Download ORTHOTOPIC LIVER TRANSPLANTATION FOR BYLER`S

ORTHOTOPIC
LIVER
BYLER’S DISEASE
TRANSPLANTATION
FOR
Torri E, Lucianetti A, Pinelli D, Corno V, Guizzetti M, Maldini
G, Zambelli M, Bertani A, Melzi ML, Alberti D, Doffria E, Torre
G, Spada M, Gridelli B, Colledan M
Ospedali Riuniti di Bergamo, ISMETT, UPMC, Palermo, ITALY
Byler’s disease (BD) is a heterogeneous group of autosomal
recessive childhood cholestatic diseases of hepatocellular origin. It
has a rare incidence of l in 90000. It is characterized by cholestasis
often arising in the neonatal period and it leads to death, due to
liver failure, without liver transplantation (OLTx). Publishes data
on survival and post OLTx complications are limited. From
October 1997 through January 2004, 242 pediatric OLTx were
performed in 215 patients (pts) at “Ospedali Riuniti di Bergamo”.
BD was the indication in 12 pts, with age ranging from 0.7 to 13
years (median 1.32). We reviewed the data of these pts for
technical features of the OLTx, incidence and type of
complications, need for re-transplantation and patient and graft
survival. There were 8 males and 4 females with a median weight
of 10 kg (6-35). 7 Pts (58.3%) received a LLS graft (one of these
was a reduced LLS graft), 5 pts (41.6%) received a whole graft.
Median follow up was 670 days (3-2051). During the same period
no pts died while on the waiting list. Major surgical complication
requiring a reintervention occurred in 3 pts (25%) (Bowel
perforation, skin flap for necrotic lesions of the abdominal wall,
portal vein thrombosis). No thrombosis of the hepatic artery was
observed. Infections with positive blood cultures were diagnosed in
4 pts (33.3%). One patient (8.3%) had biliary anastomotic stenosis
successfully treated by percutaneous techniques. Four pts (33.3%)
had an episode of acute rejection that was successfully treated with
steroids. Two pts (16.6%) were retransplanted, one for portal and
caval thrombosis with a LLS graft and the other for primary
dysfunction with a right extended split graft. Both of them died in
the early post operative period after reOLTx, respectively for
sovra-hepatic vein thrombosis and for veno-enteric fistula. All the
other pts are currently alive with an actuarial pt and graft survival
of 83% at 1 yr and 83% at 5 yrs. Our results confirm that Tx is an
effective treatment for children with BD. Split liver technique
satisfies the need for grafts for these pts.