Idiopathic Non Cirrhotic Portal Hypertension and Spleno-Portal Axis Abnormalities in Patients with Severe Primary Antibody Deficiencies

Background and Aim. Portal hypertension has been reported in association with acquired and primary immune deficiencies without a comprehensive description of associated spleno-portal axis abnormalities. Pathological mechanisms are poorly defined.Methods. Observational, single centre study with the aim of assessing the prevalence of spleno-portal axis abnormalities in an unselected cohort of 123 patients with primary antibody deficiencies and without known causes of liver diseases regularly followed up for a mean time of 18 ± 14 years. A cumulative period of 1867 patients-year was analysed. Clinical and immunological data, abdominal ultrasounds, CT scans, and endoscopy features were included in the analysis.Results. Twenty-five percent of patients with primary antibody deficiencies had signs of portal vein enlargement but only 4% of them had portal hypertension, with portal systemic collaterals. Liver biopsies showed liver sinusoids congestive dilatation, endothelization, and micronodularity fulfilling the criteria for noncirrhotic portal hypertension. Patients with portal vein enlargement had severe clinical and immunological phenotypes.Conclusions. In primary antibody deficient patients, infections, inflammations, splenomegaly, increased blood venous flow, and lymphocyte abnormalities contribute to establishment of liver damage possibly leading to noncirrhotic portal hypertension. Patients with primary antibody deficiency should be considered a good model to give insight into the pathological mechanisms underlying noncirrhotic portal hypertension.

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Pathology of Noncirrhotic Portal Hypertension: Clinicopathologic Study in Pediatric Patients

Pediatric and Developmental Pathology ◽

10.1007/s10024-003-1002-8 ◽

2003 ◽

Vol 6 (5) ◽

pp. 421-426 ◽

Cited By ~ 21

Author(s):

Carlos Abramowsky ◽

Rene Romero ◽

Thomas Heffron

Keyword(s):

Portal Hypertension ◽

Portal Vein ◽

Hepatopulmonary Syndrome ◽

Intestinal Bleeding ◽

Liver Transplants ◽

Venous Flow ◽

Noncirrhotic Portal Hypertension ◽

Clinicopathologic Study ◽

Esophageal Bleeding ◽

Portal Vein Obstruction

From 1995–2002, 14 patients with predominantly prehepatic, noncirrhotic portal hypertension were evaluated. At presentation, the eight females and six males had a mean age of 9 years (range 2–18). Seven were admitted with gastrointestinal, mostly esophageal bleeding, three with splenomegaly, three with hepato-pulmonary syndrome, and one with hyperammonemia. Imaging studies showed portal vein obstruction in six patients and non-obstructed but frequently anomalous vascular patterns, including hypoplasia of the portal vein, in the remaining eight patients. At the onset, liver function was marginally abnormal in all patients, but thrombocytopenia of approximately 100 × 109/L was consistently observed, probably reflecting chronic mild consumption coagulopathy and hypersplenism. The most striking and frequent histopathologic finding in 25 liver samples, was the presence of hypoplastic portal triads with collapsed portal vein radicles. In contrast, other triads showed markedly distended and misshapen portal vein radicles and likely lymphatics. These two patterns of collapse and distention presumably reflect areas of impaired versus overloaded intrahepatic portal venous flow. Some of the biopsies showed variable portal/sinusoidal fibrosis. Four patients (two with intestinal bleeding, two with hepatopulmonary syndrome) required liver transplants and are doing well. Eight patients are doing well clinically after surgical or spontaneous vascular shunting. Two patients with intestinal bleeding and hepato-pulmonary syndrome, respectively) who had congenital dyskeratosis, underwent bone marrow transplantation and died of nonhepatic-related complications. It is possible to suggest prehepatic causes of portal hypertension even in needle biopsies when collapsed portal vein radicles are present in portal triads, but more than one biopsy sample with larger bore bioptomes may be required to see the changes. Conversely, identifying these changes may suggest to the clinicians the need to work-up a patient for portal hypertension.

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Sonographic Evaluation of a Unique Meso-Rex Shunt: A Case Study

Journal of Diagnostic Medical Sonography ◽

10.1177/8756479320920212 ◽

2020 ◽

Vol 36 (6) ◽

pp. 567-571

Author(s):

Danielle E. Cain ◽

Sharlette Anderson

Keyword(s):

Portal Hypertension ◽

Portal Vein ◽

Bypass Surgery ◽

Main Portal Vein ◽

Extrahepatic Portal Vein Obstruction ◽

Venous Flow ◽

Sonographic Evaluation ◽

Portal Vein Obstruction ◽

Rex Shunt

Portal hypertension is a result of an increase in intrahepatic resistance in the main portal vein. The Meso-Rex shunt is used to bypass the obstructed portal vein and restore the venous flow into the liver. This procedure alleviates the need for a hepatic transplant. The Meso-Rex shunt has proven to be an effective treatment for extrahepatic portal vein obstruction, thus saving children from a complete transplant. There are variants to this bypass surgery, and sonography is commonly used to assess the condition pre- and postoperatively. In this case, the shunt was uniquely different from the typical Meso-Rex bypass surgery. Particular vasculature made it imperative for the sonographer to review the prior sonograms and review the chart information before preforming the examination. It should also be noted that sonographers must adapt the protocols to give the utmost treatment.

