Extrahepatic Portal Vein Obstruction Research Articles

Do Heavy Metals Have A Role In Extrahepatic Portal Vein Obstruction In Children: A Pilot case-control study

ObjectiveExtrahepatic portal venous obstruction (EHPVO) is a common cause of portal hypertension in Southeast Asia as compared to the western world, where chronic liver disease is the most common cause. Pollution and inadvertent exposure to heavy metals in poor socio-economic status in this part of the world are quite common. Therefore, this study was proposed to determine the association between heavy metal levels and oxidative stress in children with EHPVO. MethodsThis pilot case-control study was conducted in the department of Pediatrics, of a tertiary health care centre from January 2020 to October 2021. Children between 1-14 years, diagnosed with EHPVO were included. Controls were the healthy volunteers from OPD and near discharge indoor pediatrics patients. Metal analysis for Copper, Lead, Zinc, and Manganese, & Antioxidant activity for superoxide dismutase (SOD), Glutathione reductase (GR) and Lipid peroxidase (LPO) in form of malondialdehyde (MDA) was measured in both the groups. ResultsA total of 80 subjects were enrolled (40 EHPVO cases and 40 controls) mean age for cases was 8.15±3.53, and for control, was 7.69±3.55 years (p=0.560). Lead values were found to be significantly higher (p=0.003) in EHPVO cases (5.39±3.13) in comparison to controls (3.30±2.91). Mean superoxide dismutase (SOD) and Glutathione reductase (GR) values were decreased (p=0.035- GR) and the malondialdehyde (MDA) value was increased in the EHPVO cases as compared to controls. ConclusionChildren with high blood levels of lead might be at risk of portal vein thrombosis resulting in EHPVO, and lead-induced oxidative stress.

Abdominal surgery in patients with chronic noncirrhotic extra hepatic portal vein obstruction: A multicenter retrospective study.

In patients with noncirrhotic chronic extra-hepatic portal vein obstruction (EHPVO), data on morbimortality of abdominal surgery are scarce. We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the VALDIG network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complication within 1 month after surgery. Fifteen percent had ≥1 portal hypertension related complication within 3 months after surgery. Three patients died within 12 months after surgery. An unfavorable outcome (i.e. ≥1 above-mentioned complications or death) occurred in 37% of the patients and was associated with a history of ascites and with non-wall, non-cholecystectomy surgical intervention: 17% of the patients with none of these features had an unfavorable outcome, versus 48% and 100% when one or both features were present, respectively. We then compared 63/76 EHPVO patients with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, incidence of major bleeding (p<0.001) and portal-hypertension related complication (p<0.001) was significantly higher in patients with EHPVO, but not that of grade ≥3 postoperative complication nor of death. The incidence of unfavorable post-operative outcome was significantly higher in patients with EHPVO than in those without (33% vs. 18%, p=0.01). Patients with EHPVO are at high-risk of major peri- or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.

Extrahepatic portal vein obstruction in children of the first year of life: diagnostic and treatment approaches

Extrahepatic portal vein obstruction in children of the first year of life: diagnostic and treatment approaches

A clinico-etiological profile of adult extrahepatic portal venous obstruction in patients from Northern India. Is it a novel subset of occult extrahepatic portal vein obstruction?

Background: Extrahepatic portal vein obstruction (EHPVO) is caused by occlusion and cavernomatous transformation of the portal vein. The EHPVO is well characterized in children. However, the same is not valid for adults. We aimed to evaluate the clinico-etiological profile along with the management of adult patients. Aims and Objectives: We evaluated the clinico-etiological profile along with the management and outcomes of adult patients presenting with EHPVO. Materials and Methods: In a descriptive, observational study, patients between ages 15 and 75 years diagnosed with EHPVO on ultrasonography abdomen were included in the study. Liver cirrhosis and malignancy were considered as an exclusion criterion. There was evaluation of clinical and imaging findings along with biochemical analysis, workup for thrombophilia, treatment given to the patients, and follow-up. Results: Out of total 109 patients enrolled in the study, the median age of patients was ±40.25. Main clinical features were dyspepsia, abdominal discomfort, and splenomegaly. Out of 109, 27.5% patients showed fatty liver and 5.5% had both splanchnic vein thrombosis and fatty liver. Idiopathic EHPVO in majority of patients (80%), adult EHPVO with polycythemia rubra vera in 7.33% patients, adult EHPVO with heterozygous MTHFR mutation in 7.33% patients, and adult EHPVO with antiphospholipid antibody in 3.66%. No mortality was seen in this 5-year observational study and majority of the patients, i.e., 96 (88.1%) did not require any treatment. Conclusion: Relatively benign nature of adult EHPVO was found in the selected group of patients. Majority of the cases in our study were idiopathic and few had positive thrombophilia profile.

