Ehlers-Danlos Syndrome (Vascular type) Criteria
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Major Criteria
☐Thin, translucent skin (Especially on chest or abdomen)
☐Arterial / intestinal / uterine fragility or rupture
☐Easy bruisability
☐Characteristic facial appearance (Thin lips and philtrum, small chin, thin nose, large eyes)
Minor Criteria
☐Acrogeria (Aged appearance of extremities & hands)
☐Hypermobility of small joints
☐Tendon/muscle rupture
☐Clubfoot
☐Early-onset varicose veins
☐Arteriovenous carotid-cavernous sinus fistula
☐Pneumothorax or pneumohemothorax
☐Gingival recession
☐Positive family history (Consistent with autosomal dominant inheritance)
R e s u l t s
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Ehlers Vascular Criteria
 
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Ehlers-Danlos Syndrome (Vascular type) Criteria

Vascular Ehlers-Danlos Syndrome (EDS) is the most serious form of EDS as patients may have arterial or organ rupture spontaneously.

Clinical diagnostic criteria requires 2 Major criteria to be met. Even though Minor criteria are listed; none are required for clinical diagnosis.

Major Criteria

  • Thin, translucent skin (Especially on chest or abdomen)
  • Arterial/intestinal/uterine fragility or rupture
  • Easy bruisability
  • Characteristic facial appearance (Thin lips and philtrum, small chin, thin nose, large eyes)

Minor Criteria

  • Acrogeria (Aged appearance of extremities & hands)
  • Hypermobility of small joints
  • Tendon/muscle rupture
  • Clubfoot (Talipes equinovarus)
  • Early-onset varicose veins
  • Arteriovenous carotid-cavernous sinus fistula
  • Pneumothorax or pneumohemothorax
  • Gingival recession
  • Positive family history (Consistent with autosomal dominant inheritance)

References:

  1. Beighton P, De Paepe A, Steinmann B, et al. Ehlers-Danlos syndromes: Revised nosology, Villefranche, 1997. Am J Med Genet . 1998;:77(1):31-7.