Extrahepatic Portal Vein Research Articles

A case report: mucinous carcinoma of extrahepatic bile ducts with periportal calcification suggestive of lymphatic calcification.

Mucinous carcinoma is a rare subtype of extrahepatic bile duct (EHBD) cancer in which abundant mucin surrounds cancer cell nests. Calcification is often observed in mucus-producing tumors, and we herein report a case of mucinous carcinoma of EHBD with diffuse periportal calcifications. A 92-year-old woman was seen for jaundice. Non-contrast CT showed diffuse semi-linear branching calcifications extending from the hepatoduodenal ligament along the long axis of the intra- and extrahepatic portal veins. A bile duct mass and multiple lymph nodes were hyperintense on T2-weighted MRI images, and periportal abnormal intensity was visible along the intrahepatic portal tract. On contrast-enhanced CT, the mass and enlarged lymph nodes showed a weak enhancing effect. In addition, a lesion with a similar enhancing effect was observed in parallel with periportal calcification, raising suspicion of tumor extension. An endoscopic bile duct biopsy was performed, which resulted in the tumor being diagnosed as a mucinous carcinoma. Considering the imaging findings, it was determined to be a mucinous carcinoma of EHBD origin, with lymphatic vessel infiltration and multiple lymph node metastases. The lymphatic tracts presented lymphangitic carcinomatosis extending from the hepatoduodenal ligament to intrahepatic portal tracts, and the diffuse periportal calcification was presumed to be calcification associated with the mucin component of tumor infiltration.

Current approaches to the management of jejunal variceal bleeding at the site of hepaticojejunostomy after pancreaticoduodenectomy.

Jejunal variceal bleeding at the site of hepaticojejunostomy after pancreaticoduodenectomy due to portal hypertension caused by extrahepatic portal vein obstruction is a life-threatening complication and is very difficult to treat. Pharmacotherapy, endoscopic methods, transcatheter embolization of veins supplying the jejunal afferent loop, portal venous stenting, and surgical procedures can be used for the treatment of jejunal variceal bleeding. Nevertheless, the optimal management strategy has not yet been established, which is due to the lack of randomized controlled trials involving a large cohort of patients necessary for their development.

Extrahepatic Portal Vein Obstruction in Children.

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.

Effectiveness of Preoperative Intrahepatic Portal Venous Classification System in Guiding Preoperative Surgical Decisions and Predicting Hypotensive Effects After Meso-rex Bypass for Children With EHPVO

BackgroundThe categorization of intrahepatic portal venous system (IHPS) patterns using wedged hepatic venous portography (WHVP) has proven to be an effective tool in the preoperative evaluation of Rex recessus and in identifying pediatric patients with extrahepatic portal vein obstruction (EHPVO) who are suitable for meso-Rex bypass (MRB). Despite this classification system being proposed a decade ago, its clinical application remains underutilized. MethodsA single-center retrospective study of 182 children with EHPVO was conducted between October 2014 and July 2023 when MRB was attempted. Data on demographics, etiology, imaging examinations, procedures, and follow-up were collected for 161 patients included. Two interventional radiologists used deVille’s method to classify patients into types A to E based on WHVP imaging, with interobserver agreement analyzed. Associations between IHPS patterns and surgical outcomes following MRB were investigated. ResultsTwo radiologists had a high level of agreement on identifying IHPS patterns and suitable patients for MRB. Of the 161 cases, 130 were type A, 10 were type B, 5 were type C, 7 were type D, and 9 were type E. One hundred and forty-five patients with types A, B and C underwent successful MRB, showing feasibility for 90% of patients. Children categorized as types A and B experienced more significant benefits than type C, including intraoperatively decreased portal vein pressure, esophageal/gastric varices relief, decreased portal venous collaterals and a lower rate of bypass occlusion after one year. The surgical outcomes of patients with types A and B were not influenced by the diameter of the Rex recessus as suggested by WHVP. ConclusionsThe majority of pediatric patients with EHPVO in mainland China have opportunities to receive successful MRB. There are potential differences in the etiology of Chinese and Western patients. The IHPS classification system aids in guiding preoperative surgical decisions and predicting hypotensive effects after MRB. Type C patients should be carefully chosen for MRB.

Handheld ultrasound for portal vein evaluation in pediatric patients.

