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HPB (Oxford). 2019 Jul 29. pii: S1365-182X(19)30612-4. doi: 10.1016/j.hpb.2019.07.001. [Epub ahead of print]

Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries.

Author information

1
Pediatric Liver and Gut Research Group and Section of Pediatric Surgery, Helsinki University Children's Hospital, Helsinki, Finland.
2
Section of Pediatric Surgery, Oslo University Hospital, University of Oslo, Oslo, Norway.
3
Section of Pediatric Surgery, Queen Silvia Children's Hospital, Gothenburg, Sweden.
4
Section of Pediatric Surgery, Tampere University Hospital, Tampere, Finland.
5
Section of Pediatric Surgery, Rigshospitalet, Copenhagen.
6
Surgical Department A, Odense University Hospital, Odense, Denmark.
7
Section of Pediatric Surgery, Skåne University Hospital, Lund University.
8
Section of Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.
9
Pediatric Liver and Gut Research Group and Section of Pediatric Surgery, Helsinki University Children's Hospital, Helsinki, Finland. Electronic address: mikko.pakarinen@hus.fi.

Abstract

BACKGROUND:

Incidence and long-term outcomes of choledochal malformations (CMs) in children remain unclear.

METHODS:

Clinical characteristics, operative details, complications, and follow-up data were collected from eight pediatric surgical centers in Sweden, Norway, Denmark, and Finland, which also answered a questionnaire addressing management practices.

RESULTS:

During 2000-2017, 126 pediatric CMs were diagnosed, corresponding an incidence of 1:37,400. Diagnostic, treatment, and follow-up practices varied markedly. Of patients with complete clinical data (n = 119), 85% and 11% had type I and IV CMs and were managed by open hepaticojejunostomy at median age of 2.5 (interquartile range 0.46-5.8) years. Associated malformations were more common in fusiform and type IV (23%) than cystic CMs (8%, p = 0.043). Pancreaticobiliary maljunction was more frequently confirmed in patients presenting with pancreatitis (26% vs. 7%, p = 0.005) and with fusiform CMs (56% vs. 25%, p = 0.001). Cholangitis/pancreatitis episodes, occurring in 12% during postoperative follow-up of 4.0 (2.0-7.9) years, associated with longer surveillance (OR 1.32, 95% CI 1.13-1.54, p < 0.001). However, only two thirds of centers continued follow-up until adulthood. No malignancies were reported.

CONCLUSIONS:

CM incidence was higher than traditionally reported among Western populations. Although open hepaticojejunostomy carries good short-term outcomes, long-term morbidity is noteworthy. Standardized evidence-based management strategies and long-term follow-up are encouraged.

PMID:
31371244
DOI:
10.1016/j.hpb.2019.07.001

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