2023 Update of Indian National Association for Study of the Liver Consensus on Management of Intermediate and Advanced Hepatocellular Carcinoma: The Puri III Recommendations.

Hepatocellular carcinoma (HCC) presents significant treatment challenges despite considerable advancements in its management. The Indian National Association for the Study of the Liver (INASL) first published its guidelines to aid healthcare professionals in the diagnosis and treatment of HCC in 2014. These guidelines were subsequently updated in 2019. However, INASL has recognized the need to revise its guidelines in 2023 due to recent rapid advancements in the diagnosis and management of HCC, particularly for intermediate and advanced stages. The aim is to provide healthcare professionals with evidence-based recommendations tailored to the Indian context. To accomplish this, a task force was formed, and a two-day round table discussion was held in Puri, Odisha. During this event, experts in their respective fields deliberated and finalized consensus statements to develop these updated guidelines. The 2023 INASL guidelines offer a comprehensive framework for the diagnosis, staging, and management of intermediate and advanced HCC in India. They represent a significant step forward in standardizing clinical practices nationwide, with the primary objective of ensuring that patients with HCC receive the best possible care based on the latest evidence. The guidelines cover various topics related to intermediate and advanced HCC, including biomarkers of aggressive behavior, staging, treatment options, and follow-up care.

Recurrent Hepatic Encephalopathy Due to Surgically Created Shunt During Living Donor Liver Transplantation.

Hepatic encephalopathy due to portosystemic shunts is a well-defined entity in patients with cirrhosis. Rarely, liver transplant recipients develop hepatic encephalopathy owing to persistence or surgically created portosystemic shunts. We present a case of post-transplant recurrent hepatic encephalopathy due to a surgically created portocaval shunt. The patient was managed by a covered metal stent placement in the inferior vena cava, thus causing functional closure of the shunt.

Endovascular management of portal steal syndrome due to portosystemic shunts after living donor liver transplantation.

BACKGROUND AND AIM: After liver transplant, pre-existent porto-systemic shunts (PSS) may persist, causing "portal steal," leading to graft dysfunction, hepatic encephalopathy (HE), and eventual rejection. In recipients of small-for-size transplant liver grafts, shunts may be created intraoperatively, facilitating diversion of portal flow to systemic circulation to avoid ill-effects of portal overperfusion. These iatrogenic shunts may also subsequently lead to portal steal. We aim to evaluate safety and efficacy of endovascular techniques in management of portal steal due to PSSs in living donor liver transplantation (LDLT) recipients. METHODS: Between 2013 and 2020, we encountered five LDLT recipients with large PSS, who presented with graft dysfunction and/or HE. One patient had a surgically created shunt and four had spontaneous shunts, not surgically ligated during transplant. Endovascular techniques including plug-assisted or balloon-occluded retrograde transvenous obliteration (PARTO/BRTO) or covered inferior vena cava (IVC) stent grafts were to occlude these PSS and counter the portal steal in all patients. Technical success and clinical outcomes at 1-year-follow-up were assessed. RESULTS: Imaging showed large PSS causing portal steal syndrome in all five patients. IVC stent graft was used to isolate the shunt in two patients and PARTO/BARTO was performed in three patients. One patient had guarded prognosis due to multiple organ dysfunction and died 5 days after endovascular procedure. At 1-year follow up, graft functions normalized in four patients with no recurrence of HE. No procedure-related complications were seen. CONCLUSION: Endovascular techniques can be safely and effectively used to counter portal steal syndrome in LDLT recipients, thus avoiding surgical re-exploration in these patients.

Corrigendum to "Joint Consensus Statement of the Indian National Association for Study of the Liver and Indian Radiological and Imaging Association for the Diagnosis and Imaging of Hepatocellular Carcinoma Incorporating Liver Imaging Reporting and Data System" [J Clin Expt Hepatol 9 (2019) 625-651].

[This corrects the article DOI: 10.1016/j.jceh.2019.07.005.].

Experience With LDLT in Patients With Hepatocellular Carcinoma and Portal Vein Tumor Thrombosis Postdownstaging.

