Transjugular intrahepatic portosystemic shunt (TIPS) and portal hypertension by different causes: Our initial experience on children

Abstracts / Digestive and Liver Disease 40 (2008) A41–A118

and mild steatosis, ductular proliferation, portal and perivenous fibrosis, with porto-portal fibrous bridgings in the girl. Genetic analysis of SDBD gene revealed the same two mutations of exon 2 (258+2T > C and 183–184 TA > CT) in both children, confirming the diagnosis of SDS. Both children were treated with pancreatic enzymes, and liposoluble vitamins supplementation and ursodeoxycholic acid (30 mg/kg) with improvement of clinical conditions and decrease of aminotransferase activity to AST 1.5 × N and ALT 2 × N. as well for the patients with a longer follow up. Conclusion. Patients with SBD syndrome may present with a liver disease and with few clinical element suggesting the diagnosis. Liver damage in patients with SDS is characterized by inflammatory bile duct damage and early fibrosis which may be severe. SBD syndrome should been included in the etiologic spectrum of crhronic cryptrogenic elevation of liver enzymes doi:10.1016/j.dld.2008.07.297 PA65 EXTRAHEPATIC BILIARY ATRESIA IN 71 CHILDREN: A SINGLE CENTRE EXPERIENCE M. Fornaro a , M. Candusso a , P. Stroppa a , M. Bravi a , V. Quadri a , V. Casotti a , M. Colusso b , S. Camagni b , A. Lucianetti a , M. Colledan a , D. Alberti b , G. Torre a a

Pediatric Transplant Unit, OORR Bergamo, Italy Surgery, OORR Bergamo, Italy

b Pediatric

Early diagnosis, surgical treatment, medical therapy and timing for liver transplant (OLTx) still represent the main challenges in extrahepatic biliary atresia (EHBA), a rare but severe disease. We retrospectively analysed the clinical course of 71 children who underwent surgery for portoenteroanastomosis at our Centre in Bergamo, in a 7-year period (2000–2007). The specificity of the Centre is the presence of a multidisciplinary team, in which paediatric surgeons, paediatricians, transplant surgeons and pathologists take care of any child affected by liver disease, especially if EHBA is suspected. For our intervention protocol, if EHBA is suspected, percutaneous or surgical liver biopsy is obtained, before Kasai operation; after intervention, we usually suggest steroids for 4 months and antibiotic prophylaxis for the first yr. At discharge, the child is admitted for follow-up at the Paediatric Liver Unit, to correctly and early define risk factors and eventually precocious indications for OLTx. In the study period, 10 Kasai’s operations were performed each year in 71 children (51 coming outside Lombardia); the mean age at surgery was 69.23 days (S.D. 4.2, r 22–133 days); survival rate is 100%. Listing for OLTx has been activated for 30 pts, 28 out of these underwent transplant, 26 in Bergamo, 2 in other Centres (1 in Italy, 1 out of Italy); 43 pts are still on follow-up, 34 out of them in our Unit. The mean age at listing was 8 months (S.D. 15.6, r 4–62), the mean weight 8.9 kg (S.D. 4.46, r 4–18.3) and the mean bilirubin level was 12.3 g/dl (S.D. 7.3); portal hypertension (60.7%), malnutri-

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tion (42%), hepatopulmonary syndrome (7%) and nodules (7%) were the most frequent indications for list, being often associated; enteral feeding was initiated in 39.2% of cases before list procedure. Transplant was performed in 26 patients in Bergamo at a mean age of 16.6 months (S.D. 16.2, r 6–62; median value 9 mo), after 2.4 mo. waiting time; survival rate is 96.4% at a 30.8 (S.D. 3.5) month mean follow-up time. Complications after Oltx are the following: biliary stenosis 54%, acute rejection 42.3%, chronic rejection 30.7% (after 28 mo on average), PTLD in 11.5% of cases; re-transplantation was required in 11.5% of cases (3 pts): for hepatic artery thrombosis in 2 cases, for chronic rejection in 1. For patients still on follow-up after Kasai operation at our centre, 2 are on list, 32 have now a mean age of 29 months (S.D. 20.6) for 26.8 mo follow-up; the mean weight of the children is 12.8 kg (S.D. 5.3); their bilirubin level is now 0.7 mg/dl (±0.35), ␥GT 222.6 (±199) UI/ml; during the follow-up, at least one cholangitis was detected in 35.2%, portal hypertension is reported in 26.4% with ascites in 5.8% and varices in 8.8%. The data refer to the experience in EHBA in a single Italian Centre; the most important result seems to be the low incidence of transplants in this series (39.4%), even if the relative short duration of follow-up could represent a misleading measure. The low incidence of complications after porto-enteroanastomosis underlies the absolute need to perform it, if EHBA is suspected in order to avoid or delay time for transplant. Complications related to transplant procedure are similar to those reported by other Centres, and in our experience on transplant for EHBA (n = 175 pts) survival rate in these babies is 96%. doi:10.1016/j.dld.2008.07.298 PA66 TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT (TIPS) AND PORTAL HYPERTENSION BY DIFFERENT CAUSES: OUR INITIAL EXPERIENCE ON CHILDREN M. Candusso, P. Stroppa, M. Bravi, V. Quadri, M. Colledan, R. Agazzi, G. Torre Transplant Paediatric Unit and Radiology Department, Ospedali Riuniti, Bergamo, Italy Portal hypertension and variceal bleeding are frequent complications of hepatic cirrhosis; in adults, TIPS could restore a normal portal flow thus limiting the bleeding risk. Experience in children is lacking, due to the difficulty to obtain adequate size devices, for portal vein dimension, or necessity to perform TIPS in general anaesthesia. We report our initial experience, started on 2005, of TIPS insertion in portal hypertension (PH) in 4 children. Case 1. A 4-year old girl (14 kg) underwent liver transplantation (OLTx) for extrahepatic biliary atresia at the age of 10 months; by the age of 3 years, repeated episodes of intestinal bleeding occurred, due to portal thrombosis

