RECURRENT SEPSIS IN A PATIENT WITH BILIARY HAMARTOMAS

Maria Beatriz Santos; Magda Ponta Garça; Bárbara Vieira; Paulo Ávila; Alexandra Freitas

doi:10.12890/2023_003764

RECURRENT SEPSIS IN A PATIENT WITH BILIARY HAMARTOMAS

Eur J Case Rep Intern Med. 2023 Mar 10;10(3):003764. doi: 10.12890/2023_003764. eCollection 2023.

Authors

Maria Beatriz Santos¹, Magda Ponta Garça¹, Bárbara Vieira², Paulo Ávila¹, Alexandra Freitas¹

Affiliations

¹ Internal Medicine Department, Hospital de Santo Espírito da Ilha Terceira, Angra do Heroísmo, Portugal.
² General Surgery Department Hospital de Santo Espírito da Ilha Terceira, Angra do Heroísmo, Portugal.

Abstract

Biliary hamartomas or von Meyenburg complexes (VMCs) are hepatic tumour-like lesions related to congenital malformation of the ductal plate, and are part of the ciliopathy spectrum of disorders. The exact pathogenesis of VMCs is unclear and it remains controversial whether they have the potential for malignant transformation. Patients are often asymptomatic and VMCs are usually encountered as an incidental finding on imaging. We report a case of recurrent sepsis with an unidentified focus. It was later confirmed that biliary hamartomas were acting as a sanctuary for the persistent pathogenic agent. The authors hope to draw attention to the existence of this unusual focus of recurrent sepsis.

Learning points: Hepatobiliary sepsis is an unusual clinical presentation of biliary hamartomas.Clinicians should be aware of the infectious complications of these diffuse structural biliary ductal abnormalities.Early recognition of this atypical life-threatening clinical presentation is important for the prognosis.

Keywords: Biliary hamartoma; hepatic lesions; sepsis; von Meyenburg complex.