Posterior spinal fusion in patients with Ehlers-Danlos syndrome: a report of six cases.

ABSTRACT

PURPOSE: There is a paucity of literature describing posterior spinal fusion (PSF) in the Ehlers-Danlos syndrome (EDS) patient. The vast majority of these studies diagnosed EDS clinically. The purpose of this study is to discuss the management and complications of EDS patients with scoliosis treated with PSF at a single institution. METHODS: Clinical and radiographic data are presented describing six patients who had PSF for EDS. The diagnoses were confirmed by a geneticist. RESULTS: All of the patients in the current cohort underwent posterior fusion only, with no anterior approach. Neuromonitoring was also used in the majority. Half of our patients experienced complications. One patient had a hemoperitoneum that was initially treated nonoperatively but, unfortunately, they expired 1 month after discharge from abdominal bleeding. Another patient suffered neuropathic pain attributed to the type of implant used. A third underwent a total of seven procedures beginning at the age of 3 years in a different era of spinal surgery. The mean major curve, percentage correction, and estimated blood loss of the current cohort are similar to previous studies. CONCLUSION: The fragility of the EDS patient population cannot be overlooked. Despite a conservative surgical approach, half of our patients experienced complications. The surgeon choosing to operate on EDS patients must do so with extreme caution.