Pathology of Noncirrhotic Portal Hypertension: Clinicopathologic Study in Pediatric Patients

2003 ◽  
Vol 6 (5) ◽  
pp. 421-426 ◽  
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.

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

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.

scholarly journals Very Early Presentation of Extrahepatic Portal Vein Obstruction Causing Portal Hypertension in an Infant: Uncertainties in the Management and Therapeutic Limitations

2016 ◽  
Vol 10 (2) ◽  
pp. 360-365 ◽  
Author(s):  
Parisá Khodayar-Pardo ◽  
Andrés Peña Aldea ◽  
Ana Ramírez González ◽  
Adela Meseguer Carrascosa ◽  
Cristina Calabuig Bayo
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Imaging Study ◽  
Hepatopulmonary Syndrome ◽  
Caudate Lobe ◽  
Extrahepatic Portal Vein Obstruction ◽  
Early Presentation ◽  
Pediatric Age Group ◽  
Cavernous Transformation ◽  
Portal Vein Obstruction

Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound. Neither signs of thrombosis in arteriovenous system, nor affectation of biliary tract were identified in the magnetic resonance imaging study. A significant enlargement of the caudate lobe of the liver was reported. No risk factors were detected. The differential diagnosis performed was extensive. Inherited thrombophilia and storage disorders were especially considered. Liver biopsy was normal. Upper gastrointestinal esophagogastroduodenoscopy detected two small varicose cords on the distal third of the esophagus. Finding a cavernous transformation of the portal vein with evidence of collateral circulation in such an early age is a challenging condition for professionals, since PHT may lead to severe complications during childhood and can compromise growth and development. Evidence-based guidelines for the management of PHT in adults have been published. However, follow-up and treatment of pediatric patients have not yet been standardized. Moreover, management of PHT in infants faces particular difficulties such as technical restrictions that could hinder their treatment.

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

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Federica Pulvirenti ◽  
Ilaria Pentassuglio ◽  
Cinzia Milito ◽  
Michele Valente ◽  
Adriano De Santis ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Primary Antibody ◽  
Venous Flow ◽  
Noncirrhotic Portal Hypertension ◽  
Liver Sinusoids ◽  
Primary Antibody Deficiencies ◽  
Single Centre Study ◽  
Immune Deficiencies ◽  
Liver Biopsies

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.

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

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

Well-tolerated portal hypertension and favorable prognosis in adult patients with extrahepatic portal vein obstruction in Japan

2015 ◽  
Vol 46 (6) ◽  
pp. 505-513 ◽  
Author(s):  
Tadashi Sekimoto ◽  
Hitoshi Maruyama ◽  
Kazufumi Kobayashi ◽  
Soichiro Kiyono ◽  
Takayuki Kondo ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Adult Patients ◽  
Extrahepatic Portal Vein Obstruction ◽  
Favorable Prognosis ◽  
Portal Vein Obstruction

scholarly journals Evaluation of growth and nutritional status in children and adolescents with extrahepatic portal vein obstruction and portal hypertension

2017 ◽  
Vol 30 (4) ◽  
pp. 455-461
Author(s):  
Priscila Francisco MARQUES ◽  
Maria Angela Bellomo BRANDÃO ◽  
Gabriel HESSEL ◽  
Roberta Vacari ALCANTARA ◽  
Marcela Linden FERREIRA ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Portal Hypertension ◽  
Nutritional Status ◽  
Portal Vein ◽  
Dietary Intake ◽  
Children And Adolescents ◽  
Energy Intake ◽  
Body Mass ◽  
Extrahepatic Portal Vein Obstruction ◽  
Portal Vein Obstruction

