Neonatal compartment syndrome
Synonyms: CVIC · Congenital Volkmann ischemic contracture syndrome · NCS · Neonatal Volkmann ischemic contracture syndrome · Syndrome de Volkmann congénital
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Neonatal
- Status
- active
A rare non-inflammatory vasculopathy characterized by intracompartmental pressure typically in a distal upper extremity due to intrauterine ischemia and external compression. Affected limb presents significant swelling and sentinel skin changes (desquamation, blister/bullae formation, and skin necrosis). It can lead to long-term sequelae including Volkmann contracture, limb length discrepancies,bone growth abnormalities and nerve lesions. Neurologic involvement, ranging from sensory and motor nerve palsies to complete hand paralysis, may persist.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Neonatal compartment syndrome | CVIC · Congenital Volkmann ischemic contracture syndrome · NCS · Neonatal Volkmann ischemic contracture syndrome · Syndrome de Volkmann congénital |
| fr | Syndrome des loges néonatal | Syndrome de Volkmann néonatal |
| de | Neonatales Kompartmentsyndrom | CVIC · Ischämische Volkmann-Kontraktur · Ischämisches Kompartmentsyndrom des Neugeborenen · Kompartmentsyndrom des Neugeborenen · NCS · Volkmannsche Kontraktur |
| es | Síndrome compartimental neonatal | Síndrome de Volkmann congénito · Síndrome de contractura isquémica congénita de Volkmann · Síndrome de contractura isquémica neonatal de Volkmann |
| it | Sindrome compartimentale neonatale | CVIC · NCS · Sindrome di Volkmann congenita · Sindrome di Volkmann congenita da contratture ischemiche · Sindrome di Volkmann neonatale da contratture ischemiche |
| nl | Neonataal compartimentsyndroom | CVIC · Congenitaal syndroom van Volkmann · Congenitaal syndroom van ischemische Volkmann-contractuur · NCS · Neonataal syndroom van ischemische Volkmann-contractuur |
| pt | Síndrome compartimental neonatal | CVIC · NCS · Síndrome Volkmann congénito · Síndrome Volkmann isquémico contractural congénito · Síndrome Volkmann isquémico contractural neonatal |
| pl | Zespół ciasnoty powięziowej u noworodka | = en |
| cs | Novorozenecký kompartment syndrom | = en |
| uk | Неонатальний дистрес-синдром новонароджених | Вроджений синдром ішемічної контрактури Фолькмана · CVIC · NCS · Неонатальний синдром ішемічної контрактури Фолькмана · Вроджений синдром Фолькмана |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | P01.8 | NTBT | attributed | validated |
| ICD-11 | NF0A.6 | NTBT | attributed | validated |
| MONDO | 0957432 | E | validated | |
| UMLS | C5782100 | 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:496924Non-inflammatory vasculopathy
rare circulatory system 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 | 60 cases | Worldwide | Case(s) | Validated | 35415493[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 35415493[PMID]_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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/641829
- This record as JSON: /data/d/641829.json