To illustrate imaging features of peri-odontoid CPPD deposition presenting acutely as “crowned dens syndrome”.
To review cases and discuss differential diagnosis.
CPPD is an umbrella term used for all instances of calcium pyrophosphate (CPP) crystal deposition,
as proposed by the EULAR (European League Against Rheumatism) CPPD Task Force.
Clinical presentations of CPPD include (table 1):
OA with CPPD (osteoarthritis with CPPD)
acute CPP crystal arthritis
chronic CPP crystal inflammatory
CPPD occurs most frequently in articular,
hyaline or fibro-cartilage; less frequently,
it can affect tendons and joint capsule structures.
This form of crystal deposition is well documented and commonly described as:
Imaging findings OR Procedure Details
Our review includes 6 cases.
These are examples used to illustrate imaging findings of Crowned Dens Syndrome and its differential diagnoses.
All patients presented with acute,
non-traumatic neck pain.
92-yr-old female presented to A&E withpyrexia,
suspected stroke and urosepsis.
No history of fall or trauma.
White cell count was 13 x 109/L and C-reactive protein was 215mg/L.
Clinical examination found exquisite tenderness at C2-C3 and inability to rotate the neck laterally.
A CT was requested to exclude a fracture (Fig.
Crowned Dens Syndrome (CDS) should be considered in the differential diagnosis of patients presenting with acute onset of non-traumatic neck pain,
The characteristic clinico-radiological triad of CDS includes:
peri-odontoid soft tissue calcification with or without erosions
raised inflammatory markers
CT is the modality of choice to demonstrate peri-odontoid soft tissue calcifications.
MRI helps with complications and differential diagnosis.
The radiologist should be able to recognize the typical radiologic features of this condition and its main differential diagnoses.
Early recognition of CDS...
European League Against Rheumatism recommendations for calcium pyrophosphate deposition.
Part I: terminology and diagnosis.
Zhang W et al.
Annals of the Rheumatic Diseases 2011; 70:571-575.
European League gainst Rheumatism recommendations for calcium pyrophosphate deposition.
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Frequency of Atlantoaxial Calcium Pyrophosphate Dihydrate Deposition Chang et al.
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