Köhler disease

Köhler disease is an eponymous term referring to childhood-onset osteonecrosis of the navicular in the foot. Müeller-Weiss syndrome is the adult counterpart of navicular osteonecrosis ^4,5.

Epidemiology

Köhler disease typically presents in the pediatric population (4-6 years of age) and there is a recognized male predilection.

Pathology

Although the etiology remains unknown, a vascular incident is suspected. Delayed bone age has also been noted in some cases and may play a part in the pathogenesis of this entity. The central third of the navicular is a watershed area and it is prone more to avascular necrosis and stress fractures ^ref.

Radiographic features

Plain radiograph

• the navicular may appear wafer-like (thinned) and fragmented

• demonstrates patchy sclerosis (similar to osteonecrosis elsewhere)

• often associated soft tissue swelling

CT/MRI

Cross-sectional imaging is usually not required but may be necessary if pain persists or the diagnosis is not clear. 

Treatment and prognosis

Köhler disease often tends to be self-limiting and heals spontaneously with re-ossification and reconstitution within a few years. Application of a below-knee weight-bearing cast may improve symptoms and is recommended, typically for 6-7 weeks. 

History and etymology

It was first described in 1908 by Alban Köhler, a German radiologist (1874-1947) ^1,6. Interestingly, the radiograph with which Köhler made his original case description is now not thought to be Köhler disease ⁷!

Differential diagnosis

Usually, there is little difficulty in making the diagnosis. If symptoms persist then findings of tarsal coalition should be sought. This can be misinterpreted as infection ^ref.

See also

• Müeller-Weiss syndrome

• osteonecrosis