PMC9459495_01
Cervical magnetic resonance image (MRI) (A, B, C) showing areas of
altered signal, hyperintense in T2-weighted images involving the
articular surfaces of the joint between the atlas and epistrophe
on the right side (A: axial fat suppression T2-weighted
sequences), gaining the contrast medium and surrounding a large
area of osteorarefaction compatible with osteolysis (B: axial
postcontrast T1-weighted images). Voluminous anterior somatic
osteophytes (not significantly compressing the esophagus) and
posterior osteophytic disk bars which mark the anterior profile
of the medulla at several levels (C: sagittal fat suppression
T2-weighed sequences). No areas of altered spinal cord signal
are detected. Neck computed tomographic (CT) scan (D, E). As
partially visible in neck MRI, in CT images the focal osteolytic
alteration involving the right side of the apex of the tooth and
the atloaxial joint, associated with irregular bone margins due
to the presence of fine erosions, is confirmed (D: coronal view;
E: axial view). (F) shows lateral radiography of the cervical
spine, which demonstrated a posterior dislocation of the atlas
with respect to the axis. Simultaneously, aspects of
spondyloarthrosis are appreciable in the C3-C7 cervical tract
with reduction in thickness of almost all the related
intersomatic spaces.
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After admission, neck MRI revealed hyperintensity in T2-weighted sequences on the articular surfaces of the right atlo-epistrophic joint (Figure 1A), with contrast enhancement surrounding a large area of osteolysis (Figure 1B). Anterior somatic and posterior disk osteophyte bars slightly imprinting the medulla anterior profile without impairing the spinal cord were also appreciable (Figure 1C). Computed tomographic (CT) scan confirmed the presence of the osteolytic lesion on the right side of the atloaxial joint with irregular margins due to the presence of fine erosion (Figure 1D and E). A mild posterior subluxation of the atlantoaxial joint with right dislocation of the epistrophic tooth with respect to the atlas was documented, together with the presence of an areola of cortical erosion on the right side of the apex of the tooth (Figure 1F).
Whole-body [18F]2-Deoxy-2-fluoro-d-glucose positron
emission tomography imaging showing the presence of modest focal
hypercaptation of the radiotracer (SUV max 4.8) in
correspondence with the known structural alterations of the
articular surfaces between the atlas and the epistrophe on the
right side (A: sagittal view; B: coronal view).
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Consequently, the patient underwent total-body [18F]2-Deoxy-2-fluoro-d-glucose (FDG) positron emission tomography (PET) examination, which only revealed isolate mild hypercaptation at the atlantoepistrophic level (SUV max 4.8) (Figure 2).
Bone scintigraphy exhibiting modest hyperfixation in correspondence
with the articular passage between the atlas and the epistrophe
of the right side, site of known osteo-structural alterations
described in magnetic resonance imaging and computed tomographic
images. Mild hyperfixation at the scapular girdle, lumbar L4-L5
levels (site of known spondylolisthesis), and trapeziometacarpal
joint of both hands, on a degenerative basis.
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Bone scintigraphy was also performed, confirming isolate mild hypercaptation at the same level (Figure 3).
(A and B) Histopathological finding revealing the band-like
complete loss of elastic fibers in the mid-dermis, annotated
with dotted lines, properly studied by elastic stains; elastic
fiber fragmentation is also detected (Weigert stain, 5x
magnification/A and 20x magnification/B). (C) Hematoxylin and
eosin staining did not reveal pathological findings except for
slight lymphohistiocytic perivascular infiltrates around the
vessels of the superficial plexus in the lesional sites. There
is no evidence of dermal actinic damage such as elastosis (5x
magnification). (D) Immunolabelling for CD163 (a marker of
monocytic/histiocytic lineage) revealed a discrete increased
number of interstitial histiocytes/macrophages engulfing elastic
fibers within the mid-dermis and occasional figures of
elastophagocytosis (5x magnification).
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Chest magnetic resonance imaging. At the level of the right
sternoclavicular joint, a periarticular and intra-articular
effusion is observed (A: axial T1-weighed image; B: axial Short
tau inversion recovery [STIR] image). Reactive sponge edema of
the medial extremity of the right clavicle is appreciated, in
the context of millimetric areas of hyperintense signal in the
long TR sequences in the subchondral area, referable in first
hypothesis to pseudocystic aspects or erosions (C: coronal STIR
image).
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Chest MRI revealed intra-articular effusion with marked bone edema of the medial extremity of the right sternoclavicular joint and millimetric pseudocystic aspects of the bone (Figure 5).