The Etiology of Portal Hypertension in Egyptian Children and Adolescents

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
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Pressure Gradient ◽  
Portal Vein Thrombosis ◽  
Hepatic Fibrosis ◽  
Venous Pressure ◽  
Vena Cava ◽  
Clinical Manifestations ◽  
Vein Thrombosis ◽  
Egyptian Children

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.

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 ◽  
...  
Keyword(s):  
Risk Factors ◽  
Portal Hypertension ◽  
Gastrointestinal Bleeding ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Bacterial Infections ◽  
Umbilical Vein ◽  
Clinical Manifestations ◽  
Vein Thrombosis ◽  
Vein Catheterization

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.

scholarly journals MesoTIPS: Combined Approach for the Treatment of Portal Hypertension Secondary to Portal Vein Thrombosis – A Brief Report

2017 ◽  
Vol 01 (01) ◽  
pp. 20-26
Author(s):  
Abbas Chamsuddin ◽  
Lama Nazzal ◽  
Thomas Heffron ◽  
Osama Gaber ◽  
Raja Achou ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Transjugular Intrahepatic Portosystemic Shunt ◽  
Portosystemic Shunt ◽  
Vein Thrombosis ◽  
Intrahepatic Portosystemic Shunt ◽  
The Right ◽  
Minilaparotomy Approach ◽  
Mean Time

AbstractIntroduction: We describe a technique we call “Meso-transjugular intrahepatic portosystemic shunt (MTIPS)” for relief of portal hypertension secondary to portal vein thrombosis (PVT) using combined surgical and endovascular technique. Materials and Methods: Nine adult patients with PVT underwent transjugular intrahepatic portosystemic shunt through a combined transjugular and mesenteric approach (MTIPS), in which a peripheral mesenteric vein was exposed through a minilaparotomy approach. The right hepatic vein was accessed through a transjugular approach. Mechanical thrombectomy, thrombolysis, and angioplasty were performed when feasible to clear PVT. Results: All patients had technically successful procedures. Patients were followed up for a mean time of 13.3 months (range: 8 days to 3 years). All patients are still alive and asymptomatic. Conclusion: We conclude that MTIPS is effective for the relief of portal hypertension secondary to PVT.

Development of Portal Vein Thrombosis Complicating Idiopathic Portal Hypertension

1985 ◽  
Vol 88 (4) ◽  
pp. 1034-1040 ◽  
Author(s):  
Kunihiko Ohnishi ◽  
Masayuki Saito ◽  
Hidetaka Terabayashi ◽  
Fumio Nomura ◽  
Kunio Okuda
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Idiopathic Portal Hypertension ◽  
Vein Thrombosis

Covered TIPS versus endoscopic band ligation plus propranolol for the prevention of variceal rebleeding in cirrhotic patients with portal vein thrombosis: a randomised controlled trial

Gut ◽  
2017 ◽  
Vol 67 (12) ◽  
pp. 2156-2168 ◽  
Author(s):  
Yong Lv ◽  
Xingshun Qi ◽  
Chuangye He ◽  
Zhengyu Wang ◽  
Zhanxin Yin ◽  
...  
Keyword(s):  
Portal Hypertension ◽  
Adverse Events ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Decompensated Cirrhosis ◽  
Covered Stents ◽  
Band Ligation ◽  
Vein Thrombosis ◽  
Cirrhotic Patients ◽  
Endoscopic Band Ligation

ObjectiveLimited data are available on the prevention of variceal rebleeding in cirrhotic patients with portal vein thrombosis (PVT). This study aimed to compare transjugular intrahepatic portosystemic shunt (TIPS) with covered stents versus endoscopic band ligation (EBL) plus propranolol for the prevention of variceal rebleeding among patients with cirrhosis and PVT.DesignConsecutive cirrhotic patients (94% Child-Pugh class A or B) with PVT who had variceal bleeding in the past 6 weeks were randomly assigned to TIPS group (n=24) or EBL plus propranolol group (EBL+drug, n=25), respectively. Primary endpoint was variceal rebleeding. Secondary endpoints included survival, overt hepatic encephalopathy (OHE), portal vein recanalisation and rethrombosis, other complications of portal hypertension and adverse events.ResultsDuring a median follow-up of 30 months in both groups, variceal rebleeding was significantly less frequent in the TIPS group (15% vs 45% at 1 year and 25% vs 50% at 2 years, respectively; HR=0.28, 95% CI 0.10 to 0.76, p=0.008), with a significantly higher portal vein recanalisation rate (95% vs 70%; p=0.03) and a relatively lower rethrombosis rate (5% vs 33%; p=0.06) compared with the EBL+drug group. There were no statistically significant differences in survival (67% vs 84%; p=0.152), OHE (25% vs 16%; p=0.440), other complications of portal hypertension and adverse events between groups.ConclusionCovered TIPS placement in patients with PVT and moderately decompensated cirrhosis was more effective than EBL combined with propranolol for the prevention of rebleeding, with a higher probability of PVT resolution without increasing the risk of OHE and adverse effects, but this benefit did not translate into improved survival.Trial registration numberClinicalTrials.gov: NCT01326949.