Clinical features of extrahepatic portal vein obstruction: Myeloproliferative neoplasms eliminate hypersplenic hematologic changes in extrahepatic portal vein obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease. Most EHPVO patients are usually referred to a gastroenterologist for intestinal bleeding and hypersplenic thrombocytopenia; however, hypercoagulative diseases may be occult in these patients and require anticoagulation. The purpose of this study was to elucidate the clinical characteristics of EHPVO. We conducted a retrospective analysis of the hospital database, evaluating the medical records of 15 patients (7 males, 8 females, mean age of onset 42.0 years, range 5-74 years). Thirteen of 15 EHPVO patients (86.7%) had intestinal varices. These included 10 esophageal (66.7%), 12 gastric (80.0%), and 6 ectopic varices (40.0%). Nine (60.0%) of 15 had a history of intestinal bleeding. Regarding comorbidities, 5 of 15 (33.3%) suffered from vascular diseases, including acute myocardial infarction, cerebral infarction, pulmonary embolism, Budd-Chiari syndrome, and mesenteric vein thrombosis. The former 3 vascular commodities manifested at less than 32 years of age. Four patients (26.7%) with JAK2V617F mutation were diagnosed as myeloproliferative neoplasm (MPN). 72.3% of EHPVO patients without MPN experienced thrombocytopenic state. No EHPVO patients with MPN experienced thrombo-leukocytopenia. The elevation of white blood cell and platelet counts, and decrease of protein S were seen in EHPVO with MPN, compared with EHPVO without MPN. EHPVO is frequently associated with underlying hypercoagulative factors, causing a dilemma between thrombotic complications and portal hypertensive bleeding. Most EHPVO patients experience an evident thrombocytopenic state due to severe hypersplenism; however, hypersplenic hematologic changes are eliminated in EHPVO with MPN. MPN should be suspected in EHPVO patients negative for thrombo-leukocytopenia.

Safety and effectiveness of SARS-CoV-2 vaccines for patients with intractable hepatobiliary diseases: A multicenter, questionnaire-based, cross-sectional study.

There are few data regarding the safety and effectiveness of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines in patients with intractable hepatobiliary diseases. We conducted a multicenter, questionnaire-based, cross-sectional study to determine the safety and effectiveness of the SARS-CoV-2 vaccines in Japanese patients with intractable hepatobiliary disease. Patients aged ≥18years with autoimmune hepatitis (AIH), primary biliary cholangitis, primary sclerosing cholangitis, Budd-Chiari syndrome, idiopathic portal hypertension, and extrahepatic portal vein obstruction at each center were consecutively invited to join the study. Participants were asked to complete a questionnaire regarding their characteristics, vaccination status, post-vaccination adverse effects, and SARS-CoV-2 infection. Additionally, liver disease status, treatment regimens, and liver function test values pre- and post-vaccination were collected. The survey was conducted from September 2021 to May 2022, and 528 patients (220 AIH, 251 primary biliary cholangitis, 6 AIH- primary biliary cholangitis/primary sclerosing cholangitis overlap, 39 primary sclerosing cholangitis, 4 Budd-Chiari syndrome, 5 idiopathic portal hypertension, and 3 extrahepatic portal vein obstruction) participated in the study. Post-vaccination adverse effects were comparable to those observed in the general population. Post-vaccination liver injuries classified as grade 1 or higher were observed in 83 cases (16%), whereas grades 2 and 3 were observed in only six cases (1.1%); AIH-like liver injury requiring treatment was not observed. Overall, 12 patients (2.3%) were infected with SARS-CoV-2, and only one patient was infected 6months after the second vaccination. SARS-CoV-2 vaccines demonstrated satisfactory safety and effectiveness in Japanese patients with intractable hepatobiliary diseases.

Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Lieno-Adrenal Shunt a Rescue for Porto-Systemic Decompression in 23-Year-Old Female

Extra hepatic portal vein obstruction is one of the commonest cause of portal hypertension and upper gastrointestinal bleed in the third world. Portal vein cavernous transformation is one of the hallmarks of EHPVO. Despite various lesser invasive interventional approaches for management of EHPVO, surgical shunts still play an important role in long term treatment of patients with EHPVO. Here we present a case of 23 years old female who presented to the OPD with complains of dull aching abdominal pain for a year which was insidious on onset and gradually progressive. The pain was non-radiating, localized to the left upper quadrant of the abdomen which aggravated on lying on the left side. She underwent lieno-adrenal shunt for porto-systemic decompression and management of EHPVO. This case aims to highlight the use of leino-adrenal shunt as a feasible alternative to Linton’s shunt.

The role of variceal bleeding primary prophylaxis in the management of extrahepatic portal vein obstruction in children

Extrahepatic portal vein obstruction is a major cause of pediatric symptomatic portal hypertension and can lead to profuse variceal hemorrhaging. Purpose - to evaluate the role of primary prophylaxis of the variceal bleeding in children with extrahepatic portal vein obstruction. Materials and methods. A one-center prospective cohort clinical study involved 120 patients with extrahepatic portal vein obstruction who underwent primary or secondary variceal bleeding prophylaxis in 2016-2021. The laboratory, ultrasound, and endoscopy data before and after treatment were collected and evaluated. Results. Variceal bleeding episodes occurred in 5.8% (n=3) of patients who underwent primary prophylaxis and rebleeding occurred in 27.9% (n=19) of those who underwent secondary prophylaxis. Bleeding episodes occurred less frequently in patients from primary prophylaxis group (p=0.013). In patients treated endoscopically (n=53, 44.17%) Variceal bleeding appeared less frequently (n=3, 5.66%) after treatment compared to patients who underwent surgery (n=67 (55.83%), 19 had rebleeding (28.35%)) (p=0.001). In 69.17% patients (n=83) esophageal varices eradication was achieved: in 53.01% (n=44) patients from the primary prophylaxis group and 46.99% (n=39) from the secondary prophylaxis group. Bleeding episodes occurred less frequently after the eradication achievement (p&lt;0.001). The primary prophylaxis led to varices eradication more often than secondary (p=0.003). The varices recurrence episodes rates after the eradication achievement were not different between groups (p=0.51). Conclusions. Primary prophylaxis can reduce the bleeding risk in extrahepatic portal vein obstruction with high risk of variceal bleeding. The prophylaxis by endoscopic variceal banding is an important bridge in the treatment for pediatric patients with portal hypertension that along with portosystemic shunting could potentially improve prophylactic treatment results. The Committee on Clinical Investigation of Bogomolets National Medical University approved this study (Protocol No.141, 27.01.2021). All the studies were conducted according to implemented guidelines in consideration of GCP-ICH and Declaration of Helsinki. The written informed consent of all participants’ parents/guardians was achieved. No conflict of interests was declared by the authors.

A CLINICAL CASE OF PORTAL BILIOPATHY

Background. The term «portal biliopathy» (PB) refers to abnormalities of the entire biliary tract including intrahepatic and extrahepatic bile ducts, the cystic duct as well as the gallbladder in patients with portal hypertension. Objective. To describe a clinical case of portal biliopathy. Material and methods. Clinical observation of a patient aged 42, who was examined and treated at the 2nd City Clinical Hospital, Minsk, Belarus in February 2023. Results. Esophagogastroduodenoscopy and ultrasonography revealed portal hypertension, grade 2-3 esophageal varices and changes in the bile ducts. The subsequent use of computed tomography, magnetic resonance imaging, endoscopic retrograde cholangiopancreatography made it possible to identify cavernous transformation of portal vein due to occlusive thrombosis as well as portal biliopathy with extensive stenosis of the ductus choledochus. Conclusions. The presented clinical case demonstrates the asymptomatic course of a severe pathology known as extrahepatic portal vein obstruction (EHPVO) with the development of portal hypertension and portal biliopathy.

Navigating portal hypertension: Unlocking safe passage to healthy pregnancy in EHPVO.

