, in whom gastrointestinal bleeding is the most frequent and potentially lifethreatening clinical presentation. Ultrastructure of the liver in noncirrhotic portal fibrosis. Idiopathic noncirrhotic portal hypertension journal of rare. Flow chart showing how the articles were selected and excluded. It occurs when blood flow in the major vein in the liver the portal vein.
Noncirrhotic portal hypertension pubmed central pmc. We describe the epidemiological, clinical and histological features of 12 hivpositive individuals presenting with noncirrhotic portal. Mar 21, 2018 noncirrhotic portal hypertension ncph comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver. The etiology of our patients liver disorder re mains obscure. Noncirrhotic nodular transformation of the liver with.
In the second, a patient who developed portal hypertension after unresolved pvt suffered a fatal variceal bleed this patient had never been anticoagulated. Noncirrhotic portal hypertension associated with didanosine. Thank you, polly, for presenting the case of a young man with portal hypertension without a definitive diagnosis of cirrhosis who presented with aki and profound anemia. Idiopathic non cirrhotic portal hypertension and spleno portal axis abnormalities in patients with severe primary antibody. Extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal hypertension, especially in third world ehlvo. Read noncirrhotic portal hypertension in the pediatric population, clinical liver disease on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Patients with incph usually present with signs or symptoms of portal hypertension such as gastroesophageal varices, variceal bleeding or. This article focuses on noncirrhotic pediatric portal hypertension, which is not well documented in the literature. We found a strong correlation between the exposure to ddi and the development of ncph.
Idiopathic noncirrhotic portal hypertension gastroenterology. We found a strong correlation between the exposure to. It is a common cause of portal hypertension in children up to 70% and young adults in india and other developing countries. Idiopathic portal hypertension radiology reference. Noncirrhotic portal hypertension represents a heterogeneous group of conditions that have distinct clinical and hemodynamic features that often help distinguish them from cirrhosis. Noncirrhotic portal hypertension a journal of hepatology. Natural history of noncirrhotic portal hypertension the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Noncirrhotic portal hypertension in feltys syndrome. In ehpvo, there are additional concerns of growth faltering, portal. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. Case report of hepatopulmonary syndrome in patients with. Noncirrhotic portal hypertension ncph comprises a group of diseases that are characterized by increased portal pressure in the absence of cirrhosis of the liver.
Pdf noncirrhotic portal hypertension ncph is a heterogeneous group of liver disorders of vascular origin, leading to portal hypertension. Extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal hypertension, especially in third world countries. The etiology of incph can be classified in five categories. The fda has issued a warning about an association between use of ddi didanosine and the development of noncirrhotic portal hypertension. Think about noncirrhotic portal hypertension in terms of anatomical causes. Noncirrhotic portal hypertension ncph has recently been reported as a liver complication in human immunodeficiency virus hivinfected patients and has been found to be associated with exposure to didanosine. Idiopathic noncirrhotic portal hypertension incph is an underrecognized clinicopathologic entity of the liver characterized by histologic changes secondary to phlebosclerosis without cirrhosis.
Nov 11, 2015 idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Idiopathic portal hypertension noncirrhotic portal hypertension or banti syndrome is a term that has been given to portal hypertension occurring without hepatic cirrhosis, parasitic infection, or portal venous thrombosis. Increased splenic blood flow may be a contributing factor to the development of the regenerative nodules. Genetic determinants of idiopathic noncirrhotic portal. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal dilatation.
The natural course of ncph is largely indolent and benign with an overall better outcome than cirrhosis, as long as the. Noncirrhotic portal hypertension ncph comprises a broad group of disorders characterized by elevated portal pressures in the absence of cirrhosis. We had a great report discussing our approach to noncirrhotic portal hypertension. We evaluated the frequency of this syndrome in noncirrhotic portal hypertension 1, 3. If you do not see its contents the file may be temporarily unavailable at the journal website or you do not have a pdf plugin installed and enabled in your browser. Pht iph, hepatoportal sclerosis and obliterative venopathy, is a disorder of unknown etiology. However, 3 months later, the patient died of upper gastrointestinal bleeding and respiratory failure. Of the two modalities of endotherapy, est is an established modality to tackle varices in children table 1.
Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Noncirrhotic portal hypertension ncph is a term used to describe a heterogeneous group of diseases characterized by the presence of portal hypertension with preserved liver structure and function. Noncirrhotic portal hypertension portal hypertension that is not caused by cirrhosis of the liver is rare in the united states. The sites of portal flow resistance may not be precisely localized to one area of the hepatic lobule and may extend beyond the site where the pathogenetic process. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized by of intrahepatic portal hypertension in the absence of cirrhosis, other causes of liver disease and splanchnic venous thrombosis 17. Ultrastructure of the liver in noncirrhotic portal. Noncirrhotic portal hypertension ncph is a heterogeneous group of vascular diseases that lead to portal hypertension ph with normal or mildly elevated hepatic venous pressure gradient and preserved liver synthetic function. Patients with incph present with portal hypertension, thus recognition of the subtle histologic findings and exclusion of cirrhosis is crucial for correct. Histologic features of idiopathic noncirrhotic portal hypertension in incidental liver biopsies and their implication for practicing pathologists. Noncirrhotic idiopathic portal hypertension radiology. Hypoxaemia and digital clubbing are rare but well recognised associations of hepatic cirrhosis with portal hypertension. Noncirrhotic portal hypertension ucsf internal medicine. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension ncph must be considered. Pathophysiology of portal hypertension jaime bosch, m.
The effect of ehhpvo portal pressure in ehpvo is not localized saein the esophagus and stomach. Hepatopulmonary syndrome hps, which consists of intrapulmonary vascular dilatation and impaired oxygenation in the setting of liver dysfunction or portal hypertension 1, 2, has predominantly been reported in patients with cirrhosis. In these cases, mortality and resolution of hypoxemia are related to the severity. Portosystemic shunt surgery in patients with idiopathic noncirrhotic portal hypertension. In 2011, maganty and ghanta reported the case of a 26 year old patient with portal hypertension secondary to nodular regenerative hyperplasia who had dyspnea and platypnea and who required home oxygen. Noncirrhotic idiopathic portal hypertension radiology case. Cirrhosis is the most common cause of portal hypertension, but it can also be present in the absence of cirrhosis, a condition referred to as noncirrhotic portal hypertension. The third died due to complications of liver failure, the result of noncirrhotic portal hypertension arising due to unresolved pvt this patient had previously received warfarin. Pathology and nomenclature, clinical liver disease on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Noncirrhotic portal fibrosis ncpf is a chronic liver disease and type of noncirrhotic portal hypertension ncph. Presented here is the case of a alyrojd man with portal hypertension and bleeding esophageal varices in which the gross and microscopic pathological findings have the features of two rare hepatic condi. Cirrhosis is the most common cause of portal hypertension, but portal hypertension can also be present in the absence of cirrhosis, a condition referred to as noncirrhotic portal hypertension. It may arise as a result of either primary hepatic disease or the effects of systemic disorders on liver.
May, 2011 however, in a variety of disorders, portal hypertension develops in the absence of cirrhosis. Idiopathic noncirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or. The efficacy and safety of direct oral anticoagulants in. Idiopathic noncirrhotic portal hypertension incph is a rare disorder consisting of intrahepatic portal. Abstract morphological changes of the liver were studied in 24 autopsy cases of noncirrhotic portal hypertension of unknown etiology idiopathic portal hypertension, iph, and in 123 surgical. Surgical management of noncirrhotic portal hypertension.
The portal hypertension is postulated to be a result of intrahepatic vascular compression. Portal hypertension is a clinical manifestation of cirrhotic scarred liver disease. Non cirrhotic portal hypertension authorstream presentation. The disease course appears to be indolent and benign. Based on the number of welldocumented cases and exclusion of other causes of portal hypertension such as alcoholrelated cirrhosis or hepatitis c. In other parts of the world it is variously named, as hepatoportal sclerosis 4, 5, noncirrhotic intrahepatic portal hypertension 6, and idiopathic noncirrhotic intrahepatic portal hypertension 7. Noncirrhotic portal hypertension journal of hepatology. The causes of portal hypertension and the treatment of its complications are discussed in detail elsewhere. Noncirrhotic portal hypertension ncph is a heterogeneous group of vascular diseases that lead to portal hypertension ph with normal or mildly elevated hepatic. It is the result of resistance to portal blood flow and may lead to complications such as variceal bleeding and ascites. Love, sammy top pearls schistosomiasis is one of the most common causes of noncirrhotic portal. Case report of hepatopulmonary syndrome in patients with non. Ddi associated with noncirrhotic portal hypertension. When portal hypertension occurs in the absence of liver cirrhosis, noncirrhotic portal hypertension ncph must.