To assess diagnostic capabilities of a modern handheld ultrasound device (HUD) for portal vein (PV) evaluation in pediatric patients and to verify if age, body mass index (BMI) and object depth correlate to imagequality. 45 patients (3.9 months- 17.9 years; 10.3 years±5.4) were examined using a HUD and cart-based high-end ultrasound system (HEUS). The intra-and extrahepatic PV was scanned using B-Mode and Color-coded Doppler sonography (CCDS). A five-point Likert scale was applied to evaluate image quality. Results were interpreted by two readers in consensus. Scores were compared between the scanners and correlated to age, BMI and skin-to-portal-vein-distance (SPVD). The influence on image quality and the difference between the two devices were analyzed using ordinal and Bayesian logistic regression models. ≥4 points (mild or no limitations) were achieved by the HUD in 60% for B-Mode and 56% for CCDS. There was a statistically significant negative correlation between the image quality achieved by the HUD and the SPVD for B-Mode and CCDS. The effect of BMI and SPVD on having≥4 points differed significantly between the HUD and HEUS for B-Mode (OR = 0.191, p = 0.005, and OR = 0.040, p = 0.008) and for CCDS (OR = 0.209, p = 0.009, and OR = 0.084,p = 0.011). Sufficient to excellent image quality for portal vein assessment in pediatric patients was achieved by the HUD except for the most deep-lying structures. Compared to HEUS, diagnostic performance of the HUD is lower, based on a negative correlation with object depth.

Medium-to Long-term Outcomes of Rex Shunt in 105 Children With Extrahepatic Portal Vein Obstruction in China

PurposeThe aim of our study was to analyze the medium-to long-term outcomes of Rex shunts in a large series of children with extrahepatic portal vein obstruction (EHPVO). MethodsThe clinical data of 105 children aged between 6 months and 16 years with EHPVO who underwent Rex shunt between October 2014 and June 2021 at our center were retrospectively reviewed after more than 2 years of follow-up. ResultsThe overall patency rate of the Rex shunt was 91.43% (96/105) during a median follow-up of 41 months (range, 24–98 months). Eighty-seven (82.86%) of the 105 patients underwent classical Rex shunt with internal jugular vein (IJV) bypass, and the remaining 18 patients (17.14%) underwent modified Rex shunt with intra-abdominal vein bypass. Patients with a patent shunt experienced portal hypertension resolution, which was characterized by a reduction in portal pressure, disappearance of variceal bleeding, relief of gastroesophageal varices, and relief of splenomegaly or hypersplenism. The rate of Rex shunt thrombosis in our center was 8.57% (9/105), and a repeat Rex shunt was effective for the treatment of graft thrombosis. Anastomotic stenosis occurred in 14.26% (15/105) of the children, 38.46% (5/13) of whom received successful endovascular intervention therapy and experienced remission of portal hypertension symptoms. The patency rate of the classical Rex shunt was higher than that of the modified Rex shunt (97.70% vs. 61.11%), whereas the rate of vascular complications, including anastomotic stenosis and graft thrombosis, of the classical Rex shunt was lower than that of the modified Rex shunt (11.49% vs. 77.78%). Further comparison revealed that the risk of vascular complications was substantially greater in the modified Rex shunt group than in the classical Rex shunt group in the nonadjusted model, minimally adjusted model, and fully adjusted model (RR ranged from 6.77 to 7.07, all p < 0.001). ConclusionsThe Rex shunt provides medium-to long-term benefits for children with EHPVO. The classical Rex shunt with IJV bypass provides the best patency rate and the fewest vascular complications. Levels of EvidenceⅢ Type of StudyRetrospective comparative study.