BACKGROUND: Median survival in patients with hepatocellular carcinoma (HCC) and portal vein tumor thrombosis (PVTT) is 2-6 months; conventionally liver transplantation is contraindicated. METHODS: We studied outcomes following living donor liver transplantation (LDLT) post-PVTT downstaging (DS) with stereotactic body radiotherapy (SBRT), and tumor ablation (with transarterial chemo- or radio-embolization). RESULTS: Of 2348 consecutive LDLTs, 451 were for HCC, including 25 with PVTT (mainly Vp1-3) after successful DS and 20 with Vp1/2 PVTT without previous treatment. DS was attempted in 43, was successful in 27 (63%), and 25 underwent LDLT. Median alpha fetoprotein (AFP) at diagnosis and pre-LDLT were 78.1 ng/mL (3-58 200) and 55 ng/mL (2-7320), respectively. Mean DS to LDLT time was 10.2 weeks (5-16). Excluding 2 postoperative deaths, 1- and 5-year overall survival (OS) and recurrence-free survival (RFS) were 82%, 57%, and 77%, 51%, respectively, comparable to survival in 382 HCC patients without PVTT undergoing upfront LDLT (5-y OS 65%, P = 0.06; RFS 66%, P = 0.33, respectively). There was a trend toward better OS in DS+LDLT versus non-DS LDLT group (5-y OS/RFS-48%/40%). OS was significantly better than in HCC-PVTT patients receiving no intervention or palliative Sorafenib alone (1-y OS of 0%) or Sorafenib with TARE/SBRT (2-y OS of 17%) at our center during the study period. Initial AFP <400 ng/mL and AFP fall (initial minus pre-LDLT) >2000 ng/mL predicted better RFS; Grade III/IV predicted worse OS in DS patients. CONCLUSIONS: HCC patients with PVTT can achieve acceptable survival with LDLT after successful DS. Low initial AFP level, a significant drop in AFP with DS and low tumor grade, favorably influence survival in these patients.

Joint Consensus Statement of the Indian National Association for Study of the Liver and Indian Radiological and Imaging Association for the Diagnosis and Imaging of Hepatocellular Carcinoma Incorporating Liver Imaging Reporting and Data System.

Hepatocellular carcinoma (HCC) is the 6th most common cancer and the second most common cause of cancer-related mortality worldwide. There are currently no universally accepted practice guidelines for the diagnosis of HCC on imaging owing to the regional differences in epidemiology, target population, diagnostic imaging modalities, and staging and transplant eligibility. Currently available regional and national guidelines include those from the American Association for the Study of Liver Disease (AASLD), the European Association for the Study of the Liver (EASL), the Asian Pacific Association for the Study of the Liver, the Japan Society of Hepatology, the Korean Liver Cancer Study Group, Hong Kong, and the National Comprehensive Cancer Network in the United States. India with its large population and a diverse health infrastructure faces challenges unique to its population in diagnosing HCC. Recently, American Association have introduced a Liver Imaging Reporting and Data System (LIRADS, version 2017, 2018) as an attempt to standardize the acquisition, interpretation, and reporting of liver lesions on imaging and hence improve the coherence between radiologists and clinicians and provide guidance for the management of HCC. The aim of the present consensus was to find a common ground in reporting and interpreting liver lesions pertaining to HCC on imaging keeping LIRADSv2018 in mind.

Superselective vesical artery embolization in the management of intractable hematuria secondary to hemorrhagic cystitis.

PURPOSE: To evaluate the efficacy and outcome of superselective vesical arterial embolization in the management of severe intractable hematuria secondary to hemorrhagic cystitis. MATERIALS AND METHODS: We retrospectively reviewed the medical records of nine patients with severe intractable hematuria treated with superselective vesical artery embolization at our institution between March 2003 and February 2015. There were six males and three females with a mean age of 56.1 years. Seven patients had transitional cell carcinoma (TCC) of urinary bladder and had undergone transurethral resection of bladder tumor and pelvic radiotherapy. One patient had synchronous renal pelvis and bladder TCC. One patient had aortoarteritis and was receiving cyclophosphamide therapy and another patient had carcinoma cervix post-pelvic radiotherapy. Following the failure of conservative management, superselective vesical artery catheterization and embolization was performed with 300-500-µ PVA particles in all patients. Coil embolization of inferior gluteal artery followed by particle embolization of vesical arteries was done in one patient in whom superior, inferior vesical and inferior gluteal arteries were arising as a trifurcation. RESULTS: The technical success rate was 100% with complete cessation of hematuria within 48 h in all patients. No significant complications were noted, except for post-embolization syndrome in one patient, which improved on symptomatic treatment. During a mean follow-up period of 14.45 months (ranging from 3-28 months), one patient had mild recurrent hematuria (at 2 months) which resolved spontaneously. CONCLUSIONS: Superselective vesical artery embolization is a safe and effective procedure in controlling intractable life-threatening hematuria in a select group of patients who have failed conventional treatment protocols. This procedure may be considered as the treatment of choice since it usually obviates the need for emergency surgery in these severely ill patients.

Modified palliative biliary stenting in situs inversus totalis patient with carcinoma gallbladder: feasibility and technical details.

