Kohler Disease

Updated: May 28, 2021
Author: Bernardo Vargas, MD; Chief Editor: Vinod K Panchbhavi, MD, FACS, FAOA, FABOS, FAAOS 


Practice Essentials

Köhler disease, first described by Alban Köhler in 1908, is a rare bone disorder of the foot that occurs in children, most commonly boys aged 4-7 years.[1] It belongs to a group of conditions known as osteochondroses, which have been reported since 1903. Approximately 40 different osteochondroses have been described in the literature. In these self-limited diseases, there is avascular necrosis (AVN) of primary or secondary centers of ossification; almost all of the epiphyses, apophyses, and small bones can be implicated.[2]  In Köhler disease, AVN of the navicular bone occurs.

The etiology of these conditions is not well known, but vascular accidents, coagulation anomalies, and heredity have been implicated.[3]  The most common osteochondroses are Kienbock, Freiberg, and Panner diseases.

Surgery is not indicated for Köhler disease; treatment is nonoperative (see Treatment).


Vascularization of the navicular bone occurs in two ways and is identical in adults and in children. A branch from the dorsalis pedis artery crosses the dorsal surface of the navicular and gives off three to five branches. Some small branches come from the medial plantar artery to supply the plantar surface. These blood vessels create a dense network around the bone and come from the perichondrium toward the center of the cartilage. Less commonly, a single dorsal or plantar artery is found in anatomic specimens.

Köhler suggested that the changes in this disease might be the result of an abnormal strain that acts on a weak navicular, but a definitive explanation has not been found. Of the theories put forward to explain the nature of this lesion, a more satisfactory one is a mechanical basis that is associated with a delayed ossification.[4]

The navicular is the last tarsal bone to ossify in children. This bone might be compressed between the already ossified talus and the cuneiforms when the child becomes heavier. Compression involves the vessels in central spongy bone, leading to ischemia, which then causes clinical symptoms. Thereafter, the perichondral ring of vessels sends the blood supply, allowing rapid revascularization and formation of new bone. The radial arrangement of the vessels of this bone is of great importance in explaining why the prognosis of this lesion is always excellent.

In his original paper, Köhler also reported a single instance of an osteochondrosis of the primary patellar ossification center; this clinical entity has been rarely reported in the years since. Dharamsi and Carl reported a case of isolated bilateral Köhler disease of the patella in a male athlete aged approximately 7 years.[5]


The etiology of Köhler disease, like those of the other osteochondroses, is unknown, but the condition is thought to result from compressive stress-related injury at a critical time of growth.[6] Vascular insults, retarded bone age, and genetic predilection have also been implicated.[7]


Köhler disease is a rare condition thought to occur in less than 2% of the population.[6] However, no accurate epidemiologic figures are currently available for this condition. The disorder can begin very early, after age 2 years, but it is more frequent in children aged 5-10 years.[8] Köhler disease is far more common in boys than in girls; however, girls with this condition are often younger than boys with the disease are. This is probably due to the onset of ossification in girls, which occurs at age 18-24 months, whereas in boys, ossification occurs at age 24-30 months.


The evolution of the x-ray appearance in Köhler disease is variable. Normal x-rays may be obtained 6-18 months following onset. At adulthood, the navicular bone is expected to be normal. Patients recover excellent function.[9]



History and Physical Examination

In this uncommon condition, children present with an antalgic limp and local tenderness of the medial aspect of the foot over the navicular. The child can walk with an increased weight on the lateral side of the foot. Frequently, there is swelling and redness of the soft tissues.



Imaging Studies

Radiography is indicated in the workup of Köhler disease (see the first image below). The lateral view shows a flat tarsal scaphoid (see the second image below). The space between the talus and the cuneiforms is not decreased. Frequently, there is an irregular ossification of the tarsal navicular bone or radiologic changes that resemble Köhler disease, but the diagnosis must not be made in the absence of clinical signs.[10, 11]

Radiograph from 16-year-old patient who had Köhler Radiograph from 16-year-old patient who had Köhler disease. Normal x-rays at adulthood are the rule for Köhler disease.
Radiograph from patient with Köhler disease. Image Radiograph from patient with Köhler disease. Image is typical for this disease. Note flat aspect of tarsal scaphoid.

Bone scanning reveals decreased uptake of radionuclide in the right midtarsal area.[12] This study is not necessary for the diagnosis. In view of the radiation dose involved in nuclear medicine imaging, coupled with the limited diagnostic and prognostic benefit, bone scans should be considered contraindicated for the evaluation of this condition.[13]  

If pain persists for 6 months after appropriate treatment (to include casting), magnetic resonance imaging (MRI) or computed tomography (CT) should be considered for the purpose of excluding a tarsal coalition.[14]



Medical Therapy

A walking short leg cast with toe extension is recommended. Total cast time is approximately 6-8 weeks. The cast is better in moderate varus (10-15º) that is associated with moderate equinus (10-20º). In this position, the navicular is relaxed from posterior tibialis strain. Arch supports can be prescribed after the cast period and used for an average of 6 months. In mild cases, soft arch supports may be the only treatment necessary.

Surgery is not indicated for Köhler disease. 


In 1984, Ertel and O'Connell reported a case of acquired talonavicular coalition that followed avascular necrosis (AVN) of the tarsal navicular bone.[15] This complication is very rare.

Long-Term Monitoring

In treated patients, symptoms may last less than 3 months.[11]  In untreated patients, symptoms may be present for 15 months. Because immobilization in a short leg cast decreases the duration of symptoms, treating all patients for at least 6 weeks is recommended.

If pain persists after a 6-week period of casting, a new cast must be applied for 6 supplementary weeks.[16]  If pain does not disappear after the cast period, other causes of foot pain, including talar coalition or an accessory navicular, should be investigated.[17]