Hepatic Venous Obstruction Research Articles

Prevalence of minimal hepatic encephalopathy in extra hepatic portal venous obstruction patients following proximal splenorenal shunt surgery

Prevalence of minimal hepatic encephalopathy in extra hepatic portal venous obstruction patients following proximal splenorenal shunt surgery

Surgical outcome of extrahepatic portal venous obstruction: Audit from a tertiary referral centre in Eastern India.

Extra hepatic portal venous obstruction (EHPVO) is the most common cause of portal hypertension in Indian children. While endoscopy is the primary modality of management, a subset of patients require surgery. This study aims to report the short- and long-term outcomes of EHPVO patients managed surgically. All the patients with EHPVO who underwent surgery between August 2007 and December 2021 were retrospectively reviewed. Postoperative complications were classified after Clavien-Dindo. Binary logistic regression in Wald methodology was used to determine the predictive factors responsible for unfavourable outcome. Total of 202 patients with EHPVO were operated. Mean age of patients was 20.30 ± 9.96 years, and duration of illness, 90.05 ± 75.13 months. Most common indication for surgery was portal biliopathy (n = 59, 29.2%), followed by bleeding (n = 50, 24.8%). Total of 166 patients (82.2%) had shunt procedure. Splenectomy with esophagogastric devascularization was the second most common surgery (n = 20, 9.9%). Nine major postoperative complications (Clavien-Dindo > 3) were observed in 8 patients (4.0%), including 1 (0.5%) operative death. After a median follow-up of 56 months (15-156 months), 166 patients (82.2%) had favourable outcome. In multivariate analysis, associated splenic artery aneurysm (p = 0.007), isolated gastric varices (p = 0.004), preoperative endoscopic retrograde cholangiography and stenting (p = 0.015), and shunt occlusion (p < 0.001) were independent predictors of unfavourable long-term outcome. Surgery in EHPVO is safe, affords excellent short- and long-term outcome in patients with symptomatic EHPVO, and may be considered for secondary prophylaxis.

Additive Therapy of Plasmodium berghei-Induced Experimental Cerebral Malaria via Dihydroartemisinin Combined with Rapamycin and Atorvastatin.

Cerebral malaria (CM), caused by Plasmodium falciparum, is the primary cause of death from severe malaria. Even after immediate parenteral therapy with antimalarial drugs, the mortality rate remains 15 to 25%. Currently, no effective therapeutic agents are available for the radical treatment of CM. Thus, further in-depth explorations of adjuvant therapies in combination with antimalarial drugs are urgently needed. The experimental cerebral malaria (ECM) model was established by infecting C57BL/6 mice with Plasmodium berghei ANKA. Subsequently, infected mice were continuously treated with dihydroartemisinin (DHA) in combination with rapamycin (RAP) and atorvastatin (AVA) for 5 days at different time points, including day 0, day 3, and day 6 postinfection (p.i.). Treatment efficacy was evaluated by comparing behavioral scores, body weight, parasitemia, survival rate, blood-brain barrier (BBB) integrity, and histopathology. The optimal combination therapy of DHA, RAP, and AVA on day 3 p.i. was selected for ECM. This strategy significantly improved survival rate, reduced parasitemia, improved the rapid murine coma and behavioral scale scores and permeability of the BBB, attenuated cerebrovascular and hepatic central venous obstruction and hemozoin deposition in the liver, and decreased the red pulp area of the spleen, which effectively ameliorated neurological damage in ECM. It also improved histopathology and neurological damage caused by ECM. In this study, the optimal therapeutic strategy for ECM was selected, which is expected to be a potential therapy for human CM. IMPORTANCE Although artemisinin-based combination therapies (ACTs) have greatly improved the clinical outcome of cerebral malaria (CM) as a fatal disease that can permanently disable a significant proportion of children even if they survive, new treatment options are needed as Plasmodium falciparum develops resistance to antimalarial drugs. Recent reports suggest that basal treatment with artemisinin derivatives often fails to protect against cell death, neurological damage, and cognitive deficits. In this study, the combination of dihydroartemisinin with rapamycin and atorvastatin improved the current antimalarial outcomes by overcoming the limitations of current antimalarials for CM morbidity and neurological sequelae. This combination offers a new adjunctive treatment for the clinical treatment of human CM in susceptible populations, including children under 5 years old and pregnant women.