Here, we describe the case of an hivinfected patient with portal hypertension. Ddi associated with noncirrhotic portal hypertension in summary, we described a case series of 15 hiv infected patients with ncph and assessed associated risk factors in a nested casecontrol analysis. Noncirrhotic portal fibrosis ncpf variously called as idiopathic. It is characterized by obliterative portovenopathy, which.
Amati2 1department of clinical medicine, center for the diagnosis and treatment of portal hypertension, 2department of radiological, oncological, and pathological sciences, sapienza university of rome, rome, italy. Cumulative risk of noncirrhotic portal hypertension in human immunodeficiency virusinfected patients with allelic variants at 4 loci at 2 genes coding for enzymes involved in the purine metabolism. May 03, 2017 we had a great report discussing our approach to noncirrhotic portal hypertension. Aug 18, 2019 extrahepatic portal vein obstruction ehpvo is an important cause of noncirrhotic portal hypertension, especially in third world ehlvo. Noncirrhotic portal hypertension in hivinfected patients. Noncirrhotic presinusoidal portal hypertension is common. Imaging is suggestive of noncirrhotic idiopathic portal hypertension. In an attempt to elucidate the pathophysiology of this condition, the hepatic ultrastructure in nine cases of noncirrhotic portal fibrosis with portal hypertension has been studied. Jun 06, 2000 hepatopulmonary syndrome hps, which consists of intrapulmonary vascular dilatation and impaired oxygenation in the setting of liver dysfunction or portal hypertension 1, 2, has predominantly been reported in patients with cirrhosis. The patient had a meld of 10, and her only indication for transplant was lung compromise.
This condition, referred to as noncirrhotic portal hypertension, is often classified based on the site of obstruction i. Schistosomiasis is one of the most common causes of noncirrhotic portal hypertension worldwide. Liver transplantation, with 5 year survival rates of about 75%, is the only therapeutic option 16. Find support for a specific problem on the support section of our website. Listing a study does not mean it has been evaluated by the u. Histological features of incph comprise a wide spectrum of nonspecific features, ranging from minor changes, sinusoidal. Idiopathic noncirrhotic portal hypertension schouten 2011. Portal hypertension is defined by a pathologic increase in the pressure of the portal venous system. Idiopathic non cirrhotic portal hypertension and spleno portal axis. Hepatopulmonary syndrome in noncirrhotic portal hypertension.
So far there is no effective medical therapy for hps. Pathology of noncirrhotic liver disease request pdf. May 30, 2015 idiopathic noncirrhotic portal hypertension incph is a rare disease characterized of intrahepatic portal hypertension in the absence of cirrhosis or other causes of liver disease and splanchnic venous thrombosis. Patients with incph present with portal hypertension, thus recognition of the subtle histologic findings and exclusion of cirrhosis is. The natural course of ncph is largely indolent and benign with an overall better outcome than cirrhosis, as.
In 1902, gilbert and carnot introduced the term portal hypertension to describe this. Noncirrhotic nodular transformation of the liver with portal. A case of feltys syndrome with nodular regenerative hyperplasia of the liver, presinusoidal portal hypertension, and bleeding esophageal varices is reported. May 30, 2015 idiopathic noncirrhotic portal hypertension incph is a rare disease characterized by of intrahepatic portal hypertension in the absence of cirrhosis, other causes of liver disease and splanchnic venous thrombosis 17. Portal hypertension is characterized by an increase in portal pressure 10 mmhg and could be a result of cirrhosis of the liver or of noncirrhotic diseases. We report the first european patient with idiopathic noncirrhotic portal hypertension complicated by these features. In the western world, liver cirrhosis is the most frequent cause of portal hypertension. Idiopathic non cirrhotic portal hypertension incph is a rare disorder consisting of intrahepatic portal.