European Radiology

, Volume 27, Issue 4, pp 1335–1343 | Cite as

Infraorbital nerve involvement on magnetic resonance imaging in European patients with IgG4-related ophthalmic disease: a specific sign

  • J. Ben Soussan
  • R. Deschamps
  • J. C. Sadik
  • J. Savatovsky
  • L. Deschamps
  • M. Puttermann
  • M. Zmuda
  • F. Heran
  • O. Galatoire
  • H. Picard
  • A. Lecler
Head and Neck



To measure the frequency of infraorbital nerve enlargement (IONE) on magnetic resonance imaging (MRI) in European patients suffering from an IgG4-related ophthalmic disease (IgG4-ROD) as compared to patients suffering from non-IgG4-related ophthalmic disease (non-IgG4-ROD).


From January 2006 through April 2015, 132 patients were admitted for non-lymphoma, non-thyroid-related orbital inflammation. Thirty-eight had both pre-therapeutic orbital MRI and histopathological IgG4 immunostaining. Fifteen patients were classified as cases of IgG4-ROD and 23 patients as cases of non-IgG4-ROD. Two readers performed blinded analyses of MRI images. The main criterion was the presence of an IONE, defined as the infraorbital nerve diameter being greater than the optic nerve diameter in the coronal section.


IONE was present in 53% (8/15) of IgG4-ROD cases whereas it was never present (0/23) in cases of non-IgG4-ROD (P < 0.0001). IONE was only present in cases where, on MRI, the inflammation of the inferior quadrant was present and in direct contact with the ION canal.


In European patients suffering from orbital inflammation, the presence of IONE on an MRI is a specific sign of IgG4-ROD. Recognition of this pattern may facilitate the accurate diagnosis for clinicians and allow for the adequate management and appropriate care of their patients.

Key points

IONE on an MRI is a specific sign of IgG4-ROD.

IONE recognition allows for a quicker diagnosis and appropriate management.

IONE appears when inflammation is in direct contact with the ION canal.


IgG4-related ophthalmic disease Orbital inflammation MRI Infra-orbital nerve enlargement Diagnosis 



IgG4-related ophthalmic disease


non-IgG4-related ophthalmic disease


Magnetic resonance imaging


Infra-orbital nerve


Infra-orbital nerve enlargement


Mild infra-orbital nerve enlargement



The scientific guarantor of this publication is Augustin Lecler, MD. The authors of this manuscript declare no relationships with any companies, whose products or services may be related to the subject matter of the article. The authors state that this work has not received any funding. One of the authors has significant statistical expertise. Institutional Review Board approval was obtained. Written informed consent was obtained from all subjects (patients) in this study. Methodology: retrospective, diagnostic or prognostic study, performed at one institution.


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Copyright information

© European Society of Radiology 2016

Authors and Affiliations

  1. 1.Department of RadiologyFondation Ophtalmologique Adolphe de RothschildParisFrance
  2. 2.Department of NeurologyFondation Ophtalmologique Adolphe de RothschildParisFrance
  3. 3.Department of PathologyBichat Hospital, APHPParisFrance
  4. 4.Department of PathologyNecker-Enfants Malades Hospital, APHPParisFrance
  5. 5.Department of Orbitopalpebral SurgeryFondation Ophtalmologique Adolphe de RothschildParisFrance
  6. 6.Clinical Research UnitFondation Ophtalmologique Adolphe de RothschildParisFrance

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