scholarly journals Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children

2021 ◽  
Vol 10 (12) ◽  
pp. 2703
Author(s):  
Alina Grama ◽  
Alexandru Pîrvan ◽  
Claudia Sîrbe ◽  
Lucia Burac ◽  
Horia Ştefănescu ◽  
...  

One of the most important causes of portal hypertension among children is extrahepatic portal vein thrombosis (EHPVT). The most common risk factors for EHPVT are neonatal umbilical vein catheterization, transfusions, bacterial infections, dehydration, and thrombophilia. Our study aimed to describe the clinical manifestations, treatment, evolution, and risk factors of children with EHPVT. Methods: We analyzed retrospectively all children admitted and followed in our hospital with EHPVT between January 2011–December 2020. The diagnosis was made by ultrasound or contrast magnetic resonance imaging. We evaluated the onset symptoms, complications, therapeutic methods, and risk factors. Results: A total of 63 children, mean age 5.14 ± 4.90 (33 boys, 52.38%), were evaluated for EHPVT during the study period. The first symptoms were upper gastrointestinal bleeding (31 children, 49.21%) and splenomegaly (22 children, 34.92%). Thrombocytopenia was present in 44 children (69.84%). The most frequent risk factors were umbilical vein catheterization (46 children, 73.02%) and bacterial infections during the neonatal period (30 children, 47.62%). Protein C, protein S, antithrombin III levels were decreased in 44 of the 48 patients tested. In 42 of these cases, mutations for thrombophilia were tested, and 37 were positive. Upper digestive endoscopy was performed in all cases, revealing esophageal varices in 56 children (88.89%). All children with gastrointestinal bleeding received an octreotide infusion. In 26 children (41.27%), variceal ligation was performed, and in 5 children (7.94%), sclerotherapy. Porto-systemic shunt was performed in 11 children (17.46%), and Meso-Rex shunt was done in 4 children (6.35%). The evolution was favorable in 62 cases (98.41%). Only one child died secondary to severe sepsis. Conclusions: EHPVT is frequently diagnosed in the last period in our region due to the increased use of umbilical vein catheterization. Furthermore, genetic predisposition, neonatal bacterial infections, and prematurity certainly play an important role in this condition. A proactive ultrasound assessment of children with risk factors for EHPVT should be encouraged for early diagnosis and treatment.

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 261-263
Author(s):  
L Tsang ◽  
J Abraldes ◽  
E Wiebe ◽  
G S Sandha ◽  
S van Zanten

Abstract Results A 41-year old Asian male, who immigrated to Canada many years ago, and who had previously been successfully treated for Helicobacter pylori infection underwent gastroscopy for investigation of dyspepsia. His gastroscopy was normal except for a large subepithelial abnormality that was noted close to the gastroesophageal junction. Routine gastric biopsies from the antrum and body were normal. Subsequent endoscopic ultrasound revealed flow through the anechoic tortuous lesion and confirmed it was a very large isolated gastric varix type 1. Abdominal CT scan revealed chronic occlusion of the portal vein, splenic vein, and the portal confluence with extensive collateralization in the upper abdomen. There was complete cavernous transformation of the portal vein. Of the numerous varices in the upper abdomen, a very large varix drained into the left renal vein and indented into the posterior wall of the fundus of the stomach which accounted for the endoscopic finding. Multiple mesenteric veins were identified that connected to varices adjacent to the inferior aspect of the pancreas and duodenum. Notably, there was no evidence of cirrhosis or chronic pancreatitis. Liver enzymes, albumin, and INR were normal. Further collateral history revealed that he was hospitalized as a neonate for pneumonia with catheterization of the umbilical vein, which is known to be associated with thrombosis of the portal vein. Conclusions Detection of congenital absence of the portal vein (CAPV) is recognized more often due to advances in diagnostic imaging. Radiologically, the absence of the portal vein in CAPV is distinguished from portal vein thrombosis by the lack of venous collaterals or sequalae of portal hypertension, such as ascites or splenomegaly. A more gradual thrombosis of the portal vein may permit collaterals to develop without acute changes and is not equivalent to portal vein aplasia or agenesis as intrahepatic bile ducts are normal. The gold standard for diagnosis of CAPV is histologic absence of the portal vein in the liver on catheter angiography. CAPV is associated with abnormal embryologic development of the portal vein and frequently presents with complications of portal hypertension or portosystemic encephalopathy or the sequalae of venous shunts, hepatic or cardiac abnormalities found on imaging. Our case is an incidentally discovered absence of the portal venous system due to chronic thrombosis with extensive collateralization and an enlarged gastric varix protruding into the proximal stomach. It is well documented that canalization of the umbilical vein in infancy is associated with portal vein thrombosis, with incidences up to 68%. This case highlights the importance of eliciting a childhood hospitalization history in cases of non-cirrhotic portal hypertension. Funding Agencies None


