Abstract
The authors present the case of a 64-year-old woman with a destructive calcium pyrophosphate dihydrate (CPPD) crystal deposition disease of the temporomandibular joint. Progressive pain, swelling and a malocclusion were her chief complaints. A few granular calcified masses surrounding the left condylar head and extending to the infratemporal fossa and middle cranial base were presented in CT images. It occurred alone without other joints being affected. A provisional diagnosis of occupying lesion with invasion was made preoperatively, but histologically, the mass contained numerous deposits of rod-shaped or rhomboid crystals, which were positively birefringent under a polarising microscope, suggesting a CPPD deposition disease. The histopathological diagnosis was further supported by scanning electron microscopy with energy dispersive X-ray spectroscopy. The diagnosis, differential diagnosis and treatment of this disease are discussed.
Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease is characterized by the accumulation of pyrophosphate dihydrate crystals in articular and periarticular tissues. Large joints such as the knee and wrist are the most common sites affected. CPPD deposition disease of the temporomandibular joint (TMJ) is rare . The authors present the case of a 64-year-old woman with a destructive and invasive facial mass extending to the infratemporal fossa. A provisional diagnosis of an occupying lesion with invasion was made preoperatively.
Case report
A 64-year-old woman was admitted to hospital for the diagnosis and treatment of a swelling and pain in the left preauricular region. The patient presented a 5-year history of chronic pain and swelling. The symptoms were aggravated and followed a malocclusion with a deviation of mandible and a limitation of mouth opening for 1 year. She had a history of hypertension, hypercholesterolaemia and a gallbladder stone. There was no history of trauma to the orofacial region and no history of metabolic disturbances, such as hyperparathyroidism, chronic renal failure or diabetes. There was no history of disease involving any other joints. The preoperative blood investigations showed slightly higher phosphate, 1.49 mmol/l (normal 0.81–1.46) and cholesterol 5.89 mmol/l (normal 3.10–5.70), but other electrolytes, including urate and calcium, were normal.
Clinical examination showed an obvious preauricular swelling with tenderness on the left side. Stenosis of the external ear canal was found because of the swelling, but there was no hearing loss. Interincisal mouth opening was limited to 25 mm. A malocclusion was present with a slight deviation of mandible to the right side. She had a maxillary complete denture and removable partial denture in the mandible with only eight teeth left.
A panoramic image showed three big masses with gravel-like appearance and calcified foci in the left joint region. The glenoid fossa was destructive and the condyle was displaced anteroinferiorly due to the extrusion of the masses ( Fig. 1 A ). On an axial CT, there were a few granular calcified masses surrounding the left condylar head and extending into the infratemporal fossa ( Fig. 1 B). Coronal CT scans revealed calcified mass in the joint space. Destruction and sclerosis of the middle cranial base were presented and the lesion seemed to extend into the middle cranial fossa ( Fig. 1 C). Although sclerosis was shown in the condyle, the right joint had no evidence of a similar manifestation to the left side on the CT scan. A provisional diagnosis of occupying lesion with invasion such as chondroma/osteochondroma or chondrosarcoma/osteochondrosarcoma was made preoperatively.
Surgical exploration of the left TMJ was planned. During surgery, a few white and gritty, dough-like masses were removed from the lateral aspect of the upper joint space ( Fig. 2 ). Microscopically, the frozen section revealed much crystalline material within the biopsy specimen. A large amount of similar white, gritty material was curetted from the anteromedial and posterior aspects of the upper joint space. Although the glenoid fossa was destructive, it was not perforated with the skull base. The meniscus appeared normal in colour and texture. No additional masses were found in the inferior joint space. There were no destructive changes in the condyle.
Histologically, under light microscopy, an amorphous substance or chondromyxoid tissue containing abundant crystal deposits was observed. The crystals were positively birefringent under a polarising microscope and rod- or rhomboid-shaped, which strongly suggested a diagnosis of CPPD deposition disease of the TMJ ( Fig. 3 ).
Scanning electron microscopy (SEM) revealed rod- and rhomboid-like crystals mainly ranging from 1 to 5 μm in size. Energy dispersive X-ray spectroscopy (EDS) of these crystals showed peaks corresponding to calcium and phosphorus ( Fig. 4 ). Qualitative analysis indicated that the calcium and phosphorus ratio was close to 1, which further supported the histological diagnosis of CPPD deposition disease.
