Clinical Images: Crowned mandible: A case of calcium pyrophosphate deposition disease involving the temporomandibular joint

The patient, a 68-year-old man, was referred to rheumatology following a computed tomography scan done for right ear pressure changes that revealed extensive changes of calcium pyrophosphate deposition (CPPD) involving the right temporomandibular joint (TMJ) ([A] coronal view), including extensive calcification and erosion through the skull base and into the tympanic cavity, abutting the middle ear ossicles ([B] sagittal view). He reported intermittent episodes of frontal headaches, vertigo, low grade temperature, and right ear pressure worsening over the last six months. Physical examination demonstrated fullness around the right mandible and bulky metacarpophalangeal joints without synovitis. The patient did not report any history of acute warm, swollen, or tender peripheral joints. Evaluation for secondary causes of CPP arthritis was unremarkable including comprehensive metabolic profile, phosphorus, parathyroid hormone, magnesium, iron panel, and ferritin. Radiographs of the bilateral hands and knees demonstrated chondrocalcinosis. Surgical pathology from open biopsy confirmed CPPD. Age and history of possible remote trauma to the jaw most likely contributed to development of CPPD within the right TMJ. The patient underwent a middle fossa craniotomy for CPPD disease removal with mandibular condylectomy and TMJ arthroplasty. He was started on low dose colchicine before surgical intervention and continued for six weeks afterward to prevent acute flare of pseudogout following surgery. There are few reported cases of CPP arthritis involving the TMJ, rarely involving the skull base.1 Symptomatic patients may present with pain, swelling, and conductive hearing loss.1, 2 Management is typically surgical.1, 2 Disclosure form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.