- Autosomal dominant
Major Diagnostic criteria
- Severe generalised joint hypermobility with recurrent subluxations
- Congenital bilateral hip dislocation.
Minor Diagnostic criteria
- Skin hyper-extensibility
- Tissue fragility, including atrophic scars
- Easy bruising
- Muscle hypotania
- Radiologically mild osteopenia
- Congenital hip dislocation has been present in all biochemically proven individuals.
- Short statue is not a manifestation, unless it is a complication of severe kyphoscoliosis and / or hip dislocation.
- Larsen syndrome should be considered in the differential diagnosis.
- For management see (Steinmann et al. 1993).