“ Multisystem connective tissue disorder with a 70% autosomal dominant inheritance . Cardiac disease is the cause of death in 90%. There a...
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Multisystem connective tissue disorder with a 70% autosomal dominant inheritance.
Cardiac disease is the cause of death in 90%.
There are numerous musculoskeletal manifestations.
SPINE X-RAY
• Progressive scoliosis (about 60% affected)
• Scalloping of the posterior lumbosacral vertebral bodies (due to dural ectasia)
• Spondylolisthesis
HANDS/FEET X-RAY
• Arachnodactyly (i.e. elongated metacarpals) is non-specific.
• Pes planus.
• Hallux valgus.
PELVIS X-RAY
• Acetabular protrusion.
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