ABSTRACT Objective: This study aims to evaluate dietary intake, nutritional status, and growth rate in children and adolescents with extrahepatic portal vein obstruction and portal hypertension. Methods: Outpatients aged 1-18 years, diagnosed with extrahepatic portal vein obstruction and portal hypertension, who had no associated diseases, and who had not been subjected to a venous shunt were included in this study. Two evaluations were carried out in this study: an initial (evaluation 1) and a final evaluation (evaluation 2), with a three-month minimum interval between them. In each evaluation, dietary intake was analyzed comparing the results with recommended energy intake using the Harris & Benedict equation and participants’ anthropometric data, such as weight, height, mid-arm muscle circumference, weight-for-age, height-for-age, and body mass index-for-age, based on the World Health Organization 2006 standards. Results: A total of 22 patients participated in this study. There was a significant improvement in weight, height, body mass index, and mid-arm muscle circumference measurements (p<0.001; p<0.001; p<0.017; p=0.0018 respectively) and in the relationship between dietary intake and energy recommended energy intake, according to the Harris & Benedict equation (p=0.0001) from the first and second evaluation. Conclusion: Extrahepatic portal vein obstruction and portal hypertension were not shown to be factors predisposing to malnourishment.

scholarly journals Vascular stasis, intestinal hemorrhage, and heightened vascular permeability complicate acute portal hypertension in cd39-null mice

2009 ◽  
Vol 297 (2) ◽  
pp. G306-G311 ◽  
Author(s):  
Xiaofeng Sun ◽  
Andrés Cárdenas ◽  
Yan Wu ◽  
Keichi Enjyoji ◽  
Simon C. Robson
Keyword(s):  
Nitric Oxide ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Vascular Permeability ◽  
Extracellular Nucleotides ◽  
Intestinal Bleeding ◽  
Portal Vein Ligation ◽  
Dependent Manner ◽  
Wild Type ◽  
Intestinal Hemorrhage

Vasoactive factors that regulate splanchnic hemodynamics include nitric oxide, catecholamines, and possibly extracellular nucleosides/nucleotides (adenosine, ATP). CD39/ectonucleoside triphosphate diphosphohydrolase-1 (NTPDase1) is the major vascular ectonucleotidase that hydrolyzes extracellular nucleotides. CD39 activity may be modulated by vascular injury, inflammation, and altered oxygen tension. Altered Cd39 expression by the murine hepatosplanchnic vasculature may impact hemodynamics and portal hypertension (PHT) in vivo. We noted that basal portal pressures (PPs) were comparable in wild-type and Cd39-null mice ( n = 9). ATP infusions resulted in increments in PP in wild-type mice, but, in contrast, this significantly decreased in Cd39-null mice ( n = 9) post-ATP in a nitric oxide-dependent manner. We then studied Cd39/NTPDase1 deletion in the regulation of portal hemodynamics, vascular integrity, and intestinal permeability in a murine model of PHT. Partial portal vein ligation (PPVL) was performed in Cd39-null ( n = 44) and wild-type ( n = 23) mice. Sequential measurements obtained after PPVL were indicative of comparable levels of PHT (ranges 14–29 mmHg) in both groups. There was one death in the wild-type group and eight in the Cd39-null group from intestinal bleeding ( P = 0.024). Circulatory stasis in the absence of overt portal vein thrombosis, portal congestion, intestinal hemorrhage, and increased permeability were evident in all surviving Cd39-null mice. Deletion of Cd39 results in deleterious outcomes post-PPVL that are associated with significant microcirculatory derangements and major intestinal congestion with hemorrhage mimicking acute mesenteric occlusion. Absent Cd39/NTPDase1 and decreased generation of adenosine in the splanchnic circulation cause heightened vascular permeability and gastrointestinal hemorrhage in PPVL.

Postoperative Portal Vein Obstruction in Patients with Idiopathic Portal Hypertension

1990 ◽  
Vol 12 (5) ◽  
pp. 607 ◽  
Author(s):  
Hector Orozco ◽  
Takeshi Takahashi ◽  
Miguel Angel Mercado ◽  
Guadalupe Garcia-Tsao ◽  
Aurora Gonzalez-Lopez-Lira ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Idiopathic Portal Hypertension ◽  
Portal Vein Obstruction

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

1992 ◽  
Vol 83 (1) ◽  
pp. 41-45 ◽  
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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