Ignite Creation Date:
2024-05-06 @ 7:43 PM
Last Modification Date:
2024-10-26 @ 3:12 PM
Study NCT ID:
NCT06117488
Status:
NOT_YET_RECRUITING
Last Update Posted:
2023-11-07
First Post:
2023-10-06
Brief Title:
Risk Factors and Management Outcome of Chronic Portal Vein Thrombosis in Children
Sponsor:
Assiut University
Organization:
Assiut University
Study Overview
Official Title:
Risk Factors and Management Outcome of Chronic Portal Vein Thrombosis in Children
Status:
NOT_YET_RECRUITING
Status Verified Date:
2023-11
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
None
Brief Summary:
The aim of study is to evaluate different etiological and risk factors that lead to chronic portal vein thrombosis and to delineate a management plan for chronic portal vein thrombosis in children
Detailed Description:
Chronic Portal vein thrombosis PVT is defined as thrombosis that develops in the trunk of the portal vein including its right andor left intrahepatic branches of more than 5 weeks duration This thrombus may extend to the splenic or superior mesenteric veins The causes of chronic PVT in children are not entirely known but several factors that predispose to this pathology are described These are classified into three categories local factors that can cause injury to the portal vein abdominal infections abdominal surgery umbilical catheter general factors procoagulant status and less often vascular malformation The most common cause is umbilical vein catheterization UVC Among the general factors that predispose to venous thrombosis are thrombophilia sepsis and dehydration Deficiency or qualitative abnormalities of anti-coagulation factors antithrombin III protein C protein S and activated protein C resistance often predispose to thrombotic events including PVT Thrombophilia is incriminated in 35 of cases of PVT in children For this reason children with PVT and especially those that associate other risk factors UVC should be screened for inherited prothrombotic disorders prothrombin 20210 mutation PTHR factor V Leiden FVL methylenetetrahydrofolate reductase MTHFR genes deficiency or metabolic defects like hyper homocysteinemia Congenital abnormalities portal vein stenosis atresia or agenesis are rarely involved in PVT Furthermore early PVT after liver transplantation with cadaveric graft was described in adults Even less often in children PVT after splenectomy for hematologic diseases was also described An association between more than one factor is frequently observed which further increases the risk of thrombosis In almost 50 of cases the etiology of PVT remains unknown
PVT patients initially present with upper gastrointestinal bleeding UGIB or splenomegaly on routine clinical examination in asymptomatic individuals The initial presence of hematemesis is usually dramatic in a previously healthy child with past history of morbidity often without remarkable intercurrent events Melena may also be observed but it is less common than hematemesis The child can be lethargic with signs of orthostatic hypotension The clinical examination revealing splenomegaly in a child with UGIB indicates esophageal varices as the most probable site for the bleeding Less frequently the diagnosis can be based on the investigation of a child with abdominal pain or with complications related to hypersplenism The physical examination may reveal splenomegaly hepatomegaly is not common in children with PVT without underlying liver disease as well as stigmata of chronic liver disease PVT should be suspected in all children with splenomegaly without hepatomegaly and hematemesis with normal liver function test results Liver biopsy is normal in children without associated cirrhosis
Abdominal Doppler ultrasonography is the most widely used diagnostic exam in pediatric patients with a high sensitivity and specificity even though it is an operator-dependent diagnostic method Chronicity of PVT is defined by Doppler ultrasonography by means of visualization of the formation of new vessels around the thrombus cavernoma Some diagnostic exams should not be routinely used in pediatric patients due to their risk splenoportography and arterial portography nuclear magnetic resonance angiography computed tomography portogram All patients must be submitted to upper gastrointestinal endoscopy to check for the presence of esophagogastric varices which will allow for a better planned therapeutic approach Laboratory exams show normal liver function in most patients except in those who have a prolonged decrease in portal circulation or portal biliopathy
The treatment of portal venous obstruction depends upon the patients age the site and nature of the obstruction and the clinical features Endoscopic variceal ligation EVL is the primary choice for the management of variceal bleeding in children This treatment may be technically difficult in young and small children sclerotherapy is then recommended as an alternative approach in such cases Beta adrenergic blockade may play a role in secondary prophylaxis as they reduce the risk of rebleeding and improve survival after variceal bleed Decompressive shunt surgery should be considered in cases with failed endotherapy It is also indicated for correcting symptomatic portal hypertensive biliopathy symptomatic hypersplenism and on demand one-time treatment Oesophageal transection with or without splenectomy is less useful to control bleeding because of a high risk of late rebleeding and reappearance of varices but can be resorted to as a nonshunt option in patients with portosystemic encephalopathy hepatopulmonary syndrome or portopulmonary syndrome Portal vein thrombosis was considered a major obstacle to liver transplantation which led to increased surgical complexity and perioperative morbidity and mortality
PVT patients initially present with upper gastrointestinal bleeding UGIB or splenomegaly on routine clinical examination in asymptomatic individuals The initial presence of hematemesis is usually dramatic in a previously healthy child with past history of morbidity often without remarkable intercurrent events Melena may also be observed but it is less common than hematemesis The child can be lethargic with signs of orthostatic hypotension The clinical examination revealing splenomegaly in a child with UGIB indicates esophageal varices as the most probable site for the bleeding Less frequently the diagnosis can be based on the investigation of a child with abdominal pain or with complications related to hypersplenism The physical examination may reveal splenomegaly hepatomegaly is not common in children with PVT without underlying liver disease as well as stigmata of chronic liver disease PVT should be suspected in all children with splenomegaly without hepatomegaly and hematemesis with normal liver function test results Liver biopsy is normal in children without associated cirrhosis
Abdominal Doppler ultrasonography is the most widely used diagnostic exam in pediatric patients with a high sensitivity and specificity even though it is an operator-dependent diagnostic method Chronicity of PVT is defined by Doppler ultrasonography by means of visualization of the formation of new vessels around the thrombus cavernoma Some diagnostic exams should not be routinely used in pediatric patients due to their risk splenoportography and arterial portography nuclear magnetic resonance angiography computed tomography portogram All patients must be submitted to upper gastrointestinal endoscopy to check for the presence of esophagogastric varices which will allow for a better planned therapeutic approach Laboratory exams show normal liver function in most patients except in those who have a prolonged decrease in portal circulation or portal biliopathy
The treatment of portal venous obstruction depends upon the patients age the site and nature of the obstruction and the clinical features Endoscopic variceal ligation EVL is the primary choice for the management of variceal bleeding in children This treatment may be technically difficult in young and small children sclerotherapy is then recommended as an alternative approach in such cases Beta adrenergic blockade may play a role in secondary prophylaxis as they reduce the risk of rebleeding and improve survival after variceal bleed Decompressive shunt surgery should be considered in cases with failed endotherapy It is also indicated for correcting symptomatic portal hypertensive biliopathy symptomatic hypersplenism and on demand one-time treatment Oesophageal transection with or without splenectomy is less useful to control bleeding because of a high risk of late rebleeding and reappearance of varices but can be resorted to as a nonshunt option in patients with portosystemic encephalopathy hepatopulmonary syndrome or portopulmonary syndrome Portal vein thrombosis was considered a major obstacle to liver transplantation which led to increased surgical complexity and perioperative morbidity and mortality
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None