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Assessment of Rex vein patency in children with Extra hepatic Portal Venous Obstruction

Occurrence of Chronic Budd-Chiari Syndrome in a Patient with Coeliac Disease

The association of Budd-Chiari Syndrome (BCS) with coeliac disease has been understudied and underreported. Majority of the cases of this association have been reported from North Africa and Southern Europe, due to a higher prevalence of coeliac disease. BCS can present as acute, subacute, or chronic form and is characterised by hepatic venous obstruction, either thrombotic or non-thrombotic without any evidence of sinusoidal obstruction syndrome, right heart failure or constrictive pericarditis. Unlike patients with BCS alone, an underlying hypercoagulable state cannot be identified in most patients if coeliac disease is associated with BCS. Its incidence is found to be low in the rest of the world. Here, we present a case of coeliac disease and BCS from an Indian setting in a patient who was previously diagnosed with coeliac disease six years back, maintained on a gluten-free diet, and now presented with complaints of chronic Budd-Chiari Syndrome without any identifiable hypercoagulable aetiology.

S3305 A Unique Case of Extra Hepatic Portal Venous Obstruction Managed by Meso-Rex Shunt

S3305 A Unique Case of Extra Hepatic Portal Venous Obstruction Managed by Meso-Rex Shunt

S1476 Efficacy and Clinical Outcomes of Endoscopic Variceal Ligation With N-butyl-2-cyanoacrylate in Pediatric Patients in a Tertiary Care Center—A Retrospective Study

Introduction: Acute variceal bleeding (AVB) from gastroesophageal varices is a life-threatening complication in patients with portal hypertension. Bleeding from gastric varices is less common and treatment options are not standardized. Endoscopic variceal obliteration (EVO) with N-butyl-2-cyanoacrylate glue or balloon-occluded retrograde transvenous obliteration (BRTO) are the 2 most common treatment modalities and information on these are limited to adult series. There is paucity of information about gastric varices and its treatment in children. Methods: We conducted a retrospective cohort study among pediatric patients (1-18 years) who presented to our center with AVB from gastric varices. Between 2011-2021, we identified 538 among 4539 cases (12%) of portal hypertension with bleeding from gastric varices. Of these only 14 cases (2.6%) had a diagnosis of gastric variceal bleeding in children. Details of 13 patients were extracted with non-availability of records of 1 patient. Cyanoacrylate glue in 0.5-1 ml were injected in to the gastric varices. Patients were evaluated for efficacy, safety and complications associated with the treatment. Endoscopy was repeated after 3 weeks for assessment of variceal size and need for repeat glue injection. Results: The mean age of the patients was 13.3±5.2, with males (54%) and females (46%). The mean age at first presentation to hospital with gastrointestinal bleeding was 8.7±3.6, with hematemesis (62%) being the most common presentation. On endoscopy, 8 patients had type 1 gastro-esophageal varices and 3 patients had type 2 gastroesophageal varices. All 13 patients had stigmata of recent gastric variceal bleeding. The most common cause of portal hypertension was extra hepatic portal venous obstruction (EHPVO) (54%). The mean age at glue therapy treatment was 11.3±5.3. While immediate hemostasis was achieved in all 13 patients (100%) with gastric variceal bleeding, re-bleeding was seen among 3 patients, which occurred after 1 month of glue therapy. 1-3 sessions were required for obliteration of gastric varices. There was one case of death among the 13 children: a patient with decompensated Wilson’s disease. We did not encounter complications like anaphylactic shock, treatment-associated infection, gastric perforation, and distant emboli. Conclusion: The experience from our series suggests that endoscopic vascular obliteration using cyanoacrylate glue injection is safe and effective in children with gastric variceal bleeding.

