Maria Hukkinen1, Kristin Björnland2, Vladimir Gatzinsky3, Tarja Iber4, Lars S Johansen5, Niels Qvist6, Pernilla Stenström7, Jan F Svensson8, Mikko P Pakarinen9. 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.
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.
Authors: Jan B F Hulscher; Joachim F Kuebler; Janneke M Bruggink; Mark Davenport; Stefan Scholz; Claus Petersen; Omid Madadi-Sanjani; Nagoud Schukfeh Journal: J Clin Med Date: 2022-02-21 Impact factor: 4.241