Imaging Manifestations of Hepatic Involvement in Blue Rubber Bleb Nevus Syndrome: A Preliminary Retrospective Study
Qin Yang, Aolan Ran, Dajing Guo, Xiaojing He, Jun Zhou, Haiping ZhangIntroduction:
The imaging characteristics of hepatic involvement in Blue Rubber Bleb Nevus Syndrome (BRBNS) have not been systematically described, with the limited available literature merely describing it as a cavernous hemangioma. The purpose of this study is to retrospectively characterize the imaging features of hepatic involvement in BRBNS.
Materials and Methods:
Imaging findings from four patients with clinically confirmed BRBNS were retrospectively reviewed. Available examinations included helical Computed Tomography (CT) in four patients, Magnetic Resonance Imaging (MRI) in two patients, and Transabdominal Ultrasonography (TUS) in three patients. Lesion morphology, distribution, attenuation/signal characteristics, enhancement patterns, and associated extrahepatic involvement were evaluated. The Short-axis to Long-axis ratio (S/L ratio) of each lesion was calculated, and the relative attenuation values between the lesions and background liver parenchyma during each phase of contrast-enhanced scanning were assessed. Endoscopic examinations were available in four patients.
results:
CT showed multiple (n=3) or solitary (n=1) lesions within the liver. The lesions were predominantly located in the peripheral regions of the liver, exhibiting a "serpentine" or "sheath-like" growth pattern. Multilocular calcifications were observed in four patients. All lesions demonstrated an enhancement pattern characterized by scattered nodular hyperenhancement within the lesion or along its periphery during the arterial phase, with progressive centrifugal enhancement during the portal venous and delayed phases. MR imaging showed lesions exhibiting T2 hyperintensity similar to that of classical hepatic cavernous hemangioma. The short-axis to long-axis ratio (S/L ratio) was < 1 in all cases. The ultrasound examination did not effectively diagnose hemangiomas or vascular malformations. Endoscopic examinations showed venous tumors in the four patients.
Results:
CT showed multiple (n=3) or solitary (n=1) lesions within the liver. The lesions were predominantly located in the peripheral regions of the liver, exhibiting a “serpentine” or “sheath-like” growth pattern. Multilocular calcifications were observed in four patients. All lesions demonstrated an enhancement pattern characterized by scattered nodular hyperenhancement within the lesion or along its periphery during the arterial phase, with progressive centrifugal enhancement during the portal venous and delayed phases. MR imaging showed lesions exhibiting T2 hyperintensity similar to classical hepatic cavernous hemangiomas. The Short-axis-to-Long-axis ratio (S/L ratio) was < 1 in all cases. The ultrasound examination did not effectively detect hemangiomas or vascular malformations. Endoscopic examinations showed venous tumors in all four patients.
Discussion:
The distinctive imaging features observed in this series may reflect the venous malformation nature of BRBNS and its characteristic pattern of hepatic involvement.
Conclusion:
Hepatic involvement in BRBNS may demonstrate characteristic imaging features, including subcapsular distribution, serpentine morphology, intralesional calcifications, and progressive centrifugal enhancement on contrast-enhanced imaging, which may aid in the recognition of this rare disease.