S2943 Budd-Chiari Syndrome Diagnosis Achieved With High Clinical Suspicion Raised by EUS-Guided Portal Pressures and Liver Biopsy

Introduction: Budd Chiari syndrome (BCS) is a rare syndrome due to hepatic venous obstruction in the absence of cardiac cause, mostly secondary to thrombosis. As high as 80%of patients present with ascites. The diagnosis should be suspected in patient with acute or chronic liver disease without identified cause. Venous obstruction can be seen on Doppler ultrasound, CT, or MRI of the hepatic veins and inferior vena cava. In some cases, hepatic venogram is needed for its diagnostic and therapeutic advantages. We report a 50-year-old woman with BCS who underwent successful wire recanalization, angioplasty and thrombolysis of the right hepatic vein. Case Description/Methods: 50-year-old woman with no significant past medical history presented with abdominal distension and bilateral lower extremity swelling of two weeks duration. On exam, abdomen was distended with positive signs of fluid collection with +3 bilateral lower extremity edema. Labs revealed total bilirubin 3.3 direct bilirubin 1.4, ALP 477, ALT 38, AST 96, PT 14.4, INR 1.35. Ultrasound showed Heterogeneous appearance of liver and large volume ascites. Doppler study showed normal hepatic and portal venous flow. Abdominal MRI showed hepatic steatosis and caudate lobe hypertrophy. Large volume paracentesis was done with analysis showing SAAG of 3.1, WBC 364, Neutrophils 8. EGD revealed large esophageal varices. Viral hepatitis panel, ceruloplasmin, alpha-1antitripsin, ASMA, AMA antibodies were negative. EUS guided liver biopsy and portal pressure measurement was done showing venous pressure gradient of 13 consistent with portal hypertension. Liver biopsy showed grade 2 fibrosis and zone 3 congestion. Hepatic venogram showed chronically occluded right hepatic vein on which successful wire recanalization was done. Pt was worked up for hypercoagulable state and was found to have JAK 2 mutation and Protein C deficiency. Apixaban, Beta blocker and diuretics were started. Patient showed significant improvement with ascites and MELD score. Discussion: First line investigation for diagnosis of BCS is doppler ultrasound. Doppler US and MRI venography didn’t reveal the hepatic venous thrombosis in our patient. However, high index of suspicion, finding of elevated IVC pressure on EUS, and caudate lobe hypertrophy found on imaging as well as congestion liver biopsy findings led us to further investigate with hepatic venogram which confirmed the diagnosis and provided therapeutic advantages. Table 1. - Prothrombotic risk factors for BCS ACG Clinical Guideline A. Acquired thrombophilia B. Inherited thrombophilia C. Systemic factors D. Hormonal factors Myeloproliferative disorderPolycythemia veraEssential thrombocytosisIdiopathic myelofibrosisJAK 2 mutationPNH Hyperhomocysteinemia Factor V LeidenProthrombin gene G20210A mutationMTHFR C677T mutationThalassemiaPC deficiencyProtein C deficiencyAntithrombin deficiency SarcoidosisVasculitisBehcet’s diseaseConnective tissue diseaseInflammatory bowel diseases OCP usePregnancy Disorders of the Hepatic and Mesenteric Circulation Simonetto, Douglas A.; Singal, Ashwani K.; Garcia-Tsao, Guadalupe; Caldwell, Stephen H.; Ahn, Joseph; Kamath, Patrick S. Official journal of the American College of Gastroenterology | ACG115(1):18-40, January 2020. doi: 10.14309/ajg.0000000000000486

Long-Term Improvement in Liver Function Following Transjugular Intrahepatic Portosystemic Shunt in Patients With Budd–Chiari Syndrome