Pregnancy is associated with hyperdynamic circulatory state and increased risk of portal hypertension related complications in patients with extra-hepatic portal vein obstruction (EHPVO). We aim to study the impact of EHPVO on pregnancy-related outcomes with focus on subset of patients with UGIB (upper GI bleed). Retrospective analysis of obstetric, maternal and neonatal outcomes of patients with EHPVO registered between January 2006 and December 2022. Forty-five patients were included. Forty-five healthy females with low-risk pregnancies formed the control group. Adverse obstetric and neonatal outcomes were comparable between EHPVO and control group (22% vs. 28.6%; p > .05; low birth weight/ small for gestational age 17.8% vs. 36%, p = .0918 and 14.2% vs. 10%, p = .5698 respectively). Adverse outcomes were similar in patients with and without history of UGIB (26.3% vs. 19.4%, p = .0814; 17.8% vs. 36%, p = .0918; 14.2% vs. 10%, p = .5698). There was no maternal mortality in both the groups. A total of 7% pregnancies in EHPVO patients were complicated by ascites. EHPVO pregnancies have successful obstetric and neonatal outcomes with adequate management of portal hypertension.

Ectopic Variceal Bleeding from the Hepaticojejunostomy due to Extrahepatic Portal Vein Occlusion: How to Treat?

Atypical variceal bleeding, which primarily stems from extrahepatic portal vein obstruction (EHPVO), is a severe complication of pancreatic hepatobiliary surgery. This review provides insights into this condition's incidence, diagnosis, and management strategies. The treatment modalities for atypical variceal bleeding resulting from EHPVO range from endoscopic intervention to surgical procedures, including direct variceal ligation and shunt surgery. Here, we discuss the efficacy and potential limitations of each treatment approach. Additionally, we explored the utility and therapeutic advantages of the meso-Rex shunt, a particularly promising surgical technique for mitigating the hemodynamic and metabolic impacts of EHPVO.

Feasibility of laparoscopic cholecystectomy for symptomatic gallstone disease with portal cavernoma: Can prior portal vein decompression be avoided?

Biliary surgery in patients with extrahepatic portal vein obstruction with portal cavernoma (PC) is technically challenging, and associated with the risk of bleeding. Therefore, prior portal vein decompression is usually recommended before definitive biliary surgery. Only a few studies have so far reported the safety of isolated laparoscopic cholecystectomy. We aimed to evaluate our experience of laparoscopic cholecystectomy in patients with PC without prior portal decompression. Prospectively maintained data for patients with PC who underwent laparoscopic cholecystectomy for symptomatic gallstone disease without portal decompression were analyzed. Clinical features, imaging, intraoperative factors, conversion rate, complications of surgery, and long-term outcomes were assessed. Sixteen patients underwent cholecystectomy without portal decompression from 2012 to 2021, of which interventions 14 were laparoscopic cholecystectomies. One patient required conversion (7.1%) to open surgery. Jaundice was present in 5 patients (35.7%), and underwent endoscopic stone clearance before surgery. Median intraoperative blood loss, operative time, and hospital stay were 100 mL (20-400 mL), 105 min (60-220 min), and 2 days (1-7 days), respectively. Blood transfusion was required in two patients (14.2%). Prior endoscopic or percutaneous intervention was associated with significant blood loss and prolonged intraoperative time. In centers with experience, prior portal decompression can be avoided in patients with PC requiring isolated cholecystectomy to treat gallstones or their complications. Laparoscopic surgery is safe and feasible for these patients, and gives excellent outcomes in the selected group.

An Audit of Extrahepatic Portal Vein Obstruction: Experience from Tertiary Referral Center.

Extrahepatic portal vein obstruction (EHPVO) is a common cause of portal hypertension in India. (1) To evaluate the clinical presentation and the natural history of EHPVO; (2) to describe the risk factors, rebleeding rates and development of portal biliopathy on follow-up; and requirement of surgery in EHPVO at a tertiary care center. Data from 318 consecutive patients with EHPVO from June 2012 to October 2020 were analyzed. All patients underwent liver biochemistry, ultrasonography (USG) abdomen, upper gastrointestinal (GI) endoscopy, and viral serology. Color Doppler, computed tomography (CT) abdomen and magnetic resonance cholangiopancreatography (MRCP) were done as indicated. Mean age of presentation was 15.08 years [standard deviation (SD) 12.74; 6 months-60 years; 210 males)]. The presenting features were upper GI bleed (n = 227) (age at first bleed 11 years; 4 months-56 years), left hypochondrium pain or lump (n = 67), and only lower GI bleed (n = 1). Incidentally detected EHPVO on USG was seen in 10.69% (n = 34) patients. Postbleed ascites were seen in 10.69% (n = 34) patients. Six patients had symptomatic portal biliopathy and 14 had hypersplenism. Around 14.77% (n = 47) of patients had a history of being delivered at home, while 3.45% (n = 11) had a history of umbilical sepsis. During follow-up, 35.3% (n = 82) of patients had rebled. On imaging, associated splenic vein (SV) collaterals and superior mesenteric vein (SMV) collaterals were seen in 35.84% (n = 114) and 11.01% (n = 35) patients, respectively. Gallbladder varices were seen in 44.3% (n = 106), while gallstones in 5.66% (n = 18). On endoscopy, 87.42% (n = 278) patients had esophageal varices, 18.86% (n = 60) had isolated fundic varices, and three had ectopic varices. Only two patients had rectal varices and colopathy. Emergency devascularization was required in 3.45% (n = 14) patients for the failure of variceal bleed control, 1.88% (n = 7) underwent splenectomy, and four patients had proximal splenorenal shunt (PSRS) surgery. Extrahepatic portal hypertension (EHPVO) is an important cause of portal hypertension (PHT) in our country. The majority of them present with GI bleed; postbleed ascites were seen only in ~10%. Rebleed occurs in one-third of cases. Gallbladder varices were common; portal biliopathy occurred in 10% and were usually asymptomatic.

