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Start free with EleplanNeonatal compartment syndrome
ORPHA:641829Clinical syndrome
Also called CVIC · Congenital Volkmann ischemic contracture syndrome · NCS · Neonatal Volkmann ischemic contracture syndrome · Syndrome de Volkmann congénital
What it is
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.
Key facts
- Prevalence
- <1 / 1 000 000
- Age of onset
- Neonatal
- Inheritance
- Not applicable
- Classified as
- Clinical syndrome
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
ICD-10 codes
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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