John T. Mather Memorial Hospital Medical Center Port Jefferson, NY, United States
Faraz Badar, MD1, Haseeb Siddique, MD1, Siddhant Kulkarni, DO1, Ramona Rajapakse, MD, FRCP, FACG2 1John T. Mather Memorial Hospital Medical Center, Port Jefferson, NY; 2Zucker School of Medicine at Hofstra/Northwell Health, Port Jefferson, NY
Introduction: Multiple Biliary Hamartomas (MBHs), also known as “Von Meyenberg Complexes”', are rare, benign, liver cysts, usually discovered incidentally. We describe 2 cases with these lesions, one asymptomatic, and one symptomatic.
Case Description/Methods: Case 1: A 43 yo female without GI complaints or past medical history, was referred for evaluation of multiple small liver cysts seen on CT chest performed for dyspnea. CT abdomen revealed multiple cysts with one containing protein and another septations. CBC, LFTs, CA 19-9, Echinococcus antibodies and Alpha-fetoprotein were normal. MRI showed multiple nonenhancing subcentimeter lesions compatible with MBHs throughout the liver and solitary cysts with thin septations and slight protein content. The patient was recommended to follow up in 6 months for surveillance MRI. Case 2: A 53 yo female presented with intermittent, mild to moderate, dull, RUQ abdominal pain for 6 months, aggravated by heavy lifting. CT scan revealed a large left lobe liver cyst with numerous other smaller cysts. MRI revealed multiple simple cysts throughout the liver consistent with MBHs. The largest was 6 cm in size, as seen on CT scan. As she continued to have abdominal pain, the patient was referred to a liver surgeon for evaluation and management.
Discussion: MBHs are rare, benign, cystic liver lesions with an incidence of 5.6% in autopsy series(1) and < 1% in imaging series. Sonographically, MBHs are seen as innumerable tiny hypoechoic, hyperechoic or mixed foci. CT shows hypoattenuating, nonenhancing lesions whereas MRI shows hypo signal on T1WI and hyper signal on T2WI. Since technical advances in radiology have made MBHs easier to diagnose by imaging, particularly MRI, biopsy is recommended only when the diagnosis is in doubt(2). Most authors suggest clinical, biochemical and imaging surveillance once the diagnosis of MBH is made because, although usually stable, there are a few reports describing malignant transformation to Cholangiocarcinoma. However, screening and treatment guidelines have yet to be defined.
References: 1. Redston MS, Wanless IR. The hepatic von Meyenburg com-plex: prevalence and association with hepatic and renal cysts among 2843 autopsies. Mod Pathol 1996; 9:233–23 2. Liu S, Zhao B, Ma J, Li J, Li X. Lesions of biliary hamartomas can be diagnosed by ultrasonography, computed tomography and magnetic resonance imaging. Int J Clin Exp Med. 2014;7(10):3370-3377
Figure: Case 1 A. Axial CT demonstrates multiple hypodense round lesions throughout the liver. Two cysts have slightly increased Hounsfields units. B. Axial T2 MRI & C. Axial T1 Post-contrast MRI lesions seen on CT demonstrate T1 hypointensity, T2 hyperintensity, and no post-contrast enhancement. One cyst contains an internal septation and another contains internal proteinaceous material. Case 2 D. Axial CT Large hypodense cystic lesion in the left hepatic lobe measuring 5.9 x 6 cm, multiple other subcentimeter hypodense round lesions scattered throughout the liver. E. Axial T2 MRI & F. Axial T1 Post-contrast MRI lesions seen on CT demonstrate T1 hypointensity, T2 hyperintensity with no septations, and no post-contrast enhancement.
Faraz Badar indicated no relevant financial relationships.
Haseeb Siddique indicated no relevant financial relationships.
Siddhant Kulkarni indicated no relevant financial relationships.
Ramona Rajapakse indicated no relevant financial relationships.
Faraz Badar, MD1, Haseeb Siddique, MD1, Siddhant Kulkarni, DO1, Ramona Rajapakse, MD, FRCP, FACG2. P1924 - Multiple Biliary Hamartomas: A Lesser Known Differential for Cystic Lesions of the Liver, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.