Atresia de vías biliares: variabilidad en su evolución posterior a portoenterostomía de Kasai. Reporte de dos casos

Kerly Fiestas; Wilma Geraige; Mariana Torres; Paula Roitman; Karina Arco; Giuliana Vaquer

doi:10.59594/iicqp.2025.v3n2.145

Authors

Kerly Fiestas Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina. https://orcid.org/0009-0002-6128-5647
Wilma Geraige Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina. https://orcid.org/0009-0009-1652-1947
Mariana Torres Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina. https://orcid.org/0009-0006-8086-7457
Paula Roitman Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina. https://orcid.org/0009-0002-3471-5170
Karina Arco Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina. https://orcid.org/0009-0002-4964-6577
Giuliana Vaquer Servicio de Gastroenterología Pediátrica, Hospital Dr. Humberto Notti, Mendoza M5500, Argentina.

DOI:

https://doi.org/10.59594/iicqp.2025.v3n2.145

Keywords:

Biliary Atresia, Hepatic Portoenterostomy, Liver Transplantation, Case Reports

Abstract

Background: Biliary atresia (BA) is a leading cause of neonatal cholestasis and the primary indication for liver transplantation in children. Its etiology is unknown and is characterized by the obliteration of the bile ducts, resulting in impaired bile flow, which manifests as jaundice, dark urine, pale stools, hepatomegaly, and/or splenomegaly. Kasai portoenterostomy (KP) can restore bile drainage, although its success decreases with increasing age at surgery.

Case presentation: We report two infants who underwent intraoperative cholangiography followed by KP due to suspected BA. Liver biopsies confirmed the diagnosis. Outcomes were influenced by the timing of surgery: one patient, who underwent surgery at a later age, experienced an unfavorable course and required early liver transplantation.

Conclusion: These cases highlight age at KP as a key determinant of BA outcomes. Late diagnosis remains a challenge despite existing policies at various levels of care, emphasizing the need to strengthen national screening programs.

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