Introduction
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 are reported in the literature. In these self-limited diseases, there is avascular necrosis of primary or secondary centers of ossification; almost all of the epiphyses, apophyses, and small bones can be implicated.2 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.
Radiograph from a 16-year-old patient who had Köhler disease. Normal x-rays at adulthood are the rule for Köhler disease.
Radiograph from a patient with Köhler disease. This image is typical for Köhler disease. Note the flat aspect of the tarsal scaphoid.
Problem
Avascular necrosis of the navicular bone occurs.
Frequency
Köhler disease is rare. To the authors' knowledge, no accurate prevalence figures are available. This 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 are boys with the disease. 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.
Etiology
As with the other osteochondroses, the etiology of Köhler disease is unknown. Nevertheless, a vascular incident and a retarded bone age have been implicated.
Pathophysiology
Vascularization of the navicula occurs in 2 ways and is identical in adults and children. A branch from the dorsalis pedis artery crosses the dorsal surface of the navicula and gives off 3-5 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 navicula, but a definitive answer has not been found. Among the theories to explain the nature of this lesion, a more satisfactory one is a mechanical basis that is associated with a delayed ossification.4 The navicula 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.
Presentation
In this uncommon condition, children present with an antalgic limp and local tenderness of the medial aspect of the foot over the navicula. 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.
Indications
Surgery is not indicated for Köhler disease. Clinical management is discussed in Treatment, Medical therapy.
Relevant Anatomy
See Pathophysiology.
Contraindications
Surgery is not indicated for Köhler disease.
More on Köhler Disease |
 Overview: Köhler Disease |
| Workup: Köhler Disease |
| Treatment: Köhler Disease |
| Follow-up: Köhler Disease |
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| References |
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References
Kohler A. Uben eine häubige bisher ansheinend unbekannte Erkrankung einzelner kindlicher Knochen Verh deutsch Röntg-Ges 1908;4:110.
DiGiovanni CW, Patel A, Calfee R, Nickisch F. Osteonecrosis in the foot. J Am Acad Orthop Surg. Apr 2007;15(4):208-17. [Medline].
Khan AQ, Sherwani MA, Gupta K, Siddiqui YS, Hali NZ. Kohler's disease. Saudi Med J. Sep 2008;29(9):1357-8. [Medline].
Waugh W. The ossification and vascularisation of the tarsal navicular and their relation to Köhler's disease. J Bone Joint Surg Br. Nov 1958;40-B(4):765-77. [Medline]. [Full Text].
Berard J, Fournet-Fayard J. [Idiopathic ostonecrosis of the scaphoid tarsal bone (Köhler's second disease)] [French]. Rev Rhum Mal Osteoartic. Feb 1983;50(2):163-5. [Medline].
Williams GA, Cowell HR. Köhler's disease of the tarsal navicular. Clin Orthop Relat Res. Jul-Aug 1981;158:53-8. [Medline].
Khoury J, Jerushalmi J, Loberant N, Shtarker H, Militianu D, Keidar Z. Kohler disease: diagnoses and assessment by bone scintigraphy. Clin Nucl Med. Mar 2007;32(3):179-81. [Medline].
Gips S, Ruchman RB, Groshar D. Bone imaging in Kohler's disease. Clin Nucl Med. Sep 1997;22(9):636-7. [Medline].
Ippolito E, Ricciardi Pollini PT, Falez F. Köhler's disease of the tarsal navicular: long-term follow-up of 12 cases. J Pediatr Orthop. Aug 1984;4(4):416-7. [Medline].
Ertel AN, O'Connell FD. Talonavicular coalition following avascular necrosis of the tarsal navicular. J Pediatr Orthop. Aug 1984;4(4):482-4. [Medline].
Borges JL, Guille JT, Bowen JR. Köhler's bone disease of the tarsal navicular. J Pediatr Orthop. Sep-Oct 1995;15(5):596-8. [Medline].
Khan AQ, Sherwani MA, Gupta K, Siddiqui YS, Hali NZ. Kohler's disease. Saudi Med J. Sep 2008;29(9):1357-8. [Medline].
Keywords
Köhler disease, Köhler's disease, avascular necrosis, avascular necrosis of tarsal navicula, osteochondritis of tarsal navicula, osteochondroses, osteonecrosis of the foot, foot osteonecrosis. limping child
