Seventeen-year outcome of surgical management of severe early onset kyphoscoliosis in a patient with arthrochalasia-type Ehlers-Danlos.

Abstract

We report on the surgical management of severe early onset kyphoscoliosis in a 5-year-old patient with the rare arthrochalasia subtype of Ehlers-Danlos syndrome, who we have followed for 17 years post-operatively. Successful correction of the deformity with an excellent outcome was achieved with minimal morbidity using MAGEC™ (MAGnetic Expansion Control) growth rods instead of traditional growth rods, undertaken with the close involvement of plastic surgical colleagues. Our patient suffered only one minor surgical complication (thought to be allergic rather than traumatic in origin), despite having skin so fragile that rubbing his skin with a disinfectant wipe was sufficient to cause skin breakdown. The non-invasive lengthening that the MAGEC rods allowed enabled us to avoid repeated open surgeries which would have had a high risk of complications, most notably wound breakdown with poor healing, and we advocate their use in similar cases.