Hereditary hemorrhagic telangiectasia of liver: Pathophysiology with role of radiology in diagnosis and treatment

Hereditary hemorrhagic telangiectasia (HHT) or Osler–Weber–Rendu syndrome is a rare condition which can result in significant systemic and hepatobiliary abnormalities. Liver involvement in HHT consists primarily of the consequence of various intrahepatic shunts. Even though these vascular shunts are present in the majority of patients with HHT, symptoms occur only in minority with clear predilection to female gender. The symptoms and imaging findings of liver vascular malformations can be easily overlooked or misdiagnosed which can result in delay in treatment or potentially harmful vascular interventions. In this case report, we discuss the pathophysiology of HHT in liver involvement, role of imaging in diagnosis, and the possible role of interventional radiologist in the treatment

An unusual cause of breathlessness and profuse micronodules

We describe a 21-year-old male with a history of smoking and subacute onset of breathlessness with normal cardiorespiratory examination. The presence of “track marks” and digital infarcts prompted evaluation for infective endocarditis and confrontational history taking revealed anorexia, weight loss over 3 months along with intravenous drug abuse of reconstituted tablets of tapentadol. Echocardiography was normal and blood cultures were sterile; computed tomography showed bilateral, diffuse, small centrilobular nodules with “tree-in-bud” appearance. In this clinicopathologic conference, we discuss the clinical and radiological differential diagnosis of centrilobular nodules, lung biopsy findings, and management options for patients with such a presentation