Techniques and Outcomes of Transjugular Intrahepatic Portosystemic Shunting in Infants with Budd-Chiari Syndrome.

PURPOSE: To describe the technical aspects, feasibility, and outcomes in children with Budd-Chiari syndrome (BCS) undergoing transjugular intrahepatic portosystemic shunt (TIPS) creation during infancy. MATERIALS AND METHODS: A retrospective review of infants with BCS undergoing TIPS creation between January 2012 and December 2018 was performed. Eight infants (5 males) underwent TIPS creation (7 for refractory ascites and 1 for refractory variceal bleeding) during the study period. The median age at TIPS creation was 10.5 months (range, 8-16 months). The median elapsed time between presentation and TIPS creation was 6.5 months (range, 0-13 months). The median weight and median pediatric end-stage liver disease score of the infants at the time of TIPS creation were 6.7 kg (range, 5.4-10 kg) and 13 kg (range, 8-18 kg), respectively. RESULTS: TIPS creation was successful in all patients. There were no immediate postprocedural complications. An 18-gauge hollow needle was manually curved, through which a 21-gauge Chiba needle was inserted to access the portal vein. All patients received 1 or 2 overlapping bare metal stents. One patient was lost to follow-up after the procedure. The median follow-up duration was 32 months (range, 14-51 months). Four of 7 infants needed reintervention. Two children died during the follow-up period. Two children successfully underwent living donor liver transplant, whereas the remaining 3 children were asymptomatic at the follow-up. CONCLUSIONS: TIPS creation was found to be safe and efficacious in improving portal hypertension and growth in these children, although, with a higher rate of reinterventions, possibly due to the use of small, bare metal stents.

Positional outflow obstruction as a cause of early refractory ascites post-pediatric living donor liver transplantation.

Refractory ascites post-liver transplantation can be a challenging problem. Causes of refractory ascites include venous outflow anastomotic stenosis, vessel kinking by the regenerating liver, pre-existing graft disease, and positional outflow obstruction. We present a case report of a child presenting with high drain output and refractory ascites post-LDLT secondary to a positional kinking. Repeating the Doppler studies with patients both supine and sitting may be helpful.

Preoperative successful thrombectomy and thrombolysis of acute extensive splanchnic venous system and TIPSS thrombosis in a child with Budd-Chiari syndrome-Creating a window to enable living donor liver transplantation.

Preoperative extensive PV thrombosis can pose a technical challenge during liver transplantation surgery. Several strategies adopted to mitigate this problem include creation of a superior mesenteric vein-PV jump graft, use of a polytetrafluoroethylene graft, renoportal anastomosis, or cavoportal hemitransposition. Extensive and diffuse thrombosis of the splanchnic venous system may even necessitate multivisceral transplantation. We describe the case of a pediatric patient with Budd-Chiari syndrome and decompensated cirrhosis, who developed extensive thrombosis of the porto-spleno-mesenteric venous system prior to liver transplantation. We used a combination technique of thrombus aspiration by a novel trans-TIPPS approach followed by thrombolysis. Complete preoperative resolution of the extensive thrombosis was achieved. This allowed the creation of a brief window to enable planned LDLT. In prudently selected patients, performing an early mechanical and chemical thrombolysis of an extensive acute splanchnic venous thrombosis can thus help expedite a planned LDLT.

Therapeutic coil embolization of dominant shunt in hepatopulmonary syndrome enhances post-liver transplant respiratory recovery.

Coil embolization of the atypical enlarged pulmonary artery/arteriole with visible shunting may improve hypoxemia in patients with hepatopulmonary syndrome (HPS). When used selectively in cases with large shunts, either pre- or post-liver transplantation (LT), it can aid an early recovery and reduce morbidity. We present a case where a large intrapulmonary shunt was embolized preoperatively to improve hypoxemia associated with HPS and enhance post-operative recovery.

Major hemobilia--experience from a specialist unit in a developing country.

BACKGROUND AND AIM: Hemobilia is a rare but potentially life threatening problem, which can be difficult to diagnose and treat. In the last few decades there has been a change in the etiologic spectrum and management of this problem in the West. The aim of this study was to analyze the etiology, clinical features, management and outcome of major hemobilia in a tertiary referral centre from western India. METHODS: A retrospective analysis was undertaken on 22 patients (16 males, 6 females; mean age 39 years, range 13 to 74) who presented with major hemobilia over a 5-year period. RESULTS: The etiology was iatrogenic in 13 patients (percutaneous transhepatic biliary drainage 8, post laparoscopic cholecystectomy 3, endoscopic retrograde cholangiopancreatography 1, and liver biopsy 1), liver trauma in 6 and liver tumors in 3 patients. Twenty patients presented with gastrointestinal bleeding (melena 20 patients, hemetemesis with melena 8 patients), 5 with jaundice and 8 had fever. Abdominal angiography was performed in 20 patients. Angiography revealed pseudoaneurysm of the right hepatic artery or its branches in 14 patients, left hepatic artery in 2, an arterio-biliary fistula in 1, tumor blush in 1 and the source could not be located in 2 patients. Seventeen of the 22 patients were treated with radiological intervention, 3 required surgery (liver resection for tumors 2, laparotomy for venous collateral bleeding of portal cavernoma 1) and two were managed conservatively. Radiological intervention involved embolisation with coils and/or glue in 16, and chemoembolisation in 1 patient. Sixteen of 17 patients responded to embolisation. Overall there were two deaths. CONCLUSION: The spectrum of hemobilia seen in India is now similar to that in the developed world with iatrogenic causes being the commonest. Interventional radiology can treat a majority of patients reducing the need and morbidity associated with surgery.

