There are many Chiari patients who also have EDS. An added caution is needed due to the diagnosis of EDS. Chiari is typically treated in one of two ways: drug management and/or surgery. With EDS, the surgical protocol must be altered to protect connective tissue, deal with arachnoid scarring or adhesions and prevent cranial-cervical instability. The surgical techniques are different, too. They are designed to protect fragile tissues and prevent or minimize bleeding, and post operatively, there is more emphasis on preventing or fixing would dehiscence. Seek out and consult with Chiari-experts before undergoing any treatment or surgery. It is generally not an emergency, unless you have any of the three red flag symptoms: choking, breathing problems or drop attacks. Those indicate more severe brainstem involvement, so don't delay in seeking treatment for them. Resource: (Grubb 2002 - Rowe er.al 1999)
Hereditary disorders of connective tissue may present with Chiari I malformation, occipitoatlantoaxial hypermobility and functional cranial settling. C. Francomano, T. Milhorat, P. Bolognese, M. Nishikawa, N. McDonnell. We report an association of hereditary disorders of connective tissue (HDCT) and Chiari malformation 1 (CM1), presenting with lower brain stem symptoms attributable to occipito-atlantoaxial hypermobility and functional cranial settling. The prevalence of hereditary disorders of connective tissue (HDCT) was determined in a prospectively collected cohort of 2,813 patients with CM1. The diagnosis of Ehlers-Danlos syndrome (EDS) or other HDCT was made in 357 of 2,813 of patients with CM1 (12.7%). The clinical features of HDCT/CM1 were distinguished from those of CM1 alone by clinical stigmata of HDCT, a greater female preponderance (7:1 vs. 3:1), a greater incidence of lower brain stem symptoms (0.43 vs. 0.05), retroodontoid pannus formation (0.71 vs. 0.16) and hypoplasia of the oropharynx (0.45 vs. 0.02). In patients with HDCT/CM1, upon sitting or standing there was reduction of the basal-dens interval (3.6 mm), enlargement of the basal-atlas interval (3.0 mm) reduction of the clivus-axis angle(10.8o), reduction of clivus-atlas angle (5.8o) and reduction of the atlas-axial angle (5.3o). These changes were reducible by cervical traction or returning to the supine position. In normal controls and patients with CM1 alone, these measurements did not change with position. The identification of HDCT in 12.7% of patients with CM1 establishes an association between these previously unrelated disorders. Patients with HDCT and symptoms suggestive of CM1 should be evaluated with brain MRIs in the supine and upright positions. Full paper available from http://bit.ly/xesPlJ.