Osteochondrosis of the tarsal bone
Synonyms: Aseptic necrosis of the tarsal bone · Avascular necrosis of the tarsal bone · Kohler disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Childhood
- Status
- active
A rare bone disease characterized by avascular necrosis of the navicular bone in children. Patients present with sudden unexplained foot pain, inability to bear weight, and limping. Radiographic features include flattening, fragmentation, and patchy sclerosis of the navicular bone. Soft tissue swelling may be associated. The condition is most commonly unilateral and self-limiting. Boys are more often affected than girls.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Osteochondrosis of the tarsal bone | Aseptic necrosis of the tarsal bone · Avascular necrosis of the tarsal bone · Kohler disease |
| fr | Ostéochondrose du tarse | Maladie de Kohler · Nécrose aseptique du tarse · Nécrose avasculaire du tarse · Ostéochondrite du tarse |
| de | Osteochondrose des Fußwurzelknochens | Aseptische Nekrose des Fußwurzelknochens · Avvaskuläre Nekrose des Fußwurzelknochens · Kohler-Krankheit |
| es | Osteocondrosis de hueso tarsiano | Enfermedad de Kohler · Necrosis aséptica de hueso tarsiano · Necrosis avascular de hueso tarsiano |
| it | Osteocondrosi del tarso | Malattia di Kohler · Necrosi asettica del tarso · Necrosi avascolare del tarso |
| nl | Osteochondrose van tarsaal bot | Aseptische necrose van tarsaal bot · Avasculaire necrose van tarsaal bot · Ziekte van Kohler |
| pt | Osteocondrose do osso tarso | |
| pl | Osteochondroza kości stępu | Choroba Kohlera |
| cs | Osteochondróza nártní kosti | = en |
| uk | Остеохондроз передплеснової кістки | Асептичний некроз передплеснової кістки · Аваскулярний некроз передплеснової кістки · Хвороба Колера |
| zh | 跗骨软骨病 | 跗骨无菌性坏死 |
| tr | Tarsal kemiğin osteokondrozu | Kohler hastalığı · Tarsal kemiğin aseptik nekrozu · Tarsal kemiğin avasküler nekrozu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M92.6 | E | specific | validated |
| ICD-11 | FB82.1 | NTBT | index | validated |
| MONDO | 0016086 | E | validated | |
| MONDO | 16086 | E | validated | |
| UMLS | C0022765 | E | validated | |
| GARD | 6842 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:399319Osteochondrosis
rare systemic and rheumatological diseases; rare bone diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
13 annotations · validated 2021-06-11| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0008369 | Abnormal tarsal ossification | |
| HP:0030840 | Ankle pain | |
| HP:0031955 | Antalgic gait | |
| HP:0100662 | Chondritis | |
| HP:0008144 | Flattening of the talar dome | |
| HP:0025238 | Foot pain | |
| HP:0010741 | Pedal edema | |
| HP:0031051 | Tarsal sclerosis | |
| HP:0008131 | Tarsal stippling | |
| Occasional (29-5%) | ||
| HP:0001369 | Arthritis | |
| HP:0010885 | Avascular necrosis | |
| HP:0100323 | Juvenile aseptic necrosis | |
| HP:0010886 | Osteochondritis Dissecans | |
Annotation source: 30838149[PMID]_30685015[PMID]_31083875[PMID]_30949007[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/563991
- This record as JSON: /data/d/563991.json