Ischemic Cholangiopathy: A Consequence of Sickle Cell Disease?

Abstract

Introduction: Hepatobiliary complications in sickle cell disease (SCD) such as acute hepatic ischemia, veno-occlusive disease, sequestration, and benign cholestasis are directly related to the sickling process and have been well documented in the medical literature. We present a case of ischemic cholangiopathy (IC) in a patient with SCD.Figure 1Case Report: A 16-year-old male with history of SCD was admitted with intermittent right upper quadrant (RUQ) abdominal pain, jaundice, nausea, and vomiting for 2 weeks. On admission, hemoglobin 8.7 g/dl, WBC of 15.2 k/cu mm, AST 89 U/L, ALT 108 U/L, alkaline phosphatase 537 U/L, total bilirubin 2.8 mg/dL, and direct bilirubin 1.5 mg/dL. An ultrasound revealed intrahepatic duct dilation (IHD) and 2 echogenic foci with posterior shadowing near the porta hepatis. The common bile duct (CBD) measured 4 mm. An endoscopic retrograde cholangiopancreatogram (ERCP) revealed multiple focal areas of stenosis and dilations in the left and right IHD, common hepatic duct (CHD), and stenosis of the CBD. Multiple small pigment stones and sludge were found in the CHD and IHD. A sphincterotomy was performed, multiple stones were removed, and a biliary stent was placed. Bile duct brushings were negative for malignancy. Serology testing for primary sclerosing cholangitis (PSC) and a colonoscopy were negative. Given these findings a diagnosis of IC was made. Discussion: IC is increasingly recognized in association with various processes that reduce arterial blood supply to the bile ducts. Most common cause of IC is following liver transplantation. The main diagnostic challenge is to distinguish it from more common causes of biliary obstruction such as choledocholithiasis and malignancy. The biliary abnormalities may also closely resemble those seen in PSC. A limited number of case reports of IC have been described in SCD. SCD causes marked microcirculatory alterations. The hepatic artery through the peribiliary plexus provides the sole blood supply to the major bile ducts. The terminal effect of sickling such as hypoxia/ischemia has been proposed as the contributing factor in the pathogenesis of IC in this population. We postulated cholangiopathy in our case was due to repeated episodes of hypoxic/ischemic injury to the biliary tract secondary to the sickling process. Given the fact that our patient had no features of inflammatory bowel disease, PSC was felt to be an unlikely diagnosis. Ischemic cholangiopathy remains a diagnostic challenge but should be considered in patients with SCD.