Ehlers-Danlos syndrome: Difference between revisions
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*Hereditary [[connective tissue disorder]] | *Hereditary [[connective tissue disorder]] | ||
*Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis | *Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis | ||
*Causes increased risk of: | |||
**[[Thoracic Aortic Aneurysm|Thoracic]] and [[abdominal aortic aneurysm]] | |||
**[[Mitral valve prolapse]] | |||
**Spontaneous [[pneumothorax]] | |||
**Myopia and retinal detachment | |||
**Premature degenerative arthritis | |||
==Clinical Features== | ==Clinical Features== | ||
*Hyper-extensible skin, classically "doughy" or "velvety" | *Hyper-extensible skin, classically "doughy" or "velvety" | ||
*Hypermobile joints | *Hypermobile joints | ||
*Tissue fragility and poor wound healing | *Tissue fragility and poor wound healing | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Marfan syndrome]] | *[[Marfan syndrome]] | ||
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*Stickler syndrome | *Stickler syndrome | ||
*Arteria tortuosity syndrome | *Arteria tortuosity syndrome | ||
==Evaluation== | ==Evaluation== | ||
==Management== | ==Management== | ||
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*Ophthalmologic evaluation to screen for myopia, retinal detachment, and glaucoma | *Ophthalmologic evaluation to screen for myopia, retinal detachment, and glaucoma | ||
*Monitoring of skin fragility, wound healing, easy bruising | *Monitoring of skin fragility, wound healing, easy bruising | ||
==Disposition== | |||
==See Also== | ==See Also== | ||
*[[Connective tissue | *[[Connective tissue disease]] | ||
==External Links== | ==External Links== | ||
==References== | ==References== | ||
Revision as of 06:19, 15 July 2017
Background
- Hereditary connective tissue disorder
- Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis
- Causes increased risk of:
- Thoracic and abdominal aortic aneurysm
- Mitral valve prolapse
- Spontaneous pneumothorax
- Myopia and retinal detachment
- Premature degenerative arthritis
Clinical Features
- Hyper-extensible skin, classically "doughy" or "velvety"
- Hypermobile joints
- Tissue fragility and poor wound healing
Differential Diagnosis
- Marfan syndrome
- Cutis laxa
- Loeys-Dietz syndrome
- Osteogenesis imperfecta
- Larsen Syndrome
- Stickler syndrome
- Arteria tortuosity syndrome
Evaluation
Management
- Cardiac evaluation to screen for valvular disease, vascular disease, and aortic aneurysm
- Ophthalmologic evaluation to screen for myopia, retinal detachment, and glaucoma
- Monitoring of skin fragility, wound healing, easy bruising