Multiple Biliary Hamartoma Masquerading Polycystic Liver Disease-A Case Report

Sri keerthi. K, Siddharth Srinivasan, Aniket Khadatkar, JK Banerjee, Mangal Mahajan, Mrunal N Ketkar

Dr. Sri keerthi. K, Resident, Department of Surgery, BHRC, Pune;
Dr. Siddharth Srinivasan, Resident, Department of Surgery, BHRC, Pune;
Dr. Aniket Khadatkar, Asst Professor (General Surgery) and Fellow in Hepatobiliary surgery, BHRC, Pune;
Dr. JK Banerjee, Professor, Department of GI and Hepatopancreaticobiliary surgery; BHRC, Pune;
Dr. Mangal Mahajan- Professor, Department of Radiodiagnosis, BHRC, Pune;
Dr. Mrunal N Ketkar, Professor and HOD, Department of Surgery, BHRC, Pune.

Conflict-of-interest statement: The author(s) declare(s) that there is no conflict of interest regarding the publication of this paper.

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Correspondence to: Dr. Aniket Khadatkar, Asst Professor (General Surgery) and Fellow in Hepatobiliary surgery, BHRC, Pune.
Email: kskasturi811@gmail.com
Telephone: +9975017057

Received: December 16, 2019
Revised: December 26, 2019
Accepted: December 29, 2019
Published online: March 16, 2020


INTRODUCTION: Multiple Biliary Hamartomas are rare and asymptomatic and so are diagnosed less in vivo. They are benign malformation of ductal plate of intrahepatic biliary radicals. Patients are usually asymptomatic and the lesions are an incidental radiological finding. They maybe mistaken with liver metastasis, microabscess and other cystic lesions of liver. We report a 45 year old female who had vague right upper quadrant pain on and off for a year. She was diagnosed as extensive multiple biliary hamartomas on CT and MRI of the abdomen. Tumors markers were negative suggesting absence of malignancy. She was managed with symptomatic treatment and is on regular follow up.

Key words: Multiple Biliary Hamartomas; Von Meyenberg complexes (VMC)

© 2020 The Authors. Published by ACT Publishing Group Ltd. All rights reserved.

Keerthi K, Srinivasan S, Khadatkar A, Banerjee JK, Mahajan M, Ketkar MN. Multiple Biliary Hamartoma Masquerading Polycystic Liver Disease-A Case Report. International Journal of Radiology 2020; 7(1): 234-236 Available from: URL: http://www.ghrnet.org/index.php/ijr/article/view/2758

Case report

We report a 45 year old female patient who presented with vague right upper quadrant pain on and off since one year. There wasno history of jaundice, weight loss, loss of appetite or any constitutional symptoms. Family and past history were not contributory. Clinically she had gross hepatomegaly with a liver span measuring 24 cm and nodular surface of liver. There was no ascites. Systemic examination was unremarkable.

Ultrasonography of abdomen revealedgross hepatomegaly with multiple cystic lesions in the liver. Abdominal computed tomography confirmedhepatomegaly andliver being studded with numerous cystic lesions. These lesions showed no enhancement on post-contrast images. No calcification, fat component or central dot sign were seen. Liver was enlarged measuring 24 cm craniocaudally. All the segments of both the lobes were studded with numerous well defined hypodense lesions, varying in sizes from 2 mm to 19 mm in size. The findings were suggestive of biliary hamartomas. On magnetic resonance imaging, few of these cysts showed fluid-fluid level on the T2WI suggesting intracystic bleed/secondary chronic infection. Four solid non-enhancing lesions in segment V, VII, IVA and VII/VIII were also noticed. Largest lesion was seated deep in segment VII/VIII indenting the intrahepatic IVC and abutting the hepatic veins, measuring 39.1 × 38.3 × 43.4 mm (Trans × AP × CC) in size showing cystic degeneration with fluid-fluid levels. These lesions were hypodense on CT and did not show enhancement on arterial, portal, venous and delayed phases. They showed restricted diffusion on MR.

Tumor markers AFP, CEA,CA-125, CA 19-9, Beta HCG were normal. Patient was managed symptomatically and advised regular follow-up.

Figure 1 and 2 Showing multiple cystic lesions in liver on CT-scan.These lesions do not show enhancement on post-contrast images. No calcification and fat component seen within it. No central dot sign is seen.

Figure 3 and 4 T1WI and T2WI on axial MRI showing well defined hypointense and hyperintense cysts respectively. Cysts were also indenting the intrahepatic IVC.


Multiple benign hamartomas were first described by von Meyenberg in 1918, so also known as von Meyenberg complexes (VMC). The prevalence of multiple biliary hamartomas is approximately 3% at autopsy and on imaging it is around 1% because lesions less than 5mm are often difficult to find out on imaging[1]. It is three times more common in females than in male[2]. VMCs are benign malformations of ductal plate of intra hepatic bile duct. It can be associated with fibrocystic diseases of the liver, including congenital hepatic fibrosis, autosomal recessive polycystic kidney disease, bile duct atresia, Caroli's disease and mesenchymalhamartomas[1,3,4,5,]. Occasionally patient may present with jaundice due to mass effect. It is known to be associated with autosomal dominant polycystic kidney disease (ADPKD) and polycystic liver disease.There is a rare possibility of malignant transformation of multiple biliary hamartomas into hepatocellular carcinoma and cholangiocarcinoma. The malignant transformation is due to the toxic effect of chronic biliary stasis[6].The pathogenesis of VMC is interruption of remodeling of the ductal plates during the late phase of embryonic development of intra hepatic bile ducts[7]. They are well circumscribed, unencapsulated lesions throughout the liver although they may aggregate. On histology they appear as dilated small bile ducts with single layer of cuboidal epithelium with surrounded fibrous stroma. Biliary hamartomas are classified into three classes - class 1- predominantly solid pattern with narrow bile channels, class 2- intermediate pattern, class 3- marked cystic dilatation of bile ducts within the lesions[7]. Because of different histological pattern, biliary hamartoma may exhibit different echogenicity on ultrasonography.  There is association between biliary hamartomas and polycystic liver disease.In a study containing 70 cases done by Melnick, 29 cases had polycystic liver disease co existing with multiple biliary hamartomas. Melnick postulated that bile duct hamartomas arise when embryonic bile ducts fail to involute and polycystic liver disease result from gradual dilatation of hamartomas[8]. On ultrasonography, biliary hamartomas can be hypo echoic or hyper echoic. CT scans can detect multiple, nodular and irregular cystic liver lesions, smaller than 15 mm. The use of CA 19-9 to diagnose malignant transformation is not routinely indicated because of persistent elevation of this tumor marker has been described in patients with multiple biliary hamartomas without malignancy. On MRI, the biliary hamartomas exhibit hypointensity compared with liver parenchyma on T1-weighted image, and hyperintensity, but less than that of simple fluid, on T2-weighted image[9,10,11]. Liver biopsy is not contra indicated and should be done if there is suspicion of malignancy.As evident in studies conducted by Homer, malignant transformation, though rare, is a possibility. Hence, when in doubt, a liver biopsy is warranted[12].


Multiple biliary hamartoma is a rare entity.Most of the patients are asymptomatic and are diagnosed incidentally. The radiological appearance of the same can typically mimic polycystic liver disease or metastatic liver disease. Even though subtle differences have been reported in imaging, evaluation in the form of histopathology or tumor markers is warranted to rule out malignancy. These particularly require regular follow-up to watch for malignant transformation and hence deserve emphasis.


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