scholarly journals Difficulties of differential diagnosis of portal hypertension: case report

2021 ◽  
pp. 92-101
Author(s):  
T. V. Penkina ◽  
O. E. Berezutskaya ◽  
D. T. Dicheva ◽  
E. V. Partsvania-Vinogradova ◽  
V. S. Larina ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Portal Hypertension ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Clinical Case ◽  
Clinical Symptoms ◽  
Varicose Veins ◽  
Small Diameter ◽  
Magnetic Resonance Tomography ◽  
Vein Thrombosis

In the article we analysed the difficulties of differential diagnosis of portal hypertension, considers a clinical case that illustrates the presented theoretical material. In the presented clinical observation, the patient’s disease was manifested by bleeding from the varicose veins of the esophagus. In most cases, portal hypertension syndrome in practicing clinicians is associated with liver cirrhosis, however, it is necessary to remember about the possibility of developing subhepatic portal hypertension, in particular as a result of the formation of portal vein thrombosis. If there are signs of portal hypertension, it is necessary to specify the level of obstruction to blood flow, that is, the form of portal hypertension (subhepatic, hepatic, suprahepatic). Often, portal vein thrombosis can be formed due to undiagnosed blood diseases that occur without any clinical symptoms. The provided clinical example demonstrates a case of portal hypertension in the outcome of a chronic form of myeloproliferative syndrome. Portal cavernoma is quite rare and it is formed due to multiple small-diameter venous structures that gradually replace the occluded vessel with a system of collaterals proximal and distal to the portal vein thrombosis site. In the formation of the diagnosis the main are radiation research methods, but the conclusions should be considered only in conjunction with the clinical evidence. The clinical case is interesting because a large cavernoma of the portal vein in a patient with subhepatic portal hypertension was regarded as a «solid formation» according to magnetic resonance tomography. According to the literature data, cavernous transformation has an external similarity to the tumor process, which expands the range of differential diagnosis and requires the exclusion of oncological formations. 

scholarly journals A226 INCIDENTAL FINDING OF A LARGE GASTRIC VARIX ASSOCIATED WITH NEONATAL UMBILICAL VEIN CANALIZATION

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 261-263
Author(s):  
L Tsang ◽  
J Abraldes ◽  
E Wiebe ◽  
G S Sandha ◽  
S van Zanten
Keyword(s):  
Portal Hypertension ◽  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Umbilical Vein ◽  
Gastroesophageal Junction ◽  
Splenic Vein ◽  
Gastric Varix ◽  
Vein Thrombosis ◽  
Inferior Aspect ◽  
Upper Abdomen

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

scholarly journals Pelvic lipomatosis associated with portal vein thrombosis and hydronephrosis: a case report

2019 ◽  
Vol 47 (6) ◽  
pp. 2674-2678 ◽  
Author(s):  
Özkacmaz Sercan
Keyword(s):  
Portal Vein ◽  
Portal Vein Thrombosis ◽  
Superior Vena ◽  
Vena Cava ◽  
Vascular Compression ◽  
Fatty Tissue ◽  
Vein Thrombosis ◽  
Urinary Infections ◽  
Pelvic Lipomatosis ◽  
Uncommon Disease

Pelvic lipomatosis is an uncommon disease with no clear etiology and it occurs secondary to deposition of a large amount of fatty tissue in the pelvis. This deposition causes compression to the rectum, bladder, and venous structures. Because of this compression, various symptoms, such as recurrent urinary infections, dysuria, tenesmus, and constipation, have mostly been reported. However, iliac or superior vena cava thrombosis secondary to vascular compression of pelvic lipomatosis is rare. This report describes a case of pelvic lipomatosis, which was associated with right-sided mild hydronephrosis and portal vein thrombosis.

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