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To meet the diagnostic criteria for kEDS, a person must meet: Major criterion 1 AND major criterion 2 AND major criterion 3 OR Major criterion 1 AND major criterion 2 AND three minor criteria (general or gene-specific) Major Criteria Congenital muscle hypotonia Congenital or early onset kyphoscoliosis (progressive or non-progressive) Generalized joint hypermobility with dislocations/subluxations (shoulders, hips, and knees in particular) Minor Criteria Skin hyperextensibility Easily bruisable skin Rupture/aneurysm of a medium-sized artery Osteopenia/osteoporosis Blue sclerae Hernia (umbilical or inguinal) Pectus deformity Marfanoid habitus Talipes equinovarus Refractive errors (myopia, hypermetropia) Gene-Specific Minor Criteria PLOD1 Skin fragility (easy bruising, friable skin, poor wound healing, widened atrophic scarring) Scleral and ocular fragility/rupture Microcornea Facial dysmorphology FKBP14 Congenital hearing impairment (sensorineural, conductive, or mixed) Follicular hyperkeratosis Muscle atrophy Bladder diverticula Musculocontractural EDS (mcEDS) If a person meets the diagnostic criteria for mcEDS, genetic testing should be done to confirm the diagnosis

Computerized axial tomography and MRI can also outline the cyst more accurately and allows assessment for rupture of the cyst, hemorrhagic transformation, and accompanying pyomyositis or osteomyelitis [1]

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