Calcium pyrophosphate dihydrate deposition disease

Case contributed by Dr Matt Skalski

Presentation

Poly-trauma victim.

Patient Data

Age: 35
Gender: Female

There is calcification within the radiocapitellar, ulnotrochlear , and proximal radioulnar joints which parallels the articular surfaces to follow the contour of the hyaline cartilage.

If one does not appreciate that this finding extends into the ulnotrochlear joint and proximal radioulnar joint, the chondrocalcinosis within the radiocapitellar joint could be mistaken for a radial head fracture in the setting of trauma. The absence of elevated fat pats mitigates this concern. In this case, the emergency department did mistakenly believe the patient had a radial head fracture based on the imaging. 

Remember that the hyaline cartilage of the radial head extends circumferentially around the radial head, so chondrocalcinosis would be expected to extend similarly to anywhere around the circumference of the radial head. 

Bilaterally, there is extensive chondrocalcinosis within the triangular fibrocartilage complex (TFCC), radiocarpal, intercarpal, and carpometacarpal joint spaces. On the right, there is chondrocalcinosis within the distal radioulnar joint. On the left, chondrocalcinosis is noted within the 1st and 3rd MCP joints. 

There is a thin area of calcification adjacent to and paralleling the articular surfaces of the humeral heads bilaterally, representing chondrocalcinosis. There is also mild degenerative changes of the glenohumeral joints, with mild osteophyte proliferation at the inferior aspect humeral heads at the attachment of the inferior glenohumeral ligament.

There is thin, irregular calcification noted immediately superior to the humeral heads and greater tuberosities bilaterally, within the supraspinatus and infraspinatus tendons.

There is also calcification within the acromioclavicular joints and their joint capsules bilaterally. The AC joints also demonstrate minimal osteophyte proliferation representing degenerative changes.

no fractures are identified.

Chondrocalcinosis is observed within the articular cartilage on the femoral heads bilaterally, as well as calcification of the superior labrum.

Mild osteophyte proliferation is noted at the superolateral aspect of the right acetabulum, with mild collar osteophyte proliferation around the right femoral head-neck junction bilaterally.

no fractures are identified.

Extensive tricomparmental chondrocalcinosis, as well as meniscal calcification is noted. There is also mild tricompartmental osteophyte formation.

There is no suprapatellar effusion. 

Chondrocalcinosis is noted within the ankle mortise joint, as well as sparsely throughout the intertarsal joints. 

There are multiple areas of irregular calcification within the proximal aspect of the plantar fascia.

An os peroneum is noted.

Throughout the cervical spine, extensive calcification is observed within the disc spaces, as well as chondrocalcinosis within the uncovertebral joint spaces.  

Chondrocalcinosis is also seen within the synovial atlantoaxial joint spaces at the lateral masses, and around the dens in the atlantodental space. Calcification of the transverse ligament is also demonstrated. Chondrocalcinosis is observed within both of the atlantooccipital joints as well.

There are multiple areas of calcification of the ligamentum flavum posteriorly within the neural canal, at the levels of  C3/4 and C5/6, with significant narrowing of the neural canal at C5/6. Thin but diffuse, faint areas of calcification of the thecal sac is noted.

Case Discussion

This case nicely demonstrates the extensive polyarticular involvement that can be seen with calcium pyrophosphate deposition disease (CPPD).  With extensive involvement of the periodontoid region (giving the "crowned dens" appearance with a halo of calcification around the dens) crowned dens syndrome should be expected if the patient reports severe neck pain and headaches.3-4  Hydroxyapatite crystals have also been described causing similar radiographic findings and clinical symptoms.5 

Due to the widespread extent of chondrocalcinosis in this relatively young patient, familial calcium pyrophosphate dihydrate deposition disease is suspected, which has an autosomal-dominant mode of inheritance.1-2 Other considerations could include hemochromatosis or hyperparathyroidism as underlying causes of the chondrocalcinosis, but there are no other features of these diseases to raise suspicion in this case.

This case is courtesy of Deborah M. Forrester, MD who kindly shared it with me. 

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Case Information

rID: 25455
Case created: 26th Oct 2013
Last edited: 16th Apr 2017
Inclusion in quiz mode: Included

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