Köhler Disease

Köhler Disease

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Köhler Disease is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Kohler's Disease (of the Tarsal Navicular)
  • Kohler's Osteochondrosis of the Tarsal Navicular
  • Navicular Osteochondrosis

Disorder Subdivisions

  • None

General Discussion

Köhler 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 of three and seven, and it affects males five times more often than it does females. Typically, just one foot is affected.



Children appear to grow out of the disorder, and the affected bones regain their size, density and structure within a year. For some, however, symptoms may last as long as two years.

Symptoms

Köhler disease is a rare bone disorder characterized by a painful swollen foot. The foot is especially tender along the length of the arch. 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 relative 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.

Causes

The exact cause of Köhler disease is unknown. It does not appear to be hereditary and, until recently, was not thought to be linked to an injury.



Some orthopedic specialists now believe that Köhler disease may be linked to an injury in the area around a bone (navicular bone) in the foot and may be the result of delayed bone formation (ossification). 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 foru to six, 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).

Affected Populations

Köhler 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 Köhler 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.



It is thought that the incidence of the disorder in the population is about 2%.

Standard Therapies

Diagnosis

Köhler disease is diagnosed with an X-ray of the foot and the presence of weight-bearing pain in the middle of the foot.



Treatment

Treatment ranges from weight-bearing short-leg plaster casts at one extreme to "watchful waiting" at the other. Special supportive shoes may also be considered. Staying off the foot as much as possible helps in recovery. Symptoms can last for a few days or persist for up to two years. However, symptoms usually resolve within a year.

Investigational Therapies

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 web site.



For information about clinical trials being conducted at the 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



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Cook RA, O'Malley MJ. Navicular Osteochondritis (Köhler 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.



Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:2414-15.



JOURNAL ARTICLES

Tsirikos AI, Riddle EC, Kruse R. Bilateral Köhler'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 Köhler's disease. Clin Nucl Med. 1997;22:636-37.



Devine KM, Van Demark RE Sr. Köhler'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. Köhler'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. Köhler's disease of the tarsal navicular. Clin Orthrop. 1981;158:53-58.



FROM THE INTERNET

Köhler's disease. Gpnotebook. 2003:1p.

www.gpnotebook.co.uk/cache/-1241120759.htm



Vargas-Barreto B. Köhler Disease. eMedicine. Last Updated: May 22, 2002:5pp.

www.emedicine.com/orthoped/topic410.htm



Questions and Answers About Avascular Necrosis. National Institute of Arthritis and Musculoskeletal and Skin Diseases. NIAMS. Publication Date: January 2001. 7pp.

www.niams.nih/gov/hi/topics/avascular_necrosis/index.htm

Resources

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Email: NIAMSinfo@mail.nih.gov

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TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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