Treatment outcomes and prognostic factors in children with hepatoblastoma using a risk-stratified approach.

BACKGROUND: Not all the significant progress made in the management of children with hepatoblastoma (HB) has translated into improved outcomes in limited-resource settings. There are limited data on outcomes in children with HB from India. METHODS: All patients diagnosed with HB between July 2013 and December 2020 were risk-stratified and treated as per International Liver Tumor Strategy Group (SIOPEL). Patients with standard-risk HB received cisplatin monotherapy and those with high-risk HB received alternating cycles of cisplatin and the combination of carboplatin plus doxorubicin. Data regarding demographic details, chemotherapy, surgery, liver transplantation, outcomes, prognostic factors, and toxicity were collected. RESULTS: Of 157 patients with HB, 117 (74%) were high risk, 31 (20%) were standard risk, and nine (6%) unknown. Patients with standard-risk disease had excellent outcomes, with 3-year event-free survival (EFS) and overall survival (OS) of 96% and 100%, respectively. Among high-risk HB, six underwent orthotopic liver transplantation of which four were alive at last follow-up. The 3-year EFS and OS of patients with high-risk disease was 56% and 66%, respectively. Outcomes of patients with PRETEXT IV (3-year EFS: 42%, 3-year OS: 50%) and metastatic disease (3-year EFS: 30%, 3-year OS: 50%) were dismal. Patients with serum alpha-fetoprotein (AFP) reduction greater than 90% following two courses of chemotherapy had favorable outcomes; 3-year EFS: 80% versus 58% (p = .013) and 3-year OS: 95% vs. 68% (p < .01). Only two (6%) of 31 patients with relapse/refractory HB were alive at a median follow-up of 36 months, and both had received salvage chemotherapy and surgery. CONCLUSIONS: While children with standard-risk HB had excellent outcomes, those with high-risk disease continue to do poorly. Serial monitoring of serum AFP values is a cost-effective and reliable predictor of outcomes. Orthotopic liver transplantation remains a viable option for inoperable disease in resource-limited settings as well.

Archimedes Absorbable Internal Biliary Stent in Liver Transplants to Prevent Bile Leak.

BACKGROUND: Biliary complications, especially bile leaks, are an important cause of early postoperative morbidity and, rarely, mortality after liver transplant. The risk is higher in living donor liver transplant (LDLT) compared to deceased donor liver transplant (DDLT). Attempts to reduce bile leaks have included refinements in the biliary anastomosis technique and use of various external and internal stents, with inconsistent benefits. Recent availability and successful use of the absorbable Archimedes stent has prompted its intrabiliary placement across the anastomosis. METHODS: In this retrospective study, we analyzed the data of 20 adult patients who underwent a liver transplant with duct-to-duct biliary anastomosis using the Archimedes stent. Both DDLT and LDLT were performed using cava-preserving hepatectomy followed by standard implantation methods. Duct-to-duct biliary anastomosis was performed in all cases using interrupted sutures with extracorporeal knots over an absorbable intrabiliary stent. In addition to standard postoperative care, patients were monitored for bile leak. RESULTS: Nine DDLTs had a single anastomosis over a 10-Fr stent. Out of 11 LDLT patients, 7 had a single anastomosis and 4 patients had 2 anastomoses, all over a 6-Fr stent. Two patients died, 1 as a result of graft primary nonfunction and another because of multidrug-resistant pneumonia. One patient had ascending cholangitis owing to stent migration in the duodenum. This episode was treated with endoscopic stent removal and appropriate antibiotics, with good recovery. None of the other patients had bile leaks, biloma, or stent-related complications. CONCLUSIONS: Archimedes internal absorbable biliary stents can be safely used in both living and deceased donor liver transplants to prevent bile leaks.

Acute liver failure secondary to yellow phosphorus rodenticide poisoning: Outcomes at a center with dedicated liver intensive care and transplant unit.

