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

Matur, Abhijith V.; Nouri, Aria; Huang, Shenwen; Elson, Nora C.; Jeong, William; Bierbrauer, Karin; Mangano, Francesco T.

doi:10.1016/j.wneu.2019.09.046

Cited by 13 publications

(12 citation statements)

References 19 publications

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“…This is consistent to the findings of Matur et al. who in a study of fifty-six pediatric deformity EDS patients, found that they do not have a greater risk of perioperative adverse events [17] . Nonetheless, this different from Rabenhorst et al.…”

Section: Discussionsupporting

confidence: 91%

Single-level posterior lumbar fusions in patients with Ehlers Danlos Syndrome not found to be associated with increased postoperative adverse events or five-year reoperations

Gouzoulis¹,

Kammien²,

Zhu³

et al. 2022

North American Spine Society Journal (NASSJ)

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Section: Discussionsupporting

confidence: 91%

Single-level posterior lumbar fusions in patients with Ehlers Danlos Syndrome not found to be associated with increased postoperative adverse events or five-year reoperations

Gouzoulis¹,

Kammien²,

Zhu³

et al. 2022

North American Spine Society Journal (NASSJ)

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“…This study challenges many of the others in that it does not report an overall high complication rate in EDS patients, but there are several limitations including the small sample size of 13 EDS patients and the three-fold incidence of dislocations (Guier et al, 2020). Another study by Matur et al (2020) analyzed pediatric patients with EDS undergoing spine surgery and compared them to patients without EDS undergoing the same surgery. After studying 56 patients with EDS undergoing spine surgery, they concluded that there were no differences between patients with and without EDS in unplanned reoperations, wound infections or disruptions, total amount of blood transfused, length of hospital stay, or overall complication rate (Matur et al, 2020).…”

Section: Procedure-specific Outcomesmentioning

confidence: 83%

“…Another study by Matur et al (2020) analyzed pediatric patients with EDS undergoing spine surgery and compared them to patients without EDS undergoing the same surgery. After studying 56 patients with EDS undergoing spine surgery, they concluded that there were no differences between patients with and without EDS in unplanned reoperations, wound infections or disruptions, total amount of blood transfused, length of hospital stay, or overall complication rate (Matur et al, 2020). However, since this study obtained its data from the Pediatric National Surgery Quality Improvement Program, long-term outcomes could not be reported, significantly limiting the results of this study (Matur et al, 2020).…”

Section: Procedure-specific Outcomesmentioning

confidence: 99%

Orthopedic considerations and surgical outcomes in Ehlers–Danlos syndromes

Yonko

LoTurco

Carter

et al. 2021

American J of Med Genetics Pt C

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The Ehlers-Danlos syndromes (EDS) are a clinically and genetically heterogeneous group of connective tissue disorders with varying physical manifestations. There are no clear guidelines for addressing orthopedic concerns or reporting surgical outcomes in this population. This article reviews the literature, reports on a new study, and offers considerations prior to surgical intervention. The new study seeks to determine the effectiveness of surgical intervention in individuals with EDS. It is a retrospective chart review of 154 individuals clinically diagnosed with EDS who had orthopedic surgery >2 years ago at Hospital for Special Surgery. A total of 120 individuals were included in the study. One hundred eleven females and 9 males underwent a total of 320 orthopedic surgeries, of which 204 surgeries had available postoperative follow-up. The average age at surgery was 38.2 years (range: 7.6-83.3).Multiple post-operative complications (290) were reported in 91% of cases. Common complications were persistent pain/discomfort (45), continued subluxation/ dislocation (20), instability (19), pain/discomfort from hardware (17), and infection (16). Our results suggest that surgical outcomes are worse for individuals with EDS compared to the general population, a finding which is similar to other studies. Complications occurred more frequently in the EDS population than the average population, suggesting that surgery should be undertaken by a multidisciplinary team of clinicians with careful pre-operative planning and full knowledge of the risks and benefits. Guidelines for the care of this unique population must be established.

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“…examining 279 patients with Ehlers-Danlos syndromes did not find any increased bleeding or vascular risk. 21 In orthopaedics specifically, little recent data exist on how patients with EDS fare postoperatively. A survey of 214 joint-related procedures of the shoulder, elbow, knee, and ankle by Weinberg et al 22 published in 1999 found postoperative complications occurred more often compared with patients without connective tissue disorders.…”

Section: Figurementioning

confidence: 99%

Patients With Ehlers-Danlos Syndromes Experience Higher Rates of Prosthetic Dislocation After Total Hip Arthroplasty and Worse Implant Survival at 5 Years

Moore

Burroughs

Rubin

et al. 2021

J Am Acad Orthop Surg

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Introduction: Ehlers-Danlos syndromes (EDS) are genetic connective tissue disorders affecting multiple organ systems that frequently result in connective tissue hyperlaxity and early osteoarthritis. Short-and longterm outcomes after total hip arthroplasty in this cohort remain poorly characterized. Methods: Data were abstracted from the 2010 to 2018 PearlDiver Mariner administrative database. Patients with EDS undergoing total hip arthroplasty for osteoarthritis were identified and matched 1:10 to patients without a diagnosis of EDS. Ninety-day incidence of postoperative complications and hospital readmission were identified and compared. Odds of 90-day adverse events were compared using multivariate logistic regression. Finally, 5-year implant survival was compared using a log-rank test. Significance was set at P , 0.005 after Bonferroni correction. Results: Matching was done for 354 patients with EDS to 3,518 patients without EDS. Patients with EDS had markedly higher rates of periprosthetic dislocation within 90 days of surgery (4.2% dislocation rate in EDS vs. 1.7% in matched control subjects, P = 0.001). On multivariate analysis, patients with EDS continued to have increased odds of dislocation in the 90-day postoperative period (Odds Ratio=2.64, P = 0.001), whereas the rates of the other studied adverse events were not markedly different between groups. At 5 years, 92.7% of patients with EDS and 96.1% of the matched control subjects remained unrevised (significant log rank difference, P = 0.004). Conclusion: Compared with patients without EDS, patients with EDS were found to have a higher rate of perioperative dislocation and markedly lower implant survival at 5 years. These findings are consistent with the increased connective tissue laxity associated with EDS. Ehlers-Danlos syndromes (EDS) are a family of genetic connective tissue disorders affecting roughly one in 5,000 to 20,000 people worldwide and is inherited in either an autosomal dominant, recessive, or X-lined

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

Cited by 13 publications

References 19 publications

Single-level posterior lumbar fusions in patients with Ehlers Danlos Syndrome not found to be associated with increased postoperative adverse events or five-year reoperations

Single-level posterior lumbar fusions in patients with Ehlers Danlos Syndrome not found to be associated with increased postoperative adverse events or five-year reoperations

Orthopedic considerations and surgical outcomes in Ehlers–Danlos syndromes

Patients With Ehlers-Danlos Syndromes Experience Higher Rates of Prosthetic Dislocation After Total Hip Arthroplasty and Worse Implant Survival at 5 Years

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