Transjugular intrahepatic portosystemic shunt (TIPS) relieves hepatic venous obstruction in Budd-Chiari syndrome (BCS), but the effect on liver function is unclear, particularly outside the immediate post-treatment period. This study aims to evaluate the long-term impact of TIPS on liver function and outcomes in BCS patients. Twenty patients with BCS who underwent TIPS from 1999 to 2018 were included. Demographic data and clinical data at the time of TIPS procedure, 6 months, 12 months, 2 years, 5 years, and 10 years post-TIPS were collected. There were 13 (13/20, 65%) women and 7 (7/20, 35%) men with a mean age at the time of TIPS of 42.6±12.8 years. The median time from BCS diagnosis to TIPS was 41 (IQR: 4-165) days. The number of patients with severe ascites decreased significantly from 10/17 (58.8%) at the time of TIPS, to 1/16 (7.7%), 1/13 (7.7%), 2/16 (12.5%), 1/14 (7.1%), and 0/8 (0%) at 6 months, 12 months, 2 years, 5 years and 10 years post-TIPS, respectively.4/20 (20%) patients developed new hepatic encephalopathy post-TIPS procedure. Child-Pugh score significantly decreased from a score of 9.4±1.8 pre-TIPS to 7.6±1.8at 6 months, 7.4±1.5at 12 months, 7.3±1.6at 2 years, 6.8±1.5at 5 years, and 6.4±0.7at 10 years post-TIPS. Fifteen (15/20, 75%) patients required TIPS revision including 4 (4/15, 26.7%) within 30 days, 2 (2/15, 13.3% within 1 month to 1 year, and 9 (9/15, 60%) at more than 1 year. Eight (8/20, 40%) patients underwent liver transplantation (LT) at median time of 7.3 (IQR 3.2-12.9) years after TIPS. TIPS placement for BCS results in sustained resolution of symptoms and improved liver function. Despite the frequent need for revisions, the long-term durability of TIPS can forgo the need for LT in the majority of patients.

Role of interventional radiology in management of post-liver transplant anastomotic complications

BackgroundLiver transplantation is considered to be the treatment of choice in cases of end-stage liver disease; however, as a major procedure, the operation is fraught with complications. The etiology, symptoms, and diagnostic methods for arterial, portal, and biliary issues are thoroughly discussed. Interventional procedures such as balloon angioplasty and stent placement in the arterial and portal systems, as well as biliary interventional procedures, are described.ResultsIn our study, we reviewed 25 cases of post-living donor transplanted liver, with anastomotic complications including biliary stenosis 40%, hepatic vein stenosis 20%, portal vein stenosis 16%, biliary leakage 16%, and hepatic artery stenosis or pseudo-aneurysm 16%. We had 10 cases of biliary stenosis, 7 of which were successfully treated with the insertion of an internal/external drain, and one case failed. Two patients died. We had four cases of hepatic venous obstruction with successfully implanted stents and a perfect outcome, efficacy, and patency rate of 100%. We also had two cases of hepatic artery stenosis that were perfectly managed by stent placement, with a patency rate of 100%. We came across two cases of hepatic artery pseudo-aneurysm. One case failed due to large sac size, while the other was successful. Finally, in our study, we had a 100% success rate in 5 cases of portal vein stenosis in the early postoperative period.ConclusionsPercutaneous IR was effective treatment for hepatic vein occlusion, portal vein stenosis, hepatic artery stenosis, and anastomotic biliary stricture after living donor liver transplantation. The interventional radiology team is now an integral part of the multi-disciplinary care of transplant patients. As new interventional instruments are developed and experience is gained, the outcomes of interventional treatments will continue to improve.

Budd chiari syndrome: A case report on a rare disorder

Budd Chiari syndrome (BCS) is a rare disease that threatens life due to a hepatic vein blood flow obstruction. As per the literature, thrombosis is a significant factor for hepatic venous obstruction. It can treat BCS based on the severity of the patient’s condition by three methods like hepatic vein angioplasty, interventional radiology stenting with direct intrahepatic portosystemic shunt, or liver transplantation. We describe the case of a 34-years-old female patient who presented with abdominal distension and generalized weakness with a history of BCS. A cooperative collaboration of hepatologists and interventional radiologists helps in effectively treating the syndrome.