Knowledge of the anatomical variants is essential for all invasive hepatobiliary procedures such as endoscopy, surgery and radiologic interventions. Modification in standard therapeutic interventions may be required based on variant anatomy. We report a technical modification in a 75-year-old female with known situs inversus (SI) totalis with carcinoma gallbladder. Present case highlights the finer technical details of the modified percutaneous transhepatic biliary drainage (PTBD) procedure and biliary stenting in a SI patient with carcinoma gallbladder causing malignant biliary obstruction.

ß-Blocker therapy ameliorates hypersplenism due to portal hypertension in children.

BACKGROUND/PURPOSE: Thrombocytopenia due to hypersplenism precludes percutaneous liver biopsy in many cases of chronic liver disease (CLD). The aim of this study was to assess the efficacy of propranolol in correcting platelet counts (>100,000/mm(3)) to ensure percutaneous liver biopsy in children with CLD. METHODS: From January 2005 to December 2012, 51 consecutive children (mean age 11.5 ± 3.0 years, 34 boys) with CLD who needed liver biopsy but could not be done due to hypersplenism-related thrombocytopenia (platelets <100,000/mm(3) and/or total leukocyte counts <4,000/mm(3) with splenomegaly) were recruited and given a 4-week trial of long-acting propranolol (1.5-2 mg/kg/day). Hemodynamic parameters and splenic artery hemodynamics by Doppler ultrasound were recorded before and after the propranolol trial. Response to therapy was defined as improvement of platelet counts to ≥10(5)/mm(3). RESULTS: Thirty-two (62.7%) children responded to propranolol therapy and their mean platelet counts increased from 57.5 ± 13.0 × 10(3) to 140.7 ± 43.3 × 10(3)/mm(3), p = 0.0001. Liver biopsy could be done in 29. While comparing responders with non-responders, baseline spleen size (7.4 ± 3.3 vs. 12.7 ± 4.5 cm, p = 0.0001) and platelet counts (57.5 ± 13.0 × 10(3) vs. 39.5 ± 14.5 × 10(3), p = 0.0001) were found to be significant. ROC curve suggested a cut-off value of ≤8.5 cm of spleen and ≥53,000 platelets as predictors of response. With propranolol, mean arterial pressure and spleen size reduced (p < 0.05) and splenic artery resistance increased significantly (p = 0.005) in responders. CONCLUSIONS: Propranolol corrects thrombocytopenia and makes liver biopsy possible in almost two-thirds of cases by reducing splenic sequestration through splenic artery vasoconstriction. The baseline spleen size and platelet counts determine the effectiveness of therapy. A trial of ß-blocker is worth carrying out in cases where liver biopsy is contraindicated due to hypersplenism-related thrombocytopenia.

Budd-Chiari syndrome in children: clinical features, percutaneous radiological intervention, and outcome.

OBJECTIVES: 'Radiological intervention' to restore venous patency is the preferred therapy in adults with Budd-Chiari syndrome (BCS). The published literature on pediatric BCS is scarce. This study evaluated the clinical profile and role of a therapeutic radiological intervention in children with BCS. PATIENTS AND METHODS: Forty-six BCS children [29 boys, median age 10.5 (2-16) years] were enrolled. Standard medical therapy was administered to all. A radiological intervention, angioplasty [hepatic vein (HV) (n=3)], stenting [HV (n=18), inferior vena cava (IVC) (n=5)], transjugular intrahepatic portosystemic shunt (TIPS) (n=3), was performed in 25 cases. Clinical, biochemical, and radiological follow-up was carried out. RESULTS: Doppler ultrasonography was diagnostic in 95% of cases. All patients had chronic BCS, with ascites in 82.6%, hepatomegaly in 84.8%, splenomegaly in 69.6%, prominent abdominal veins in 69.6%, and variceal bleed in 34.8% cases. The most common site of block was HV (n=33), followed by combined HV and IVC block (n=11), and isolated IVC block (n=2). Eight of 12 (75%) cases had abnormal procoagulant workup. Radiological intervention was technically successful in 100%. Clinical and biochemical improvement was observed in the intervention group. Complications included neck hematoma and hemorrhagic ascites in one patient each. One child in the intervention group (post-TIPS sudden cardiac event) and two children in the nonintervention group [end-stage liver disease (n=1), head injury (n=1)] died. Stent was patent in 15/20 (75%) children over a median follow-up of 6.5 months. CONCLUSION: HV block and a chronic presentation are most common in BCS children. Doppler ultrasonography establishes the diagnosis in 95% of cases. Radiological intervention is an effective and safe therapeutic modality for children with BCS.

