Müller-Weiss-Syndrom

Mueller Weiss syndrome, also known as Brailsford disease , refers to spontaneous multifactorial adult onset osteonecrosis of the tarsal navicular. This syndrome is distinct from Köhler disease, the osteonecrosis of the tarsal navicular bone that occurs in children.

Epidemiology

It occurs in adults between 40 and 60 years of age and is more common in females. Patients present with mid- and hind foot pain and pes planovarus.

Radiographic features

Plain radiograph

Plain radiographic features can include:

  • comma-shaped deformity due to collapse of the lateral part of the bone
  • medial or dorsal protrusion of a portion of the bone or the entire navicular bone

The disease may be bilateral or asymmetric and associated with pathologic fractures.

There is a radiographic staging.

CT
  • sclerosis with comma-shaped deformity of the lateral aspect
  • more advanced cases demonstrate further collapse and fragmentation
MRI
  • can show edema on STIR / PD FS images and is more sensitive in picking up the early changes due to its ability to detect marrow signal changes

Treatment and prognosis

The disease can be progressive at times, and it is associated with severe pain and disability. Initially, it is treated conservatively with analgesics and orthotics which if they fail then surgical treatment is considered.

History and etymology

Schmidt reported the first case in 1925. However W Muller was the first to propose that the pathophysiology was due to an abnormal compressive force upon the ankle or a congenital abnormality. K Weiss concluded that the appearances on plain film were analogous to those seen with Kienbock disease, i.e. an osteonecrotic etiology.

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