Etiology, Presenting Features, and Endoscopic Findings of Portal Vein Hypertension in Children (original) (raw)
Related papers
Portal hypertension among Egyptian children and adolescents (single center study)
Current Pediatric Research, 2021
Portal hypertension is a clinical syndrome in which the portal venous pressure gradient between the portal vein and inferior vena cava exceeding 5 mmHg. 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. This study aimed to evaluate the aetiology, presentation and quality of life in pediatric portal hypertension patients. This cross-sectional study was done on ninety-one consecutive cases of portal hypertension enrolled from 2016 to 2019. Demographic data, etiology, clinical presentation, endoscopic interventions, and quality of life were all assessed. The mean age of participants was 5.55 ± 4.30 years with a male to female ratio of 1.5:1. Out of 91 children, 56.1% developed portal hypertension due to extrahepatic causes and 42.9% due to hepatic causes. In extrahepatic causes portal vein obstruction was the most common aetiology representing 39.6% Splenomegaly was the commonest presentation of PHT and esophageal varices were the commonest complication. QoL scores, total and individual domains, were lower in all our PHT children. Conclusion: Extra hepatic portal vein obstruction was the most common etiology of portal hypertension in studied cases. Poor QOL was reported in all patients irrespective of the etiology of portal hypertension.
Portal Hypertension in Children: A Tertiary Center Experience in Turkey
Pediatric Gastroenterology, Hepatology & Nutrition
Purpose: Portal hypertension (PH) and its complications have a significant impact on morbidity and mortality. This study aimed to evaluate the etiology; clinical, laboratory, and endoscopic findings; treatment approaches; long-term outcomes; and prognosis of pediatric PH. Methods: This retrospective study included 222 pediatric patients diagnosed with PH between 1998 and 2016, and data encompassing clinical, laboratory, and radiological features; treatments; and complications were analyzed. Results: The most common causes of PH were portal vein thrombosis (20.3%), progressive familial intrahepatic cholestasis (18.9%), and biliary atresia (12.2%). Among the enrolled patients, 131 (59.0%) were included in the cirrhotic group and 91 (41.0%) in the noncirrhotic group. Hepatomegaly and increased transaminase levels were more frequent in the cirrhotic group than in the non-cirrhotic group. Additionally, portal gastropathy, esophageal varices, and variceal bleeding were more frequent in the non-cirrhotic group, whereas ascites, hepatopulmonary syndrome and hepatic encephalopathy were more common in the cirrhotic group. The incidence of hepatomegaly was higher in the presinusoidal group than in the prehepatic group (p<0.001). Hyperbilirubinemia was more frequent in the prehepatic group (p=0.046). The frequency of esophageal varices was similar between the prehepatic and presinusoidal groups; however, variceal bleeding was more frequent in the prehepatic group (p=0.002). Conclusion: Extrahepatic portal vein obstruction, genetic-metabolic diseases, and biliary atresia were the most prevalent causes of PH in our country. In patients with PH, hepatomegaly, increased transaminase levels, and synthesis dysfunction were suggestive of cirrhotic PH. Notably, PH in patients without cirrhosis might be more severe than that in those with cirrhosis.
Frequency of Causes of Portal Hypertension in Children
Cureus
The most common etiology of portal hypertension (PH) in children is obstruction at the presinusoidal or sinusoidal level. In addition, portal vein thrombosis (PVT) and biliary atresia are the most prevalent extrahepatic causes. This study aims to evaluate all the possible etiologies leading to PH in the pediatric population and provide the most common cause associated with this condition along with the age group most frequently affected by it. Material and Methods From January 2018 to December 2020, a cross-sectional study was carried out in tertiary care hospitals in Pakistan. A total of 100 children, both male and female, aged one month to 15 years and diagnosed with PH, were enrolled for the evaluation for the causes of PH. The Statistical Package for the Social Sciences (SPSS), version 20, was used to analyze the data. Results The mean age of enrolled participants was 9.01 ± 2.81 years. It was found that PVT (63%) was the most common cause of PH, followed by liver cirrhosis (19%) and biliary atresia (18%). Age of more than eight years was significantly associated with PVT (p-value: 0.007). Conclusion In children, PH may be caused by a wide range of etiologies. It is imperative to understand the underlying etiologies contributing to PH for proper guidance and management, prevention, and overall outcomes.
