Long-standing Portal Hypertension Research Articles

Clinical characteristics of cirrhosis patients with umbilical vein recanalization: A retrospective analysis.

Umbilical Vein Recanalization (UVR) may occur in patients with long-standing portal hypertension and liver cirrhosis. This study aimed to investigate the clinical significance of UVR.Medical records of a cohort of patients with cirrhosis (n = 247) who were hospitalized at the Digestive Medicine Center of the Second Affiliated Hospital of Nanchang University from January 2012 to October 2015 were accessed. The UVR diagnosis was made by ultrasound examination and was confirmed by computerized tomography scan.The UVR incidence was 20.2% (50/247) in the cohort. The size of UVR was 9.9 ± 4.7 mm (range: 5–26.5 mm) in diameter. The UVR and non-UVR groups showed no difference in grades of hepatic encephalopathy (P = .496), Child-Pugh classification (P = .401), the incidence of moderately severe ascites (26% vs 26%, P = 1), the esophageal variceal bleeding rate (32% vs 39%, P = .402), or portal vein thrombosis (8% vs 12%, P = .580). However, the incidence of cavernous transformation of the portal vein was statistically different, that there was 0 case in the UVR group and 8 cases in the non-UVR group (P < .05).Our results suggested that UVR had little impact on the clinical manifestations of patients with liver cirrhosis, the significance of UVR as an intervention method requires further studies.

Cystic Fibrosis–Related Liver Disease in a 12-Year-Old: Important Questions of Portal Hypertension Etiology That Start With Pathology: A Case Presentation

Abstract Introduction Cystic fibrosis liver disease (CFLD) has long been postulated to be secondary to dysfunctional cystic fibrosis transmembrane conductance regulator in the apical biliary epithelium, leading to bile stasis and eventually cirrhosis with portal hypertension (PH). We present a considerably young patient with signs of esophageal varices and liver biopsy indicating CFLD with significant obliterative portal venopathy as a result of long-standing portal hypertension. Case Presentation A 12-year-old with CF (diagnosed at 1 year) and pancreatic insufficiency was noted to have chronically elevated liver enzymes with decreasing platelet counts (130,000 K/ µL). These findings prompted an ultrasound, which showed splenomegaly. Upper endoscopy showed grade 1 esophageal varices, and liver needle biopsy performed at this time demonstrated focal neutrophilic lobular inflammation with marked pseudoacinar transformation and biliary metaplasia. Noted was grade 1 macrovesicular steatosis, focal dense fibrosis also known as “focal biliary cirrhosis,” and obliterative portal venopathy. Discussion CF patients with liver fibrosis have significant risk of future morbidity. Of specific interest is identification of PH, and obliterative portal venopathy is a very important feature to distinguish on biopsy as it predicts clinical course. Two recent studies in young adults described obliterative portal venopathy (OPV) and noncirrhotic portal hypertension (NCPH) as the predominant pathophysiology in young adults (median, 22 years) with CFLD. One study found cirrhosis present in only 27% of patients with portal hypertension. In addition, it appears that portal hypertension can precede cirrhotic changes. This unique case in a very young patient with CFLD demonstrates that the underlying pathogenesis of noncirrhotic portal hypertension and CFLD remains to be fully solved. These observations may have important consequences for CFLD management, including opting away from “cholestasis-targeted” treatments and more often considering portosystemic shunting procedures instead of transplant, as liver function remains preserved in numerous CFLD cases.

