Surgical and Medical Aspects of the Initial Treatment of Biliary Atresia: Position Paper

Overview

Journal J Clin Med

Specialty General Medicine

Date 2022 Nov 11

PMID 36362829

Authors

Mark Davenport

Omid Madadi-Sanjani

Christophe Chardot

Henkjan J Verkade

Saul J Karpen

Claus Petersen

Affiliations

Soon will be listed here.

Abstract

Biliary atresia, a fibro-obliterative disease of the newborn, is usually initially treated by Kasai portoenterostomy, although there are many variations in technique and different options for post-operative adjuvant medical therapy. A questionnaire on such topics (e.g., open vs. laparoscopic; the need for liver mobilisation; use of post-operative steroids; use of post-operative anti-viral therapy, etc.) was circulated to delegates ( = 43) of an international webinar (Biliary Atresia and Related Diseases-BARD) held in June 2021. Respondents were mostly European, but included some from North America, and represented 18 different countries overall. The results of this survey are presented here, together with a commentary and review from an expert panel convened for the meeting on current trends in practice.

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References

de Vries W, de Langen Z, Groen H, Scheenstra R, Peeters P, Hulscher J . Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008. J Pediatr. 2011; 160(4):638-644.e2. DOI: 10.1016/j.jpeds.2011.09.061. View

Betalli P, Cheli M, Colusso M, Casotti V, Alberti D, Ferrari A . Association between Kasai portoenterostomy at low caseload centres and transplant complications in children with biliary atresia. J Pediatr Surg. 2022; 57(9):223-228. DOI: 10.1016/j.jpedsurg.2021.12.030. View

Ohya T, Miyano T, Kimura K . Indication for portoenterostomy based on 103 patients with Suruga II modification. J Pediatr Surg. 1990; 25(7):801-4. DOI: 10.1016/s0022-3468(05)80025-9. View

Serinet M, Wildhaber B, Broue P, Lachaux A, Sarles J, Jacquemin E . Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009; 123(5):1280-6. DOI: 10.1542/peds.2008-1949. View

Wu J, Jeng Y, Chen H, Ni Y, Hsu H, Chang M . Quantification of Serum Matrix Metallopeptide 7 Levels May Assist in the Diagnosis and Predict the Outcome for Patients with Biliary Atresia. J Pediatr. 2019; 208:30-37.e1. DOI: 10.1016/j.jpeds.2018.12.006. View

Toyosaka A, Okamoto E, Okasora T, Nose K, Tomimoto Y, Seki Y . Extensive dissection at the porta hepatis for biliary atresia. J Pediatr Surg. 1994; 29(7):896-9. DOI: 10.1016/0022-3468(94)90011-6. View

Abukawa D, Nakagawa M, Iinuma K, Nio M, Ohi R, Goto J . Hepatic and serum bile acid compositions in patients with biliary atresia: a microanalysis using gas chromatography-mass spectrometry with negative ion chemical ionization detection. Tohoku J Exp Med. 1998; 185(4):227-37. DOI: 10.1620/tjem.185.227. View

Kohaut J, Guerin F, Fouquet V, Gonzales E, de Lambert G, Martelli H . First liver transplantation for biliary atresia in children: The hidden effects of non-centralization. Pediatr Transplant. 2018; :e13232. DOI: 10.1111/petr.13232. View

Chardot C, Buet C, Serinet M, Golmard J, Lachaux A, Roquelaure B . Improving outcomes of biliary atresia: French national series 1986-2009. J Hepatol. 2013; 58(6):1209-17. DOI: 10.1016/j.jhep.2013.01.040. View

10.

Jiang J, Wang J, Shen Z, Lu X, Chen G, Huang Y . Serum MMP-7 in the Diagnosis of Biliary Atresia. Pediatrics. 2019; 144(5). DOI: 10.1542/peds.2019-0902. View

11.

Hery G, Gonzales E, Bernard O, Fouquet V, Gauthier F, Branchereau S . Hepatic Portocholecystostomy: 97 Cases From a Single Institution. J Pediatr Gastroenterol Nutr. 2017; 65(4):375-379. DOI: 10.1097/MPG.0000000000001685. View

12.

Hsu F, Dai S, Chou C, Huang S . The application of artificial intelligence to support biliary atresia screening by ultrasound images: A study based on deep learning models. PLoS One. 2022; 17(10):e0276278. PMC: 9581370. DOI: 10.1371/journal.pone.0276278. View

13.

Lu X, Jiang J, Shen Z, Chen G, Np Y, Xiao X . Effect of Adjuvant Steroid Therapy in Type 3 Biliary Atresia: A Single-Center, Open-Label, Randomized Controlled Trial. Ann Surg. 2022; 277(6):e1200-e1207. DOI: 10.1097/SLA.0000000000005407. View

14.

Parolini F, Boroni G, Milianti S, Tonegatti L, Armellini A, Garcia Magne M . Biliary atresia: 20-40-year follow-up with native liver in an Italian centre. J Pediatr Surg. 2018; 54(7):1440-1444. DOI: 10.1016/j.jpedsurg.2018.10.060. View

15.

Starzl T, Marchioro T, VONKAULLA K, Hermann G, BRITTAIN R, Waddell W . HOMOTRANSPLANTATION OF THE LIVER IN HUMANS. Surg Gynecol Obstet. 1963; 117:659-76. PMC: 2634660. View

16.

Fanna M, Masson G, Capito C, Girard M, Guerin F, Hermeziu B . Management of Biliary Atresia in France 1986 to 2015: Long-term Results. J Pediatr Gastroenterol Nutr. 2019; 69(4):416-424. DOI: 10.1097/MPG.0000000000002446. View

17.

Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O . Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005; 41(2):366-71. DOI: 10.1002/hep.20547. View

18.

Xiao H, Huang R, Chen L, Diao M, Li L . The Application of a Shorter Loop in Kasai Portoenterostomy Reconstruction for Ohi Type III Biliary Atresia: A Prospective Randomized Controlled Trial. J Surg Res. 2018; 232:492-496. DOI: 10.1016/j.jss.2018.07.002. View

19.

Russo P, Magee J, Anders R, Bove K, Chung C, Cummings O . Key Histopathologic Features of Liver Biopsies That Distinguish Biliary Atresia From Other Causes of Infantile Cholestasis and Their Correlation With Outcome: A Multicenter Study. Am J Surg Pathol. 2016; 40(12):1601-1615. PMC: 5123664. DOI: 10.1097/PAS.0000000000000755. View

20.

Asai A, Miethke A, Bezerra J . Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes. Nat Rev Gastroenterol Hepatol. 2015; 12(6):342-52. PMC: 4877133. DOI: 10.1038/nrgastro.2015.74. View