Journal of Shaheed Suhrawardy Medical College
Background: Portal hypertension is a clinical syndrome defined by a pathologic increase of portal venous pressure. The objectives of this study were to evaluate etiological and clinical presentation of portal hypertension admitted in a tertiary care centre of Bangladesh. Materials and Methods: This cross sectional study was done at the Department of Paediatric Gastroenterology & Nutrition, BSMMU on 100 consecutive cases admitted during the period from July 2013 through June 2015. Confirmation of the presence of portal hypertension was done by demonstration of oesophageal varices during upper GI endoscopic examination. The diagnosis of chronic liver disease was based on a combination of clinical, biochemical (abnormal liver function tests) and ultrasonographic (coarse echotexture of liver) features. Extrahepatic portal hypertension was diagnosed on the basis of clinical, normal liver function test and ultrasonographic evidence of portal or splenic vein thrombosis, with or without cavernous transformation. Doppler ultrasound showed increase portal venous pressure. Results: Patient's age group was 2 to 15 years. 69 cases were extra hepatic portal hypertension. Haematemesis and/or melaena were found in 42(60.9%) and splenomegaly was found in 68(98.6%) cases. Of extra hepatic 69 cases 10% had history of umbilical sepsis. Of the 31cases of CLD with portal hypertension, haematemesis and/or melaena was found in 20(64.5%) cases, splenomegaly in 30(96.8%), ascites 8(25.8%).Most (50%) of CLD cases were cryptogenic followed by Wilson's disease 29%. Of the 100 cases, endoscopy revealed grade 3 esophageal varices in 45% cases. All the patients were treated with propranolol. EVL was done in 70% cases. Conclusion: In this study, most of the cases were extra hepatic portal hypertension. Gastrointestinal bleeding & splenomegaly were found in most of the cases. No risk factor was found in most of extra hepatic cases. Portal vein thrombosis & cryptogenic were the most common cause in extra-hepatic and intra-hepatic cases respectively. [
Journal of Pediatric Gastroenterology and Nutrition, 2011
Objectives: The aim of the study was to determine the frequency of portal gastropathy (PG) and duodenopathy (PD) in children, to document the correlation of various clinical and laboratory parameters associated with portal hypertensive gastroduodenal lesions, to compare the endoscopic portal hypertensive lesions with different histologic findings, and to evaluate the use of a possible histologic scoring system. Methods: All children undergoing endoscopic investigation for portal hypertension (PH) between January 2006 and November 2007 were analysed retrospectively. Clinical and demographical data and endoscopic and histologic findings were recorded. Histologic findings suggestive of PG and PD (capillary dilation, increased numbers of capillaries, histologic bleeding, and edema) were scored. Results: Of 51 consecutive children (29 boys, mean age 10.1AE 3.6 years [range 2.5-15.8 years]), 28 were cirrhotic. PG was diagnosed in 58.8% endoscopically. Children with cirrhotic PH had the highest rate of PG (64.3%), whereas those with extrahepatic or intrahepatic noncirrhotic PH were alike (50% and 54.5%, respectively). Baveno PG scores were higher in children with cirrhosis with higher Child-Pugh scores. Capillary dilation was the only histologic finding showing significant association with the endoscopic diagnosis. Only 9% had PD on endoscopy. None of the histologic findings correlated with endoscopic diagnosis of PD. Conclusions: PG and PD are seen in children with extrahepatic and intrahepatic PH at rates similar to those reported in adult studies. Baveno PG scores increased in parallel with Child-Pugh class in children with cirrhosis. Capillary dilation was the only histologic finding showing significant association with the endoscopic diagnosis of PG in this study.
Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children
Journal of Clinical Medicine
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 fre...
