Diagnosis Of Noncirrhotic Portal Hypertension Research Articles

Non-Cirrhotic Portal Hypertension: An Update of Diagnosis and Management

Portal hypertension is not the only caused by liver cirrhosis. Non-cirrhotic portal hypertension (NCPH) is characterized by elevated portal pressure in the absence of cirrhosis and is often underdiagnosed in daily clinical practice. The clinical manifestations of NCPH are similar to liver cirrhosis. The diagnosis of NCPH is still challenging due to the various underlying etiologies and often require either non-invasive or invasive examination. Laboratory examination, abdominal ultrasound, endoscopic procedure, hepatic venous gradient measurement, and liver biopsy have role in diagnosis of NCPH. The principle management of NCPH is to treat portal hypertension and its complications, such as prophylaxis and acute management of variceal bleeding, anticoagulants, surgery, splenectomy, and liver transplantation.

Noncirrhotic portal hypertension misdiagnosed as liver cirrhosis: A rare case

Noncirrhotic portal hypertension misdiagnosed as liver cirrhosis: A rare case

Porto-sinusoidal vascular disorder.

It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term.

The Role of Magnetic Resonance Elastography in the Diagnosis of Noncirrhotic Portal Hypertension

Portal hypertension (PH) is defined as abnormal elevation of portal venous pressure with cirrhosis accounting for 90% of cases and 10% of cases classified as noncirrhotic PH (NCPH).1,2 The differentiation of cirrhotic PH (CPH) from NCPH is difficult (Supplementary Figure1), with recent research efforts focusing on noninvasive evidence of increased hepatic stiffness.3,4 Magnetic resonance elastography (MRE) is an established imaging technique in the assessment of hepatic stiffness, and is now the most efficacious, noninvasive method to assess for hepatic fibrosis.5-8 The aim of this study was to assess the ability of magnetic resonance imaging (MRI) and MRE to differentiate between CPH and NCPH.

Non-cirrhotic Portal Hypertension from Chemotherapy-induced Intrahepatic Non-tumorous Arterioportal Fistula

Purpose: Intrahepatic arterioportal fistulas (APF) are rare, are mostly caused by trauma or malignancy, and can cause presinusoidal portal hypertension. We report a case of chemotherapy-induced non-tumoral intrahepatic APF (CI-APF) presenting with non-cirrhotic portal hypertension (NCPH). A 57-year-old white man presented with chronic abdominal pain and abnormal liver tests for one year (3/13). He underwent right hemicolectomy for cecal adenocarcinoma (2/12), and received adjuvant chemotherapy with FOLFOX (4/12-9/12). His laboratory data showed thrombocytopenia and fluctuating liver tests (AST 43-90 U/L; ALT 49-91U/L; alkaline phosphatase 173-280U/L, and total bilirubin 0.4-1.7mg/dL). Viral and autoimmune hepatitis were excluded. Physical exam was normal. Upper endoscopy showed new trace esophageal varices. Abdominal magnetic resonance imaging reported a large APF in both lobes of the liver, with hyperarterial vascularity perfusing the right hepatic lobe, early shunting to the portal vein, moderate atrophy of right lobe of the liver, distension of the portal vein, and splenomegaly with otherwise patent hepatic vasculature (A). Trans-jugular liver biopsy with wedge pressure and hepatic arteriography showed hepatic vein pressure gradient of 3 mmHg and hepatic arterial to portal vein shunting (B). Liver biopsy was notable for focal sinusoidal dilatation, unremarkable bile ducts, portal tracts, and hepatocytes, with no fibrosis. Prior imaging showed normal hepatic anatomy (2/2012, 6/2012) and fatty infiltration of the liver without vascular abnormalities (11/2012). The occurrence of APF with presinusoidal portal hypertension and the temporal relationship with chemotherapy led to the final diagnosis of NCPH from CI-APF. Several cases of NCPH in patients undergoing chemotherapy for metastatic colorectal cancer have been reported. It is believed that severe hepatic injury from chemotherapy results in benign transformation of the hepatic parenchyma into nodular regenerative hyperplasia (NRH). In our case, the presence of pre-sinusoidal portal hypertension is more supportive of obliterative portal venopathy leading to APF, rather than NRH. Shunt reduction with minimally invasive techniques such as transcatheter hepatic artery embolization have been successfully performed without severe complications.Figure

Clinical Factors That Predict Noncirrhotic Portal Hypertension in HIV-Infected Patients: A Proposed Diagnostic Algorithm

Noncirrhotic portal hypertension (NCPH) is a rare but important clinical entity in human immunodeficiency virus (HIV) populations. The purpose of this study was to describe the clinical factors associated with the condition in an effort to formulate a diagnostic algorithm for easy and early diagnosis. We performed a multicenter, retrospective case-control study of 34 patients with NCPH and 68 control HIV patients. The study found that thrombocytopenia, splenomegaly, didanosine use, elevated aminotransferases, and an elevated alkaline phosphatase level were all significantly more prevalent in the NCPH cohort. Using these easily available clinical parameters, we developed an algorithm for early diagnosis of NCPH in HIV.

