Uremic leontiasis ossea

Case contributed by Dalia Ibrahim
Diagnosis almost certain

Presentation

Right palatal swelling.

Patient Data

Age: 25 years
Gender: Male
ct

Diffuse expansion of the maxillary and mandibular bones showing cortical thinning, dense trabeculae, and serpiginous lucencies/tunnels (feline stripes) within the bone marrow. Also a focal asymmetric right hard palatal bone swelling showing the zonal pattern with alternating hypodense and hyperdense rings

Other features of renal osteodystrophy include:

  • diffuse calvarial thickening with widened diploic space, cortical resorption, and salt and pepper appearance
  • vertebral osteomalacia with multiple osteolytic lesions
  • erosive changes of both temporomandibular joints

The 3D surface rendered images show widening of the nares, flattened nasal bridge, and widened interdental spacing.

Photo

Annotated images show the characteristic appearances of the intramedullary serpiginous lucent tunnels (feline stripes) (yellow arrows), and asymmetric focal right hard palatal bone swelling, also showing the zonal pattern (blue arrows).

Case Discussion

The patient was known to have chronic renal failure on hemodialysis. He had a history of progressive facial swelling with current right hard palatal hard swelling.

Uremic leontiasis ossea is a rare condition that occurs in patients with renal failure on hemodialysis with secondary hyperparathyroidism. It is characterized by swelling of the jaws, widening of the nares, flattened nasal bridge, and increased interdental spacing.

Radiographic features include:

  • radiological criteria of renal osteodystrophy and secondary hyperparathyroidism

    • osteomalacia

    • skull thickening with salt and pepper appearance

    • diffuse maxillary and mandibular expansion with:

      • characteristic intramedullary serpiginous tunnels (feline stripes), or

      • zonal pattern which is characterized by alternating hypoattenuation and hyperattenuation rings caused by osteoclastic activity (lytic lesions) and osteoblastic activity (sclerosis) respectively

      • focal asymettric maxillary or mandibular bone swellings

Differential diagnoses for this craniofacial appearance include fibrous dysplasia and Paget disease.

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