Skip to main content

Lund University Publications

LUND UNIVERSITY LIBRARIES

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

Hukkinen, Maria ; Björnland, Kristin ; Gatzinsky, Vladimir ; Iber, Tarja ; Johansen, Lars S. ; Qvist, Niels ; Stenström, Pernilla LU orcid ; Svensson, Jan F and Pakarinen, Mikko P. (2020) In HPB 22(1). p.161-168
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)

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.

(Less)
Please use this url to cite or link to this publication:
author
; ; ; ; ; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
HPB
volume
22
issue
1
pages
8 pages
publisher
Elsevier
external identifiers
  • pmid:31371244
  • scopus:85069814243
ISSN
1365-182X
DOI
10.1016/j.hpb.2019.07.001
language
English
LU publication?
yes
id
05ef4d86-c0cd-4306-b069-a88e0fa24034
date added to LUP
2019-08-16 09:44:43
date last changed
2024-06-26 00:39:50
@article{05ef4d86-c0cd-4306-b069-a88e0fa24034,
  abstract     = {{<p>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 &lt; 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.</p>}},
  author       = {{Hukkinen, Maria and Björnland, Kristin and Gatzinsky, Vladimir and Iber, Tarja and Johansen, Lars S. and Qvist, Niels and Stenström, Pernilla and Svensson, Jan F and Pakarinen, Mikko P.}},
  issn         = {{1365-182X}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{161--168}},
  publisher    = {{Elsevier}},
  series       = {{HPB}},
  title        = {{Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries}},
  url          = {{http://dx.doi.org/10.1016/j.hpb.2019.07.001}},
  doi          = {{10.1016/j.hpb.2019.07.001}},
  volume       = {{22}},
  year         = {{2020}},
}