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Kohler Disease

  • Author: Bernardo Vargas, MD; Chief Editor: Anthony E Johnson, MD  more...
 
Updated: Jul 26, 2016
 

Practice Essentials

In 1908, Köhler first described the disease named in his honor.[1]  This clinical entity belongs to a group of conditions called 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 Legg-Perthes-Calve, Osgood-Schlatter, Sinding-Larsen-Johansson, Kienbock, Freiberg, and Panner diseases.

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

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Pathophysiology

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]

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Etiology

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]

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Epidemiology

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. 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.

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Prognosis

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.[8]

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Contributor Information and Disclosures
Author

Bernardo Vargas, MD Consulting Staff, Department of Orthopedic Surgery, University Hospital of Geneva, Switzerland

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Anthony E Johnson, MD Chairman, Department of Orthopaedic Surgery, San Antonio Military Medical Center; Research Director, US Army–Baylor University Doctor of Science Program (Orthopaedic Physician Assistant); Custodian, Military Orthopaedic Trauma Registry; Associate Professor, Department of Surgery, Baylor College of Medicine; Associate Professor, The Norman M Rich Department of Surgery, Uniformed Services University of the Health Sciences

Anthony E Johnson, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Healthcare Executives, American College of Sports Medicine, American Orthopaedic Association, Arthroscopy Association of North America, Association of Bone and Joint Surgeons, International Military Sports Council, San Antonio Community Action Committee, San Antonio Orthopedic Society, Society of Military Orthopaedic Surgeons, Special Operations Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Society of Military Orthopaedic Surgeons; American Academy of Orthopaedic Surgeons<br/>Received research grant from: Congressionally Directed Medical Research Program<br/>Received income in an amount equal to or greater than $250 from: Nexus Medical Consulting.

Additional Contributors

Heidi M Stephens, MD, MBA Associate Professor, Department of Surgery, Division of Orthopedic Surgery, University of South Florida College of Medicine; Courtesy Joint Associate Professor, Department of Environmental and Occupational Health, University of South Florida College of Public Health

Heidi M Stephens, MD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Orthopaedic Surgeons, American Medical Association, American Orthopaedic Foot and Ankle Society, Florida Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Mark Clayer, MD, MBBS, FRACS, FAOrthA, is gratefully acknowledged for the contributions made to this article.

References
  1. Kohler A. Uben eine häubige bisher ansheinend unbekannte Erkrankung einzelner kindlicher Knochen Verh deutsch Röntg-Ges 1908;4:110.

  2. DiGiovanni CW, Patel A, Calfee R, Nickisch F. Osteonecrosis in the foot. J Am Acad Orthop Surg. 2007 Apr. 15(4):208-17. [Medline].

  3. Khan AQ, Sherwani MA, Gupta K, Siddiqui YS, Hali NZ. Kohler's disease. Saudi Med J. 2008 Sep. 29(9):1357-8. [Medline].

  4. Waugh W. The ossification and vascularisation of the tarsal navicular and their relation to Köhler's disease. J Bone Joint Surg Br. 1958 Nov. 40-B(4):765-77. [Medline]. [Full Text].

  5. Dharamsi AS, Carl RL. Bilateral osteochondrosis of the primary patellar ossification centers in a young athlete: a case report. Clin J Sport Med. 2014 Jan. 24(1):80-2. [Medline].

  6. Köhler disease. National Organization for Rare Disorders. Available at http://http://rarediseases.org/rare-diseases/kohler-disease. 2004; Accessed: July 23, 2016.

  7. Tsirikos AI, Riddle EC, Kruse R. Bilateral Köhler's disease in identical twins. Clin Orthop Relat Res. 2003 Apr. 195-8. [Medline].

  8. Borges JL, Guille JT, Bowen JR. Köhler's bone disease of the tarsal navicular. J Pediatr Orthop. 1995 Sep-Oct. 15(5):596-8. [Medline].

  9. Berard J, Fournet-Fayard J. [Idiopathic ostonecrosis of the scaphoid tarsal bone (Köhler's second disease)] [French]. Rev Rhum Mal Osteoartic. 1983 Feb. 50(2):163-5. [Medline].

  10. Williams GA, Cowell HR. Köhler's disease of the tarsal navicular. Clin Orthop Relat Res. 1981 Jul-Aug. 158:53-8. [Medline].

  11. Khoury J, Jerushalmi J, Loberant N, Shtarker H, Militianu D, Keidar Z. Kohler disease: diagnoses and assessment by bone scintigraphy. Clin Nucl Med. 2007 Mar. 32(3):179-81. [Medline].

  12. Wall BF, Hart D. Revised radiation doses for typical X-ray examinations. Report on a recent review of doses to patients from medical X-ray examinations in the UK by NRPB. National Radiological Protection Board. Br J Radiol. 1997 May. 70 (833):437-9. [Medline].

  13. Gips S, Ruchman RB, Groshar D. Bone imaging in Kohler's disease. Clin Nucl Med. 1997 Sep. 22(9):636-7. [Medline].

  14. Ertel AN, O'Connell FD. Talonavicular coalition following avascular necrosis of the tarsal navicular. J Pediatr Orthop. 1984 Aug. 4(4):482-4. [Medline].

  15. Ippolito E, Ricciardi Pollini PT, Falez F. Köhler's disease of the tarsal navicular: long-term follow-up of 12 cases. J Pediatr Orthop. 1984 Aug. 4(4):416-7. [Medline].

  16. Ramachandran KR, Sasidharan K, Kusumakumary P, Ittiyavirah AK, Krishnakumar AS. Osteochondritis of the tarsal navicular bone (Kohler's disease) occurring in a child with acute lymphoblastic leukemia. Indian J Radiol Imaging. 1999. 9:153-4.

 
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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 is typical for this disease. Note flat aspect of tarsal scaphoid.
 
 
 
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