The growing spine in marfan and loeys–dietz syndromes

Abstract

Marfan and Loeys-Dietz Syndromes both involve connective tissues and TGF-beta signaling. Both may lead to spinal deformity which becomes severe at an early age. Bracing may only be of value if started before 25°. Growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically-controlled rods may not be feasible if the patients require periodic MRI for the cardiovascular system. Growing rods produce good deformity control in both conditions. The rate of rod fracture is higher in Loeys-Dietz syndrome, perhaps because of the greater tissue laxity. The surgeon should be aware of cervical deformity in this population, either at presentation or later in the course.