Shaji Marar

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 ≤4 mm), 2 underwent HV stent placements (vein size ≥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.

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

BackgroundHepatic 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 presentationA 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.ConclusionAt five years, she remains asymptomatic, with normal liver functions, no ascites, and normal flow through the stent on Colour Doppler examination.

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.

Ruptured hepatocellular carcinoma in a child with Budd-Chiari syndrome

BackgroundHepatocellular carcinoma is an uncommon complication described in patients with Budd-Chiari syndrome.Case characteristicsA 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.OutcomeIt was diagnosed to be a case of ruptured hepatocellular carcinoma.MessageSuccessful TIPS may not prevent the development of hepatocellular carcinoma, and children with Budd Chiari syndrome should be monitored for the same.

Anticoagulation and Budd-Chiari syndrome

To the Editor, We read with interest the article by Shukla and Bhatia [1] in a recent issue of the Journal. The authors found that 6 of their patients could not undergo the standard radiological procedures because of technical reasons; reasons for this technical failure were however, not provided. In our experience of adult patients, the TIPSS procedure is usually possible. Of 58 patients who underwent TIPSS, we faced technical difficulty in only one patient who had acute Budd-Chiari syndrome; the massive hepatomegaly caused an increase in distance between the IVC and narrowed portal vein (unpublished data). Technical difficulties usually arise in infants when the vein size is too small for stenting. In 14 children with Budd-Chiari syndrome, we could perform radiological intervention successfully in 11; the procedure failed due to small vessel size in 3 patients [2]. The authors found that those with more severe disease did not do well in the long term with anticoagulation alone. These are precisely the patients who benefit with radiological intervention [3]. We have successfully done TIPSS in a fouryear-old child with Budd-Chiari syndrome who presented with fulminant liver failure [4]. The use of anti-coagulation alone in the treatment of Budd Chiari syndrome is a compromise rather than an alternative treatment.

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.