A 7-year-old girl with giant congenital biliary dilatation

A 7-year-old girl presented to our out-patient clinic with complaints of abdominal mass and pain with yellowish skin for over 3 months. The patient was also accompanied by clinical manifestations such as nausea and vomiting, skin itching and clay-colored stools. She denied any past history of gastrointestinal bleeding or trauma. Examination revealed a non-tender swelling as it moved with breathing (Panel A). Laboratory tests were as follows: alanine aminotransferase (ALT): 318 U/L, aspartate aminotransferase (AST): 345 U/L, total bilirubin (TBIL): 311.2 µmol/L, direct bilirubin (DBIL): 149.6 µmol/L, albumin (ALB): 30.8 g/L, alkaline phosphatase (ALP): 3,928 U/L, K: 3.1 mmol/L, Na: 131.8 mmol/L, amylase (AMY): 174 U/L, lipase (LIP): 203 U/L. CT/MRI of the abdomen showed a cystic hypodense lesion in the intrahepatic bile duct with a maximum cross-sectional area of approximately 15.0 cm × 12.0 cm, which was considered to be a congenital choledochal cyst (Panels B,C). The patient underwent a transhepatic percutaneous cholangial drainage (PTCD) to relieve bile pressure and improve liver function at the first (Panel D). Subsequently, the patient underwent a laparoscopic excision of choledochal cyst + cholangiojejunostomy + cholecystectomy. Histopathological examination indicated biliary dilation. The deposition of mucous bile pigments and the infiltration of lymphocytes were correlated with changes in chronic inflammation (Panel E). One month after the operation, a CT scan showed normal abdominal appearance (Panel F).

Acknowledgments

Funding: This study was supported by the National Key R&D Program Project (No. 2017YFC0909900), Qinghai Science and Technology Department 2022 Science and Technology Plan Projects (Qinghai Research Key Laboratory for Echinococcosis Research), Young and Middle-aged Scientific Research Fund Project of the Affiliated Hospital of Qinghai University (No. ASRF-2021-YB-17), Tsinghua University Institute of Precision Medicine Strategic Project (Nos. 2022ZLA005, 2022ZLB004) and Beijing Natural Science Foundation (No. Z190024).

Footnote

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://hbsn.amegroups.com/article/view/10.21037/hbsn-22-551/coif). The authors report that this study was supported by the National Key R&D Program Project (No. 2017YFC0909900), Qinghai Science and Technology Department 2022 Science and Technology Plan Projects (Qinghai Research Key Laboratory for Echinococcosis Research), Young and Middle-aged Scientific Research Fund Project of the Affiliated Hospital of Qinghai University (No. ASRF-2021-YB-17), Tsinghua University Institute of Precision Medicine Strategic Project (Nos. 2022ZLA005, 2022ZLB004) and Beijing Natural Science Foundation (No. Z190024). The authors have no other conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.