Description

Köhler disease is a rare, idiopathic self-limiting osteochondrosis caused by avascular necrosis of the tarsal navicular bone presenting in young children with dorsomedial foot pain and limp

Most common in young children (usually 3-9 yrs); 80% of cases are in males; and unilateral in 75% of cases. Most likely caused by excessive strain on the tarsal navicular bone and its associated blood vessels before the bone is completely ossified. 

Signs and symptoms include swelling and redness to the dorsomedial midfoot; tenderness along the length of the arch; limp or abnormal gait.

Treatment is usually nonoperative with NSAIDs, and a short period of cast immobilisation.

Kohler disease is distinct from Müller-Weiss disease, a complex idiopathic foot condition with deformity of the tarsal navicular presenting in adulthood

Case example

Below are images of right foot Navicular avascular necrosis (Köhler disease) in a 5 year old male

Right navicular avascular necrosis 2
Right navicular avascular necrosis 2
Right navicular avascular necrosis – drag marker to reveal labels
Left comparison - normal navicular 1
Left comparison - normal navicular 1
Left comparison – normal navicular – – drag marker to reveal labels

Spontaneous adult onset osteonecrosis of the tarsal navicular is known as Mueller-Weiss syndrome (also Brailsford disease)


History of Köhler disease

1908 – First described by German radiologist Alban Köhler (1874-1947) in his MWW article Uber eine häufige, bisher anscheinend unbekannte Erkrankung einzelner kindlicher Knochen [About a common, so far apparently unknown disease of individual children’s bones]

Köhler disease. Erkranktes os naviculare, Kohler 1908
Fig 3. Erkranktes os naviculare, Kohler 1908

Es handelte sich um Knaben im Alter von 5-9 Jahren (..) Alle drei Patienten klagten über mehr oder weniger heftige Schmerzen in der Gegend des Os naviculare (…) Die Röntgenuntersuchung ergab einen recht eigenartigen (…) Befund. Ueberall zeigte sich das Os naviculare in die Augen fallend erkrankt, während alle anderen Knochen des Fusses einen normalen Anblick boten. Das Navikulare war in vierfacher Beziehung verändert, und zwar in seiner Grösse, seiner Gestalt, seiner Architektur und seinem Kalkgehalt.

Der Krankheitsverlauf zog sich auf 2-3 Jahre hin (…) es erfolgte Heilung (…) nicht nur im klinischen Sinne, sondern wie die Röntgenbilder anzunehmen zwingen, auch in anatomischem Sinne. Die Prognose des Leidens ist also durchaus günstig zu stellen.

Köhler, 1924

In question were young lads aged 5-9 years old. All patients complained of more or less severe pains in the region of the navicular bone. The Röntgen examination demonstrated a most peculiar finding. Everywhere the navicular bone appeared diseased, whilst all other bones of the foot presented normally. The navicular was changed in four aspects, namely its size, shape, architecture, and calcification.

The disease took its course in 2-3 years (…) healing was evident(…) not only in the clinical sense, but also in the anatomical sense, as the X-rays force us to acknowledge. The prognosis of this suffering is therefore quite favourable.

Köhler, 1924


Associated Persons

Alternative names
  • Kohler disease, Koehler disease
  • Osteonecrosis; Osteochondrosis; Osteochondritis of the tarsal navicular

References

Original articles

Review articles


Cite this article as: Mike Cadogan and Aparicio Gomez, "Köhler disease," In: LITFL - Life in the FastLane, Accessed on October 1, 2022, https://litfl.com/koehler-disease/.

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Associate Professor Curtin Medical School, Curtin University. Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM Sir Charles Gairdner Hospital.  Passion for rugby; medical history; medical education; and asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | Twitter |

Resident medical officer in emergency medicine MB ChB (Uni. Dundee)  MRCS Ed. Avid traveller, yoga teacher, polylinguist with a passion for discovering cultures.

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