1438 A Starry Sky Liver: Klebsiella Pneumoniae Septic Shock Caused by Infected Multiple Biliary Hamartomas

Abstract

INTRODUCTION: Biliary hamartomas or von Meyenburg complexes are an exceedingly rare benign cystic disease of the liver. They are diagnosed radiographically due to a distinct appearance. We present an unusual case of a patient presenting with septic shock from multiple infected biliary hamartoma cysts. CASE DESCRIPTION/METHODS: A 52-y/o caucasian female presented to the emergency department with 20 pounds of unintentional weight loss over 3 months, painless jaundice, rigors and worsening altered mental status. Medical history included: Down's syndrome with progressive dementia, depression, and hypothyroidism. Physical exam notable for hypotension, fever, and tachycardia. Laboratory evaluation illustrated a leukocytosis of 10.9, total bilirubin of 7.6, direct bilirubin 6.1, alkaline phosphatase 926, ALT 44, AST 71, INR 2.3. An abdominal ultrasound revealed numerous hepatic cysts without cholelithiasis. MRCP confirmed innumerable uniform hepatic cysts without connection to the biliary tree consistent with multiple biliary hamartomas. The hepatic cysts lacked mural nodules. She was treated for presumed septic shock from ascending cholangitis with empiric IV antibiotics and vasopressors. With continued supportive care, her liver enzymes and coagulopathy normalized. Blood cultures grew Klebsiella pneumoniae. Her septic shock resulted from cholangitis due to infected biliary hamartomas. She discharged after 7 days in healthy condition. DISCUSSION: Multiple biliary hamartomas (MBH) are rare, benign lesions of the liver. They occur roughly 0.6-2.8% of the general population. Multiple biliary hamartomas are thought to arise from ductal plate malformations involving the small interlobular bile ducts. The cysts are typically uniform in size and most are asymptomatic found incidentally on imaging. MBH are associated with autosomal polycystic kidney disease, hepatic fibrosis, and polycystic liver disease. Gold standard for diagnosis of MBH is MRCP. It depicts striking stereotypical multiple T2 hyper-intense cystic lesions distributed homogeneously within the liver without communication to the bile ducts. Differential diagnosis for this imaging appearance includes metastasis, simple liver cysts, liver cancer, biliary adenomas, and Caroli's disease. Albeit unclear, MBH may be a risk factor for cholangiocarcinoma. This case highlights a peculiar case of biliary septic shock caused by infected liver biliary hamartomas.