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Last updated:
November 16, 2018
Years published: 1990, 1995, 2004, 2018
NORD gratefully acknowledges Maral Karalekian, NORD Editorial Intern from the Keck Graduate Institute, and Bernardo Vargas, MD, Department of Orthopedic Surgery, University Hospital of Geneva, Switzerland, for assistance in the preparation of this report.
Kohler disease is a rare bone disorder of the foot in children that may be the result of stress-related compression at a critical time during the period of growth. It is characterized by limping caused by pain and swelling in the foot. It most often occurs in children between the ages 3-7, and affects males five times more often than females. Usually, just one foot is affected so children typically walk on the side portion of the foot.
Children appear to grow out of the disorder, and the affected bones usually regain their size, density and structure within three months. Rarely, symptoms may last as long as two years.
Kohler disease is a rare bone disorder characterized by a painful swollen foot. The foot is especially tender along the length of the arch. It may include redness of the affected area. Putting weight on the foot or walking is difficult, causing further discomfort and a limp. For reasons that are not understood, the flow of blood to one of the bones in the foot (navicular bone) is interrupted, resulting in progressive degeneration of that bone. In a relatively short time, however, the bone heals itself.
Usually, symptoms will be mild, and patients may not seek treatment until the pain and swelling have persisted for a while.
The exact cause of Kohler disease is unknown.
Some orthopedic specialists think that Kohler disease may be related to an injury in the area around the navicular bone in the foot and may be the result of delayed bone formation (ossification). Bone ossification usually begins at age 18-24 months in girls and at age 24 to 30 months in boys. Structural weakness might result from an increase in the ratio of cartilage to bone. Since the navicular bone is part of the mechanism by which the foot moves (articulation), it is subject to weight-bearing pressures and stresses from twisting and turning.
Under normal circumstances, the navicular bone is served by a blood vessel from which smaller arteries supply blood to the regions of bone growth. At around the ages of 4-6, the blood supply to these regions of bone growth increases as other blood vessels reach them. If ossification is delayed and the child gains weight, the effect is to compress the blood vessels, thus causing tissue destruction (ischemia).
It has been suggested that genetic factors may play a role in the development of Kohler disease, but a specific gene has not been found to be associated with this disease.
Kohler disease is a rare bone disorder of the foot that affects males more often than females. The disorder strikes children between the ages of 1 and 10 years with a peak occurring at ages 3 to 7 years. The center of bone growth that is affected in Kohler disease develops in young girls about one year before it appears in young boys. Nevertheless, the disorder is five times more prevalent in boys than girls.
The incidence of the disorder in the population is estimated to be less than 2%.
A diagnosis of Kohler disease is suspected based on the presence of the signs of symptoms in the child. The clinical diagnosis of Kohler disease is reinforced by X-rays that show flattening, sclerosis, and fragmentation of the navicular bone, which can be compared to the unaffected side to make the diagnosis. However, these abnormalities of the navicular bone are bilateral in approximately 25% of patients and may be seen in children who do not have Kohler disease.
Treatment
Kohler disease typically resolves over time with or without treatment. Symptoms can last for a few days or persist for up to two years, but symptoms usually resolve within six months. Treatment can include pain relievers or weight-bearing short-leg plaster casts. Special supportive shoes may also be considered. Staying off the foot as much as possible helps in recovery. The long-term outlook (prognosis) for people with Kohler disease is usually excellent. People affected by the condition typically recover all function of the affected foot and have no lasting consequences.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
TEXTBOOKS
Cook RA, O’Malley MJ. Navicular Osteochondritis (Kohler Disease). In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:21.
Myerson MS. Foot and Ankle Disorders. WB Saunders Co., Philadelphia, PA. 2000:793-96.
JOURNAL ARTICLES
Tsirikos AI, Riddle EC, Kruse R. Bilateral Kohler’s disease in identical twins. Clin Orthop. 2002;409:195-98.
Harty MP. Imaging of pediatric foot disorders. Radiol Clin North Am. 2001;39:733-48.
Toth AP, Easley ME. Ankle chondral injuries and repair. Foot Ankle Clin. 2000;5:119-33.
Bui-Mansfield LT, Lenchik L, Rogers LF, et al. Osteochondritis dissecans of the tarsal navicular bone: Imaging findings in four patients. J Comput Assist Tomogr. 2000;24:744-47.
Gips S, Ruchman RB, Groshar D. Bone imaging in Kohler’s disease. Clin Nucl Med. 1997;22:636-37.
Devine KM, Van Demark RE Sr. Kohler’s osteochondrosis of the tarsal navicular: case report with twenty-eight year follow-up. S D J Med.1989;42(9): 5-6.
Ippolito E, et al. Kohler’s disease of the tarsal navicular: long-term follow-up of 12 cases. J Pediatr Orthop.1984;4(4):416-417.
Williams GA, Cowell HR. Kohler’s disease of the tarsal navicular. Clin Orthrop. 1981;158:53-58.
INTERNET
Vargas B. Kohler Disease.Medscape.
www.emedicine.com/orthoped/topic410.htm Updated: Dec 18, 2017. Accessed November 7, 2018.
Kohler Disease. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/6842/kohler-disease Last updated: 2/18/2014. Accessed November 7, 2018.
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