S1427 Lime-A-Way: Limy Bile Syndrome Complicated by Biliary Obstruction

Abstract

INTRODUCTION: Limy bile syndrome (LBS), also known as milk of calcium bile, is a rare disorder of unclear etiology, characterized by the accumulation of thick, paste-like radiopaque calcium salts in the gallbladder and common bile duct (CBD). It is prevalent in 0.1% of patients that undergo cholecystectomies and can lead to life-threatening complications, necessitating early recognition. We report a case of this rare clinical entity presenting with obstructive jaundice and causing debilitating abdominal pain and chronic cholecystitis. CASE DESCRIPTION/METHODS: A 51-year-old man with no past medical history presented with 4 months of worsening sharp, post-prandial right upper quadrant abdominal pain and nausea. Vital signs were normal. Physical exam was notable for scleral icterus, jaundice, tenderness to palpation in the right upper quadrant of his abdomen and negative Murphy’s sign. Laboratory data was notable for AST 195 U/L, ALT 520 U/L, alkaline phosphatase 186 U/L, total bilirubin 6.2 mg/dL (conjugated 2.8/unconjugated 0.9). Abdominal ultrasound showed CBD dilation to 10 mm without gallstones. Abdominal CT showed a dilated CBD and densely hyperattenuating material along the inferior gallbladder wall and within the CBD (Figure 1). Given the absence of stones, the diagnosis of LBS was suspected. He underwent ERCP with sphincterotomy and copious amounts of thick paste-like sludge was swept from the duct, confirming the diagnosis of LBS (Figures 2, 3). His symptoms and biochemical abnormalities resolved rapidly following ERCP, and he underwent laparoscopic cholecystectomy with pathology confirming chronic cholecystitis. DISCUSSION: LBS is a rare disorder with poorly defined pathogenesis. Abnormalities in calcium metabolism, variable gallbladder pH and gallbladder stasis are factors that may cause calcium salt precipitation. Most patients present with biliary colic or cholecystitis. LBS can also present as incidental radiopaque shadows below the liver on abdominal x-rays in asymptomatic individuals. The identification of these calcifications should prompt further investigation with ultrasound and CT to distinguish LBS from other calcifying pathologies such as porcelain gallbladder in which gallbladder wall calcification is demonstrated as opposed to radiopaque material within the gallbladder itself. Timely recognition of this rare syndrome and early cholecystectomy is crucial as it can lead to complications such as biliary obstruction, cholangitis, biliary pancreatitis and cholecystitis.Figure 1.: CT showing hyperattenuating material along the inferior gallbladder wall.Figure 2.: EGD showing thick paste-like sludge.Figure 3.: ERCP images.