Budd-Chiari syndrome in children: experience with therapeutic radiological intervention.

OBJECTIVES: Budd-Chiari syndrome (BCS) in children is not uncommon. Published literature on therapy for this condition is scarce. We therefore attempted radiological interventions in these patients to determine their efficacy and safety. PATIENTS AND METHODS: Fourteen of 16 children with a median age of 22 months diagnosed as having BCS were subjected to an inferior vena cava/hepatic venogram with the aim to establish a normal antegrade flow in at least 1 hepatic vein (HV). RESULTS: A normal antegrade flow in at least 1 of the HVs could be established in 11 children. Three patients had angioplasty of the HV (vein size or=5 mm), and 6 had transjugular intrahepatic porta systemic shunt ([TIPSS] total occlusion of all 3 HVs or veno-occlusive disease). The youngest child undergoing a successful stenting was 7 months of age and the child undergoing TIPSS was 3 years of age. One patient had reversal of fulminant liver failure following a successful TIPSS. Postprocedure, 2 patients developed reversible encephalopathy and 1 had a neck hematoma. There was no procedure-related mortality. The procedure was successful in both patients with stenting (100%), 5 of the 6 patients with TIPSS (80%), and only 1 of the 4 patients (25%) with angioplasty. The median follow-up was 31 months. CONCLUSIONS: Radiological therapeutic intervention is feasible and safe in children with BCS. The overall results of stenting/TIPSS are better than with angioplasty; however, long-term results of these interventions need to be evaluated.

Chronic abdominal pain--a radiological solution.

Chronic mesenteric ischaemia is not an uncommon disorder. It is associated with high morbidity and mortality. It presents with chronic abdominal pain and the diagnosis is often missed because of nonspecific clinical findings and limitations of diagnostic studies. Although surgery has been considered to be the mainstay of treatment, it is associated with significant morbidity. We report two cases of chronic mesenteric ischaemia managed effectively with endovascular therapy with no morbidity and good long term pain relief.

Successful treatment of extensive splanchnic vein thrombosis in a patient with mycosis fungoides.

A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.

Recanalization of TIPSS via transsplenic puncture.

Transjugular intrahepatic portosystemic shunt (TIPSS) has important role in the management of Budd-Chiari syndrome (BCS). Stent dysfunction rate up to 80% at 2 years limits the use of TIPSS. Reintervention is required if shunt develops occlusion or stenosis. Different techniques have been reported for the catheterization of TIPSS in cases of an occluded stent. In those failing a standard transvenous approach, the Colapinto needle technique, a combined transvenous transhepatic approach or transplenic approaches have been described. Here, we report our experience in a challenging TIPSS recanalization using a transsplenic approach.

Ruptured Hepatocellular Carcinoma in a Child with Budd-Chiari Syndrome.

BACKGROUND: Hepatocellular carcinoma is an uncommon complication described in patients with Budd-Chiari syndrome. CASE CHARACTERISTICS: A 12-year-old boy with Budd-Chiari syndrome, who was earlier treated with Transjugular intrahepatic porto-systemic shunt (TIPS), presented with acute onset hemoperitoneum and hypotension. OUTCOME: It was diagnosed to be a case of ruptured hepatocellular carcinoma. MESSAGE: Successful TIPS may not prevent the development of hepatocellular carcinoma, and children with Budd Chiari syndrome should be monitored for the same.

Long-term clinical outcome of Budd-Chiari syndrome in children after radiological intervention.

OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon cause of chronic liver disease in children. The literature on the management of pediatric BCS is scarce. Our aim was to determine the long-term outcome of patients undergoing a radiological intervention for the treatment of BCS. METHODS: Thirty-two children diagnosed with BCS between 2004 and 2014 were included. Data on the course of disease, medical management, response, and complications related to radiological interventions and outcome were collected. MAIN RESULTS: Twenty-five patients who were on regular follow-up were analyzed. The median age of the patients at presentation was 9 months (4.5-214). Sixteen patients initially received anticoagulation alone. This was associated with a high failure rate of 66%. Twenty patients underwent a radiological intervention in the form of angioplasty (n=7), hepatic vein stenting (n=3) or transjugular intrahepatic portosystemic shunt (TIPS) (n=14). Success with angioplasty was achieved in 43% of cases. Hepatic vein stenting was successful in 66%, whereas TIPS was successful in 72% of cases. TIPS was feasible in all patients. The median follow-up duration was 44 months (5-132). Four patients developed hepatopulmonary syndrome after a median period of 3 years (1.5-5.25) and one patient developed hepatocellular carcinoma. CONCLUSION: BCS commonly presents during infancy. Anticoagulation alone and angioplasty of the hepatic veins are associated with a high failure rate. Hepatic vein stenting or TIPS is feasible and efficacious in improving liver function, portal hypertension, and growth. It is associated with good long-term outcome and delays the need for liver transplantation, but may not prevent complications such as hepatopulmonary syndrome and hepatocellular carcinoma.

Stent angioplasty of narrowed portocaval shunt in Budd Chiari syndrome: a case report.

BACKGROUND: Hepatic vein thrombosis (Budd-Chiari Syndrome) is a rare disorder resulting from an obstruction to the outflow of blood from the liver. Early decompression is needed to prevent liver dysfunction and death. Radiological intervention includes angioplasty of stenosis and webs and the placement of transjugular intrahepatic portosystemic shunts (TIPPS). Side-to-side portacaval shunt (SSPCS) remains the gold standard for achieving good long-term results. CASE PRESENTATION: A 37-year old lady underwent side-to-side portacaval shunt for Budd Chiari syndrome. She had early shunt blockage and this was successfully treated with the placement of a metallic stent across the shunt. CONCLUSION: At five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.