Eur J Pediatr Surg
DOI: 10.1055/s-0041-1723994
Original Article

Centralization of Biliary Atresia: Has Germany Learned Its Lessons?

Omid Madadi-Sanjani*
1  Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
David Fortmann*
1  Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
2  Department of Pediatric Surgery and Pediatric Urology, Goethe University Frankfurt /M., Germany
,
Burkhard Rodeck
3  Department of Pediatric Gastroenterology, Christliches Kinderhospital Osnabrueck, Osnabrueck, Germany
,
Ekkehard Sturm
4  Department of Paediatric Gastroenterology and Hepatology, University Hospital for Children and Adolescents, University of Tuebingen, Baden-Württemberg, Germany
,
Eva-Doreen Pfister
5  Division of Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany
,
Joachim F. Kuebler
1  Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
,
Ulrich Baumann
5  Division of Pediatric Gastroenterology and Hepatology, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany
6  Liver Unit, Birmingham Women’s and Children’s Hospital, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham
,
Peter Schmittenbecher
7  Department of Pediatric Surgery, Municipal Hospital, Karlsruhe, Baden-Wuerttemberg, Germany
,
Claus Petersen
1  Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany
› Author Affiliations

Abstract

Introduction The majority of pediatric surgeons and hepatologists recommend the centralization of biliary atresia (BA) treatment within experienced liver units. We aimed to investigate whether voluntary self-restriction and acceptance of the need for this change in practice changed the BA referral policy in Germany during the last decade.

Materials and Methods In cooperation with pediatric surgeons, gastroenterologists or hepatologists, and pediatric liver transplant units, the 2-year follow-up data of infants with BA born in Germany between 2010 and 2014 were collected using www.bard-online.com or pseudonymized data transfer. Results were compared with our previous analysis of the outcome data of infants with BA born between 2001 and 2005 in Germany.

Result Overall, 173 infants with BA were identified, of whom 160 underwent Kasai portoenterostomy (KPE; 92.5%) and 13 (7.5%) underwent primary liver transplantation at 21 German centers. At 2-year follow-up, overall survival was 87.7% (vs. 81.9% in 2001–2005 [p = 0.19]), survival with native liver post-KPE was 29.2% (vs. 22.8% in 2001–2005 [p = 0.24]), and jaundice-free survival with native liver post-KPE was 24.0% (vs. 20.1% in 2001–2005 [p = 0.5]). Compared with the 2001–2005 analysis, all criteria showed improvement but the differences are statistically not significant.

Conclusion Our observation shows that KPE management requires improvement in Germany. Centralization of BA patients to German reference liver units is not yet mandatory. However, European and national efforts with regard to the centralization of rare diseases support our common endeavor in this direction.

* Equal contribution.




Publication History

Received: 27 October 2020

Accepted: 04 January 2021

Publication Date:
04 March 2021 (online)

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