Non-cirrhotic Portal Fibrosis and Gamma-Gandy Bodies

A 22-year-old female with no comorbidities presented with complaints of early satiety and feeling of mass abdomen in left upper quadrant of 5-year duration. There was history of upper gastrointestinal bleed 2 months back, for which, an endoscopic variceal ligation was done at a referral hospital. On examination, patient was pale, with massive splenomegaly. Rest of the systemic examination was unremarkable. Complete blood counts revealed pancytopenia with hemoglobin of 7 g/dL, platelet count of 30000/mm3, and a total leukocyte count of 1700/mm3. Liver function tests were normal, and gastroscopy showed large varices. Magnetic resonance imaging abdomen with venography showed features of non-cirrhotic portal fibrosis with normal liver, grossly dilated splenoportal axis, and massive splenomegaly. T1(Figure 1) and T2(Figure 2) weighted images showed diffuse hypointense foci suggestive of Gamma-Gandy bodies. These nodules are characterized by signal void on all pulse sequences, both of T1and T2-weighted images, due to their hemosiderin content.1 Gamma-Gandy bodies or siderotic nodules are small, firm nodules of fibrous tissue, impregnated with iron pigment (hemosiderin) and calcium salts that occur in the spleen in 9–12% of patients with long-standing portal hypertension.2,3 The pathophysiologic process leading to the formation of Gamma-Gandy bodies is hemorrhage in the parenchyma followed by scarring of the collagen and elastic fibers and impregnation with iron and calcium.

Splenic artery aneurysm: a diagnostic challenge in the setting of extensive portal venous collaterals

We present a 16-year-old boy with autoimmune liver disease and longstanding portal hypertension in whom a CT arteriogram demonstrated a large aneurysm arising from the distal, extra-parenchymal portion of the splenic artery. Because of its location adjacent to multiple venous collaterals, the aneurysm was indistinguishable from splenic varices on initial imaging with Doppler sonography and on portal venous-phase CT. There is an increased risk of rupture of splenic artery aneurysms in the post-liver transplant period, with high associated mortality, and therefore diagnosis of splenic artery aneurysm prior to liver transplantation is clinically important. It is quite possible that the diagnosis of splenic artery aneurysm in this case would have been missed in the absence of dedicated arterial-phase imaging. As radiologists strive to reduce radiation exposure in children, this case highlights a potential diagnostic pitfall of both Doppler sonography and venous or single-acquisition arterial/venous-phase CT angiogram in children with venous collaterals and an undiagnosed splenic artery aneurysm.

Multidetector CT findings of splenic artery aneurysm in children with chronic liver disease

Splenic artery aneurysm (SAA) is a well-known complication of chronic liver disease and portal hypertension in adults. The incidence of SAA in children undergoing selective hepatic angiography prior to liver transplantation is reported as 4%, but there are few systematic studies. To investigate the SAAs detected by multidetector CT angiography (MDCTA) among children with chronic liver disease. A total of 124 children (71 girls, 53 boys; mean age 118 months; age range 5 days to 204 months) with chronic liver disease underwent MDCTA to display the vascular anatomy and any vascular complications during the pretransplantation period. Of these children, 23 also underwent coeliac angiography. The digital subtraction angiography (DSA) and MDCTA findings were compared. SAAs were detected in 13 children (10.4%); none was detectable by US. All patients had more than one aneurysm; ten patients had more than three. In all except one patient, the SAAs were located only in the intraparenchymal branches of the splenic artery; in one patient they were located in the intraparenchymal segment and in the distal third of the splenic artery. The mean size of the aneurysms was 6.5 mm (range 2.5-18 mm). All patients with aneurysms had splenomegaly and vascular collaterals. Nine of the children with SAAs had portal vein pathologies (two occlusions, two stenoses, five dilatations). A statistically significant difference existed with regard to the size of spleen (P < 0.05) and patient age (P < 0.05) between children with SAAs and children without SAAs. There was an increased risk of SAAs in patients with portal vein pathologies. In 19 patients without SAAs on MDCTA, no SAAs were seen on DSA. It is likely that the incidence of SAA in children with chronic liver disease will increase with improved survival of children with long-standing portal hypertension and chronic liver disease. MDCTA with multiplanar reconstruction is a noninvasive and effective means of imaging paediatric patients with SAAs, especially during the peritransplantation period, which is considered to be a time of significant risk for SAA rupture in this patient population.

End‐stage Liver Disease and Liver Transplant: Current Situation and Key Issues

The aim of this article is to identify the current challenges in pediatric OLT. It is directed to all physicians who care for pediatric patients from the early prospect of liver transplant to posttransplant life. Besides assessing current information on transplant results, the coauthors of this article have identified a number of issues that they consider as the most important that need to be addressed in 2008. Some are only an update of old questions, and others are newly identified problems. The recommendations from the group are summarized at the end of each specific issue and in the consensus guidelines.