Splenectomy unveils thrombocytosis in underlying myeloproliferative neoplasms with extrahepatic portal vein obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a rare disease with myeloproliferative neoplasm (MPN) as the most common cause. We report that hypersplenic hematologic changes in EHPVO might be eliminated by MPN. Through experience with splenectomy for variceal control with EHPVO, we suspected that spleen might mask MPN-induced thrombocytosis, and that MPN might have a significant influence on excessive thrombocytosis after splenectomy. To clarify the influence of MPN and spleen on platelet trends, we conducted a retrospective hospital database analysis, evaluating 8 EHPVO patients with splenectomy (2 males, 6 females; from 17 years to 64 years, mean 38.3 years). Three (37.5%) of 8 were diagnosed as MPN by JAK2V617F mutation. The perioperative serum platelet counts in EHPVO without MPN were 10.5, 35.4, and 36.6 (x104/μL) preoperatively, after 1 week and 3 weeks, respectively. The platelet counts in EHPVO with MPN were 34.2, 86.4, and 137.0 (x104/μL), respectively. Splenectomy and MPN showed positive interaction on platelet increasing with statistical significance. We also examined the spleen volume index (SpVI: splenic volume (cm3) / body surface area (m2) and postoperative platelet elevations ratio (PER: 3-week postoperative platelet counts / preoperative platelet counts). However, both SpVI and PER showed no significant difference with or without MPN. Histological examination revealed splenic congestion in all 8 EHPVO cases, and splenic extramedullary hematopoiesis in 2 of 3 MPN. In EHPVO with MPN, hypersplenism causes feigned normalization of platelet count by masking MPN-induced thrombocytosis; however, splenectomy unveils postoperative thrombocytosis. Spleen in EHPVO with MPN also participates in extramedullary hematopoiesis.

Portal vein stenting blocked the inflow tract and completely resolved bile duct varices, formed by cavernous transformation of the portal vein.

There is no established treatment for bleeding bile duct varices (BDVs). We report the first case of portal vein (PV) stenting completely eradicating bleeding BDVs. A 70-year-old male with malignant lymphoma developed BDVs due to PV obstruction, which had caused compression and stricture of the distal bile duct. Endoscopic retrograde cholangiography was performed to evaluate the stricture and bleeding from the ruptured BDV was observed. Endoscopic hemostasis was difficult, requiring reopening of the extra-hepatic PV and reducing the blood flow to the BDVs for hemostasis. Therefore, PV stenting was performed. During the procedure, portal angiography confirmed an inflow tract to the BDVs. Therefore, covered stents were placed in the PV and adjusted to block the inflow tract to the BDVs at the distal end. After stenting, the BDVs were successfully blocked and all PV blood flowed through the stent placed in the extra-hepatic PV. Two weeks after stenting, the BDVs had disappeared completely and the bleeding has not recurred for months. We experienced a case in which PV stenting not only reopened an obstructed PV but also successfully occluded the inflow tract. This case demonstrates the potential of PV stenting for the treatment of hemorrhagic BDVs.

Beyond the Rex: an innovative meso-intrahepatic portal vein bypass for late-onset extrahepatic portal vein occlusion after living donor liver transplantation.

The Meso-Rex bypass (MRB) is recognized as an effective treatment for portal hypertension secondary to extrahepatic portal vein occlusion (EHPVO) both in the pediatric and adult population, within or outside the context of liver transplantation. It is the preferred surgical treatment in most centers because not only does it addresses the portal hypertension, but also restores physiologic portal hepatopetal flow. However, the Rex recess, the landmark for this technique, may not be safely accessible in some patients. We present a 22-year-old male who underwent living donor liver transplant (LDLT) for neonatal hepatitis. He presented with variceal bleeding due to EHPVO at 13 years after transplant. Various endoscopic, radiologic, and surgical interventions were employed to address the recurrent gastrointestinal bleeding, but results have been unsatisfactory. We performed a meso-intrahepatic portal vein bypass (MIPVB), an innovative alternative to the MRB, for this patient with extensive post-operative adhesions, perihilar collaterals, and cavernous transformation. MIPVB creation in patients where the Rex recess is inaccessible is technically challenging. But with a multidisciplinary team approach, meticulous preoperative planning, and close follow-up, the authors have demonstrated that it is a safe and feasible option for patients with late-onset EHPVO after liver transplantation.

Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction.

Meso-Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%. We aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency. Transjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A-E), and factors associated with patency of Rex vein were studied. The patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso-Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under-5-year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65-90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity. Less than one-third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.