Portal Hypertension Secondary to Isolated Liver Tuberculosis

In this report, we present a case of isolated liver tuberculosis (TB) as a cause of non-cirrhotic portal hypertension leading to bleeding esophageal varices. Although TB has been known to cause portal hypertension in a variety of ways, this case was notable for the presence of periportal inflammation and granulomas, also seen in hepatic schistosomiasis. Herein, we discuss isolated liver TB and the differential diagnosis of non-cirrhotic portal hypertension. In endemic areas, TB should be considered in the differential diagnosis of non-cirrhotic portal hypertension.

Reply to Chang and Garcia‐Pagan

To the Editor—We thank Chang and Garcia-Pagan [1] for their interest in our study [2], and we offer the following reply. First, in our case-control study of human immunodeficiency virus (HIV)-infected patients with noncirrhotic portal hypertension (NCPH), inclusion criteria were the presence of endoscopically documented esophageal varices or hepatic venous pressure gradient (HVPG) ⩾10 mmHg, absence of hepatic cirrhosis on liver biopsy, and no common cause of liver disease. All of our case patients had endoscopically documented esophageal varices, and no patient was excluded because of a HVPG <10 mmHg. Eight of 15 case patients underwent hepatic hemodynamic evaluation; the median HVPG was 24.5 mmHg (range, 7–54 mmHg). Except in 1 case patient, all HVPG values were ⩾10 mmHg. Second, because most case patients received a diagnosis of NCPH at a time before liver elastography was regularly conducted, we did not evaluate liver stiffness values systematically. Third, we were not able to search for prothrombotic disorders, because cases were included retrospectively. However, in contrast to Chang et al [3], who did not find coagulopathies in their 8 patients, other reports have noted thrombophilic abnormality in affected patients [4–6]. This supports a multifactorial pathogenesis of NCPH in HIV infection, with antiretroviral therapy and a prothrombotic state leading to microthrombosis and vascular obstruction.

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.

Pregnancy and its outcome in patients with noncirrhotic portal hypertension.

There is paucity of data in the literature on the occurrence of pregnancy and its outcome in patients with noncirrhotic portal hypertension. The present study was undertaken to assess the fertility pattern, fetal loss, and consequences of variceal bleeding during pregnancy in these patients. Fifty-five women of child-bearing age with a diagnosis of noncirrhotic portal hypertension (including 32 with noncirrhotic portal fibrosis and 23 with extrahepatic portal venous obstruction) and 44 age-matched controls were studied. A detailed history of obstetric events and bleeding episodes was recorded in all patients. Any complication of portal hypertension was managed accordingly. Patients were classified into three groups for analysis: group 1-obstetric events after the diagnosis of portal hypertension in patients; group II-obstetric events before the diagnosis of portal hypertension in patients; and group III-obstetric events in controls. A total of 116 pregnancies took place in 44 patients. Of these, 36 occurred after and 80 before the diagnosis of portal hypertension was made. In the 44 controls, 118 pregnancies took place. The mean fertility rates of groups I, II and III were 0.20+/-0.24, 0.18+/-0.21, and 0.22+/-0.12 pregnancies per year, respectively, which were not statistically different (P>0.05). The numbers of fetal losses in groups I, II and III were 3/36 (8.3%), 6/80 (7.5%), and 12/118 (10.17%) pregnancies, respectively. Menstrual irregularities were found in 7/55 (12.72%) patients and 6/44 (13.6%) controls (P>0.05). Five episodes of variceal bleeding occurred in 36 pregnancies after the diagnosis of portal hypertension (13.88%). All five were successfully managed with endoscopic sclerotherapy. In conclusion, noncirrhotic portal hypertensive patients have normal fertility and outcome of pregnancy. Variceal bleeding episodes are not common and endoscopic sclerotherapy is safe and effective in pregnancy.