The postoperative course was uneventful. The patient’s clinical symptoms improved soon after surgery. Conventional radiographs of the knee and wrist were examined postoperatively and showed no similar image findings in these joints. The patient remained free of symptoms at the 8-month follow-up and had no evidence of recurrence.
Discussion
CPPD deposition disease is usually a benign condition characterized by crystal deposition of CPPD in synovial membranes, joint cartilages and surrounding soft tissues. CPPD deposition disease of the TMJ is rare; the disease is most commonly found in the knee joint. Other joints that may be affected are the wrists, elbows, shoulders and ankles. P ritzker et al. first described it in the TMJ in 1976. It is also termed tophaceous pseudogout or chondrocalcinosis .
To the authors’ knowledge, 42 cases of CPPD deposition disease affecting the TMJ have been described in the literature and are summarized in Table 1 . The ratio of females to males was 1.8:1 (27:15), the average age was 63 years (35–85 years, median 56 years). Most of the cases were unilateral lesions, and the ratio of left to right was close to 1.5 (22:15). Two cases were bilateral, and three cases were not stated. The common clinical manifestations include pain (30/42), swelling or mass (31/42), trismus or limitation of condylar movement (19/42), hearing loss or deafness (6/42) and malocclusion (3/42). Radiologically, calcified, radiopaque or dense masses (32/41) were most commonly found in the joint space, surrounding the condyle or extending into neighbouring tissues. Over half of the cases (27/41) showed bony changes to different extents, from sclerosis and erosion of articular cortex to severe osseous destruction. Five cases demonstrated destruction of the skull base and two extended into the middle cranial fossa.
| Author | Side | Age | Sex | Clinical findings | Radiological findings | Treatment modalities | Recurrence (follow-up) |
|---|---|---|---|---|---|---|---|
| P ritzker et al. | R | 55 | M | Painless mass | Radiopaque mass, articular erosion | Excision | No (2 yr) |
| de V os et al. | L | 51 | F | Chronic pain, deviation of mandible | Calcified mass on leading edge of condyle | Excision | No (1 yr) |
| G ood & U pton | L | 56 | M | Painful swelling | Flattened condyle, sclerosis of articular surfaces | Medication | No (2 yr) |
| Z emplenyi & C alcaterra | L | 51 | F | Painful swelling, trismus | Dense mass between condyle and coronoid process, no bony destruction | Excision | Yes (2 yr) |
| K amatani et al. | L | 57 | M | Malocclusion | Dense mass between condyle and coronoid process, hypertrophic condyle | Excision | NA |
| G ross et al. | L | 59 | F | Painful joint, trismus | Destructive changes in condylar head | Excision | No (10 mo) |
| M ogi et al. | R | 54 | F | Pain, swelling and trismus | Irregular changes to fossa, calcified mass | Excision | No (20 mo) |
| H utton et al. | R | 78 | F | Painful joint, trismus | No abnormality | None | NA |
| H utton et al. | R | 76 | F | Acute ear pain, trismus | Calcified mass in joint space, loss of normal condylar shape | Medication | NA |
| H utton et al. | R | 68 | F | Acute pain, trismus | Nonspecific changes | Medication | NA |
| L ambert et al. | R | 41 | M | Chronic painless mass, restriction of movement and deafness | Calcified mass around condyle, erosion and sclerosis of zygomatic process, temporal bones, destruction of skull base | Excision | NA |
| Magno et al. | L | 53 | F | Ear pain with deafness | Calcareous masses in TMJ, erosion into temporal bone, irregular condyle | Excision | No (2 yr) |
| Dijkgraaf et al. | L | 53 | F | Acute painful swelling, trismus, malocclusion | Calcified material in joint space, lysis of the condyle and articular eminence | Excision | 1st – yes (11 mo); 2nd – no (22 mo) |
| Chuong & P iper | B | 65 | F | Painful swelling, trismus | Abnormal signal intensity within joint space, severe osteoarthrosis | Excision | No (2 yr) |
| Pynn et al. | L | 58 | M | Painless swelling and malocclusion | A cloudy and diffuse radiopacity, flattened and sclerotic articular surfaces | Excision | No (3 yr) |