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Systemic and splanchnic haemodynamic effects of pentifylline in rats with portal hypertension

Clinical Science ◽

10.1042/cs0830041 ◽

1992 ◽

Vol 83 (1) ◽

pp. 41-45 ◽

Cited By ~ 6

Author(s):

M. Dagenais ◽

G. Pomier-Layrargues ◽

B. Rocheleau ◽

L. Giroux ◽

P.-M. Huet

Keyword(s):

Cardiac Output ◽

Portal Hypertension ◽

Portal Vein ◽

Portal Pressure ◽

Reference Sample ◽

Peripheral Resistance ◽

Haemodynamic Effects ◽

Portal Vein Ligation ◽

Portal Venous Flow ◽

Venous Flow

1. The systemic and splanchnic haemodynamic effects of pentifylline (40 mg/kg body weight intravenously) were assessed in rats with portal hypertension associated either with CCl4-induced cirrhosis (n= 13) or portal vein ligation (n=13). 2. Heparinized catheters were placed into the portal vein, inferior vena cava, aorta and left ventricle with exits from the neck. Haemodynamic studies were performed 4 h after consciousness was regained. Cardiac output and regional blood flows were measured using radiolabelled microspheres and the reference sample method in seven rats in each group; portal-systemic shunting was measured using microsphere injection in the ileo-colic vein in six rats in each group. 3. Forty-five minutes after injection, pentifylline had no effect on mean arterial pressure, cardiac output, peripheral resistance, portal venous flow, hepatic artery flow or portal-systemic shunting in either group of rats with portal hypertension. The drug lowered portal pressure (−18%) in cirrhotic rats, but not in portal-vein-ligated rats. 4. These data demonstrate that pentifylline lowers portal pressure in cirrhotic rats without affecting portal venous flow and portal-systemic shunting; this effect is possibly mediated by changes in intrahepatic resistance related to the effects of pentifylline on blood viscosity and/or on intrahepatic vasomotor tone.

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Magnetic Resonance Elastography and Portal Hypertension: Influence of the Portal Venous Flow on the Liver Stiffness

Stem Cells and Regenerative Medicine - Biomedical and Health Research ◽

10.3233/bhr210022 ◽

2021 ◽

Author(s):

Simon Chatelin ◽

Raoul Pop ◽

Céline Giraudeau ◽

Khalid Ambarki ◽

Ning Jin ◽

...

Keyword(s):

Portal Hypertension ◽

Magnetic Resonance ◽

Portal Vein ◽

Peak Flow ◽

Peak Velocity ◽

Liver Stiffness ◽

Portal Venous Flow ◽

Venous Flow ◽

Velocity Magnitude ◽

Magnetic Resonance Imaging Mri

The invasive measurement of the hepatic venous pressure gradient is still considered as the reference method to assess the severity of portal hypertension. Even though previous studies have shown that the liver stiffness measured by elastography could predict portal hypertension in patients with chronic liver disease, the mechanisms behind remain today poorly understood. The main reason is that the liver stiffness is not specific to portal hypertension and is also influenced by concomitant pathologies, such as cirrhosis. Portal hypertension is also source of a vascular incidence, with a substantial diversion of portal venous blood to the systemic circulation, bypassing the liver. This study focuses on this vascular effect of portal hypertension. We propose to generate and control the portal venous flow (to isolate the modifications in the portal venous flow as single effect of portal hypertension) in an anesthetized pig and then to quantify its implications on liver stiffness by an original combination of MRE and 4D-Flow Magnetic Resonance Imaging (MRI). A catheter balloon is progressively inflated in the portal vein and the peak flow, peak velocity magnitude and liver stiffness are quantified in a 1.5T MRI scanner (AREA, Siemens Healthcare, Erlangen, Germany). A strong correlation is observed between the portal peak velocity magnitude, the portal peak flow or the liver stiffness and the portal vein intraluminal obstruction. Moreover, the comparison of mechanical and flow parameters highlights a correlation with the possibility of identifying linear relationships. These results give preliminary indications about how liver stiffness can be affected by portal venous flow and, by extension, by hypertension.