Solid pseudopapillary epithelial neoplasia of the pancreas causing sinistral portal hypertension: a case report

Solid pseudopapillary epithelial neoplasm (SPEN), also referred as Franz’s tumour or Hamoudi’s tumour is a low malignant potential epithelial neoplasm of the pancreas. It occurs at a much lower frequency than other cystic neoplasms of the pancreas. It occurs rarely and in young females. Although infrequently, large sized tumours are known to compress the splenic vein thereby resulting in extra hepatic portal venous obstruction (EHPVO) resulting in left sided portal hypertension (PHT). The resulting periportal collateral circulation poses an intraoperative challenge while approaching these tumors. Meticulous pre-operative planning can go a long way in managing such a case and providing complete surgical cure. We present a case of SPEN in a 37 years old female which was discovered incidentally and managed surgically to achieve a complete cure to both the tumour and the left sided PHT.

Abstract No. 28 Sustained improvement in hepatic function following transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome

While transjugular intrahepatic portosystemic shunt (TIPS) has been shown to relieve hepatic venous obstruction and is a viable alternative to liver transplantation (LT) in Budd-Chiari syndrome (BCS), the effect of TIPS on liver function in patients with BCS is unclear, particularly outside the immediate post-treatment period. 20 patients with BCS who underwent TIPS from 1999-2018 were included. Demographic data and clinical data at the time of TIPS procedure, 6-month, and 12-month post TIPS were collected. There were 13 (65%) women and 7 (35%) men with a mean age of 42.6 ± 12.8 years. 8 (40%) patients had a JAK-2 mutation. Time from BCS diagnosis to TIPS procedure was 10.3 ± 23.3 months. Median follow-up time was 8.4 years. The ascitic burden significantly decreased from pre-TIPS (moderate ascites 10/17 (58.8%)) at 6 (moderate ascites 1/16 (6.3%), P = 0.0001) and 12 (1/13 (7.7%, P = 0.0007)) months follow-up, respectively. Hepatic encephalopathy was present in 5/17 (29.4%) pre-TIPS, 3/16 (18.8%) 6-month post TIPS, and 4/13 (30.8%) 12-months post-TIPS. Serum albumin significantly improved from pre-TIPS (3.0 ± 0.6) through 6 (3.5 ± 0.7 (P = 0.03)) and 12 (3.7 ± 0.6 (P< 0.01)) month follow up. Similarly, total protein significantly improved at 6-months after TIPS (pre-TIPS 6.4 ± 1.4, 6-month 7.3 ± 1 (P = 0.03)). Child-Pugh Score significantly decreased with a score of 9.4 ± 1.8 pre-TIPS, as compared to 7.6 ± 1.8 at 6-months (P = 0.007), and 7.4 ± 1.5 (P = 0.05) at 12-months post TIPS. 15 (75%) patients required TIPS revision with 4/15 (26.7%) within 30 days; 2/15 (13.3%) between 1 month and 1 year, and 9/15 (69.2%) after 1 year. 8 (40%) patients underwent LT at mean 7.68 ± 6 years after TIPS. 3 (15%) patients died at 1.9, 9.8, and 16.9 years following TIPS. In patients with BCS, TIPS significantly resolves symptoms and improves liver synthetic function at 1-year. Despite the frequent need for revision, TIPS for BCS also has good long-term durability and is able to prolong time to LT in a large proportion of patients.

Comparison of unenhanced and contrast-enhanced 3T magnetic resonance portovenography in children with extra hepatic portal venous obstruction.

