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Basics ⬇

Author: Kevin E.Burroughs, MD, CAQSM

Description

  • Pediatric osteochondrosis of the tarsal navicular, first described by Alban Köhler in 1908 (1)
  • Osteochondrosis is a disease process that causes necrosis of the ossification center of a developing bone and is followed by regeneration.

Epidemiology

ALERT
  • Predominant age: occurs in children 2 to 7 yr of age (average age 5 yr and 10 mo) (2)
  • Predominant gender: males > females (4 to 6:1)
  • Usually occurs unilaterally; bilateral in only ~15–20% of cases
  • Appears to be no relation between weight and incidence

Consideration:

  • Adult onset navicular osteonecrosis is known as Mueller-Weiss disease (also known as Brailsford disease).

Etiology and Pathophysiology

  • Navicular development:
    • The navicular becomes evident on radiographs between 18 and 24 mo in girls and between 30 and 36 mo in boys.
    • A more irregular and dense navicular is often present in children whose navicular ossifies at later times than these, and these findings are similar to those in Köhler disease.
    • The navicular ossifies from a single center in 2/3 of children and from multiple centers in the remainder. Those ossifying later than normal tend to be from multiple centers.
    • A dense perichondral network of blood vessels has been described on the nonarticular surfaces, with numerous penetrating arteries.
  • Theories of etiology:
    • Questionably a normal variant but does not explain why some individuals are asymptomatic
    • Some believe that it is avascular necrosis; however, this wouldn’t explain acute onset of pain, lack of correlation with radiographic changes in that short amount of time, and no matter the treatment, a normal navicular is the end result (true necrosis should cause deformity).
    • Most likely a syndrome or continuum of disease because radiographic changes can be seen in asymptomatic individuals. Perhaps those who are symptomatic have experienced a stress injury to the ossifying bone. This would explain radiographic changes, with remodeling to a normal-appearing bone.

Risk-Factors

  • May be caused by repetitive microtrauma to the maturing navicular ossification center (the last tarsal bone to ossify) (3)
  • Compression of the bony nucleus at a critical phase of growth may occlude the penetrating blood vessels and produce ischemia and aseptic necrosis of the bone.
  • Delayed ossification leading to irregular ossification centers may predispose to this condition.
  • Occurrence is not related to acute macrotrauma, age at first walking, foot type, or family history.

Diagnosis ⬆ ⬇

Although variations in morphology of the navicular have been described, the diagnosis of Köhler disease is made on the basis of both clinical and radiographic findings.

History

  • It is often difficult for young children to localize pain, but it should be over the midfoot area.
  • Repetitive microtrauma may be a risk factor in sports.
  • A history of macrotrauma should lead the practitioner to consider other causes of foot pain.

Physical Exam

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Signs and symptoms:
  • Insidious onset of foot pain and limp aggravated by activity
  • Time to presentation varies from days to months after onset of pain.

Physical examination:

  • Look for localized edema and warmth in the area of the tarsal navicular.
  • Palpate the entire foot and ankle; tenderness should be localized to the medial midfoot.
  • Check the range of motion of the ankle and subtalar joints, which should be normal.
  • Examine the knee and hip, which can be the source of referred pain and limp.

Differential Diagnosis

  • Normal variants: Variations of size and shape of the navicular ossification center may be indistinguishable from Köhler disease except for the absence of symptoms.
  • Osteochondritis dissecans
  • Localized involvement on the articular surface
  • Well demarcated from the normal bone by a crescent-shaped area of radiolucency

Diagnostic Tests & Interpretation

ALERT
Radiographs:
  • Standard anteroposterior, lateral, and oblique radiographs of the foot should be obtained.
  • Findings (4):
    • Varying degrees of navicular sclerosis
    • Diminished size or flattening of the navicular (“Alka-Seltzer-on-end” appearance)
    • Occasional loss of trabecular pattern and fragmentation
  • A person with an asymptomatic foot with abnormal radiographic features found incidentally does not have Köhler disease.
  • Bone scintigraphy (4)[C]:
    • If diagnosis not evident, bone scan can show decreased uptake in the navicular, indicating decreased or interrupted blood supply early when radiographs still normal.
    • In the later phases, can show increased uptake indicating revascularization
  • Pathologic findings:
    • Patchy areas of bone destruction and dead trabeculae with interference of normal ossification