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

Case Reports in Pathology ◽

10.1155/2013/826284 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

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.

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Treatment of Noncirrhotic Portal Hypertension Secondary to Portal Vein Thrombosis With Endovascular Mesocaval Shunt Creation

Cureus ◽

10.7759/cureus.8086 ◽

2020 ◽

Author(s):

Parkpoom Phatharacharukul ◽

Maximilian Pyko ◽

Nabil Fayad

Keyword(s):

Portal Hypertension ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Noncirrhotic Portal Hypertension ◽

Mesocaval Shunt ◽

Vein Thrombosis

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Combination of modified Sugiura technique and proximal splenorenal shunt for the management of portal vein thrombosis in noncirrhotic portal hypertension

Clinical and Experimental Gastroenterology ◽

10.2147/ceg.s188200 ◽

2019 ◽

Vol Volume 12 ◽

pp. 149-156

Author(s):

Hendry Irawan ◽

I Made Mulyawan

Keyword(s):

Portal Hypertension ◽

Portal Vein ◽

Portal Vein Thrombosis ◽

Splenorenal Shunt ◽

Noncirrhotic Portal Hypertension ◽

Vein Thrombosis

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A Bypassing Technique for the Remedy of Portal Hypertension through Extra Hepatic Portal Vein Obstruction by CFD Analysis

GANIT Journal of Bangladesh Mathematical Society ◽

10.3329/ganit.v38i0.39789 ◽

2019 ◽

Vol 38 ◽

pp. 89-104

Author(s):

Mst Khorseda Atkar ◽

Md Tajul Islam

Keyword(s):

Portal Hypertension ◽

Portal Vein ◽

Control Volume ◽

Clinical Signs ◽

Cfd Analysis ◽

Main Portal Vein ◽

Venous Flow ◽

Portal Vein Obstruction ◽

Hepatic Portal Vein ◽

Hepatic Portal

Extra-hepatic portal vein obstruction (EHPVO) is the blockage to the flow of blood in the portal vein before reaches to the liver. EHPVO is the common cause of portal hypertension in children in the most Asian countries. Examination reveals that the presence of block in the main portal vein may be responsible for the shrinkage of vein with manifold pernicious complication. The “shunt” policy is a fruitful source of restoration of the hepatic portal flow. This study shows that a new approach of bypassing (or shunting) to the blocked (thrombosed) region of the portal vein is a significant way of reducing portal hypertension and restoration of blood circulation. We studied EHPVO case through computational fluid dynamics (CFD) analysis by considering partial block formation and side to side shunt scheme inside the main portal vein. The constitutive equation for non-Newtonian fluidand energy equation are solved by control volume technique. Our study reveals that the shunting technique is strongly effective for the reconstitution of portal venous flow to the liver with lower tissue stress and rapid regression of clinical signs of portal hypertension. This new technique may potentially applicable for medication of EHPVO when shunting procedures are indicated. GANIT J. Bangladesh Math. Soc.Vol. 38 (2018) 89-104

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Extrahepatic Portal Hypertension following Liver Transplantation: a Rare but Challenging Problem

HPB Surgery ◽

10.1155/1998/81832 ◽

1998 ◽

Vol 10 (6) ◽

pp. 357-364 ◽

Cited By ~ 12

Author(s):

B. Malassagne ◽

O. Soubrane ◽

B. Dousset ◽

P. Legmann ◽

D. Houssin

Keyword(s):

Liver Transplantation ◽

Portal Hypertension ◽

Portal Vein ◽

Surgical Reconstruction ◽

Clinical Feature ◽

Complete Relief ◽

Venous Flow ◽

Extrahepatic Portal Hypertension ◽

Portal Vein Stenosis ◽

Vein Stenosis

This study reports our experience of 8 cases of extrahepatic portal hypertension after 273 orthotopic liver transplantations in 244 adult patients over a 10- year period. The main clinical feature was ascites, and the life-threatening complication was variceal bleeding. Extrahepatic portal hypertension was caused by portal vein stenosis in 6 patients, and left-sided portal hypertension in 2 patients after inadventent ligation of portal venous tributaries or portasystemic shunts. All patients with portal vein stenosis had complete relief of portal hypertension after percutaneous transhepatic venoplasty (n=4) or surgical reconstruction (n=2), after a median follow-up of 33 (range: 6–62) months. Of the 2 patients with left-sided portal hypertension, one died after splenectomy and one rebled 6 months after left colectomy. This study suggests that extrahepatic portal hypertension is a series complication after liver transplantation that could be prevented by meticulous portal anastomosis and closure of portal tributaries or portasystemic shunts to improve the portal venous flow. However, any ligation has to be performed under ultrasound guidance to avoid inadventent venous ligations.

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