Evaluation of antigen detection and polymerase chain reaction for diagnosis of amoebic liver abscess in patients on anti-amoebic treatment.

BACKGROUND: Diagnosis of amoebic liver abscess (ALA) in patients on anti-amoebic drugs is difficult. There is scanty data on this issue using Entamoeba histolytica (E. histolytica) lectin antigen and polymerase chain reaction (PCR). We studied utility of lectin antigen, PCR, and IgG antibody in diagnosis of liver abscess in patients on anti-amoebic treatment. Liver aspirate of 200 patients, of which 170 had anti-amoebic drug prior to drainage, was tested for E. histolytica lectin antigen by (ELISA), PCR, bacterial culture, and serum IgG antibody by (ELISA). Classification of abscesses was based on result of anti-amoebic IgG antibody and bacterial culture, E. histolytica PCR and bacterial culture, and E. histolytica lectin antigen and bacterial culture. FINDINGS: Using anti-amoebic IgG antibody and bacterial culture, 136/200 (68.0%) were classified as ALA, 12/200 (6.0%) as pyogenic liver abscess (PLA), 29/200 (14.5%) as mixed infection, and 23/200 (11.5%) remained unclassified. Using amoebic PCR and bacterial culture 151/200 (75.5%) were classified as ALA, 25/200 (12.5%) as PLA, 16/200 (8.0%) as mixed infection, and 8/200 (4.0%) remained unclassified. With E. histolytica lectin antigen and bacterial culture, 22/200 (11.0%) patients were classified as ALA, 39/200 (19.5%) as PLA, 2/200 (1.0%) as mixed infection, and 137/200 (68.5%) remained unclassified. CONCLUSIONS: E. histolytica lectin antigen was not suitable for classification of ALA patients who had prior anti-amoebic treatment. However, PCR may be used as alternative test to anti-amoebic antibody in diagnosis of ALA.

Identification of high-risk group and therapeutic options in children with liver abscess.

The outcome of children with liver abscess (LA) depends upon prompt diagnosis and intervention. We evaluated the etiology, clinical profile, various interventional modalities of management and outcome of children with LA. A total of 39 hospitalized children (mean age 7.2 ± 3.9 years) with radiologically proven LA were analyzed. Parenteral antibiotics, percutaneous drainage (PD) or open surgical drainage (OSD) was done as required. Cases with ruptured or impending rupture of LA, upper gastrointestinal bleed, jaundice, pleural effusion or consolidation were labeled as "high risk" cases. Triad of fever, pain and hepatomegaly was the most common presentation. Single abscess was present in 66.7% and right lobe was involved in 69.2% of cases. Majority of LA were pyogenic (PLA, 25/39). Amebic liver abscess (ALA) and PLA had similar clinical and laboratory profile except that multiloculated abscess on ultrasonography was a feature of PLA (12/25 vs. 0/11; p = 0.006). Cases with ALA settled significantly more often with antibiotics alone (5/11 vs. 3/25; p = 0.04) than PLA and none required surgery (0/11 vs. 7/25; p = 0.03). Subjects with "high-risk" LA (n - 26) had significantly larger abscesses, more polymorphonuclear leucocytosis (74 ± 15% vs. 61 ± 13%; p = 0.01) in peripheral blood and need of drainage (24/26 vs. 7/13; p = 0.03) than patients with average-risk LA. Based on the results, 38/39 children recovered, with complete abscess resolution in 28, over 48 ± 63.8 days. In conclusion, ALA, although similar in presentation, are uniloculated, and patients with ALA recover more often without drainage than patients with PLA. Patients with "high risk" LA are more common and have a good outcome with drainage. PD, being safe, efficacious and less invasive than OSD, should be the preferred drainage procedure.

Candida esophagitis in achalasia cardia: case report and review of literature.

Candida albicans is by far the most common cause of infectious esophagitis. In most patients, this infection is secondary to an immuno-compromised state. In nearly 25% of the cases, underlying causes of esophageal stasis, e.g., achalasia and scleroderma, facilitate fungal colonization of the esophagus. Double contrast esophagography is a highly sensitive tool for diagnosing candida esophagitis. This report discusses the uncommon association of Candida esophagitis with achalasia cardia, their radiographic features and a short review of the available literature.

Superior sagittal sinus thrombosis and Budd-Chiari syndrome due to paroxysmal nocturnal hemoglobinuria managed with transjugular intrahepatic portosystemic shunt: a case report.

BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH), caused by somatic mutation of hematopoietic cells, is associated with complement-mediated hemolysis and a hypercoagulable state. Thrombotic complications in this disease are associated with reduced survival. We report a patient with PNH complicated by intracranial venous thrombosis and Budd-Chiari syndrome, who was managed with transjugular intrahepatic portosystemic shunt. CASE PRESENTATION: A 26-year-old man presented with thrombosis of the superior sagittal and right sigmoid sinuses. Initial investigations did not reveal any underlying cause. Nine months later, he developed hepatic venous thrombosis. At this time, Ham test was positive. Flow cytometry confirmed the diagnosis of PNH. The patient was treated with transjugular intrahepatic portosystemic shunt; one episode of stent blockage one month later was managed successfully with balloon dilatation and restenting. CONCLUSION: PNH should be considered in patients with unexplained venous thrombosis. Thrombosis in these patients needs to be managed with prolonged anticoagulation. For Budd-Chiari syndrome in patients with underlying PNH, transjugular intrahepatic portosystemic shunt may be a good option but caution is needed to prevent stent occlusion.

Management of childhood pancreatic disorders: a multidisciplinary approach.

INTRODUCTION: Data on therapeutic endoscopy and radiologic interventions for the management of childhood pancreatic disorders are relatively limited. This study focuses on the multidisciplinary approach to the management of pancreatitis in children. PATIENTS AND METHODS: Children with pancreatic disorders were studied from January 1992 to May 2001. Acute pancreatitis (AP) was diagnosed by clinical evaluation, serum amylase more than three times normal, and morphologic abnormalities of the pancreas on imaging. Children with recurrent abdominal pain, pancreatic calcification or ductal stones on imaging, and pancreatic ductal changes on endoscopic retrograde cholangiopancreatography (ERCP) were diagnosed with chronic pancreatitis (CP). Patients were treated by gastroenterologists, surgeons, and interventional radiologists. Pancreatic exocrine insufficiency was diagnosed in appropriate settings. RESULTS: Fifteen children--6 with AP (posttrauma, 3; gallstone disease, 1; and viral, 1), 7 with CP, and 2 with pancreatic exocrine insufficiency--were diagnosed. Local complications observed in children with AP included pseudocyst in three, and infected acute fluid collection, right-sided pleural effusion, and ascites in one patient each. Complications of AP were managed with percutaneous catheter drainage (n = 3; pseudocyst, 2; infected fluid collection, 1), additional pancreatic duct stenting (n = 2), surgical drainage (n = 1), and octreotide for pleural effusion (n = 1). Signs of CP included abdominal pain (n = 7), obstructive jaundice resulting from lower common bile duct stricture (n = 2), and bleeding from gastroduodenal artery pseudoaneurysm (n = 1). Pancreatic duct stenting relieved pain in one patient, and steel coil embolization arrested bleeding from the pseudoaneurysm. Common bile duct strictures were managed by surgical bypass (n = 2), one of which required preoperative endoscopic bile duct stenting for management of cholangitis. Two other patients with CP required no intervention. CONCLUSION: A multidisciplinary approach of radiologic and endoscopic interventions and surgery are complimentary to each other in achieving successful outcomes of complicated childhood pancreatitis.

Pancreatic abscess: 10 years experience.

BACKGROUND: Secondary infections of pancreatic and peripancreatic necrosis account for most of the deaths following acute pancreatitis. These infections occur in the form of 'infected pancreatic necrosis' and 'pancreatic abscess'. The latter is a rare complication of acute pancreatitis in comparison with the former. METHODS: Twenty-one patients with pancreatic abscess were managed over a 10-year period at a tertiary care centre in Northern India. The present report details the clinical profile, investigations performed and management strategy (surgery and intervention radiology) of these patients. The role of surgery and percutaneous catheter drainage (PCD) in the management of pancreatic abscess is discussed, with emphasis on the successful outcome seen in a properly selected group of patients managed by PCD. RESULTS: Of the 21 patients, 12 were managed by percutaneous intervention, nine were managed surgically (of these, two had a prior PCD) and two patients were managed conservatively. The overall mortality was 9.5% (2/21). Thus, percutaneous management was suitable for 57% patients, was successful in 83.3%, with a mortality of 8.3%. Surgical therapy alone was offered to 33% of patients, was successful in 85.7%, with a mortality of 14.2%. Complications were seen in four of the nine patients managed by percutaneous drainage alone and eight of the nine patients managed surgically. CONCLUSIONS: Pancreatic abscess is a potentially lethal complication in patients recovering from acute pancreatitis. Early diagnosis and prompt intervention with careful selection of patients based on computed tomography imaging for surgical or percutaneous radio-logical management, is met with a successful outcome in a majority of patients. The roles of surgery and PCD are complementary.