PREDICTIVE FACTORS OF MORBIDITY ASSOCIATED WITH ESOPHAGEAL VARICEAL BLEEDING IN CHILDREN WITH PORTAL HYPERTENSION.

•Most data on the natural history of portal hypertension come from studies in adults. •The morbidity rate of upper gastrointestinal bleeding in children with portal hypertension tend to be underestimated. •This study showed the relevance of morbidity rates after variceal hemorrhage in pediatric patients, especially those with cirrhosis. •Patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications secondary to upper gastrointestinal bleeding and should be closely monitored. Background - Most data on the natural history of portal hypertension come from studies in adults. The morbidity rate of upper gastrointestinal bleeding (UGIB) in children with portal hypertension has not been systematically characterized. Objective - To describe the morbidity and mortality of UGIB in pediatric patients with portal hypertension and identify predictive factors for the occurrence of its main complications. Methods - This retrospective study included pediatric patients with cirrhotic portal hypertension or with extrahepatic portal vein obstruction (EHPVO). Mortality and UGIB complications within a period of up to 6 weeks of the bleeding were investigated. To determine the predictive factors of morbidity, a multivariate analysis was performed using logistic regression; all results were considered significant at P<0.05. Results - A total of 86 patients (51.2% with EHPVO and 48.8% with cirrhosis) had 174 bleeding events. Ascites was the most common complication (43.1% of all cases), being more prevalent in patients with cirrhosis (P<0.001). Cirrhosis was a predictor of the occurrence of any morbidity (OR 20.3). The need for blood transfusion was predictor of at least one complication (OR 5.8), ascites (OR 7.2) and infections (OR 3.8) in the general group and at least one complication (OR 11.3) and ascites (OR 5.8) in cirrhotic patients. The need for expansion was a predictor of any morbidity (OR 4.6) and infections (OR 3.9) in the general group, in addition to being predictor of infection in cirrhotic patients (OR 5.4). There were no deaths from UGIB in the six weeks post-bleeding. Conclusion - The study showed the relevance of morbidity after UGIB in pediatric patients with portal hypertension, especially in those with cirrhosis. The patients with hemodynamic instability requiring blood transfusion or expansion on admission are at increased risk of complications related to upper gastrointestinal bleeding and should be closely monitored.

THU-305 - Impact of extrahepatic portal vein obstruction on fertility and pregnancy outcomes- a tertiary center experience

THU-305 - Impact of extrahepatic portal vein obstruction on fertility and pregnancy outcomes- a tertiary center experience

Extra-hepatic portal vein obstruction leading to portal cavernoma cholangiopathy without liver dysfunction

In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. EHPVO leads to the cavernous transformation of the portal vein in the absence of recanalization. Portal cholangiopathy (also referred to as portal biliopathy) is common in patients with long-standing chronic PVT. It is due to compression of the large bile ducts by the venous collaterals (portal cavernoma) that form in patients with chronic PVT. Typically, symptoms of portal cholangiopathy include jaundice, biliary colic and pruritus.