2020 ◽  
Vol 61 (6) ◽  
pp. 663-664
Author(s):  
Anne Louise van Els ◽  
Joël Israëls ◽  
Marianne Alice van Houten ◽  
Timotheüs Gualtherus Jacob de Meij

1998 ◽  
Vol 157 (6) ◽  
pp. 461-463 ◽  
Author(s):  
H. Guimarães ◽  
L. Castelo ◽  
J. Guimarães ◽  
A. Cardoso ◽  
C. d'Orey ◽  
...  

PEDIATRICS ◽  
1979 ◽  
Vol 63 (3) ◽  
pp. 504-504
Author(s):  
Charles T. Dotter

Acute portal vein thrombosis secondary to omphalitis or umbilical vein catheterization usually leads, years later, to death from the hemorrhagic consequences of portal hypertension. While the delayed manifestation, sometimes inaccurately referred to as "cavernous transformation of the portal vein," is therapeutically approached by surgical portosystemic shunting, results are dismal. A feasible, untried, therapeutic attack on the primary problem is now available in selective fibrinolysis of the acute portal vein thrombus. Streptokinase (or urokinase), given by catheter either into the superior mesenteric artery or via transhepatic portal vein catheterization directly into the fresh thrombus, could be expected to result in complete lysis within a matter of hours.


QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Lerine B El-Din Ahmed ◽  
Asmaa W Abd Elaziz ◽  
Reham I Abdelmegeed ◽  
Mayada Abd El-Rahman F Battah

Abstract Background Portal hypertension is a clinical syndrome in which the portal venous pressure gradient between portal vein and inferior vena cava exceeding 5mmHg. Clinically significant portal hypertension is diagnosed when clinical manifestations of the disease appear or the portal pressure gradient exceeding 10 mmHg. For better management, it is important to determine the underlying cause. Objective To evaluate the aetiology of portal hypertension in pediatric who attending the hepatology clinic at El-Demerdash hospital, Ain Shams University. Materials and Methods This cross sectional study was done in the department of pediatric hepatology clinic at El-Demerdash hospital, Ain Shams University on 91 consecutive cases of portal hypertension enrolled from 2016 to 2019. All pediatric patients were subjected to full history, clinical examination, investigations and questionnaire about effect of portal hypertension and its complications on QOL. Results Age of children and adolescent was 5 months to 16 years with mean age of 5.55±4.30y and male to female ration was 1.5:1. Out of 91 children (51, 56.1%) developed portal hypertension due to extrahepatic causes and (39, 42.9%) due to hepatic causes. In extrahepatic causes (portal vein thrombosis was found 36, (39.6%) and Budd Chiari syndrome 15(16.5%)). On the other hand, hepatic causes (cong. hepatic fibrosis (CHF) was found in 21(23.1%), cases, hepatic cirrhosis 12(13.2%), extrahepatic biliary atresia 3(3.3%) and autoimmune hepatitis with secondary hepatic fibrosis 3(3.3%)). In extrahepatic cases first variant bleed much earlier than hepatic cases. Conclusion Extrahepatic disease were the most common aetiology of portal hypertension in studied cases. Portal vein thrombosis in extrahepatic cases and congenital hepatic fibrosis in intrahepatic cases were the most common causes.


2015 ◽  
Vol 2 (4) ◽  
pp. 135-137
Author(s):  
Dr Chandrashekhar C ◽  
◽  
Dr. Sushma Krishnegowda ◽  
Dr. Vikas. VM ◽  
Dr. Bhaktavatsala. H.R c ◽  
...  

