Calcium pyrophosphate dihydrate deposition disease of the temporomandibular joint- a rare differential diagnosis in the parotid area

The differential diagnosis of tumors of the parotid area includes not only neoplasias of the parotid gland but also pathologies of the temporomandibular joint. Calcium pyrophosphate dihydrate deposition disease or pseudogout is a rare disease, which emerges through accumulation of inorganic pyrophosphate in the synovia. High Age is the main risk factor, followed by other metabolic diseases.

The 78-year-old female patient was presented with an over four years progressing tumor in the parotid area, recurring abscesses and chronic otitis externa due to constriction of the inner ear canal. The audiometry revealed severe conduction hearing loss with an air-bone gap up to 60 dB. Computed tomography showed a growing calcified tumor of the parotid area, reaching into the infratemporal fossa and masticator space with progressive destruction of the mandibular condyle, the foramen ovale, the foramen spinosum and the carotid canal. Histological examination revealed calcium phosphate dehydrate deposition disease. The surgery was performed as an enucleation with partial parotidectomy, temporal craniectomy and resection of the temporomandibular joint, while saving the continuity of the facial nerve. The outer ear canal could be decompressed, and the resection cavity was obliterated with abdominal fat. After surgery, the facial nerve was completely functional. Jaw opening and eating, as well as chewing was possible. An increase in hearing could also be shown.

Surgery with resection of the crystalin material remains the standard procedure. Steroids, nonsteroidal-antiinflammatory drugs (NSAID), or Colchicine can be used to reduce the symptoms. Although there is no causal therapy, the prognosis is good. Recurrences in the jaw joint have not yet been described.

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Artikel online veröffentlicht:
10. Juni 2020