| Ishida et al. | NA | 47 | F | Painless mass | Calcified lesion, erosion of condyle | Excision | NA |
| Ishida et al. | NA | 50 | F | Painless swelling | NA | Excision | Yes (2 yr) |
| Ishida et al. | NA | 55 | F | No symptoms | Tumourous mass in ITF and TMJ | Excision | NA |
| Onodera et al. | L | 48 | F | Painful swelling, trismus | Radiopacity around the area of TMJ | Excision | No (18 mo) |
| Kurihara et al. | R | 85 | M | Painful swelling | A calcified mass protruding from the joint space | Excision | No (6 mo) |
| Jordan et al. | R | 80 | M | Hearing loss, middle ear effusion | Mottled mass involving temporal bone, skull base and middle cranial fossa, indenting temporal lobe | Excision | NA |
| Strobl et al. | L | 51 | F | Pain and trismus, deviation of mandible | Irregular radiopaque mass around condyle, erosion of condyle | Excision | NA |
| Goudot et al. | L | 63 | F | Painful swelling | A calcified mass filling joint space and destroying the roof of joint | Excision | No (1 yr) |
| Nakagawa et al. | R | 60 | F | Painful swelling, restricted mouth opening | A large calcified mass around condyle, extending into ITF, erosion and sclerosis of condyle | Excision | No (3 yr) |
| Nakagawa et al. | L | 45 | F | Pain | Faint calcification in joint space | Medication | NA |
| Nakagawa et al. | R | 76 | M | Painful swelling, limitation of mouth opening | Joint effusion in joint space on MRI, no bony abnormality | Irrigation | No (18 mo) |
| Aoyama et al. | L | 45 | F | Painful swelling | Radiopaque images around TMJ, no destructive bony changes | Excision | No (7 mo) |
| Eriksson et al. | R | 72 | M | Painful swelling | A well-defined mass with heterogenous signal intensity, sclerosis of condyle | Excision | No (5 yr) |
| Olin et al. | L | 51 | F | Painless swelling | Radiodense in joint, osseous destruction of sphenoid | Partial excision | No (18 mo) |
| G reaves & F ordyce | B | 56 | M | Bilateral painful swelling, trismus | Calcification within both TMJs, sclerosis and flattening of articular surfaces | Medication | NA |
| Cottrell et al. | R | 68 | F | Swelling and nontender mass | Large gritty and lobular radiopaque mass in joint space, around the condyle | Excision | No (1 yr) |
| Osano et al. | L | 40 | M | Painful swelling | Severe destruction of the condyle | Excision | No (2 yr) |
| Marsot-Dupuch et al. | R | 70 | M | Painful swelling, hearing loss | A calcified soft tissue with osseous remodelling, joint space widening | Biopsy | NA |
| Marsot-Dupuch et al. | L | 53 | F | Acute aural fullness and conductive hearing loss | A large mass in glenoid fossa, eroding into middle cranial fossa | Biopsy | NA |
| Goldblatt et al. | R | 57 | M | Severe pain and swelling | Enlarged and irregular condyle, calcified mass, articular sclerosis and erosions | Medication | NA |
| Dimitroulis | L | 44 | F | Persistent pain, trismus, and intermittent swelling | Perforated glenoid fossa, calcified specks within disc, condylar sclerosis | Excision | No (6 mo) |
| Smolka et al. | L | 74 | F | Painful swelling | Calcified mass in joint space, no destructive bony changes | Excision | No (1 yr) |
| Cascone et al. | L | 64 | M | Preauricular swelling, trismus | An amount of calcified material around the condyle | Excision | No (5 yr) |
| Naqvi et al. | L | 35 | M | A painful mass in TMJ area, tinnitus, along with hearing loss | A calcified mass around condyle, extending into ITF, eroding anterior wall of epitympanum and the skull base; | Excision | NA |
| Reynolds et al. | L | 52 | F | Painful swelling, limited mouth opening | Distending of joint space and soft tissue material fillings, erosion of joint cortex | Excision | No (2 yr) |
| Ascani et al. | R | 72 | F | Painful mass of TMJ with progressive trismus | An calcified mass; destruction of condyle and the skull base; disc atrophy | Excision | No (7 mo) |
| Kalish et al. | L | 71 | F | Facial pain, trismus and a large mass | Mass in ITF, sclerosis of articular eminence and condyle | Excision | No (18 mo) |
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