Ehlers-Danlos syndrome: Difference between revisions
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==Background== | ==Background== | ||
* Hereditary [[connective tissue disorder]] | *Hereditary [[connective tissue disorder]] | ||
* Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis | *Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis | ||
==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 | ||
Increased risk of: | Increased risk of: | ||
* [[Thoracic Aortic Aneurysm|Thoracic]] and [[abdominal aortic aneurysm]] | *[[Thoracic Aortic Aneurysm|Thoracic]] and [[abdominal aortic aneurysm]] | ||
* [[Mitral valve prolapse]] | *[[Mitral valve prolapse]] | ||
* Spontaneous [[pneumothorax]] | *Spontaneous [[pneumothorax]] | ||
* Myopia and retinal detachment | *Myopia and retinal detachment | ||
* Premature degenerative arthritis | *Premature degenerative arthritis | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
*[[Marfan syndrome]] | *[[Marfan syndrome]] | ||
* Cutis laxa | *Cutis laxa | ||
*Loeys-Dietz syndrome | *Loeys-Dietz syndrome | ||
*Osteogenesis imperfecta | *Osteogenesis imperfecta | ||
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*Cardiac evaluation to screen for valvular disease, vascular disease, and aortic aneurysm | *Cardiac evaluation to screen for valvular disease, vascular disease, and aortic aneurysm | ||
*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 | ||
==See Also== | ==See Also== | ||
Revision as of 03:11, 15 July 2017
Background
- Hereditary connective tissue disorder
- Six types: Classic, hypermobility, vascular, kyphoscoliosis, arthrochalasia, dermatosparaxis
Clinical Features
- Hyper-extensible skin, classically "doughy" or "velvety"
- Hypermobile joints
- Tissue fragility and poor wound healing
Increased risk of:
- Thoracic and abdominal aortic aneurysm
- Mitral valve prolapse
- Spontaneous pneumothorax
- Myopia and retinal detachment
- Premature degenerative arthritis
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