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Preterm Infants With Biliary Atresia: A Nationwide Cohort Analysis From The Netherlands

van Wessel, Daan B.E.*; Boere, Thomas*; Hulzebos, Christian V.; de Kleine, Ruben H.J.; Verkade, Henkjan J.§; Hulscher, Jan B.F.*Also on behalf of the Netherlands Study group of Biliary Atresia, Registry (NeSBAR)

Journal of Pediatric Gastroenterology and Nutrition: October 2017 - Volume 65 - Issue 4 - p 370–374
doi: 10.1097/MPG.0000000000001692
Original Articles: Hepatology

Objectives: Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BA has an unfavorable prognosis: <40% of patients are cleared of jaundice after Kasai portoenterostomy (KPE), 4-year transplant-free survival rate is 46% and the 4-year survival rate is ∼75%. Little is known on difficulties in diagnosis and the outcome of BA in preterm infants. We aimed to analyze the incidence and outcome of BA in preterm infants in the Netherlands.

Methods: Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first symptoms, and KPE and referral interval (first hospital to KPE). Outcome parameters were clearance of jaundice (COJ) and (transplant-free) survival. Data are presented as medians (ranges).

Results: Included 28 preterm infants (13 boys/15 girls) between March 1988 and December 2015. The incidence of BA was 1.06 of 10.000 preterm live births. Gestational age was 34.8 (27.3–36.9) weeks. Congenital anomalies were present in 11 of 28 (39%) infants. Time between first symptoms and KPE was 57 (9–138) days. Referral interval was 28 (8–86) days. Age at KPE was 70 (35–145) days. COJ was achieved in 23% of cases. Four-year transplant-free survival rate was 21%. Four-year overall survival was 61%.

Conclusions: BA has a higher incidence in the preterm population compared to the overall BA population. The outcome of BA in preterm infants is poor, regarding COJ and (transplant-free) survival. We speculate that timely recognition of BA-related signs and symptoms in preterm infants will improve prognosis.

*Department of Pediatric Surgery

Department of Neonatology, Beatrix Children's Hospital

Department of Hepato-Pancreatico-Biliary Surgery and Liver Transplantation

§Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.

Address correspondence and reprint requests to Daan B.E. van Wessel, BSc, Department of Pediatric Surgery, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, PO Box 30.001, 9700 RB Groningen, The Netherlands (e-mail:

Received 3 March, 2017

Accepted 12 July, 2017

UMCG Trial registry, registration number: 201600837.

The authors report no conflicts of interest.

© 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,