PMC9753944_01
The patient presented with a draining sinus tract on the cheek below the lateral half of the right eye.
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On examination, the patient appeared pale with a draining sinus tract on his cheek below the lateral half of the right eye (Figure 1), frontal bossing, bilateral horizontal nystagmus, and poor dentition with carious teeth.
Chest x-ray shows a generalized increase in bone density in the clavicles, scapulae, and ribs (arrows).
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Chest x-ray revealed a generalized increase in bone density in the visualized clavicles, scapulae, and ribs (Figure 2).
Skull radiographs show (A) periorbital sclerosis (arrows) and (B) thickening of the calvarium and the visualized cervical vertebrae (arrows).
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The diffuse osteosclerosis prompted a skeletal survey that showed periorbital sclerosis (Figure 3A). Lateral radiograph of the skull also showed thickening of the calvarium and the visualized cervical vertebrae (Figure 3B).
Radiographs of the hands show alternating bands of radiodense and radiolucent lines at the distal metaphyses of the radii and ulnae (arrows).
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Alternating radiodense and radiolucent lines were seen at the distal metaphyses of the radii and ulnae (Figure 4).
Erlenmeyer flask deformities are seen at the distal ends of (A) the radii (arrows) and (B) the femora (arrows).
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Erlenmeyer flask deformities, characterized by reduced constriction of the diaphyses, were seen at the distal ends of the radii (Figure 5A) and the femora (Figure 5B).
(A) Anteroposterior view of the spine shows a bone-within-a-bone appearance in the iliac blades (lower arrows) and a sandwich vertebrae appearance (upper arrow). (B) Lateral view of the spine shows a sandwich vertebrae appearance (arrow).
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A bone-within-a-bone appearance was visible in the iliac blades, in addition to a sandwich vertebrae appearance because of sclerosis of the vertebral endplates (Figure 6).