Complications in Children with Ehlers-Danlos Syndrome Following Spine Surgery: Analysis of the Pediatric National Surgery Quality Improvement Program Database

Ehlers-Danlos syndrome (EDS) is a group of rare congenital disorders of connective tissue that result in tissue fragility and joint hyperextensibility. Owing to its rarity, outcomes of pediatric spine surgery in patients with EDS are poorly characterized. Although it has been suggested that complication rates are high, few studies have characterized these complications. Pediatric National Surgery Quality Improvement Program data from 2012–2016 were analyzed. Patients with EDS undergoing spine surgery were identified along with patients without EDS undergoing the same surgeries using International Classification of Diseases, Ninth Revision and Current Procedural Terminology codes. Of 369,176 total patients, 279 were determined to have EDS. Of these, 56 patients underwent spine surgery; 46% were male and 54% were female (P = 0.108). Mean age at surgery was 11.59 years (P = 0.888) with a range of 1.77–17.33 years. The most common procedure was arthrodesis (n = 37). There were no differences in unplanned reoperations (n = 4, P = 0.119), wound infections or disruptions (n = 2, P = 0.670), or overall complications (n = 25, P = 0.751). Blood transfusions were required in 41% of patients with EDS, but this was not significant compared with patients without EDS undergoing the same procedures (n = 23, P = 0.580). The total amount of blood transfused (P = 0.508), length of hospital stay (P = 0.396), and total operative time (P = 0.357) were not different from control subjects. Pediatric patients with EDS 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.