To evaluate role of 3-T magnetic resonance portovenography (MRPV) in children with extra hepatic portal venous obstruction (EHPVO) and compare unenhanced and contrast-enhanced sequences. 20 Children with EHPVO underwent MRPV using unenhanced [T2, T1 pre-contrast, Balanced turbo field echo (BTFE)] and contrast-enhanced (CE) modified DIXON (mDIXON) sequences. The images were evaluated for the patency of abdominal veins, hepatic and splenic parenchyma by two radiologists for interobserver agreement. BTFE and post-contrast mDIXON sequences performed best for evaluation of abdominal veins. Concordance between the BTFE and CE sequences was good to very good for both the radiologists for splenic vein (SV) evaluation. The concordance between the two sequences for evaluation of superior mesenteric vein and its confluence with the SV was less remarkable and varied from fair to good, while the interobserver agreement was very good to perfect. The interobserver agreement between the BTFE and CE sequence for the evaluation of left renal vein was very good, while that for IVC was perfect. The agreement between BTFE and CE sequence was perfect for the evaluation of right and middle hepatic veins, while the interobserver agreement was good to perfect. The interobserver agreement was poor for evaluation of hepatic parenchyma on BTFE sequence as compared to CE sequence, and moderate for splenic parenchyma. BTFE sequence is the single best unenhanced MR pulse sequence to detect all the vascular structures in children with EHPVO. CE-MRI is not superior to BTFE sequence and should be used at the discretion of the radiologist.

Imaging Insights into EHPVO Induced Portal Biliopathy and its Grading: Retrospective Study Over A Decade at A Tertiary Care Hospital

Background and Aim: There is limited data on imaging features of extra hepatic portal venous obstruction (EHPVO) induced portal biliopathy. We describe a decade long experience of imaging spectrum of EHPVO induced portal biliopathy using MR/CT in a referral centre for liver diseases.Â

Urgent intraoperative endovascular stent placement to resolve acute hepatic or portal venous obstruction during liver surgery: a case series

BackgroundAcute obstruction of the hepatic vein (HV) or the portal vein (PV), particularly when it occurs during liver surgery, is potentially fatal unless repaired swiftly. As surgical interventions for this problem are technically demanding and potentially unsuccessful, other treatment options are needed.Case presentationWe report two cases of acute, surgically uncorrectable HV or PV obstruction during liver resection or living donor liver transplantation (LDLT), which was successfully treated with urgent intraoperative placement of endovascular stents using interventional radiology (IVR). In Case 1, a patient with colonic liver metastases underwent a non-anatomic partial hepatectomy of the segments 4 and 8 with middle hepatic vein (MHV) resection. Additionally, the patient underwent an extended right posterior sectionectomy with right hepatic vein (RHV) resection for tumors involving RHV. Reconstruction of the MHV was needed to avoid HV congestion of the anterior section of the liver. The MHV was firstly reconstructed by an end-to-end anastomosis between the MHV and RHV resected stumps. However, the reconstruction failed to retain the HV outflow and the anterior section became congested. Serial trials of surgical revisions including re-anastomosis, vein graft interposition and vein graft patch-plasty on the anastomotic wall failed to recover the HV outflow. In Case 2, a pediatric patient with biliary atresia underwent an LDLT and developed an intractable PV obstruction during surgery. Re-anastomosis with vein graft interposition failed to restore the PV flow and elongated warm ischemic time became critical. In both cases, the misalignment in HV or PV reconstruction was likely to have caused flow obstruction, and various types of surgical interventions failed to recover the venous flow. In both cases, an urgent IVR-directed placement of self-expandable metallic stents (SEMS) restored the HV or PV perfusion quickly and effectively, and saved the patients from developing critical conditions. Furthermore, in Cases 1 and 2, the SEMS placed were patent for a sufficient period of time (32 and 44 months, respectively).ConclusionsThe IVR-directed, urgent, intraoperative endovascular stenting is a safe and efficient treatment tool that serves to resolve the potentially fatal acute HV or PV obstruction that occurs in the middle of liver surgery.

