Portal hypertension among Egyptian children and adolescents (single center study).

Evolving Consensus in Portal Hypertension Report of the Baveno IV Consensus Workshop on methodology of diagnosis and therapy in portal hypertension

Noncirrhotic intrahepatic portal hypertension.

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.

Noncirrhotic portal hypertension: Medical and endoscopic management.

Portal vein thrombosis in adults: pathophysiology, pathogenesis and management

Non-cirrhotic portal hypertension refers to causes of portal hypertension (PHT) other than cirrhosis which are pre-sinusoidal in nature. The major causes are extra-hepatic portal vein obstruction (EHPVO) and congenital hepatic fibrosis (CHF). Non-cirrhotic portal fibrosis also constitutes a small proportion of paediatric PHT. EHPVO is characterized by obstruction of the extra-hepatic portal vein with or without involvement of the intra-hepatic portal veins or splenic or superior mesenteric veins. What causes EHPVO is unknown however; various theories like infection, umbilical sepsis and hypercoagulable states have been postulated. Endoscopic management is highly successful in controlling acutevariceal bleeding and eradication of esophageal varices. At present the focus in EHPVO has gradually shifted to the management of long-term problems of bleeding from gastric and ectopic varices, growth retardation, portal biliopathy, massive splenomegaly with poor quality of life, hypersplenism and minimal hepatic encephalopathy. Improved outcomes of surgical management of EHPVO along with newer surgical procedures have changed the outlook towards surgery. Congenital hepatic fibrosis has a good long-term outcome in the absence of renal cysts and recurrent cholangitis due to biliary cystic disease. Non-cirrhotic portal fibrosis is seen in adolescents and has a good outcome with endoscopic and surgical management.

Extrahepatic portal vein obstruction (EHPO) is a frequent cause of portal hypertension in children, but it also occurs in adults as a sequela to portal thrombosis. In infants, omphalitis and other forms of infection are the established causes, and in adults pylephlebitis, biliary tract infection, and injury to the portal vein during surgery are known to be associated with EHPO. The etiology is more often obscure in adult cases while in infants there may be only cavernous transformation in the absence of a recognizable portal vein remnant, and a congenital malformation or agenesis cannot be ruled out as a possible etiology. For this reason, some investigators divide this disease into primary and secondary types, but we believe that even in infant cases the majority are due to portal thrombosis, and separation of the two types is difficult and of little significance. One of the important questions regarding its etiopathology is whether EHPO and intrahepatic noncirrhotic portal hypertension (also called hepatoportal sclerosis, idiopathic portal hypertension, and noncirrhotic portal fibrosis) are two entirely different disorders or the same disease with differing sites of primary involvement [1]. Portal vein thrombosis is frequently seen in patients with the latter disorder, and some of the patients with EHPO present the same features seen in the latter [2]. In India, both noncirrhotic portal hypertension and EHPO are very common. In 1975, a research committee was founded in Japan under the auspices of the Japan Ministry of Health and Welfare to study the epidemiology, etiology, clinical features, and management of idiopathic portal hypertension and related disorders. One of the authors (K.O.) was the chairman of this research committee, and this group continues to study these problems. The two first national surveys were conducted in 1983, one for idiopathic portal hypertension (IPH) and the other for EHPO [2]. In 1987, second national surveys were carried out for more recent cases by the other author (HA). In this chapter, therefore, we wish to present the analyses of the two national surveys on EHPO in Japan made by this study group, and a review of the past studies on portal vein obstruction, mainly of the chronic type.

Aim of investigation. To estimate diagnostic value of liver and spleen elastography in patients with extrahepatic portal vein obstruction - non-cirrhotic portal vein thrombosis (PVT). Material and methods. The study group: 19 patients (Age 21-76 years) with PVT diagnosed by multispiral computed angiography without liver/pancreatobiliary tumors and/or liver cirrhosis (LC). The comparison group included 23 patients with LC Child-Pugh class A. Past history of portal hypertension, platelet count, serum albumin level, alanine and aspartate aminotransferase level; prothrombin according to international normalization ratio; grade of esophageal varices (EV); spleen longitudinal size according to abdominal ultrasound, liver and spleen stiffness were evaluated in both groups. Results. The group of patients with non-cirrhotic PVT was characterized by presence of clinically significant portal hypertension (EV, splenomegaly and hypersplenism). Following causes for PVT were established: systemic factors - myeloproliferative diseases, G20210A prothrombin gene mutation, and local factors: complications after pancreatobiliary surgery, omphalitis and neonatal umbilical sepsis. Nine patients, 1 to 2 years prior to hospitalization, were previously misclassified as «cryptogenic liver cirrhosis» in various medical institutions. Liver stiffness in PVT group was 2,8-11,5 kPa. The grade of EV tended to increase along with progression of the spleen stiffness. Statistically significant difference in serum aminotransferases levels in non-cirrhotic PVT vs Child-Pugh class A HCV-LC was observed: enzyme levels were 1,5-2 fold higher in LC (р<0,0001). No differences in the protein-synthetic liver function and severity of thrombocytopenia were found. Prevalence of EV was higher in non-cirrhotic group (р=0,028).) Statistically significant differences between groups in a liver stiffness were revealed: in non-cirrhotic PVT patients the median liver stiffness was 5,6 kPa, in LC patients it was 20,6 kPa, (p<0,0001). Conclusions. Liver elastography is rational in patients with primary diagnosed portal hypertension and PVT. For the differential diagnosis between LC-related PVT and the non-cirrhotic PVT the cut-off value of 11,5 kPa can be applied. Plotting of multifactorial model requires further studies with larger number of patients. Diagnostic value of spleen stiffness measurement as method for noninvasive diagnostic of degree of portal hypertension in non-cirrhotic PVT has been confirmed by the published data. Estimation of a cut-off value to predict EV development and EV bleeding in these patients requires larger number of patients and considering confounding factors.

