Management strategies and treatment results of pediatric choledochal malformations in the Nordic countries
(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)
- author
- Hukkinen, Maria
; Björnland, Kristin
; Gatzinsky, Vladimir
; Iber, Tarja
; Johansen, Lars S.
; Qvist, Niels
; Stenström, Pernilla
LU
; Svensson, Jan F and Pakarinen, Mikko P.
- organization
- publishing date
- 2020-01
- 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 < 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}}, }