Budd-Chiari Syndrome as A Manifestation of Right Atrium Myxoma

Budd-Chiari syndrome (BCS) is a rare condition marked by a number of symptoms due to hepatic venous obstruction. A 40-year-old female patient presented with abdominal pain, distension, and dyspnea. An abdominal ultrasonogram showed hepatomegaly, splenomegaly and ascites. A hypodense mass obstructed hepatic flow suggested thrombus formation in the hepatic and portal veins and suprahepatic part of the inferior vena cava on computed tomography. Transthoracic echocardiography was performed to assess thrombus extension to inferior vena cava and underlying pathological processes. It showed a mass lesion in the right atrium near intra atrial septum about 4 × 3 cm in diameter. The lesion manifested as an intracardiac thrombus extending from the inferior vena cava. After the successful surgical removal, the diagnosis of the mass was confirmed as atrial myxoma by histopathological examinations. We conclude that right atrial myxoma is a rare cause of Budd-Chiari syndrome.

Clinical and Diagnostic Profile and Management of Inferior Vena Cava Obstruction- A Descriptive Study at a Tertiary Care Centre, Hyderabad

Introduction: Obstruction to hepatic venous outflow is a rare disease with a potentially fatal outcome, if not recognised and managed promptly. The level of obstruction can vary, anywhere from the small hepatic venules to the entry point of the Inferior Vena Cava (IVC) into the right atrium. The original patients that Budd-Chiari described had acute hepatic vein thrombosis but this syndrome now encompasses various hepatic venous outflow blocks, of which a membrane in the IVC is the most common anatomical form of obstruction. Pure IVC obstruction is commonly seen among Asians. Aim: To study the clinical and diagnostic profile of patients presenting with IVC obstruction and to evaluate the treatment efficacy and safety of percutaneous transluminal angioplasty. Materials and Methods: For this prospective, descriptive study, from November 2015 to August 2019, a total of 21 patients were admitted with obstruction of hepatic venous flow. Most of the patients were young adults. Clinical features, aetiologies and coagulation profiles were studied. Ascites, pain abdomen and pedal oedema were common presentations. Diagnosis was established by clinical examination and was confirmed by ultrasound-doppler study, computed tomography and conventional angiography. Statistical analysis was done by using statistical package for social sciences IBM SPSS statistics for Windows, version 20.0. The data were expressed as percentages of study population while, continuous data were expressed as mean±standard deviation. Results: The mean age of the population was 24.7±2.9 years, with a female preponderance. (male: female=1:2). IVC angiography was done in 15 patients. Seven of the 15 patients had diffuse narrowing of IVC. Eight patients had focal or segmental narrowing of IVC, among them seven patients underwent Percutaneous Transluminal Angioplasty (PTA) with stenting. The venous access was obtained from femoral vein. The stenoses were crossed with either guide wire or Brocken brough needle with Mullins sheath. Three patients did not need predilatation. In four patients, obstructed IVC was dilated with Inoue balloon and then self-expanding stents were placed. Success was achieved in all cases, with significant drop in the pressure gradients from 20±5 mmHg before procedure to 3±2 mmHg immediately after the procedure. After PTA, all seven patients had either total relief or decrease of symptoms and clinical signs of hepatic venous obstruction. No major complications were observed and no repeat interventions were required. The mean postprocedural follow-up was six months that showed good outcomes with good stent patency. Conclusion: Chronic obstruction of IVC presents with abdominal pain, progressive ascites and portal hypertension in young adults, with mild abnormality of liver function tests. The IVC obstruction can be due to an obstructive membrane or segmental stenosis or diffuse narrowing of IVC. PTA with stenting can be done safely and it effectively relieves the symptoms and venous congestion and can be considered as the first line decompressive procedure in patients with IVC stenosis.

