Complications in Children With Ehlers-Danlos Syndrome following Spine Surgery: Analysis of the Pediatric National Surgery Quality Improvement Program Database. (1st September 2019) - Matur, Abhijith V
Abstract: INTRODUCTION: Ehlers-Danlos syndrome (EDS) is a group of rare congenital disorders of connective tissue that result in tissue fragility and joint hyperextensibility. Due to its rarity, outcomes of pediatric spine surgery in EDS patients are poorly characterized. While it has been suggested that complication rates are high, few studies have characterized these complications. METHODS: Pediatric National Surgery Quality Improvement Program (PNSQIP) data from 2012 to 2016 was analyzed using IBM SPSS 24. EDS patients undergoing spine surgery were identified along with patients without EDS undergoing the same surgeries using ICD-9 and CPT codes. Patient demographics and complications were identified. Chi-squared, Fisher's exact, and independent samples t tests were used where appropriate. RESULTS: A total of 279 patients of 369 176 total patients were determined to have EDS. Of these, 56 patients were determined to have undergone spine surgery. 46% were male and 54% were female ( P = .108). Mean age at surgery was 11.59 yr ( P = .888) with a range of 1.77 to 17.33 yr. The most common procedure was arthrodesis (n = 37), with 14 patients having 13 or more levels fused. There was no difference in unplanned reoperation (n = 4, P = .119), wound infection or disruption (n = 2, P = .670), or overall complication (n = 25, P = .751). 41% of EDS patients required blood transfusions but this was not significant compared to non-EDS patients undergoing the same procedures (n = 23, P = .580). The total amount transfused ( P = .508), time until transfusion was required ( P = .127), duration of anesthesia ( P = .713), length of hospital stay ( P = .396), and total operation time ( P = .357) were not different from controls. CONCLUSION: Pediatric EDS patients do not appear to be at a higher risk of bleeding or other complications during spine surgery as reported in past case series. This is the largest retrospective review of its kind that has been performed in this patient population, however, higher power studies are needed to better explore outcomes of pediatric spine surgery patients with EDS.