Accidental or suicidal poisoning with yellow phosphorus or metal phosphides (YPMP) such as aluminum (AlP) zinc phosphide (Zn3P2) commonly cause acute liver failure (ALF) and cardiotoxicity. These are used as household, agricultural and industrial rodenticides and in production of ammunitions, firecrackers and fertilizers. In absence of a clinically available laboratory test for diagnosis or toxin measurement or an antidote, managing their poisoning is challenging even at a tertiary care center with a dedicated liver intensive care unit (LICU) and liver transplant facility. PATIENTS AND METHODS: Patients with YPMP related ALF were monitored using standardized clinical, hemodynamic, biochemical, metabolic, neurological, electrocardiography (ECG) and SOFA score and managed using uniform intensive care, treatment and transplant protocols in LICU. Socio-demographic characteristics, clinical and biochemical parameters and scores were summarized and compared between 3 groups i.e. spontaneous survivors, transplanted patients and non-survivors. Predictors of spontaneous survival and the need for liver transplant are also evaluated. RESULTS: Nineteen patients with YPMP related ALF were about 32 years old (63.2% females) and presented to us at a median of 3 (0 - 10) days after poisoning. YPMP related cardiotoxicity was rapidly progressive and fatal whereas liver transplant was therapeutic for ALF. Spontaneous survivors had lower dose ingestion (<17.5 grams), absence of cardiotoxicity, < grade 3 HE, lactate < 5.8, SOFA score < 14.5, and increase in SOFA score by < 5.5. Patients with renal failure need for CVVHDF and KCC positivity on account of PT-INR > 6.5 had higher mortality risk. Patients undergoing liver transplant and with spontaneous recovery required longer ICU and hospital stay. At median follow-up of 3.4 (2.6 - 5.5) years, all spontaneous survivors and transplanted patients are well with normal liver function. CONCLUSIONS: Early transfer to a specialized center, pre-emptive close monitoring, and intensive care and organ support with ventilation, CVVHDF, plasmapheresis and others may maximize their chances of spontaneous recovery, allow accurate prognostication and a timely liver transplant.

Portal Vein Embolization for Future Liver Remnant Enhancement and Combined Modality Treatment for the Management of Post-hepatic Resection Biliary Fistula in an 18-Month Old Child With Hepatoblastoma.

Hepatic resection is the mainstay of treatment for hepatoblastoma. However, the presence of adequate future liver remnant (FLR) is essential to prevent postoperative liver failure. Portal vein embolization (PVE) is commonly utilized in adults for promoting hypertrophy of FLR, however, it is sparingly used in children. Secondly, bile leak after liver resections is a well-defined complication. Apart from conservative treatment such as drainage and antibiotic, several management strategies including endoscopic, percutaneous, and surgical approaches have been described for its management. We present an 18-month old child with hepatoblastoma for whom PVE was performed to enhance the FLR so that an extended right hepatectomy could be accomplished. The same patient endured delayed postoperative biliary leak wherein the conservative, and non-operative interventional procedure failed, however, surgery combined with intraoperative interventional radiology procedure was utilized with a favorable outcome.

Mesenteric Panniculitis-First Case Series After Bariatric Surgery.

Mesenteric panniculitis is an uncommon pathology, of poorly understood etiology, characterized by progressive inflammation and fibrosis of the small bowel mesentery. This disease has been reported usually after other abdominal surgeries. We present two cases of young male patients who underwent laparoscopic sleeve gastrectomy and developed abdominal symptoms within 45-60 days of surgery. Both were investigated for known post-bariatric complications. While first patient presented (5 months later) at an irreversible stage and died within 8-9 months of primary surgery, in second patient, the disease process could be reversed through early intervention, diagnosis, treatment, and compliance. Mesenteric panniculitis is a rapidly progressive entity, which can be adequately treated by early identification and long-term immune-suppressive therapy.

Report of Mesocaval Shunt in the Management of Variceal Hemorrhage Following Gastric Bypass Surgery.

Gastrointestinal bleeding following Roux-en- Y gastric bypass (RYGB) is rare. We report an unusual cause of upper gastrointestinal bleeding following RYGB that was complicated by porto- splenic vein thrombosis. A 35- year- old woman was successfully treated by mesocaval surgical shunt for recurrent variceal hemorrhage in the excluded stomach following RYGB.

Interventional radiological treatment of hepatocellular carcinoma: an update.

Hepatocellular carcinoma is the commonest primary liver tumor and its incidence is on an increase.Transplantation and surgical resection are the gold standard curative treatment options but less than 20%patients are surgical candidates because of advanced liver disease and/or co-morbidities.Various interventional radiological procedures have been developed and intensively investigated for treatment of inoperable HCC.This review summarizes the various interventional radiological treatments in HCC including patient selection, procedural considerations and response evaluation. Transarterial chemoembolization, radioembolization and radiofrequency ablation are mainly discussed.