Diagnostic Value of Magnetic Resonance Imaging in Fibrodysplasia Ossificans Progressiva

Abstract

Using [¹⁸F] Sodium Fuoride (NaF) Positron Emission Tomography (PET) it is not only possible to identify the ossifying potency of a flare-up, but also to identify an asymptomatic chronic stage of fibrodysplasia ossificans progressiva (FOP). The purpose of this study was to investigate the diagnostic role of a more widely available imaging modality, Magnetic Resonance Imaging (MRI), which is of special interest for studies in pediatric FOP patients. MRI and [¹⁸F]NaF PET/CT images at time of inclusion and subsequent follow-up CT scans of 4 patients were analyzed retrospectively. Presence, location, and intensity of edema identified by MRI were compared with activity on [¹⁸F]NaF PET. Occurrence or progression of heterotopic ossification (HO) was examined on the follow-up CT images. Thirteen different lesions in various muscle groups were identified: five lesions with only edema, five lesions with both edema and increased [¹⁸F]NaF uptake, one lesion with only increased [¹⁸F]NaF uptake, and two lesions with neither edema nor uptake of [¹⁸F]NaF. Mild edema, found in three lesions, was present at asymptomatic sites, which did not show increased [¹⁸F] NaF uptake or progression of HO on consecutive CT images. Moderate edema was found in three symptomatic lesions, with increased [¹⁸F]NaF on PET and progression of HO on CT. Severe edema was identified in four lesions. Interestingly, two of these lesions did not develop HO during follow-up; one of these two even gave obvious symptoms of a flare-up. MRI can identify whether symptoms are the result of an acute flare-up by the presence of moderate to severe edema. The occurrence of severe edema on MRI was not always related to an ossifying lesion. The additional diagnostic value of MRI requires further investigation, but MRI does not seem to fully replace the diagnostic characteristics of [¹⁸F]NaF PET/CT in FOP. © 2020 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.

Keywords: ANALYSIS/QUANTITATION OF BONECLINICAL TRIALSDISEASES AND DISORDERS OF/RELATED TO BONEFIBRODYSPLASIA OSSIFICANS PROGRESSIVARADIOLOGY.

Figure 1

Consecutive MRI scans and [¹⁸F]NaF PET scans of a patient with several flare‐ups. Coronal MRI T2‐weighted short‐TI inversion recovery (STIR) images are shown of a patient with multiple flare‐ups. Starting in the loin (A + D), later also the groin (B) and upper leg (C + E). Time in months. A and D; T = 0. Clinically, a flare‐up in the pelvic area with pain and swelling of the entire right loin. MRI (A) showed moderate and severe edema of the musculus psoas dextra (white arrows) and the musculus iliacus dextra (blue arrows), respectively. Also, the musculi adductors (red arrows) showed moderate edema, even though no clinical signs were noted. The MRI showed also an area of nonspecific mild edema (white circle). [¹⁸F]NaF PET showed increased high uptake of tracer in the psoas muscle, mild uptake in the mm. adductors and no uptake in the iliopsoas muscle. B; T = 1. Edema at both the musculus psoas and musculus iliacus diminished, to mild and moderate edema, respectively. Edema intensity at the musculi adductors increased to severe, the patient now reported flare‐up symptoms at the groin too. The mild edema seen in plane A resolved; no calcifications were noted. C and E; T = 11. Edema at the psoas muscle, and the iliacus and adductor muscles is completely resolved, but new edema formed in the quadriceps muscle (C). High [¹⁸F]NaF uptake in the quadriceps (E). F; T = 21. Low‐dose whole‐body CT showed heterotopic ossification (HO) in the psoas muscle, HO at the site of the adductor muscles (red arrow), and in the quadriceps muscle. No HO formed in the iliopsoas (blue arrow).