Int J Clin Exp Med 2015;8(8):13145-13153 www.ijcem.com /ISSN:1940-5901/IJCEM0012001 Original Article Imaging findings of bile duct hamartomas: a case report and literature review Qiu-Sheng Shi1*, Ling-Xi Xing1*, Li-Fang Jin1, Han Wang2, Xiu-Hong Lv3, Lian-Fang Du1 1Department of Ultrasound, Shanghai First People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China; 2Department of Radiology, Shanghai First People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China; 3Department of Pathology, Shanghai First People’s Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200080, China. *Co-first authors. Received June 27, 2015; Accepted August 11, 2015; Epub August 15, 2015; Published August 30, 2015 Abstract: Bile duct hamartomas (BHs), also called von Meyenburg complex (VMC), are benign biliary malformations that originate from disorganization of the small intrahepatic bile ducts. This disorganization is often associated with the abnormal involution of embryonic ductal end plates in the liver. This is clinically significant, as the development of BHs can cause diagnostic confusion with liver metastases and small hepatocellular carcinoma (SHCC). Currently, we report a specific case of BHs and review the literature to better define and diagnose BHs. In the following case, a 37 year-old male bearing a lesion in his liver is presented and undergoes both radiological and pathological diag- nosis. The lesion is preliminarily suspected to be a hepatic hemangioma by examination of conventional ultrasound (US), contrast enhanced ultrasound (CEUS), computerized tomographic scanning (CT) and magnetic resonance imaging (MRI). However, SHCC is suspected by follow-up analysis of US and CEUS, due to the patient’s background history of hepatitis B and growth of the lesion and a tumor-feeding vessel in BHs via CEUS. However, BHs are finally diagnosed by biopsy pathology under the guidance of ultrasound. Therefore, we believe pathology is imperative for correct diagnosis of BHs over other similar diseases when the imaging findings are atypical. Here we report the novel and unique detection of a tumor-feeding vessel, which mimicked SHCC strongly, during the course of CEUS. We also present a comprehensive review of the previous reported radiological examination related to BHs. Keywords: Bile duct hamartomas (BHs), pathology, ultrasound (US), contrast enhanced ultrasound (CEUS), com- puterized tomographic (CT), magnetic resonance imaging (MRI) Introduction 5.6% in adults in a large series of autopsy sam- ples [4, 6, 7]. Macroscopically, BHs can be char- Bile duct hamartomas (BHs), also known as von acterized by the visualization of many grayish- Meyenburg complex (VMC), were initially whitish or yellow small nodules scattered described by the Swiss pathologist Hanns von throughout the liver, either intraparenchymally Meyenburg [1] in 1918. BHs have been previ- or subcapsularly [6, 8-10]. BHs may be detect- ously described as benign liver malformations ed as single or multiple hamartomas, with a resulting from maldevelopment of the ductal diameter ranging from 1 to 15 mm [11]. During end plates, which remain in a persistent dou- diagnosis, its characteristics can mimic that of ble-layered cylindrical structure similar to the liver metastases, especially when the patient fetal ductal plate [2, 3]. Therefore, pathologi- has previously suffered a primary malignancy cally, BHs can be defined by examining dilated [9, 11]. intrahepatic bile ductules and the surrounding fibrous stroma [4, 5]. Patients with BHs are usually symptomatic. Jaundice, epigastralgia, and fever can occur. BHs are typically detected during laparotomy or Jaundice, a major symptom, is caused by autopsy, and the prevalence of BH formation is obstruction of the biliary tract owing to the age-dependent, varying from 1% in children to mucus secreted by biliary papillomas [4, 6, 12]. Bile duct hamartomas Figure 1. Imaging of patient findings via US and CEUS. (A) At initial examination, a heterogeneous hypoechoic nodule was located in the liver parenchyma via US (red arrow). (B) At initial examination, there was no blood flow in the inte- rior of the nodule (red arrow). (C) The result of a second examination shows a similar heterogeneous nodule located in the liver parenchyma (red arrow). (D) The results of a second examination show sporadic internal blood flow in the interior of the nodule (red arrow). (E-H) At first CEUS examination, enhancement of the hypervascular nodule is earlier than the surrounding liver parenchyma. The arrival time, peak time and regression time of Sonovue® are (E) 12 seconds, (F) 19 seconds and (G) 24 seconds, respectively (red arrows). (H) A tumor-feeding vessel connecting the branch of hepatic artery and the lesion (red arrow). (I-L) At second CEUS examination, enhancement of the hy- pervascular nodule is also earlier than the surrounding liver parenchyma. The arrival time, peak time and regression time of Sonovue® are (I) 10 seconds, (J) 17 seconds, and (K) 24 seconds, respectively. (L) The tumor-feeding vessel is again detected (red arrows). However, these symptoms are nonspecific. A disappearance in the delay phase in CEUS. close relationship has been reported to exist Additionally, we believe imaging strategies are between BHs and the following congenital dis- not sufficient to achieve a most definitive diag- orders: Caroli’s disease, polycystic liver dis- nosis of BHs in the absence of typical imaging ease, congenital hepatic fibrosis, mesenchymal findings. hamartomas, bile duct atresia and autosomal recessive polycystic kidney disease [5, 8, 13, Case report 14]. While VMC is a benign condition, there is an ongoing debate as to whether BHs have the Here, we report the occurrence of a hypoechon- potential to induce malignant degeneration to ical solid nodule in the right liver lobe detected cholangiocarcinoma and hepatocellular carci- by B-mode ultrasonography in a 37 year-old noma [6, 8, 11, 13-15]. Here, we report a case male. Our study had been approved by the eth- of VMC diagnosed through several radiological ics committee of Shanghai First People’s examinations, including ultrasound (US), con- Hospital and the informed consent had been trast-enhance ultrasound (CEUS), computer- waived. Considering that the patient had a his- ized tomographic scanning (CT) and magnetic tory of Hepatitis B, development of a malignant resonance imaging (MRI). We present novel tumor could not be ruled out. At the initial results via CEUS that suggest the occurrence of screening, CT scanning missed the nodule a tumor-feeding vessel. The imaging is because it was imaged in only one frame, which enhanced in the arterial phase without early was reluctantly discovered in a later retrospec- 13146 Int J Clin Exp Med 2015;8(8):13145-13153 Bile duct hamartomas Figure 2. Imaging of patient findings via CT and MRI. (A, B) CECT examination displayed no enhancement in both of hepatic arterial phase and portal venous phase (red arrows). (C-E) Conventinal MRI examination. (C) T1WI, the lesion is hypointense (red arrow). (D) T2WI, the lesion is hyperintense (red arrow). (E) DWI, the lesion shows free diffusion (red arrow). (F-H) Gadolinium-enhanced T1WI. (F) The lesion displays rim enhancement in the hepatic arterial phase, and marginal tuberculiform enhancement in both (G) portal venous phase and (H) hepatic vein (red arrows). tive analysis of the CT imaging. Additionally, the (Figure 1B). The ultrasound findings at the six- patient refused to undergo a MRI examination. month follow-up were roughly consistent with CEUS was further implemented, with the pri- that of the first time, with a slightly increased mary consideration being liver benign lesion. lesion size of 13.5×13.8 mm (Figure 1C). Six months later, follow-up with US, CEUS and However, we additionally detected an internally MRI showed that the lesion had slightly sporadic blood flow Figure( 1D), which was increased in size (on US and CEUS) and indi- initially suggestive of a malignant transforma- cated the presence of a tumor-feeding vessel. tion. The MRI findings suggested that the lesion was benign. Because SHCC could not be ruled out Contrast enhanced ultrasound (CEUS) on US and CEUS, considering the patient’s his- tory of hepatitis B, a biopsy was indispensable During the first examination, a 2.5 ml solution in order to identify the final diagnosis. The of the microbubble contrast agent Sonovue® patient had suffered right abdominal discom- was injected into the median cubital vein. fort, but did not present with other clinical Twelve seconds later, the dye began to delin- symptoms such as fever, nausea or vomiting, eate the lesion outline (Figure 1E), and reached cough and expectoration, gastrointestinal dys- peak contrast at 19 seconds (Figure 1F). The function, jaundice, hematemesis or melena. contrast lasted 24 seconds and then began to Laboratory examination found no abnormal subside (Figure 1G). In the retrospective analy- results, including α-FP and other tumor-related sis six months later, a tumor-feeding vessel was index. Next, we present the strategies used for observed during the contrast enhancement diagnosis: course, which was not detected at first exami- nation of the CEUS data (Figure 1H). Conventional ultrasound (US) At the six-month follow-up, both the arrival and The first ultrasound examination was conduct- peak contrast times were two seconds earlier ed at initial screening. A solid non-homoge- than that of the first examination, 10 seconds neous hypoechoic lesion was detected in the (Figure 1I) and 17 seconds (Figure 1J), respec- liver, measuring 8.9×13.6 mm (Figure 1A). This tively. Similar to the initial screening, imaging lesion was located in the upper segment of the started to subside at 24 seconds (Figure 1K). right anterior hepatic lobe, and was considered Again, the tumor-feeding vessel was also a benign lesion due to its clear boundary, regu- detected (Figure 1L). By CEUS examination, the lar shape and the missing internal blood flow lesion demonstrated a slight increase in size, 13147 Int J Clin Exp Med 2015;8(8):13145-13153 Bile duct hamartomas Figure 3.