Mueller-Weiss syndrome
Synonyms: Brailsford disease · Mueller-Weiss osteonecrosis of the tarsal bone
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare bone disease characterized by spontaneous adult-onset tarsal navicular osteonecrosis. Patients present with chronic mid- and hindfoot pain, swelling and tenderness over the dorsomedial aspect of the midfoot, flattening of the medial longitudinal arch, and pes planovarus. Radiographic findings include comma-shaped deformity due to collapse of the lateral part of the navicular bone and medial or dorsal protrusion of a portion or the entire bone. The condition may be bilateral or asymmetric and associated with pathological fractures.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Mueller-Weiss syndrome | Brailsford disease · Mueller-Weiss osteonecrosis of the tarsal bone |
| fr | Syndrome de Mueller-Weiss | Maladie de Brailsford · Ostéonécrose du tarse de Mueller-Weiss |
| de | Mueller-Weiss-Syndrom | Brailsford-Krankheit · Mueller-Weiss Osteonekrose des Fußwurzelknochens |
| es | Síndrome de Mueller-Weiss | Osteonecrosis de hueso tarsiano de Mueller-Weiss · Síndrome de Brailsford |
| it | Sindrome di Mueller-Weiss | Malattia di Brailsford · Osteonecrosi di Mueller-Weiss del tarso |
| nl | Syndroom van Mueller-Weiss | Mueller-Weiss-osteonecrose van tarsaal bot · Ziekte van Brailsford |
| pt | Síndrome Mueller-Weiss | |
| pl | Zespół Muellera i Weissa | Choroba Brailsforda |
| cs | Mueller-Weiss syndrom | = en |
| uk | Синдром Мюллера-Вейсса | Хвороба Брейлсфорда · Остеонекроз Мюллера-Вайса передплеснової кістки |
| zh | Muller-Weiss病（足舟骨坏死） | 足舟骨Muller-Weiss坏死 |
| tr | Mueller-Weiss sendromu | Brailsford hastalığı · Tarsal kemiğin Mueller-Weiss osteonekrozu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | M92.6 | NTBT | attributed | validated |
| ICD-11 | FB81.Y | NTBT | attributed | validated |
| MONDO | 0035452 | E | validated | |
| UMLS | C4761149 | E | validated | |
| MedDRA | 10082326 | 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:399158Osteonecrosis
rare systemic and rheumatological diseases; rare bone diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 277 cases | Worldwide | Case(s) | Validated | 27145453[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | 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
21 annotations · validated 2021-06-16| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0025238 | Foot pain | |
| Frequent (79-30%) | ||
| HP:0100339 | Abnormality of the os naviculare pedis | |
| HP:0010885 | Avascular necrosis | |
| HP:0100662 | Chondritis | |
| HP:0012098 | Edema of the dorsum of feet | |
| HP:0030871 | Facet joint arthrosis | |
| HP:0001288 | Gait disturbance | |
| HP:0001387 | Joint stiffness | |
| HP:0010505 | Limitation of movement at ankles | |
| HP:0010741 | Pedal edema | |
| HP:0005656 | Positional foot deformity | |
| HP:0100925 | Sclerosis of foot bone | |
| Occasional (29-5%) | ||
| HP:0001369 | Arthritis | |
| HP:0008110 | Equinovarus deformity | |
| HP:0005063 | Fragmented, irregular epiphyses | |
| HP:0032153 | Joint subluxation | |
| HP:0005086 | Knee osteoarthritis | |
| HP:0001763 | Pes planus | |
| HP:0008124 | Talipes calcaneovarus | |
| HP:0100694 | Tibial torsion | |
| HP:0100535 | Tibiofibular diastasis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 30838149[PMID]_27145453[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/566943
- This record as JSON: /data/d/566943.json