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and subsequent gastrointestinal hypertension. Vanishing duct syndrome as an associated feature for biliary stenosis after (OLTx) and the recurrence of severe intestinal bleeding led to consider the opportunity of TIPS insertion, at least as bridging to re-tranplant. A 8 mm stent was inserted without any technical or metabolic complication; at a 3 year follow-up, the child is doing well. Case 2. In a 10-year boy (26 kg) affected by PH in cystic fibrosis, esophageal varices and hypertensive gastropathy were diagnosed, no acute bleeding episodes occurred but repeated varices sclerosing and ligation were performed. His FEV1 was between 50 and 70%, with a progressive loss (−20% in a 2-year period), eventually indicated the need for lung Tx in the future. After a short follow-up time, TIPS was inserted in order to treat the most important symptoms due to PH, and as a possible bridge to both liver and lung Tx. The procedure was uneventfully, clinical condition at 1 yr follow-up are still stable, the flow through TIPS is normal, even if sometimes he shows high ammonia levels, a previous recorded data. Case 3. A 6-year old girl (kg), affected by autosomal recessive policystic renal disease and congenital hepatic fibrosis, required bilateral nefrectomy at the age of 2 and renal Tx at the age of 3 yrs, but later slight renal insufficiency and recurrent ascites occurred. At the age of 3, TIPS was elsewhere inserted, but the course was complicated by reduced flow through TIPS and obstruction during restoring procedures, with subsequent severe and repeated bleeding episodes, often requiring intensive support and leading to acute renal failure. In relation to the evidence of a new TIPS obstruction, the child was referred to our Centre to be evaluated for a combined liver and kidney Tx. We decided to insert a coated TIPS inside the previous one; after stenosis dilatation, a 8 mm × 10 cm stent was positioned in the lumen of the pre-inserted TIPS, without any acute complication. A normal flow through device was demonstrated 1 yr later, and the child was still on good clinical condition with no signs of liver decompensation. Combined liver and kidney Tx is likely, however in the next future. Case 4. A 10-year girl (37 kg) developed acute Budd Chiari syndrome in polycytemia JAK2 associated, a well known condition in adults but extremely rare in children. She was referred to our Centre to be evaluated for OLTx, but TIPS was the preferred option for the large experience in adults, the absence of liver failure and the presence of a primary haematological disease which required a specific therapy (hydroxyurea and/or bone marrow Tx). At procedure, a severe portal hypertension with inverted flow in both splenic and mesenteric vessels was found. The complete thrombosis of sovrahepatic veins confirmed the suspected diagnosis and two coated stents were inserted through the left portal vein and inferior cava vein thus restoring a normal flow and treating ascites and hyperammoniemia. At follow-up, she is doing well on hydroxyurea. Conclusions. TIPS is a safe technique in children as well as adults; our experience is limited but indications are increasing. It appears to be useful to treat portal hypertension in

children, whatever the cause, at least as bridging procedure to OLTx and/or to more complex ones (i.e. combined Tx) and as an alternative to surgical porto-systemic shunt, if feasible. doi:10.1016/j.dld.2008.07.299 PA67 LIVER TRANSPLANTATION IN ADOLESCENT: A SINGLE CENTRE EXPERIENCE E. Cama, N. Lopez, F. Di Francesco, D. Cintorino, M. Spada, S. Riva, M. Sciveres, P. Catalano, G. Maggiore, D. Biondo, B. Gridelli Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione-University of Pittsburgh Medical Center in Italy, Palermo, Italy Adequate size matching for liver transplantation (LTx) in adolescent using standard criteria for graft to recipient matching can be difficult. Whole livers (WL) or extended right grafts (ERG) are often oversized, while left lateral segments (LLS) do not satisfy minimal criteria of liver mass requirement. Patients and methods. From July 2003 to April 2008, 83 liver transplants were performed in 71 patients in paediatric age (68 children and 15 adolescents): 16 from whole livers, 13 from extended right grafts, 54 from left lateral segments. Adolescent patients had a median age of 10 years (range 9–15) and median body weight of 25 Kg (range 24–51 kg). Mean waiting time was of 1.32 months (range 0–6 mo). Six of them underwent primary LTx with WL from young cadaveric donors (median age 12.1 years, median body weight 40 Kg).and 3 recipients received a LLS harvested from a 13 and 49 yrs cadaveric donor with a GRWR of 1.0% and 0.6% respectively. In 7 cases we used extended right grafts harvested from young donors (mean age 11 years, range 5–20) obtaining grafts of mean weight 683 gr (range 380–880). Results. After a median follow up of 22 months patient and graft survival for adolescent patients were 92.86% and 76.47%, below 9 years were 82.91% and 71.24% respectively. Between adolescent patients 3 underwent retransplantation: 1 for hepatic artery thrombosis, 1 for hepatic artery embolization performed to control a massive haemorrhage secondary to arterobiliary fistula and 1 for delayed graft function. Conclusions. The use of a liberal policy of liver splitting allowed us to satisfy the need of LTx in adolescent population. doi:10.1016/j.dld.2008.07.300