Obstructive jaundice caused by a giant liver hemangioma with Kasabach-Merritt syndrome: a case report

Abstract

Hemangioma is the most common benign tumor of the liver. Liver hemangioma (LH) usually remains asymptomatic, but the most common symptoms associated with LH are abdominal pain and discomfort. LH is an uncommon cause of bile duct dilatation and obstructive jaundice. An 83-year-old Japanese woman who received hemodialysis at another hospital was referred to our hospital because of abnormal liver function and obstructive jaundice. Abdominal computed tomography and magnetic resonance imaging revealed a 13-cm tumor in liver segments IV-V and intrahepatic bile duct dilatation. Endoscopic retrograde cholangiopancreatography revealed extrinsic compression of the bile duct at the hepatic hilar region. Laboratory tests showed that the patient had low platelet counts and low fibrinogen levels. Because the patient had hyperbilirubinemia and Kasabach-Merritt syndrome, we performed a segmentectomy of liver segments IV and V. Histological examination showed hemangioma of the liver. The patient's thrombocytopenia and coagulopathy improved immediately after surgery. In conclusion, LH is a very rare cause of obstructive jaundice. LH has the potential to compress the bile duct and cause obstructive jaundice.

Keywords: Biliary stricture; Hemangioma; Kasabach-Merritt syndrome; Obstructive jaundice.

Fig. 1

Radiological findings. a Computed tomography (CT) scan of the abdomen showing a well-demarcated liver mass consistent with hemangioma in the anterior segment of the right hepatic lobe to segment IV. The tumor pressed a bile duct in the hepatic portal region, causing an intrahepatic bile duct to expand. b A CT scan performed 5 years prior to the study shows a low-density tumor with a diameter of 28 mm in the right lobe. c The size of the tumor was shown to be 13 cm in diameter in magnetic resonance imaging, with a clearly demarcated border and a heterogeneous signal on T2-weighted images. The tumor had a fluid-fluid level at the tumor margins. d A stricture of approximately 2 cm in length in the common bile duct was observed via endoscopic retrograde cholangiography and suspected to be a result of compression from the outside wall. This caused partial expansion of a right lobe bile duct and high expansion of the left lobe bile duct

Fig. 2

Gross and histological findings. a The tumor was wine colored and elastic. b The tumor was composed of blood-filled spaces lined by a single layer of endothelial cells without smooth muscle (H&E staining, objective; ×40). c Fibrotic, hyalinized, and calcified lesions were observed (H&E staining, objective; ×40)

Fig. 3

Clinical course of coagulopathy. After surgery, blood coagulopathy was improved immediately