Surgical management of extrahepatic portal vein obstruction in children: advantages of MesoRex shunt compared with distal splenorenal shunt

PurposeTo review surgical management of extrahepatic portal vein obstruction (EHPVO) at Red Cross War Memorial Children’s Hospital and compare MesoRex shunt (MRS) with distal splenorenal shunt (DSRS).MethodsThis is a single-centre retrospective review documenting pre- and post-operative data in 21 children. Twenty-two shunts were performed, 15 MRS and 7 DSRS, over an 18-year period. Patients were followed up for a mean of 11 years (range 2–18). Data analysis included demographics, albumin, prothrombin time (PT), partial thromboplastin time (PTT), International normalised ratio (INR), fibrinogen, total bilirubin, liver enzymes and platelets before the operation and 2 years after shunt surgery.ResultsOne MRS thrombosed immediately post-surgery and the child was salvaged with DSRS. Variceal bleeding was controlled in both groups. Significant improvements were seen amongst MRS cohort in serum albumin, PT, PTT, and platelets and there was a mild improvement in serum fibrinogen. The DSRS cohort showed only a significant improvement in the platelet count. Neonatal umbilic vein catheterization (UVC) was a major risk for Rex vein obliteration.ConclusionIn EHPVO, MRS is superior to DSRS and improves liver synthetic function. DSRS does control variceal bleeding but should only be considered when MRS is not technically feasible or as a salvage procedure when MRS fails.

Anomalies and malformations of visceral arteries in children with extrahepatic portal vein obstruction

Purpose. To study the incidence of anomalies and malformations of visceral arteries and evaluate their role in the treatment of extrahepatic portal vein obstruction (EHPVO) in childrenMaterial and Methods. The study included 155 previously not operated children with EHPVO. A retrospective analysis of the results of three-phase multislice computed tomography with angiography (MSCTA) of visceral vessels was performed. Frequency of visceral arteries anomalies of the abdominal aorta, aortomesenteric angle, the structure of the hepatic, splenic and superior mesenteric arteries (SMA) were assessed. The size of visceral arteries depending on the prevalence of portal vein thrombosis was also assessed.Results. According to analysis 35 (22.6%) children with EHPVO had abnormalities of visceral arteries. Of them, 14 (11.7%) patients presented with signs of aortomesenteric compression of the left renal vein (LRV). Coeliacomesenteric trunk was detected in 4 (2.5%) children. In one (0.64%) case there was sign of saccular splenic artery (SA) aneurysm. According to data, there was a significant correlation of the diameter of SA and SMA with the age of children (r = 0.58; p = 0.001). According to the study, diameter of arteria hepatica propria in children with portal vein thrombosis (PVT) with extension into superior mesenteric vein (SMV) was significantly lower than in PVT and its branches (2.48 ± 0.23 mm vs 3.15 ± 0.08 mm, p = 0.01). The diameter of SA in children with PVT extended into splenic vein was significantly smaller (4.26 ± 0.19 mm) in comparison to children with PVT and its branches (4.9 ± 0.12 mm, p = 0.01), and children with widespread thrombosis of portal vein system (5.45 ± 0.41 mm, p = 0.05).Conclusion. Multiple aberrations of the anatomical structures of visceral arteries make any hepatobiliary surgery challenging to surgeons. The study data suggest that MSCTA is an informative method for evaluation of associated anomalies of visceral arteries in children with EHPH.

Angiographic patterns of portal venous system in children with extrahepatic portal hypertension and its etiological and clinical relevance.

To study the relationship between the angiographic pattern of extrahepatic portal vein obstruction (EHPVO) and its etiology and clinical manifestations. Clinical, etiological, and angiographic findings in 155 children with EHPVO were reviewed. Anatomy of extrahepatic portal venous system (EPVS) was categorized into five imaging patterns. Assessment of the severity of esophageal and gastric varices (EV and GV) was performed by upper gastrointestinal endoscopy. Based on multislice CT angiography, most commonly observed pattern of EHPVO was type I (48.4%) and type II (29%). According to anamnesis, 68 (43.8%) children had pathological conditions in neonatal period. Of these, 35 (22.6%) had an umbilical vein catheterization, 11 (7.1%) had a history of omphalitis, and 9 (5.8%) had prolonged jaundice. Thirteen (8.4%) patients had various septic conditions in neonatal period and it was more common associated with widespread thrombosis throughout the EPVS (type 5)-28% of observations. Significantly lower risk of bleeding from EV (p = 0.01) was noted in children with type IV pattern, whereas children with type III and V patterns had higher grades of EV. Angiographic pattern of portomesenteric occlusion may provide a clue to its etiology, and clinical manifestation, especially in children with widespread thrombosis throughout the EPVS.