Calcium Pyrophosphate Deposition Disease of the Temporomandibular Joint

Abstract

).Calcium pyrophosphate dihydrate deposition disease (CPDD) is a rare crystal arthropathy characterized by the deposition of calcium pyrophosphate crystals in joint spaces, episodes of synovitis, and radiological features of chondrocalcinosis. 1,2While the disease is most prevalent in the shoulders, pelvis, knees, and joints of the hands, the axial skeleton can be affected, but rarely involves the skull base.2-4 When involved, CPDD can cause symptoms mimicking more common neurological and neurosurgical pathologies. 2,5 We present a patient who was recently treated at our institution whose pathology demonstrated CPDD. Case ReportThe patient is a 51-year-old right-handed Caucasian woman with a 12-month history of left ear pain that originated from the temporomandibular joint (TMJ), with some associated swelling, pain with chewing, and mild hearing loss. Her physical examination revealed mild hearing loss on the left side. Computed tomography (CT) and magnetic resonance imaging of the temporal fossa revealed a large mass emanating from the temporal bone at the TMJ (►Fig. 1A, 1B), extending into the greater wing of the sphenoid, and involving the mastoid bone and air cells posteriorly. There was erosion of the petrous carotid canal. A CT-guided biopsy was performed which diagnosed the mass as tophaceous pseudogout (tumoral CPDD). Keywords► temporomandibular joint ► calcium pyrophosphate ► CPDD ► chondrocalcinosis ► tophaceous pseudogout AbstractCalcium pyrophosphate dihydrate deposition disease (CPDD, tophaceous pseudogout) is a rare crystal arthropathy characterized by calcium pyrophosphate crystal deposition in joint spaces, episodes of synovitis, and radiological features of chondrocalcinosis. We present a case of 61-year-old woman who presented with left temporomandibular joint (TMJ) pain, difficulty chewing, left facial numbness, left-sided hearing loss, and left TMJ swelling. Imaging of the temporal fossa revealed a large mass emanating from the temporal bone at the TMJ, extending into the greater wing of the sphenoid and involving the mastoid bone and air cells posteriorly. Fine needle aspiration demonstrated polarizable crystals with giant cells. Intraoperatively, the TMJ was completely eroded by the mass. Final pathology was consistent with tophaceous pseudogout. CPDD has rarely been reported involving the skull base. None of the cases originally described by McCarty had TMJ pseudogout. Symptoms are generally pain, swelling, and hearing loss. Management is nearly always surgical with many patients achieving symptomatic relief with resection. CPDD is associated with many medical problems (including renal failure, gout, and hyperparathyroidism), but our patient had none of these risk factors. This case demonstrates that CPDD can involve the skull base and is best treated with skull base surgical techniques.

Treatment of Nongout Joint Deposition Diseases: An Update

“…Case reports suggest an inefficiency of Anakinra on chronic manifestations of CPPD [ 27 ] especially in the absence of systemic inflammation [ 28 ]. CPPD treatment is essentially medical but surgical treatment has been carried out successfully for the management of temporomandibular involvement [ 29 , 30 ].…”

Calcium pyrophosphate deposition disease of the temporomandibular joint invading the middle cranial fossa: Two case reports

“…CT can clearly show the size of the lesion, calcification and extent of bone destruction. The mandibular condyle is most often involved, as well as the mastoid process and the greater wing of the sphenoid bone[ 13 ]. Both cases in our study involved the temporal bone and the greater wing of the sphenoid bone, and one involved the middle ear mastoid process.…”

“…MRI showed a mass with abnormal signals in the articular cavity, which was mostly T1WI hypointense and T2WI hypointense with significant inhomogeneous enhancement. Masses can grow along articular cartilage, with cartilage and synovial sac involvement[ 8 , 9 , 13 - 15 ]. CT is superior to MRI in demonstrating calcification and has suggestive value for disease diagnosis.…”

A Curious Case of Crystal Deposit Disease in the Petrous Bone

“…Surgical excision should be considered treatment of choice in symptomatic cases. Incomplete excision can lead to recurrences [3]. We chose a subtotal petrosectomy with obliteration and blind sac closure since hearing preservation was not of consideration and it might reduce the risk of future paradoxical CSF leak and meningitis because of the lesion’s involvement into the middle cranial fossa.…”

“…Benign dense soft tissue calcium containing deposits are referred to as tumoral calcinosis and are build up from calcium hydroxyapatite crystals [1]. It often affects joints such as the hips, elbows and shoulders, but is rarely found in other sides [2,3]. In some cases, the temporal mandibular joint is involved as pseudogout with the accumulation of calcium pyrophosphate dihydrate (CPPD) crystals [1].…”