The effects of chronic endoscopic variceal sclerotherapy on portal pressure in cirrhotics

Purpose: Extra-hepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension and variceal bleeding. Few studies have been done on hemodynamic alterations in patients with non-cirrhotic portal hypertension especially EHPVO in contrast to many reports on hemodynamics in cirrhotics. We evaluated alterations of systemic and pulmonary vascular system in patients with EHPVO and compared them with patients with compensated cirrhosis. The rationale for this investigation was to study the role of portal hypertension per se on systemic and pulmonary hemodynamics in EHPVO as compared to the hemodynamic changes produced by hepatic dysfunction plus portal hypertension in cirrhotics. Methods: Consecutive patients of EHPVO, ≥15 years of age were included, Controls were consecutive patients with compensated cirrhosis and history of variceal bleed, matched for variceal status (to ensure that they had same degree of portal hypertension) and body surface area, attending our department during the same period. The hemodynamic studies were HVPG, right atrial pressure (RAP), pulmonary arterial pressure (PAP), pulmonary capillary wedge pressure (PCWP) and mean arterial pressure (MAP). Cardiac output (CO), systemic vascular resistance and pulmonary vascular resistance were calculated. Results: The baseline parameters in the two groups were comparable. Both EHPVO patients and cirrhotics had similar values in all the measured hemodynamic parameters. The mean cardiac output in EHPVO was 6.5 (±2.6) L/min while it was 7.9 (±3.2) L/min in cirrhosis (P= 0.212). The systemic vascular resistance in EHPVO was 1242 (±494) dyn.s.cm−5, which was similar to that in cirrhotics (1018 [± 355], P= 0.167). Similarly the values of pulmonary vascular resistance were comparable in the two groups (68 [± 60] vs. 71 [± 70], P= 0.905). A subgroup analysis was done of 8 patients of EHPVO and 8 age matched compensated cirrhotics which also revealed similar cardiac index, cardiac output, systemic vascular resistance index, systemic vascular resistance, pulmonary vascular resistance index, and pulmonary vascular resistance in the two groups. Conclusion: EHPVO has features of hyperdynamic circulation, ie increased cardiac output, decreased systemic and pulmonary vascular resistance. These changes are similar to that seen in patients with cirrhosis. This suggests a predominant role of increased resistance and thus increased porto-systemic collateral circulation per se rather than hepatocellular injury in the genesis of these hemodynamic alterations.

EUS-guided portal pressure gradient measurement: a promising tool in noncirrhotic portal hypertension

Extrahepatic portal vein obstruction (EHPVO) is a rare disease-causing form of portal hypertension. Myeloproliferative neoplasm (MPN) including essential thrombocythemia (ET) is a reported risk factor for EHPVO due to underlying persistent thrombophilia. A Japanese woman in her 40s was referred to our hospital with a 1-month history of gastric variceal bleeding due to EHPVO. Laboratory investigation showed thrombocytosis despite portal hypertension. She had a mutation in clonal marker JAK2V617F with EHPVO, which prompted us to consult a hematologist. A bone marrow biopsy revealed megakaryocyte lineage proliferation, which confirmed a diagnosis of ET. Esophagogastroduodenoscopy revealed esophagogastric varices (LsF2CbRC2, Lg-cF1RC1), and abdominal computed tomography and angiography revealed splenomegaly and portal vein thrombosis with cavernous transformation, which suggested EHPVO. The patient had a history of ruptured esophagogastric varices and required prophylaxis against further variceal bleeding before antithrombotic therapy for EHPVO with ET. We performed laparoscopic Hassab's operation followed by endoscopic variceal ligation (EVL) and hematological cytoreduction therapy. Laparoscopic Hassab's operation and three bi-monthly EVL procedures improved the esophagogastric varix (LmF0RC0, Lg-f F0RC0) at 6 months after surgery. Cytoreduction therapy reduced platelet count to 60.1 × 104/uL, and the patient was very healthy at 7 months after surgery. Patients with EHPVO are traditionally referred to a gastroenterologist for abdominal pain, intestinal bleeding, or refractory ascites; however, hypercoagulative disease may be occult in such patients and require the attention of a hematologist. When treating patients with EHPVO, gastroenterologists should screen for hematological disease, including MPN.

