[Kyphotic-scoliotic deformities of the spine in children and adolescents with Ehlers-Danlos syndrome and their treatment]

Abstract

In the article are presented the results of observation of 8 patients, aged 4-16 years, with Ehlers-Danlos++ syndrome, one of the most frequent hereditary disease with generalized affection of connective tissue. ++Clinico-roentgenologic characteristic of kyphotic-scoliotic++ deformity of vertebral column indicates its progressive nature, that determines indications for surgical intervention but concomitant changes of cardiovascular system should be considered. Preoperative preparation includes measures for vertebral column mobilization as well as pharmacotherapy with retabolil in combination with ascorbic acid, calcetrine and methyluracil++. Selected method of operative treatment is Harrington-Luck techniques in case of scoliosis prevalence and application of original endocorrector with associated influence at kyphotic component. As a result of treatment scoliosis is corrected, on average, by 49,2% and kyphosis by 62,7%.