Original article—liver, pancreas, and biliary tract
Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia

https://doi.org/10.1016/j.cgh.2011.07.024Get rights and content

Background & Aims

Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and health perception among Dutch patients who survived 20 years after therapy and investigated whether the rate of transplant-free survival increases with time.

Methods

By using the Dutch national database for biliary atresia, we identified 104 patients, born between 1977 and 1988. We collected data on clinical characteristics, liver biochemistry, and ultrasonography from all transplant-free patients who were alive at age 20 years (n = 28; 27% of the patients). General health perception data (RAND-36) were collected at the last examination.

Results

The 20-year transplant-free survival rate increased from 20% (10 of 49) in the 1977 to 1982 cohort to 32% (18 of 55) in the 1983 to 1988 cohort (P = .03). Twenty-one percent of the long-term survivors (6 of 28) had normal liver biochemistry test results and no clinical or ultrasonographic signs of cirrhosis. The general health perception of female, but not male, patients, was lower, compared with controls (RAND-36 score, 54 ± 14 vs 74 ± 18; P = .005).

Conclusions

More than 25% of patients with biliary atresia survive at least 20 years without liver transplantation in The Netherlands. Women with biliary atresia have a reduced perception of their health, compared with control patients. Twenty percent of long-term survivors are symptom-free, without clinical or ultrasonographic signs of cirrhosis or portal hypertension.

Section snippets

Patients and Methods

Between January 1, 1977, and December 31, 1988, there were 106 patients who were diagnosed with BA in The Netherlands. The diagnosis of BA was confirmed in all patients by intraoperative cholangiography and pathology of the liver and biliary remnants. Surgery was performed in 1 of the 6 Dutch university medical centers that specializes in pediatric surgery. The short-term results of the majority of this cohort were described in 1989.9 The Dutch OLT program started in 1979 in adults and in 1982

Twenty-Year Survival After Surgical Correction

Of the 106 children (62 girls), 2 were lost to follow-up evaluation at the ages of 4 (address not found) and 6 years (moved abroad). Twenty-eight of the 104 patients (27%) survived for 20 years without OLT (Table 1). Sixteen of these patients were female (57%). The overall 20-year survival with and without OLT was 43% (45 of 104). OLT was performed in 23% (24 of 104) of patients before the age of 20. The survival rate after OLT was 71% (17 of 24). Fifty percent (52 of 104) of the patients died

Discussion

Since 1977, the transplant-free 20-year survival rate of BA patients treated with surgical correction has increased from 20% to 33% in The Netherlands. The 20-year survival rate was not significantly associated with the age at surgical correction. Interestingly, one fifth of the 20-year transplant-free survivors had no signs of cirrhosis and a normal liver biochemistry. Female, but not male, transplant-free BA patients had a lower GH when compared with a reference group.

The 20-year

Acknowledgments

The author are grateful to Dr A. P. van den Berg, Professor Dr J. P. H. Drenth, Dr K. J. van Erpecum, Dr R. J. de Knegt, and Dr J. Salemans for their valuable help in retrieving patient data. The authors also thank The Netherlands Study group of Biliary Atresia and Registry (NeSBAR): D. C. Aronson, J. H. Escher, L. W. E. van Heurn, H. A. Heij, A. Kindermann, C. M. F. Kneepkens, Z. J. de Langen, G. Madern, A. M. van den Neucker, P. M. J. G. Peeters, P. N. M. A. Rieu, J. J. M. Tolboom, and D. C.

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    Conflicts of interest The authors disclose no conflicts.

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