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.

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.

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.

Extrahepatic portal vein obstruction in adults detected by ultrasound with frequent lack of portal hypertension signs

1993 ◽  
Vol 8 (2) ◽  
pp. 161-167 ◽  
Author(s):  
NOBUYUKI SUGIURA ◽  
SHOUICHI MATSUTANI ◽  
MASAO OHTO ◽  
MASAAKI EBARA ◽  
MASAHARU YOSHIKAWA ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Extrahepatic Portal Vein Obstruction ◽  
Portal Vein Obstruction

Portopulmonary Shunt by Splenopneumopexy as a Treatment for Portal Hypertension Due to Extrahepatic Portal Vein Obstruction: Case Report

1993 ◽  
Vol 27 (4) ◽  
pp. 303-307
Author(s):  
Koki Tanaka ◽  
Hiromi Nakayama ◽  
Meguru Yoshimine ◽  
Kenzo Honbo ◽  
Takashi Ohno ◽  
...  
Keyword(s):  
Case Report ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Extrahepatic Portal Vein Obstruction ◽  
Portal Vein Obstruction

scholarly journals Hyperammonemia of unknown origin

2015 ◽  
Vol 4 (3S) ◽  
pp. 11-15
Author(s):  
Francesca Molino
Keyword(s):  
Computed Tomography ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Unknown Origin ◽  
Idiopathic Portal Hypertension ◽  
Biochemical Tests ◽  
Extrahepatic Portal Vein Obstruction ◽  
Laboratory Findings ◽  
Portal Vein Obstruction ◽  
Normal Ultrasound

Idiopathic portal hypertension is a benign long-standing non-cirrhotic portal hypertension with no typical laboratory findings and absence of stigmata of chronic liver disease. The disease is diagnosed by the presence of evidence of portal hypertension with preserved liver function and absence of extrahepatic portal vein obstruction. We report the case of a 71-year-old woman who was admitted in hospital with encephalopathy and hyperammonemia. Liver biochemical tests excluded cirrhosis but revealed pancytopenia; preliminary abdomen ultrasound was normal. Ultrasound doppler and abdomen computed tomography of portal vein revealed patent portal vein with impaired portal intrahepatic perfusion and portal-systemic shunts.

scholarly journals Unusual Tumors Causing Extrahepatic Portal Venous Obstruction

HPB Surgery ◽  
1996 ◽  
Vol 9 (3) ◽  
pp. 165-167 ◽  
Author(s):  
B. C. Sharma ◽  
V. A. Saraswat ◽  
R. K. Dhiman ◽  
U. C. Ghoshal ◽  
A. S. Puri ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Variceal Bleeding ◽  
Bile Ducts ◽  
Venous Obstruction ◽  
Extrahepatic Portal Vein Obstruction ◽  
Extrahepatic Portal Venous Obstruction ◽  
Portal Vein Obstruction ◽  
Vascular Infiltration ◽  
Gastric Leiomyosarcoma

Extrahepatic portal vein obstruction has been reported to be associated with tumors of liver, bile ducts and pancreas. We report two cases, one with gastric leiomyosarcoma and another with Non Hodgkin’s lymphoma, complicated by portal vein block and presenting with gastric variceal bleeding. Portal vein block in both cases was due to direct vascular infiltration. Development of portal hypertension posed difficulties in management.

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.

scholarly journals A Computational Analysis of Blood Flow through Portal Vein under Normal and Extrahepatic Obstructions

2018 ◽  
Vol 41 (2) ◽  
pp. 183-199
Author(s):  
Mst Khorseda Aktar ◽  
Md Tajul Islam
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Flow Behavior ◽  
Flow Distribution ◽  
Main Portal Vein ◽  
Extrahepatic Portal Vein Obstruction ◽  
Portal Vein Obstruction ◽  
Computational Fluid Dynamics Cfd ◽  
Flow Through ◽  
Academy Of Sciences

Portal hypertension is an alarming disease globally whereas Extrahepatic portal vein obstruction (EHPVO) is an important cause of portal hypertension among children in most of the Asian countries. The frequent occurrence of this disease is due to the formation of thrombosis in the portal vein. To acquire insights into the behaviour of hemodynamics, EHPVO case is studied by the technique of computational fluid dynamics (CFD) by considering partial block formation with different sizes inside the main portal vein. On the basis of block sizes, three cases namely normal portal vein (without block in the portal vein) case, 40% block in the portal vein case and 85% block in the portal vein case are simulated by assuming a 2D steady, laminar and fully developed flow with no-slip wall condition using AnsysTM Fluent. Our study reveals that the flow distribution between the left and right branches of the portal vein is significantly influenced by the formation of the block in the main portal vein. It is concluded that Extrahepatic portal vein obstruction leads to the possibility of Hypertrophy and Atrophy complex to the liver and could heighten the risk of liver diseases. Our numerical examination will play a great role to the Liver Specialist for understanding the flow behavior with obstructed flow path as well as the diagnosis of associated liver disease and their treatments.Journal of Bangladesh Academy of Sciences, Vol. 41, No. 2, 183-199, 2017

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