Portal venous calcifications 20 years after portosystemic shunting: Demonstration by spiral CT with CT angiography and 3D reconstructions

Background: Evaluation of the value of spiral computed tomography (SCT), and postprocessing procedures in patients with extensive portal venous calcifications 20 years after portosystemic shunting was performed. Methods: In two patients spiral CT (SCT) examinations of the abdomen (slice thickness 3 mm, table feed 6 mm/s) were performed prior and after application of 150 ml of contrast material administered at a flow rate of 4 ml/s. Axial images were reconstructed at 2 mm increments for postprocessing procedures and 6 mm increments for axial source images. Postprocessing was performed with a maximum intensity projection (MIP) and shaded surface display (SSD) imaging program. Results: In both cases preoperative plain film radiography of the chest and abdomen showed large curvilinear calcifications located at the upper quadrant of the abdomen. The calcifications were directed along the expected axis and position of the portal vein and the portosystemic venous anastomosis. Axial CT slices and CTA showed that the calcifications were located in the vessel wall and that the portal vein lumen as well as the portosystemic venous anastomosis were patent. Conclusion: Long-standing portal hypertension is capable of causing portal venous calcifications due to mechanical stress to the vessel wall even years after performing portosystemic shunting. Typically, the calcifications are directed along the expected axis and position of the portal vein. SCT of the portal venous system is a reliable method to differentiate between calcifications in a thrombus or in the vessel wall, which may have therapeutic significance.

Histopathology of the liver in non-cirrhotic portal hypertension of unknown aetiology.

Non-cirrhotic, long-standing portal hypertension of unknown aetiology is being re-evaluated histopathologically and clinically. In this study, we examined 107 livers with this condition (92 wedge biopsy and 15 autopsy specimens) from five institutions in Japan. These cases were histologically categorized into four groups: idiopathic portal hypertension (66 cases), nodular regenerative hyperplasia (14 cases), partial nodular transformation (two cases), and incomplete septal cirrhosis (25 cases). These four groups shared several histological features: dense portal fibrosis with portal venous obliteration and intralobular slender fibrosis. In addition, the histopathological features characteristic of one group were also found to a mild degree in other groups. The histopathological lesions preceding portal venous obliteration remain speculative. However, the portal venous obliteration may be responsible for the occurrence of sustained portal hypertension and several of the pathological changes in these livers. It seems likely that idiopathic portal hypertension, nodular regenerative hyperplasia, partial nodular transformation and incomplete septal cirrhosis comprise a family of non-cirrhotic, long-standing portal hypertension in Japan, and the histological differences between them may reflect chronological progression of a single disease.

Chylous Ascites Should Suggest Constrictive Pericarditis Even in a Patient with Cirrhosis

Chylous ascites, a milky, high triglyceride fluid is usually found in patients with lymphatic obstruction from malignancy. We describe a patient with cirrhosis who developed constrictive pericarditis and chylous ascites. Long-standing portal hypertension compounded by elevated central venous pressure provided several pathophysiologic contributions to the formation of the chylous ascites. Chylous ascites even in a cirrhotic requires prompt assessment for conditions leading to elevated central venous pressure.

Hemodynamic studies in long- and short-term portal hypertensive rats: the relation to systemic glucagon levels.

