INTENSIVE CARE UNIT (ICU) RELATED CHOLANGIOPATHY-INDUCED BILIARY CAST SYNDROME WITHOUT LIVER TRANSPLANTATION - A RARE ENTITY

Introduction:
Visceral artery pseudoaneurysms include aneurysms of the celiac, superior, or inferior mesenteric arteries and their branches. Pseudoaneurysms have a high risk of rupture with life threatening consequences.

Case Presentation:
A 63-year-old male with a history of alcohol-related pancreatitis presented with obstructive jaundice. He was afebrile and hemodynamically stable on presentation. Physical exam noted scleral icterus, jaundice, and central excoriations. Labs were significant for hemoglobin 12.1 g/dL, total bilirubin 16.3 mg/dL, AST 152 U/L, ALT 131 U/L, ALP 591 U/L, and lipase 40 U/L. Computed tomography scan was significant for intra- and extra-hepatic ductal dilation, and a 3 cm cystic lesion in the head of pancreas. Endoscopic ultrasound (EUS) revealed a 30 x 30mm round lesion with turbulent swirling flow arising from the gastroduodenal artery and compressing the distal bile duct (Image 1). Doppler revealed an arterial “to-and-fro” waveform, consistent with a gastroduodenal artery pseudoaneurysm (Image 2). Endoscopic retrograde cholangiography revealed severe compression of the distal bile duct, and a covered metal stent was placed. The patient underwent angiography confirming a large gastroduodenal artery pseudoaneurysm, which was treated with vascular coils.

Discussion:
The “to-and-fro” waveform on Doppler is an important diagnostic clue to the presence of a pseudoaneurysm. The “to” represents the arterial blood going into the pseudoaneurysmal sac in systole, while “fro” illustrate blood exiting the sac in diastole. Recognition of this sign on EUS is important as pseudoaneurysms can be missed on cross-sectional imaging.

Background:
Biliary cast syndrome (BCS) without liver transplantation is a rare entity. The etiology of BCS is unknown and carries a poor prognosis. Documented risk factors include post-transplant bile duct injury, ischemia, infection, and gallbladder dysmotility. Here we present a rare case of BCS without a liver transplant.

Case:
A 41-year-old male with a past medical history of hypertension and substance use presented with a hypertensive emergency due to medication non-adherence and cocaine use. Our patient had a protracted ICU course complicated with hypoxia-induced cardiac arrest and critical illness myopathy 6-8 months prior.

On the floor, the patient was persistently febrile and complained of diffuse abdominal pain, and the physical exam was positive for left upper quadrant tenderness. Laboratory values demonstrated leukocytosis with neutrophil predominance and evidence of cholestasis with alkaline phosphatase (ALP) 802 U/L, aspartate aminotransferase (AST) 138 U/L, alanine transaminase (ALT) 284 U/L and bilirubin 0.6 mg/dl; however, the infectious workup was negative.

CT chest showed multiple ill-defined right hepatic hypodensities involving more than 50% of parenchyma consistent with hepatic abscess. Magnetic resonance cholangiopancreatography (MRCP) demonstrated innumerable hyperintense and hypointense lesions with peripheral enhancement (Fig. 1), as well as linear hypointense structures in the right hepatic duct (RHD), common hepatic duct (CHD), and common bile duct (CBD). Subsequent endoscopic ultrasound (EUS) showed an isoechoic linear structure and mildly dilated CHD (7mm).

Endoscopic retrograde cholangiopancreatography (ERCP) was performed, and a cholangiogram confirmed a filling defect in CBD, CHD, and RHD. Then Single-user SpyGlass Cholangioscopy confirmed an obstructing linear object in RHD. The balloon sweep removed a long (10-12 cm) bifurcated structure, and the cholangiogram confirmed clearance. The gross specimen was tan-brown, measuring 1 cm x 10.5 cm (Fig. 2), and histopathology demonstrates a bile cast with pigmented debris, purulent inflammation, and ductal epithelium.

The patient was improving post-ERCP, and a CT abdomen and pelvis showed unenhanced liver without biliary duct dilation; however, persistent cholestatic LFTs were noted after two months with ALP of 1410U/L, AST 147 U/L, ALT 281 U/L, and bilirubin 0.7 mg/dl.

Conclusion:
This clinical picture was consistent with critical illness cholangiopathy-induced BCS. Contributing factors include fasting, parenteral feeding, hepatic ischemia, and increased bile viscosity resulting in biliary casts. There is no established standard care for managing BCS; however, ERCP has shown promising results. Moreover, monitoring patients with protracted ICU course and abnormal LFTs may be prudent for recognizing persistent cholangiopathy and BCS-related complications.