Kohler's Disease

Introduction

Avascular necrosis of the navicular bone of unclear etiology
Epidemiology
- demographics
  - occurs in young children (usually age 4-7 yrs)
  - four times more common in boys than girls
    - 80% of cases in boys
- locations
  - can be bilateral in up to 25% of cases
Pathophysiology
- the blood supply of the central one third of the navicular is a watershed zone
  - accounts for the susceptibility to avascular necrosis and stress fractures
- the navicular is the last bone to ossify
  - increases its vulnerability to mechanical compression and injury
Associated conditions
- infection
  - Kohler's disease is often misdiagnosed as infection
Prognosis
- typically a self-limiting condition
- intermittent symptoms for 1-3 years after diagnosis
  - typically associated with activity

Classification

There is no widely used classification system for this condition

Presentation

History
- patient may not have complaints as disease can be asymptomatic
- characteristically described as midfoot pain with an associated limp
Symptoms
- pain in dorsomedial midfoot
- may have swelling, warmth, and redness
- point tenderness over the navicular
Physical exam
- antalgic limp (may place weight on lateral side of foot)

Imaging

Radiographs
- characteristic sclerosis, fragmentation, and flattening of tarsal navicular
- most tarsal navicular bones reorganize after disease has run its course
  - some continue to be deformed but almost all of those remain asymptomatic

Treatment

Nonoperative
- NSAIDs
  - short course can be used to decrease symptoms
- immobilization with short leg walking cast
  - indications
    - pain with activities
    - continued symptoms after placement of walking cast
  - outcomes
    - studies have shown treatment in a short leg walking cast will decrease the duration of symptoms
    - radiographs improve at around 6-48 months from onset of symptoms
    - no reports of long-term disability
Operative
- surgery
  - indications
    - not indicated for this disease

TAG

(OBQ08.109) A 6-year-old boy presents to your office with foot pain that is increasing in severity. His parents state he has recently been limping. There is no history of trauma and he is otherwise healthy. Radiographs are shown in Figures A and B. What is the best treatment for this child? Topic

Review Topic

FIGURES: A

1. Observation
2. Open biopsy and curettage
3. Intravenous antibiotics
4. Chopart amputation
5. Short leg walking cast

PREFERRED RESPONSE ▶

DISCUSSION: The history and radiographs are consistent with Kohler’s disease, avascular necrosis of the tarsal navicular. As discussed in the review article by Borges et al. the etiology of this disease is not well known, but it tends to affect boys more frequently than girls between ages 6-9. Treatment includes immobilization for symptom relief and observation while the navicular re-ossifies.

REFERENCES:

1. Able MF (ED). Orthopaedic Knowledge Update: Pediatrics. American Academy of Orthopaedic Surgeons. Rosemont, IL. 2006, pp 235-245

2. Borges JL, Guille JT, Bowen JR. Köhler's bone disease of the tarsal navicular. J Pediatr Orthop. 1995 Sep-Oct;15(5):596-8 PMID:7593569 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Eric Shirley MD,

Please Rate Educational Value!

3.0

Average 3.0 of 10 Ratings

TAG

(OBQ04.17) A 5-year-old boy presents with a limp and pain over the medial aspect of the foot. Radiographs are shown in figures A and B. Orthotics have failed to improve his symptoms. What is the next appropriate management of this condition? Topic

Review Topic

FIGURES: A

1. core decompression
2. triple arthrodesis
3. subtalar fusion
4. IV antibiotics with irrigation and debridement
5. short-leg walking cast

PREFERRED RESPONSE ▶

DISCUSSION: The radiographs show osteonecrosis of the navicular bone, otherwise known as Kohler’s disease. Treatment is traditionally symptomatic and pain usually can be relieved by limiting activities, and using orthotics. If this does not work, then a short leg walking cast for 4-6 weeks would suffice. Surgery is not indicated for this entity since it is self limiting.

DiGiovanni et al review variants of osteonecrosis of the foot in their JAAOS article. They emphasize the radiographic changes of Kohler's disease, including navicular sclerosis, may represent a normal variant in asymptomatic patients; thus, the diagnosis of osteonecrosis must be corroborated by clinical suspicion and supportive radiographs.

Williams et al found all patients were asymptomatic and, with nonsurgical management, had a radiographically normal navicular at 9-year follow-up. Casting provided earlier resolution of symptoms. Weight-bearing status did not affect outcome.

Ippolito et al followed a series of patients for 33 years. They found all patients were asymptomatic at follow up. Weightbearing plaster casts for 3 months rendered the patients pain-free, whereas arch supports only decreased local pain, which lasted for an average of 7 months.

REFERENCES:

1. DiGiovanni CW, Patel A, Calfee R, Nickisch F. Osteonecrosis in the foot. J Am Acad Orthop Surg. 2007 Apr;15(4):208-17. PMID:17426292 (Link to Abstract)

2. Williams GA, Cowell HR. Köhler's disease of the tarsal navicular. Clin Orthop Relat Res. 1981 Jul-Aug;(158):53-8. PubMed PMID: 7273526. PMID:7273526 (Link to Abstract)

3. 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. PubMed PMID: 6432840. PMID:6432840 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Derek Moore, Eric Shirley MD,

Please Rate Educational Value!

4.0

Average 4.0 of 6 Ratings

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Borges JL, Guille JT, Bowen JR. Köhler's bone disease of the tarsal navicular. J Pediatr Orthop. 1995 Sep-Oct;15(5):596-8 PMID:7593569 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Able MF (ED). Orthopaedic Knowledge Update: Pediatrics. American Academy of Orthopaedic Surgeons. Rosemont, IL. 2006, pp 235-245

Undefined

DiGiovanni CW, Patel A, Calfee R, Nickisch F. Osteonecrosis in the foot. J Am Acad Orthop Surg. 2007 Apr;15(4):208-17. PMID:17426292 (Link to Abstract)
Williams GA, Cowell HR. Köhler's disease of the tarsal navicular. Clin Orthop Relat Res. 1981 Jul-Aug;(158):53-8. PubMed PMID: 7273526. PMID:7273526 (Link to Abstract)
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. PubMed PMID: 6432840. PMID:6432840 (Link to Abstract)

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