It is not known whether the hyperdynamic state which has been observed in several experimental models and in patients with portal hypertension reflects a temporary phase during the evolution of the portal hypertensive syndrome or is an expression of a permanent steady state. A hemodynamic study was performed in a group of rats with long-standing portal hypertension induced by portal vein constriction performed 6.2 +/- 0.1 months earlier. A group of rats matched by age and weight with short-term (20.7 +/- 0.9 days) portal hypertension and a group of long-term (6.2 +/- 0.1 months) sham-operated rats were used as controls. Cardiac output and regional blood flows were measured using a radioactive microsphere technique. Arterial blood levels of glucagon, a known vasodilator that was implicated in the etiology of the hyperdynamic circulation, were also measured. Portal pressure in long- and short-term portal hypertensive groups (12.3 +/- 0.4 and 13.7 +/- 0.4 mm Hg; not statistically significant) was higher than in the sham group (9.0 +/- 0.3 mm Hg; p less than 0.01). Cardiac output in the long-term portal hypertensive rats was similar to the sham-operated group and lower than in the short-term portal hypertensive group (19.4 +/- 1.0 and 20.6 +/- 1.5 vs. 32.7 +/- 2.0 ml X min-1 X 100 gm body weight-1; p less than 0.01). Portal venous inflow in the long-term portal hypertensive group was also similar to the sham group and lower than in the short-term portal hypertensive group (4.51 +/- 0.36 and 4.58 +/- 0.39 vs. 6.72 +/- 0.48 ml X min-1.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary Pulmonary Hypertension and Cirrhosis: Are They Related?1–3

The relationship of primary pulmonary hypertension to longstanding portal hypertension secondary to hepatic cirrhosis was examined. An unselected series of 17,901 autopsied patients older than 1 yr of age showed that primary pulmonary hypertension occurred with a prevalence of 0.13% in all patients, but with a prevalence of 0.73% among patients with cirrhosis (p less than 0.001). A clinical series of 2,459 patients with biopsy-proved hepatic cirrhosis showed a prevalence of primary pulmonary hypertension of 0.61%, also significant (p less than 0.001) when compared with the prevalence among all patients older than 1 yr of age at autopsy. These data appear to exclude a coincidental association of cirrhosis with the development of the vascular lesions of primary pulmonary hypertension.

Estimated net transcapillary water and protein flux in the liver and intestine of patients with portal hypertension from hepatic cirrhosis

Net transcapillary (lymphatic) water, total protein, and albumin fluxes (expressed as plasma clearances into lymph) from the liver and intestine were estimated in 25 patients with hepatic cirrhosis and portal hypertension undergoing abdominal operation from a “mixture formula” (see text) based on direct measurement of thoracic duct lymph flow and protein content of plasma, liver, intestinal, and thoracic duct lymph. In the digestive tract, increased water flux (4.20 ± 0.88 ml/min, mean ± SE) was unaccompanied by increased protein leakage (0.42 ± 0.10 ml/ min) (r = 0.22), whereas in the cirrhotic liver, protein flux (1.82 ± 0.44 ml/min) closely paralleled water movement (2.8 ± 0.59 ml/min) (r = 0.95) but with persistence of permselectivity. These findings point to a predominant dissipative process of protein transport (diffusion or vesicular turnover) in the splanchnic capillary network and a primarily convective process (solvent drag) in the hepatic sinusoidal microcirculation. Further, splanchnic capillaries and hepatic sinusoids apparently resist “stretching” even under the rigor of long-standing portal hypertension, and heightened capillary permeability is not a significant factor in the pathogenesis of cirrhotic ascites.

The recognition and incidence of splenic artery aneurysms.

AbstractSplenic artery aneurysms are commonly associated with longstanding portal hypertension in the presence of a markedly enlarged spleen, and these aneurysms also occur in normal sized spleens following successful portacaval anastomosis. They are most successfully demonstrated by selective coeliac axis or splenic arteriography, but a large volume injection of concentrated contrast medium is essential for the demonstration of the intra‐splenic bifurcation aneurysms in the presence of marked splenomegaly.Most of these aneurysms are not detectable on the plain film as they are not calcified and none of the intra‐splenic aneurysms showed calcifications. The trunk aneurysms may be large and easily detectable, but the branch aneurysms may appear as a markedly tortuous artery. Their diameter is, however, greater than that of the associated branch. Intra‐splenic bifurcation aneurysms in association with portal hypertension occur more frequently than either trunk aneurysms or aneurysms at the hilum of the spleen.The common association of splenic artery aneurysms and portal hypertension is thought to be due to the increased volume and rate of blood flow through the splenic artery.