Surgical management of extrahepatic portal vein obstruction at University Hospital

Surgical management of extrahepatic portal vein obstruction at University Hospital

Embolization with or without portal vein stenting for bleeding ectopic jejunal varices in hepatopetal portal collaterals due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery

PurposeTo evaluate the efficacy and safety of embolization with or without portal vein stenting for bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery.Materials and methodsThis study included consecutive patients who underwent embolization for bleeding ectopic jejunal varices in the hepatopetal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery between September 2012 and December 2020. The safety, technical and clinical success rates (no re-bleeding within 1 month) and re-bleeding-free survival after the first therapy and overall survival were assessed.ResultsFourteen sessions in 11 patients were included. Four patients (7 sessions) underwent variceal embolization only, and the remaining seven patients (7 sessions) underwent portal vein stenting and variceal embolization. Technical success was achieved in all 14 sessions (100%). Clinical success was achieved in 13 of 14 sessions (92.9%). No treatment-related serious complications including liver failure were observed. One-year and 2-year re-bleeding-free survival rate after the first endovascular therapy in all 11 patients was 90.9 and 60.6%, respectively. Two patients who experienced re-bleeding had repeat embolization treatment. There was no significant difference in re-bleeding-free survival after endovascular therapy between the combination with stenting and embolization group and the embolization-only group (p = 0.13).ConclusionEmbolization with or without portal vein stenting of bleeding ectopic jejunal varices in the hepatopetal portal collateral due to extrahepatic portal vein occlusion or stenosis after hepatobiliary and pancreatic surgery can be considered a safe, effective, and repeatable therapy for long-term hemostasis of uncontrollable bleeding.

WED-129 Abdominal surgery in patients with chronic noncirrhotic extra hepatic portal vein obstruction: a multicenter retrospective case-control study

WED-129 Abdominal surgery in patients with chronic noncirrhotic extra hepatic portal vein obstruction: a multicenter retrospective case-control study

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 and 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 extrahepatic portal vein obstruction: A multicenter retrospective study.

In patients with noncirrhotic chronic extrahepatic portal vein obstruction (EHPVO), data on the morbimortality of abdominal surgery are scarce. We retrospectively analyzed the charts of 76 patients (78 interventions) with EHPVO undergoing abdominal surgery within the Vascular Disease Interest Group network. Fourteen percent of the patients had ≥1 major bleeding (unrelated to portal hypertension) and 21% had ≥1 Dindo-Clavien grade ≥3 postoperative complications 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 (ie, ≥1 abovementioned complication or death) occurred in 37% of the patients and was associated with a history of ascites and with nonwall, noncholecystectomy 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 patients with EHPVO with 126 matched (2:1) control patients without EHPVO but with similar surgical interventions. As compared with control patients, the 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 complications nor of death. The incidence of unfavorable postoperative outcomes 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 perioperative or postoperative bleeding and postoperative complications, especially in those with ascites or undergoing surgery other than wall surgery or cholecystectomy.

Efficacy and safety of precision-guided transjugular extrahepatic portosystemic shunt (TEPS) in the management of cavernous transformation of the portal vein with portal hypertension: a case series

Background and aimsPerforming a Transjugular intrahepatic portal system shunt (TIPS) in patients with portal vein cavernous transformation (CTPV) poses significant challenges. As an alternative, transjugular extrahepatic portal vein shunt (TEPS) may offer a potential solution for these patients. Nonetheless, the effectiveness and safety of TEPS remain uncertain. This case series study aimed to evaluate the efficacy and safety of TEPS in treating patients with CTPV portal hypertension complications.MethodsThe study encompassed a cohort of 22 patients diagnosed with CTPV who underwent TEPS procedures. Of these, 13 patients manifested recurrent hemorrhagic episodes subsequent to conventional therapies, 8 patients grappled with recurrent or refractory ascites, and 1 patient experienced acute bleeding but refused endoscopic treatment. Comprehensive postoperative monitoring was conducted for all patients to rigorously evaluate both the technical and clinical efficacy of the intervention, as well as long-term outcomes.ResultsThe overall procedural success rate among the 22 patients was 95.5% (21/22).During the TEPS procedure, nine patients were guided by percutaneous splenic access, three patients were guided by percutaneous hepatic access, five patients were guided by transmesenteric vein access from the abdomen, and two patients were guided by catheter marking from the hepatic artery. Additionally, guidance for three patients was facilitated by pre-existing TIPS stents. The postoperative portal pressure gradient following TEPS demonstrated a statistically significant decrease compared to preoperative values (24.95 ± 3.19 mmHg vs. 11.48 ± 1.74 mmHg, p < 0.01).Although three patients encountered perioperative complications, their conditions ameliorated following symptomatic treatment, and no procedure-related fatalities occurred. During a median follow-up period of 14 months, spanning a range of 5 to 39 months, we observed four fatalities. Specifically, one death was attributed to hepatocellular carcinoma, while the remaining three were ascribed to chronic liver failure. During the follow-up period, no instances of shunt dysfunction were observed.ConclusionsPrecision-guided TEPS appears to be a safe and efficacious intervention for the management of CTPV.