Pediatric Transplantation, 2006
Portal hypertension leads to a wide variety of complications, which lead to significant morbidity and mortality and are some of the leading reasons for liver transplantation in children with chronic liver disease. Evidence-based approaches to the management of adults with portal hypertension exist and have been comprehensively reviewed in a series of international meetings, including the Baveno meetings. Similar evidence-based approaches for the management of portal hypertension in children do not exist and as such international meetings on portal hypertension have not focused on this problem in children. On October 7, 2005 at The Mount Sinai School of Medicine, a panel of pediatric experts reviewed the most recent Baveno statement and crafted a statement modified with their opinions vis a vis approaches to the management of portal hypertension in children.
Management of Extrahepatic Portal Hypertension in Children
Annals of Surgery, 1974
Among 69 patients with PVT, 338 variceal bleeding episodes occurred. Only two patients died from bleeding, and both lived in remote communities and were inaccessible to medical care. Fifty-three children underwent 164 operations for the management of PVT. Once operative management was undertaken, subsequent operations frequently were necessary. Nonoperative measures controlled acute variceal hemorrhage in most instances during the past 10 years. Almost all patients who underwent splenectojny alone, variceal ligation, gastric division, splenic transposition, or makeshift shunts subsequently rebled. These operations are rarely indicated in the current management of children with PVT. Portal venography is essential to define the portal venous circulation before a shunt operation is attempted. Cavomesenteric or central splenorenal shunts prevented further bleeding in eight of 15 patients and are the most reliable operations to control bleeding in patients with PVT. Emergency operation is rarely necessary to control bleeding. Sixteen patients (average age 14.6 years) with PVT did not undergo any operations, and are alive. Each of the six patients with PVT who died from complications of portal hypertension did so within nine months of an operation. Four of these patients had previous splenectomy and died with sepsis as one of the major factors. Bleeding episodes became less frequent as the patients increased in age. Patients who underwent shunts under unfavorable circumstances or who received various other operations to treat portal hypertension appeared to have a higher risk of morbidity and mortality than those managed nonoperatively. E XTRAHEPAnC portal venous thrombosis (PVT) has been recognized as the most frequent cause of portal hypertension in children, yet scant information has been recorded to indicate the long-term prognosis for patients with this condition. Since Clatworthy and associates24 summarized the manifestations of PVT and recom-From the
Clinicopathological Correlation of Portal Hypertension in Children and Management Strategies
We studied 51 children with clinical setting of portal hypertension over a period of one and half years. Out of these, 27 (52.94%) were males and 24 (47.05%) were females. All the cases were subjected to biochemical, diagnostic, radiological investigations and liver biopsy. Portal hypertension was diagnosed by demonstrating on USG abdomen/ Doppler, the presence of collaterals of portal vein and esophageal varices on endoscopy. Treatment was initiated depending upon clinical presentation. The most common presenting complaints were hematemesis and malena (35.3%) followed by their combination with abdominal distension (19.6%). 86.3% patients had anemia mostly due to upper GI bleed. 35.3% patients had leucopenia while 47.1% had thrombocytopenia. Only 18 (i.e. 35.3%) had Hypersplenism. Maximum patients who bled more than twice had a mild (41.2%) or severe (29.4%) derangement of prothrombin time. The etiology of portal hypertension turned out to be extra-hepatic portal vein obstruction in 86.3% cases. There were 2 cases each (3.9% each) of Wilson's disease and Budd-Chiari syndrome. One (2%) was secondary to chronic liver disease due to hepatitis C infection, one was extra-hepatic biliary atresia and one was autoimmune hepatitis. 9.8% cases required drugs in the form of somatostatin drip and all these required a packed cell transfusion as well. 23 patients (45.1%) required only blood transfusion without a somatostatin infusion. Those with severe acute bleeding usually were given somatostatin infusion. The variceal size at presentation was a very important predictor of the morbidity and outcome.
Etiology of Portal Hypertension in Children-An Experience in a Tertiary Centre of Bangladesh
Bangladesh Journal of Child Health, 2016
Background: Portal hypertension is defined by a pathological increase in portal venous pressure. Any condition that interfere with blood flow at any level within the portal system can lead to portal hypertension. For better management, it is important to determine the underlying cause. Objective: To evaluate the etiology of portal hypertension in children admitted in a tertiary care centre of Bangladesh.