Extrahepatic portal vein obstruction (EHPVO) refers to obstruction of the extrahepatic portal vein that is characterized by cavernous transformation, portal hypertension, and intestinal dysfunction. Radiological interventions on EHPVO are an extraordinary challenge, although being reported to be safe and effective in selected patients by pertinent experts. Chronic intestinal dysfunction is a rare complication of EHPVO; it is unknown whether portal vein re-canalization by radiological interventions can improve chronic intestinal dysfunction. We describe a 22-year-old male patient with chronic intestinal dysfunction due to EHPVO, which was not improved by portal vein re-canalization. The patient presented with acute abdominal pain and dyspepsia for 2 weeks without hematochezia in August 2016 and was diagnosed with EHPVO. Due to cavernous transformation, systemic anticoagulation therapy was administered, and although his abdominal pain was relieved, the patient still had dyspepsia and partial jejunum dysfunction. Intestinal segmentectomy was suggested but was refused, and the patient received catheter-directed thrombolysis in another hospital. Although the portal vein was partly recanalized, the intestinal obstruction was not alleviated. Four months after onset, an emergent enterectomy was performed due to severe hematochezia with pathological examination findings of necrosis, ulcer, and granulation formation. Unfortunately, the patient developed a serious systemic infection, severe thrombocytopenia and disseminated intravascular coagulation, which was assumed to be caused by intestinal bacterial translocation and serious malnutrition. The infection was subsequently controlled. In conclusion, in patients with chronic intestinal dysfunction due to EHPVO, portal vein re-canalization may not improve intestinal function. Timely enterectomy may prevent intestinal bacterial translocation and serious malnutrition.

Objective To review the efficacy and safety of Rex shunts for patients with extrahepatic portal vein obstruction (EHPVO). Methods Ten children with extra-hepatic portal vein obstruction (EHPVO) operated between October 2008 and February 2010 were reviewed. There were 8boys and 2 girls. Their ages ranged from 19 to 144 months. Seven of the patients suffered from recurrence upper gastro-intestinal (GI) bleeding, and 3 from splenomegaly and hypersplenism. Upper GI radiography (UGIR) demonstrated esophageal and gastric varices (EGV). Ultrasonograph (US) also showed EHPVO. All patients underwent mesenteric-to-left portal vein bypass (MLPVB). Patients were followed up 4-20 (average: 9. 6 ±5.0 ) months. Results The mean duration of operation was 220 ± 14. 7 minutes (range: 200 to 240) without intraoperative complication. Operative blood loss was 10 to 50 ml with no need for blood transfusion. Portal pressure decreased after Rex shunt. The postoperative course was uneventful in 10 patients with hospital stay ranging from 8 to 15days. There was no further gastrointestinal bleeding. The spleen size dccreased,The platelet counts and white blood cell counts increased, and there was no postoperative complication during the followed-up period. Conclusions Rex shunt for patients with EHPVO is feasible, safe and effective. Key words: Portal vein; Portalcaval shunt, surgical; Child

Extrahepatic portal vein obstruction is the most common cause of portal hypertension in children. This study aimed to evaluate the causes, clinical, laboratory and endoscopic findings, treatment approaches, long-term results, and prognosis of extrahepatic portal vein obstruction in children. We retrospectively evaluated 82 patients who were followed up with the diagnosis of extrahepatic portal vein obstruction at Gazi University Pediatric Gastroenterology clinic between January 1, 2011, and October 31, 2021. Among our patients, 58.5% were male, median age at presentation was 36 months (range, 1 month to 6 years), and the follow-up period was 2 years (range, 1-14 years). The most common reasons for admission were gastrointestinal bleeding and splenomegaly. All patients diagnosed with incidentally detected obstruction were children under 5 years of age. The most frequently detected risk factors were history of umbilical vein catheterization (50%) and presence of procoagulant status (31%). Esophageal varices (94.8%), variceal bleeding (73.2%), hypersplenism (64.6%), and growth failure (13.4%) were the most common complications. Weight and/or height z-scores were lower in children with esophageal varices, variceal bleeding, and hypersplenism. Endoscopic treatments were more frequently applied to those with splenomegaly, thrombocytopenia, red color sign, and esophageal variceal bleeding. Rates of splenomegaly, hypersplenism, esophageal variceal bleeding, advanced varicose veins, and portal biliopathy were higher in patients who underwent portosystemic shunt surgery than in patients who did not undergo it. Morbidity and mortality rates of variceal bleeding in extrahepatic portal vein obstruction were lower than bleeding seen in cirrhotic prehepatic portal hypertension. In our study, no patients died from extrahepatic portal vein obstruction-related complications. One patient died from tuberculous meningitis.