At the focal point| Volume 91, ISSUE 5, P1205-1207, May 2020

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A starry sky liver: Klebsiella pneumoniae septic shock caused by infected multiple biliary hamartomas

Published:December 19, 2019DOI:
      A 52-year-old woman presented to the emergency department because of 20 pounds of unintentional weight loss over 3 months, painless jaundice, rigors, and worsening altered mental status. Her physical exam was notable for hypotension, fever, and tachycardia. Laboratory evaluation showed leukocytosis of 11 K/mcL, total bilirubin of 7.6 mg/dL, and alkaline phosphatase of 926 units/L, overall consistent with septic shock from ascending cholangitis. Empiric intravenous antibiotics and vasopressors were started. MRCP revealed innumerable uniform hepatic cysts without connection to the biliary tree, consistent with multiple biliary hamartomas (MBHs) (A-C). Blood cultures ultimately grew Klebsiella pneumoniae. On day 4 of treatment, both her total bilirubin and alkaline phosphatase improved to 1.0 mg/dL and 155 units/L, respectively. She did not undergo surgical resection and completely recovered; she was discharged on day 7.
      MBHs, or von Meyenburg complexes, are rare benign lesions of the liver. They occur in roughly 0.6% to 2.8% of the general population. They are associated with autosomal polycystic kidney disease, hepatic fibrosis, and polycystic liver disease. MRCP depicts striking stereotypical multiple T2 hyperintense cystic lesions distributed homogeneously within the liver without communication to the bile ducts. The differential diagnosis for this imaging appearance includes metastasis, simple liver cysts, liver cancer, biliary adenomas, and Caroli’s disease. Although the facts are unclear, MBH may be a risk factor for cholangiocarcinoma.


      All authors disclosed no financial relationships.
      Commentary MBHs are rare benign liver lesions, also known as von Meyenburg complexes, and were first described in the early 20th century. They consist of multiple small interlobular biliary cystic lesions or hamartomas that are not connected to the main biliary tree. MBHs are the result of biliary ductal plate malformations during embryonic development. MBHs are usually asymptomatic and are found incidentally. It is key to differentiate MBHs from metastases, simple liver cysts, and Caroli’s disease.
      MBHs are easily detectable on cross-sectional imaging, and a liver biopsy is usually not necessary. MRCP with contrast material reveals multiple small (<15-mm) irregular cystic liver lesions without attenuation or enhancement, and the extrahepatic and intrahepatic biliary trees are normal. By contrast, liver metastatic lesions and Caroli’s disease show contrast enhancement. As seen in the case described here, MBHs can become infected, which can lead to cholangitis and life-threatening septic shock, as seen in this case.
      Fewer than 15 MBH cases of cholangitis have been reported in the literature. The most common reported microorganisms are Escherichia coli, Enterococcus faecium, Enterobacter cloacae, and Klebsiella pneumoniae. Over 90% of reported cases of MBHs with cholangitis had a single cholangitis episode and responded to intravenous antibiotics. Notably, Panda et al reported a case of MBH that required liver transplantation for the treatment of recurrent cholangitis. Finally, MBHs are rare biliary duct congenital abnormalities that can be complicated by cholangitis, but they usually respond to antibiotics, and endoscopic or surgical interventions are generally not necessary in most instances.
      C. Roberto Simons-Linares, MD, MSc, Cleveland Clinic Foundation, Cleveland, Ohio
      Mohamed O. Othman, MD, Associate Editor for Focal Points