Treatment ⬆ ⬇

General Measures

  • Acute treatment:
    • Apply ice to midfoot.
    • Nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen can be used for analgesic effect.
    • Immobilization to affect relief of symptoms, although the type and duration of treatment do not influence long-term outcomes
ALERT
Despite not altering the long-term outcome, immobilization has been shown to decrease the duration of symptoms (4)[C].
  • Immobilization:
    • Patients with cast immobilization are typically pain free at 3 mo, whereas those treated without casting were symptomatic until 15 mo on average.
    • Those casted for a minimum of 2 mo were asymptomatic in the shortest time.
    • Weight-bearing status does not appear to affect outcome.

Additional Therapies

  • Orthoses have not been found to be effective.
  • Rehabilitation: usually not indicated

Surgery/Other Procedures

Rarely required

Ongoing Care ⬆ ⬇

Follow-up Recommendations

Orthopedic referral is indicated if symptoms do not resolve with conservative management.

Patient Monitoring

  • In a study of 12 cases, complete restoration of normal bone structure occurred in 8 mo (minimum 6 mo and maximum of 13 mo) (5)[C].
  • At 30-yr follow-up, no patient showed degenerative changes at the tarsonavicular joint, and no radiographic differences were seen with the unaffected foot (5)[C].

Prognosis

  • Complete resolution of symptoms with reconstitution of the navicular can be expected in all patients.
  • No evidence of cartilage degeneration in long-term follow-up studies

Additional Reading ⬆ ⬇

  • Borges JL, Guille JT, Bowen JR. Köhler’s bone disease of the tarsal navicular. J Pediatr Orthop. 1995;15(5):596–598.
  • Gips S, Ruchman RB, Groshar D. Bone imaging in Kohler’s disease. Clin Nucl Med. 1997;22(9):636–637.
  • Manusov EG, Lillegard WA, Raspa RF, et al. Evaluation of pediatric foot problems: part I. The forefoot and the midfoot. Am Fam Physician. 1996;54(2):592–606.
  • Sharp RJ, Calder JD, Saxby TS. Osteochondritis of the navicular: a case report. Foot Ankle Int. 2003;24(6):509–513.
  • Stanton BK, Karlin JM, Scurran BL. Köhler’s disease. J Am Podiatr Med Assoc. 1992;82(12):625–629.
  • Williams GA, Cowell HR. Köhler’s disease of the tarsal navicular. Clin Orthop Relat Res. 1981;(158):53–58.

References ⬆ ⬇

  1. Köhler A. A frequent disease of individual bones in children. Munch Med Wochenschr. 1908;55:1923–1925.
  2. Santos L, Estanqueiro P, Matos G, et al. Köhler disease: an infrequent or underdiagnosed cause of a child’s limping?Acta Reumatol Port. 2015;40(3):304–305.
  3. Alhamdani M, Kelly C. Kohler’s disease presenting as acute foot injury. Am J Emerg Med. 2017;35(11):1787.e5–1787.e6.
  4. Khoury J, Jerushalmi J, Loberant N, et al. Kohler disease: diagnoses and assessment by bone scintigraphy. Clin Nucl Med. 2007;32(3):179–181.
  5. 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;4(4):416–417.

Clinical Pearls ⬆

  • Although all patients eventually will have complete resolution of symptoms, immobilization with a cast for at least 8 wk shortens the duration of symptoms by ~1 yr.
  • Sports may be resumed once symptoms have resolved.
  • Long-term follow-up studies have found no increase in the rate of arthritis or other chronic foot problems in adults who had Köhler disease during childhood.