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Year : 2021  |  Volume : 54  |  Issue : 6  |  Page : 226-233

Clinical presentation and outcome of pediatric congenital biliary dilatation: A study based on pancreaticobiliary maljunction

1 Department of Surgery, Taichung Veterans General Hospital, Taichung; Faculty of Medicine, School of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan
2 Department of Surgery, Taichung Hospital, Ministry of Health and Welfare, Executive Yuan; Department of Healthcare, Central Taiwan University of Science and Technology, Taichung, Taiwan
3 Department of Surgery, Taichung Veterans General Hospital, Taichung, Taiwan

Correspondence Address:
Chia-Man Chou
Department of Surgery, Taichung Veterans General Hospital, National Yang Ming Chiao Tung University, No. 1650, Sec. 4, Taiwan Boulevard, Taichung 40705
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/fjs.fjs_186_21

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Background: The commonly used anatomical classification of congenital biliary dilatation by Todani is not directly related to types of pancreaticobiliary maljunction (PBM). This work is to investigate clinical presentation and surgical outcomes according to PBM classification proposed by the Japanese Study Group on Pancreaticobiliary Maljunction. Pancreaticobiliary junction angle, common channel length, and diameter of dilatation are studied as well. Materials and Methods: Patients of redo operation, without preoperative images, and lacking clearly documented outcomes were excluded. After exclusion, 79 patients who underwent Roux-en-Y hepaticojejunostomy between January 1994 and December 2019 were enrolled for this retrospective study. Medical records were reviewed, and perioperative parameters were collected. Todani's classification, PBM types, pancreaticobiliary junction angle, common channel length, and dilatation diameter were determined based on magnetic resonance cholangiopancreatography or computed tomography. Clinical presentation and outcome were compared between groups of different anatomical features. Results: PBM type A (stenotic), type B (nonstenotic), and type C (dilated channel) consisted of 48 (60.8%), 18 (22.8%), and 11 (13.9%) patients, respectively; and two patients (2.5%) had no PBM. Patients of PBM type A were younger and had more Todani's type Ia lesion. Patients of PBM type B and C had either Todani's type Ia or Ic lesion, but type IVa had only PBM type B. Longer common channel (1.27 vs. 0.81 cm, P < 0.001) and wider dilatation (4 vs. 2 cm, P < 0.001) were found in patients with right pancreaticobiliary angle (90°). Clinical outcome was similar in different Todani's types, PBM types, and pancreaticobiliary angle. Serum alkaline phosphatase level higher than 675 U/L was associated with major perioperative complications. Preoperative jaundice, mass, and dilatation wider than 5 cm were related to subsequent liver cirrhosis. Conclusion: PBM types and pancreaticobiliary junction groups are feasible for surgical planning, but not related directly to outcome. Palpable abdominal mass suggests higher risk of perioperative and late complications. For subsequent liver cirrhosis, laboratory data of disease onset and dilatation diameter wider than 5 cm are important risk factors.

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