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 complica...

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Veröffentlicht in:World neurosurgery 2020-01, Vol.133, p.e473-e478
Hauptverfasser: Matur, Abhijith V., Nouri, Aria, Huang, Shenwen, Elson, Nora C., Jeong, William, Bierbrauer, Karin S., Mangano, Francesco T., Cheng, Joseph S.
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container_end_page e478
container_issue
container_start_page e473
container_title World neurosurgery
container_volume 133
creator Matur, Abhijith V.
Nouri, Aria
Huang, Shenwen
Elson, Nora C.
Jeong, William
Bierbrauer, Karin S.
Mangano, Francesco T.
Cheng, Joseph S.
description 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.
doi_str_mv 10.1016/j.wneu.2019.09.046
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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. 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subjects Adolescent
Blood Loss, Surgical
Blood Transfusion
Child
Child, Preschool
Complications
Connective tissue disorder
Databases, Factual
Ehlers-Danlos
Ehlers-Danlos Syndrome - complications
Ehlers-Danlos Syndrome - surgery
Female
Hemorrhagic Disorders - genetics
Humans
Infant
Length of Stay - statistics & numerical data
Male
Operative Time
Outcomes
Pediatric
Postoperative Complications - epidemiology
Postoperative Complications - genetics
Postoperative Hemorrhage - epidemiology
Postoperative Hemorrhage - genetics
Retrospective Studies
Spinal Fusion
Spine
United States - epidemiology
title Complications in Children with Ehlers-Danlos Syndrome Following Spine Surgery: Analysis of the Pediatric National Surgery Quality Improvement Program Database
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