Outcomes of Liver Transplantation During Adulthood After Kasai Portoenterostomy Due to Biliary Atresia

doi:10.1016/j.transproceed.2015.09.058

Transplantation Proceedings

Volume 47, Issue 9, November 2015, Pages 2643-2644

https://doi.org/10.1016/j.transproceed.2015.09.058 Get rights and content

Highlights

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We present 100% survival rate of LT patients after Kasai procedure during adulthood.
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BA native liver survival of 19.01 years allowed LT during adulthood.
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The delay of LT to adulthood in BA patients does not increase mortality.

Abstract

Biliary atresia (BA) is a neonatal progressive cholangiopathy of unknown etiology and one of the most common reasons for liver transplantation (LT) in children. Kasai portoenterostomy (KP) improves survival of the native liver, although LT remains the only ultimate treatment. In some cases KP makes it possible to defer the ultimate LT until adulthood. We report our experience regarding 5 cases of BA treated with LT during adulthood. KP was performed in all patients at an average age of 176 days (range, 60–280), which allowed an average survival of the native liver of 19.01 years (range, 14.06–22.32). Five-year survival rate was 100%. Ten-year survival rate did not reach 100% because of a death 9.55 years after LT due to chronic graft rejection, in a patient who was already prepared for a new LT. Our results corroborate that KP remains the first-line treatment of BA. Early performance of the KP provides children with the best chance of survival, allowing the delay of the LT to adulthood. LT during adulthood in these patients achieves good post-LT survival rate; we have not found any data regarding this group of patients in the literature.

Section snippets

Methods

Records of patients treated with LT due to BA from 1990 to 2014 at Hospital Universitario Virgen del Rocio of Sevilla, Spain were reviewed.

Demographics, KP timing, reason for LT, LT date, native liver survival, LT technique, outcomes, complications, immunosuppressors treatment, morbidity, and mortality were recorded (Table 1). Donor characteristics were recorded as well.

Results

Five patients, 3 women and 2 men, with an average age of 19.51 years (range, 14.23–22.90) were treated with LT due to BA during their adulthood from 1990 to 2014 at Hospital Universitario Virgen del Rocio of Seville, Spain.

KP had been performed in all of them at an average age of 176 days (range, 60–280), with an average survival of the native liver of 19.01 years (range, 14.06–22.32).

The reason for LT was recurrent cholangitis in 2 cases and secondary biliary cirrhosis in the other 3; all of

Discussion

In specialized institutions, reported overall survival in BA patients who underwent a KP followed by LT if needed ranges from 66% to 100% [2]. These results match our experience where overall survival rate was 100%. The conventional approach for BA is Kasai operation first and LT if needed due to failure of the KP to re-establish biliary flow or because of complications of cirrhosis [5].

The actuarial 5-year post-LT survival rate is 82.1% in the United Network for Organ Sharing registry (1549 LT

References (8)

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E. Utterson et al.
Biliary atresia: clinical profiles, risk factors, and outcomes of 755 patients listed for liver transplantation
J Pediatr
(2005)
C. Chardot et al.
Improving outcomes of biliary atresia: French national series 1986-2009
J Hepatol
(2013)
R.J. Sokol et al.
Pathogenesis and outcome of biliary atresia: current concepts
J Pediatr Gastroenterol Nutr
(2003)

There are more references available in the full text version of this article.

Cited by (5)

Living donor liver transplantation for more than 30-year survived patients with native liver after Kasai operation for biliary atresia
2020, Transplantation Reports
Citation Excerpt :
All patients were alive and overall graft survival rate was 100% at a follow-up period of 65 (range, 12 - 92) months after LDLT. Both of living and deceased donor liver transplantation have been reported for liver failure after Kasai operation in adulthood (Table 2) [1–5]. Reported 5-year graft survivals in adult liver transplantation after Kasai operation were 69.7 - 100% [1,2,5].
A number of patients have developed liver failure after the Kasai operation for biliary atresia (BA), even after long-term postoperative course. We report our experience regarding four cases of biliary atresia required and treated with living donor liver transplantation (LDLT) during adulthood.
All four patients underwent Kasai operation for BA from 1974 to 1983 and had been followed-up for more than 30 years in our institute. Then, they developed liver failure and treated with LDLT. These four patients’ records were reviewed retrospectively. All data are described using the median value.
Three female and one male were included. Kasai operation was performed at the age of 61 days. The type of biliary obstruction was type III-b1-ν in all. The survival period of the native liver was 440 months. The age at LDLT was 37 years. The reasons of deterioration of native liver were recurrent cholangitis in two and pregnancy and delivery in other two. The age of living donor was 40 years. Right liver grafts were used in all. Percentage of the real graft volume per standard liver volume was 47%. All patients were alive and overall graft survival rate was 100% at a follow-up period of 65 months after LDLT.
Even after more than 30 year-survival with native liver after Kasai operation, liver failure may be caused by recurrent cholangitis or pregnancy and delivery. LDLT may be effective treatment for those adult patients if an adequate living donor is available.
Biliary Atresia
2020, Pediatric Liver Transplantation
Biliary atresia is a uniquely perinatal disease that 30 years ago would have been universally fatal. With the development of the palliative Kasai portoenterostomy (Morio Kasai, 1922–2008), along with advances in pediatric liver transplantation (first performed by Thomas Starzl, 1926–2017), survival into adulthood and long-term quality of life are excellent. Biliary atresia is the main indication for liver transplantation in pediatric patients.
Extended adhesion-sparing liver eversion during kasai portoenterostomy for infants with biliary atresia
2021, Children
Bowel perforation after liver transplantation for biliary atresia: a retrospective study of care in the transition from children to adulthood
2017, Pediatric Surgery International
Liver transplantation for biliary atresia in adulthood: Single‐centre surgical experience
2021, Journal of Clinical Medicine

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9th Congress of the Andalucian Transplantation SocietyLiver transplantationOutcomes of Liver Transplantation During Adulthood After Kasai Portoenterostomy Due to Biliary Atresia

Highlights

Abstract

Section snippets

Methods

Results

Discussion

Gastroenterology

J Pediatr

J Hepatol

Pathogenesis and outcome of biliary atresia: current concepts

J Pediatr Gastroenterol Nutr

9th Congress of the Andalucian Transplantation Society
Liver transplantation
Outcomes of Liver Transplantation During Adulthood After Kasai Portoenterostomy Due to Biliary Atresia