Surgical Portosystemic Shunts: History, Evolution, and Current Applications

AbstractMechanical obstruction, thrombus, intrinsic liver disease causing fibrosis or cirrhosis, or an outflow obstruction at the level of the sinusoids or hepatic venous obstruction can cause an increase in pressure or resistance, or both, leading to portal hypertension (PH). Portosystemic shunts (PSS) are usually performed to relieve the congestion that inevitably occurs in the setting of PH. Since their introduction, surgical PSS were often the treatment of choice to prevent recurrent bleeding in patients with clinically significant PH. Development of novel pharmacological therapies, continuous improvement of endoscopic approaches, the introduction of transjugular intrahepatic portosystemic shunt, and advancements in transplantation has provided an evolution in the approach for PH and has precipitated the steady decrease in the proportion of patients needing surgical shunts. Despite this, PSS remain important tools in the surgeon's armamentarium, as they are often employed in the pediatric population with extrahepatic portal vein obstruction and are frequently being used for portal inflow modulation to achieve better portal hemodynamics in resections and transplantation. This has become of great relevance to decrease the risk of small-for-size syndrome and portal hyperperfusion in liver transplantation, and to decrease the risk of posthepatectomy liver dysfunction after major resections in hepatobiliary surgery.

MRI Evaluation of Liver and Cardiac Iron Overload Impact on Hematopoietic Stem Cell Transplantation with β-Thalassemia Major

OBJECTIVE: To assess the effects of liver and cardiac iron overload detected by magnetic resonance imaging (MRI) T2* on hematopoietic stem cell transplantation in children with β-thalassemia major. METHODS:Summary of 380 cases of β-thalassemia major patients more than 5 years old in Nanfang hospital, southern medical university from 2012 to 2019.Iron concentrations in the liver and heart were calculated based on MRI T2* test results of liver and heart. Age, serum ferritin, left ventricular ejection fraction (LVEF), and liver function were compared to evaluate the effect of iron overload on organ function in patients with β-thalassemia major before transplantation.168 patients underwent allogeneic hematopoietic stem cell transplantation, 48 were HLA-mismatched transplantation, and 120 were HLA-identical allogeneic hematopoietic stem cell transplantation.To analysis the influence between implantation rate, hematopoietic reconstruction time, mortality, and common complications after transplantation such as graft-versus-host disease, hepatic venous obstruction, infection, immune hemolysis, and pancytopenia and liver and cardiac iron overload detected by magnetic resonance imaging (MRI) T2*. RESULTS:Myocardial iron overload occurred in 73 cases (19.2%), including 29 cases of cardiac T2*15~20 ms (mild), 23 cases of 10~14 ms (moderate), and 21 cases of &lt;10 ms (severe).There were 305 cases (80.2%) with liver iron overload, including 98 cases with 2.7~6.3 ms (mild), 166 cases with 1.4~2.7 ms (moderate), and 41 cases with &lt;1.4 ms (severe).LVEF decreased in 5 cases (1.6%).Liver iron was positively correlated with serum ferritin (r=0.523, P=0.001), cardiac iron concentration was positively correlated with serum ferritin (r=0.33, P=0.1), age was positively correlated with cardiac iron concentration (r=0.4, P=0.14), and age was negatively correlated with left ventricular ejection fraction (r=-0.36, P=0.001).After transplantation, liver iron concentration was positively correlated with hemoglobin implantation time (r=0.49, P=0.043), heart iron concentration was positively correlated with mortality (r=0.39, P=0.012), serum ferritin was negatively correlated with implantation rate (r=-0.26, P=0.012), and serum ferritin was positively correlated with infection incidence correlation (r=0.441, P=0.034).There were no statistically significant differences in liver, heart MRI T2*, liver iron concentration and heart iron concentration between the two groups before and after transplantation. CONCLUSION:Magnetic resonance imaging (T2*) is an effective and non-invasive method to detect the iron overload in the heart and liver caused by blood transfusion in β-thalassemia patients. Iron overload can have adverse effects on hematopoietic stem cell transplantation,and effective iron removal before transplantation can improve the success rate of transplantation.Quantitative assessment of iron overload in the liver and heart by MRI can be used as a necessary examination before transplantation. Disclosures No relevant conflicts of interest to declare.