Surgical correction of spinal deformities in patients with Ehlers-Danlos syndrome. Non-systematic review

Introduction Ehlers-Danlos Syndrome (EDS) is a group of hereditary pathological conditions caused by various defects in collagen metabolism. EDS can be accompanied by progressive spinal deformities that may require surgical correction. No review papers reporting the results of the interventions were found in the literature. The objective was to present and analyze the results of surgical correction of severe progressive spinal deformities in EDS patients. Material and methods The review is based on 11 papers published between 1990 and 2014. They report the results of the treatment of 56 patients with half describing individual observations and half reporting clinical cohorts of 7 to 9 cases. Results and discussion The results of conservative treatment indicate its low efficiency. Scoliosis ranged between 45° and 143° with low preoperative mobility (20-40 %) at baseline and corresponded to the average surgical correction (30 to 55 %) and insignificant postoperative loss of correction. The average blood loss measured 540 to 1800 ml. A total of 36 complications were noted in 56 patients, with most severe of them being neurological (three cases of paraplegia, without recovery in one of them) and vascular (damage to segmental vessels and major vascular trunks) events developing from ventral access. Conclusion Surgical correction of spinal deformities due to EDS is challenging and requires an individual approach in each specific case. Modern technology makes it possible to obtain very good results in the correction of spinal deformities in EDS. There is a paucity of published observations and new studies are needed.