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Coronary Artery Manifestation of Ormond Disease: The "Mistletoe Sign"

Pál Maurovich-Horvat1, Ferenc Imre Suhai1, Csilla Czimbalmos1

  • 1From the MTA-SE Cardiovascular Imaging Research Group, Heart and Vascular Center, Semmelweis University, Városmajor St 68, 1122 Budapest, Hungary (P.M.H., F.I.S., C.C., A.T., D.B., H.V., B.M.); National Institute of Rheumatology and Physiotherapy, Budapest, Hungary (E.K.); and Cardiac MR PET CT Program, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, Mass (M.F., U.H.).

Radiology
|August 23, 2016
PubMed

Insights

Idiopathic retroperitoneal fibrosis, or Ormond disease, can affect the heart. A rare "mistletoe sign" on cardiac imaging may help diagnose this condition.

Area of Science:

  • Cardiovascular Imaging
  • Radiology
  • Fibrotic Diseases

Background:

  • Idiopathic retroperitoneal fibrosis (IPF), also known as Ormond disease, is a rare condition.
  • Cardiac involvement in IPF is uncommon but can lead to significant morbidity.
  • Accurate and timely diagnosis of cardiac manifestations of IPF is crucial.

Observation:

  • A 69-year-old woman with presumed cardiac involvement of IPF presented with distinct pericoronary tissue proliferations.
  • These proliferations were visualized using cardiac magnetic resonance (MR) imaging and coronary computed tomographic (CT) angiography.
  • The characteristic appearance on imaging was termed the "mistletoe sign".

Findings:

  • The "mistletoe sign" represents pericoronary tissue proliferations.
  • This sign was identified on both cardiac MR and coronary CT angiography.
  • The presence of the mistletoe sign in this context is likely rare but potentially characteristic of IPF.

Implications:

  • The "mistletoe sign" may serve as a diagnostic clue for cardiac involvement in retroperitoneal fibrosis.
  • Increased utilization of noninvasive cardiac imaging may lead to more frequent identification of this sign.
  • Recognition of the mistletoe sign could aid in the diagnosis and management of patients with Ormond disease.

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