The growing spine in Marfan and Loeys-Dietz syndromes

Marfan and Loeys-Dietz syndromes both involve disorders of the connective tissue and affect the TGF-B signaling pathway. Both may lead to spinal deformity, which becomes severe at an early age. Bracing may only be of value if started before the curve is 25° and growth-friendly strategies may be necessary. VEPTR may lead to kyphosis and forward trunk balance in these patients. Magnetically controlled growing rods (MCGR) may not be feasible if the patients require periodic MRI for the cardiovascular system. Traditional growing rods (TGR) 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 and osteopenia. The surgeon should be aware of cervical deformity in this population as well, either at presentation or later in the course.