2012 ◽  
Vol 49 (1) ◽  
pp. 69-76 ◽  
Author(s):  
Priscila Menezes Ferri ◽  
Alexandre Rodrigues Ferreira ◽  
Eleonora Druve Tavares Fagundes ◽  
Shinfay Maximilian Liu ◽  
Mariza Leitão Valadares Roquete ◽  
...  

CONTEXT: Portal vein thrombosis refers to a total or partial obstruction of the blood flow in this vein due to a thrombus formation. It is an important cause of portal hypertension in the pediatric age group with high morbidity rates due to its main complication - the upper gastrointestinal bleeding. OBJECTIVE: To describe a group of patients with portal vein thrombosis without associated hepatic disease of the Pediatric Hepatology Clinic of the Hospital das Clínicas, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil with emphasis on diagnosis, presentation form and clinical complications, and the treatment of portal hypertension. METHODS: This is a descriptive study of a series of children and adolescents cases assisted from January 1990 to December 2010. The portal vein thrombosis diagnosis was established by ultrasound. RESULTS: Of the 55 studied patients, 30 (54.5%) were male. In 29 patients (52.7%), none of the risk factors for portal vein thrombosis was observed. The predominant form of presentation was the upper gastrointestinal bleeding (52.7%). In 20 patients (36.4%), the initial manifestation was splenomegaly. During the whole following period of the study, 39 patients (70.9%) showed at least one episode of upper gastrointestinal bleeding. The mean age of patients in the first episode was 4.6 ± 3.4 years old. The endoscopic procedure carried out in the urgency or electively for search of esophageal varices showed its presence in 84.9% of the evaluated patients. The prophylactic endoscopic treatment was performed with endoscopic band ligation of varices in 31.3% of patients. Only one died due to refractory bleeding. CONCLUSIONS: The portal vein thrombosis is one of the most important causes of upper gastrointestinal bleeding in children. In all non febrile children with splenomegaly and/or hematemesis and without hepatomegaly and with normal hepatic function tests, it should be suspect of portal vein thrombosis. Thus, an appropriate diagnostic and treatment approach is desirable in an attempt to reduce morbidity and mortality.


2017 ◽  
Vol 27 ◽  
Author(s):  
José Ricardo Borém Lopes ◽  
Thaís Costa Nascentes Queiroz ◽  
Bárbara Fonseca Gazzinelli ◽  
Eleonora Druve Tavares Fagundes ◽  
Alexandre Rodrigues Ferreira ◽  
...  

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Tarek A Abd El Gawad ◽  
Lerine Bahy El Din ◽  
Amel A.M El Faramawy ◽  
Asmaa Wafeeq ◽  
Hebatallah A Shaaban ◽  
...  

Abstract Background and aim of the work Umbilical venous catheter(UVC) placement is thought to be a cause of portal vein thrombosis(PVT).Our study aimed to identify the incidence of PVT and potential risk factors predisposing neonates with UVC to develop PVT and the prevalence of hereditary prothrombotic mutations among the affected group. Methods A prospective observational study was conducted in the Neonatal intensive care unit (NICU) of Ain Shams university(ASU) Children's Hospital on 100 neonates.They were subjected to abdominal X-ray initially and duplex on their portal veins initially and at 1,6 and 12 months post insertion.We studied the correlation between length of NICU stay, UVC duration, technique, birth weight(BW),gestational age (GA) and the development of PVT. Results There was 46 males and 54 females.The mean BW was 1.87±0.72 Kg, the mean GA was 33.71±2.41weeks.Mean stay in NICU was 18.22±9.78 days, with mean UVC insertion duration 6.88 ±2.55 days,75%of inserted UVC were central in position,77% of the studied neonates were premature,1 patient had NEC(Necrotizing EnteroColitis),18% had sepsis,4% had blood products transfusion through the UVC.The one year follow up of the study group showed no incidence of UVC related PVT. Conclusion Although PVT is related to UVC in the literature and in our experience in the ASU Children's Hospital Hepatology Clinic as a tertiary referral centre, yet in this study, the sole placement of UVC does not appear to increase risk of PVT if properly inserted and cared.


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