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scientific articles

Natural History of Spinal Deformity in a Patient with Ehlers-Danlos syndrome: Case Report with 20-year Follow-up

key information

source: The Spine Journal

year: 2011

authors: Natarajan D, Samartzis D, Wong YW, Luk KD, Cheung KM

summary/abstract:

BACKGROUND CONTEXT:
Scoliosis can present in patients with Ehlers-Danlos syndrome (EDS) and can be surgically treated despite possible complications. The long-term natural history of the progression of spinal deformity, particularly scoliosis, and associated complications in EDS patients remains relatively unknown.
PURPOSE:
To assess the progression of spinal deformity and associated complications in a patient with EDS, who was not surgically treated for her spine condition and was followed up for 20 years.
STUDY DESIGN:
A case report.
METHODS:
A 9-year-old female with EDS presented to the orthopedic clinic with scoliosis and mild thoracic hyperkyphosis. The patient was followed up for a 20-year period. Progression of her spinal deformity and associated complications were noted.
RESULTS:
The patient’s curve progressed rapidly in the initial follow-up period an 83° worsening from the age of 9 to 10 years. Because she refused surgical intervention, her spinal deformity continued to progress. As such, by the age of 29 years, she presented with 115° from T9 to L4 with severe hyperkyphosis. The patient eventually developed respiratory compromise and diminished functional capacity.
CONCLUSIONS:
In patients with EDS, scoliosis may progress rapidly and unrelentingly, and if not treated early may lead to respiratory compromise. Close follow-up of such patients is imperative, and appropriate management should be sought to prevent potentially fatal morbidities.

organization: University of Hong Kong

DOI: 10.1016/j.spinee.2011.02.029

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