Paediatric Liver Transplantation: Life After Portoenterostomy in Biliary Atresia By Vicente Martinez-lbafiez,

Jose Boix-Ochoa, Jose Lloret, and Jesljs Broto Barcelona, Spain

l Seventy-five percent to 80% of patients with biliary atresia (BA) will be candidates for paediatric liver transplantation (PLTx) throughout the first 14 years of life. They form the main group of recipients in our Paediatric Liver Transplant Unit. CM 49 children transplanted, 21 (44%) had BA. These patients present particular features, average weight of 12 kg, mean age of 3 years, and severe malnutrition prior to PLTx, which distinguish them from other paediatric candidates. With the advent of PLTx, portoenterostomy (PE) has ceased to be the only recourse for treating the majority of patients with BA. Different factors converge in these patients: some, including haemorrhage and adhesions, may present technical difficulties, and others, such as infections (19% in this series) due to severe malnutrition and malabsorption and consequent chronic rejection (14% in this series), often lead to death in the postoperative period (33% in this series). BA is treated by all paediatric surgeons, but the overall success rate now depends not only on PE but also on PLTx. In an attempt to facilitate possible later PLTx in pts with BA, the authors as paediatric surgeons performing PE or PLTx present surgical modifications and emphasize the most important medical aspects conducive to the improved general status of these pts. Our aim was to establish guidelines for taking full advantage of PE while preventing posterior problems and facilitating future transplant surgery. Copyright 8 1992 by W.B. Saunders Company INDEX WORDS:

Biliary atresia; liver transplantation.

A

LTHOUGH the incidence of biliary atresia (BA) among Caucasians is low (1:15,000),1 it is an important disease in paediatrics because therapeutic results have not been satisfactory. The Kasai hepatic portoenterostomy has been generally used as the preferred procedure. 2-4 Because portoenterostomy fails in 75% to 80% of patients with BA,5-8this disease supplies the majority of candidates for paediatric liver transplantation (PLTx) throughout the first 14 years of life.9 Before the advent of PLTx, what was the procedure if first surgery failed? In some cases, an intestinal stoma was performed in an attempt to control bile secretion and establish prognostic factors; in others,

From the Department of Paediam’c Surgery, Hospital Infantil Valle Heb& Barcelona, Spain. Presented at the 38th Annual International Congress of the British Association of Paediam’c Surgeons, Budapest, Hungary, July 24-26, 1991. Address reprint requests to vicente Martinez-lbariez, MD, PQ Manuel Girona 86, 7-20, 08034 Barcelona, Spain. Copyright Q 1992 by W.B. Saunders Company 0022-3468/92/2707-0010$03.00/O 830

second surgery was performed and, finally, these patients were declared hopeless cases. Faced with the continuing challenge presented by BA in paediatric surgery, our pioneering PLTx programme was started in 1985.1° With PLTx, portoenterostomy has ceased to be the only opportunity for treating the majority of paediatric patients with BA. Survival in most patients with BA is merely extended by the Kasai operation and liver replacement ultimately becomes necessary. To improve their chances of receiving an organ graft, these children with Kasai failure should immediately be referred for transplantation.7 Not all patients have the same degree of end-stage liver disease because the onset cannot be predicted in the neonatal period and hepatic fibrosis may progress inexorably despite biliary reconstruction.” Therefore, failure of surgery is probably the end product of a destructive inflammatory process that results in fibrosis of the biliary tract.’ Some known prognostic factors include: age at Kasai procedure before 60 days of life, correct hepatic hilar dissection, good bile excretion, minimum hepatic fibrosis at the time of biliary reconstruction, preexisting liver damage, and prevention of recurrent cholangitis.i1J2 Consequently, the definitive prognosis depends on the severity and reversibility of the intrahepatic process.6 Particular features distinguish these BA patients from other paediatric candidates for PLTx and include: average weight, mean age, malnutritional status, and previous surgery at the time of transplant. At present, 6 years after starting the PLTx programme, we have operated on more than 50 children (44% BA) and have obtained sufficient experience to be able to suggest improvements in medical, surgical, and social aspects for PLTx in these particular candidates. MATERIALS AND METHODS Fifty-six BA patients were studied as candidates for PLTx. Seven died while on the waiting list from infection and multiorganic failure. Of 48 children transplanted, 21 with ages ranging from 8 months to 9 years and weighing 4 to 34 kg had BA (44%). Fifteen of these had previously undergone Kasai. Two patients had the more complicated procedures in which the skin jejunostomies had been constructed and later closed. A further two patients had undergone portoduodenostomy. No anatomical alterations were observed in a patient who had previously undergone a theoretical Kasai and a further patient had had no surgery prior to PLTx. JournalofPediatric

Surgery,

Vol27, No

7 (July), 1992: pp 830-832

LIVER TRANSPLANT

Table 1. Mortali,

831

AFTER PORTOENTEROSTOMY

Survival, Morbidity, and Existing Roux Limbs

Reutilized in BA Transplanted

Patients

21 BA transplanted Died 7 (33%) 4 infection (19%) 3 chronic rejection (14%) Survived 14 (67%) Malnutrition 100%

(< 2SD

66%)

Hard hepatic-intestinal adhesions 100% Reused existing Roux limbs 11/17

The Starzl procedure patients.‘“,13

for PLTx was performed

in all these

RESULTS Of 21 BA transplanted patients, 7 died (33%), although none during surgery or in the immediate postoperative period. These seven patients had a mean age of 13.5 months and an average weight of 9 kg when transplanted. Four patients died of infection (19%) and three died of chronic rejection (14%) while on the waiting list for retransplantation. After analysis of these data (Table l), several significant medical and surgical aspects should be pointed out. Medically, general malnutrition was observed in all patients referred for PLTx with a very low heightweight curve (66.6%

Paediatric liver transplantation: life after portoenterostomy in biliary atresia.

Seventy-five percent to 80% of patients with biliary atresia (BA) will be candidates for paediatric liver transplantation (PLTx) throughout the first ...
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