The Clinical Presentation of Ehlers-Danlos Syndrome

Elizabeth J. Lawrence, RNC, MSN, NNP

Disclosures

Adv Neonatal Care. 2005;5(6):301-314. 

In This Article

Prognosis

Prognosis is dependent on the type of EDS involved. Early diagnosis of vascular-type EDS may improve life expectancy. Sudden death from vascular rupture or perforation is not uncommon.[28,31,33] Vascular complications are not limited to adults. Investigation of a basilar arterial aneurysm in a 5-year-old child revealed that she had vascular-type EDS.[32] Vascular-type EDS should be considered part of the differential diagnosis of cerebral vascular accidents or strokes in infancy and early childhood.[32]

Individuals with classical- and hypermobile-type EDS have a normal lifespan.[10,25] People affected by kyphoscoliosis-type EDS may have a normal lifespan; however, in the most severe cases, they are at risk of respiratory compromise and arterial rupture.[35] The severe hypotonia in kyphoscoliosis-type EDS leads to motor developmental delays.[35] By the second or third decade of life, many of these patients are unable to walk.[1]

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