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

Extrahepatic portal vein obstruction (EHPVO) is an important cause of symptomatic portal hypertension (PH) in pediatric population. PH may lead to serious complications including variceal bleeding and hypersplenism. Literature data on infants under 12 months old with EHPVO is poor. Aim - to analyze our experience in treatment of children with symptomatic PH in children of the first year of life, the peculiarities of the course of EHPVO in them, and acknowledge readers with diagnostic algorithm of patients with EHPVO that ensures the timely start of treatment and reduce the risk of life-threatening complications. Materials and methods. 11 patients who were diagnosed with EHPVO before age of 12 month are included into the study. All patients underwent in-hospital treatment in National Specialized Children’s hospital “Okhmatdyt” in a period since 2006 to 2022. Results. Among 11 patients of the group, 7 (63.6%) were males. Mean age at diagnosis was 6.02±0.67 months. 9 (83.8%) patients had umbilical catheter in anamnesis. 7 (63.6%) patients manifested bleeding episodes. Ultrasonography and enhanced computed tomography was performed in all patients. Endoscopically all patients had high grade varices. 8 (72.7%) patients underwent surgical interventions. Median hospital stay duration in children with bleeding was 36 (95% CI: 28-56) days, and 17 (95% CI: 14-22) days in children without bleeding (p&lt;0.003). The success of primary surgical interventions was assessed with Kaplan-Meyer test. Median overall survival was reached at 920 days after initial surgery; primary surgical intervention survival 0.417 (95% CI: 0.205-0.072). There was zero mortality in the study group. Conclusions. Newborns and infants with anamnesis of umbilical catheterization should be followed up closely by ultrasound imaging specialist. Patients with suspected PH are to be immediately referred to endoscopy and computed tomography in specialized center. Timely diagnosis, current endoscopic band ligation success in variceal bleeding primary prophylaxis would ensure further successful surgery and shorter hospital stay. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Digital subtraction angiography of intrahepatic portal vein as the key visualization for mesoportal shunting in children with extrahepatic portal vein obstruction

Аn early consideration in pediatric patients with extrahepatic portal vein obstruction (EHPVO) is to be given for mesoportal shunt. A careful investigation is required to prove intrahepatic portal system patency. Conventional noninvasive imaging is not adequately reliable in assessing patency of the intrahepatic portal venous system. Retrograde portography in children brings additional invasive procedure. Antegrade, direct intraoperative digital subtraction angiography for mesoportal shunt feasibility assessment is poorly presented in literature. Aim - to improve the rate of success of mesoportal shunting in children with EHPVO by analyzing our own experience in intraoperative digital subtraction angiography of the intrahepatic branches of the portal vein for the final assessment of the mesoportal shunting feasibility. Materials and methods. 7 pediatric patients with EHPVO were selected for the study. Angiographies and surgeries in selected patients were performed within single center in a period from May 2022 to July 2023. Mean follow up of successful mesoportal shunting was 12.38±1.46 months. Results. All patients were males. Men age at surgery was 8.71±1.72 years. 5 (71.4%) patients manifested bleeding episodes as the first sign of portal hypertension. In all patients ultrasound revealed splenomegaly and suspected portal hypertension for reduced volumetric portal flow. All 7 patients had high grade. Liver function was normal in all patients, and in none thrombophilia was confirmed. CT scans suspected patent left intrahepatic branch (Rex-zone). Digital subtraction angiography approved mesoportal shunt feasibility in 4 (57.1%) patients. The follow up period was 13.5±2.9 months. Conclusions. Digital subtraction angiography of intrahepatic portal vein is effective visualization method to achieve with radiologic evidence of intrahepatic portal branches patency and make the decision on mesoportal shunting when favorable anatomy proved. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Portal Vein Recanalisation in a Paediatric Patient: A New Paradigm in the Treatment of Non-cirrhotic Extra Hepatic Portal Vein Obstruction (EHPVO)

Extrahepatic portal vein obstruction (EHPVO) is a rare condition that usually affects young adults and is associated with risk of developing non-cirrhotic portal hypertension and portal cholangiopathy. PV recanalisation may be a suitable option in such patients to decompress the portal venous system and help treat portal hypertension and its complications.

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.