Journal article
Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure
Journal of pediatric orthopaedics, Vol.41(3), pp.E204-E210
03/01/2021
DOI: 10.1097/BPO.0000000000001730
PMID: 33370003
Abstract
Background: There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population. Methods: A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation. Results: Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (+/- 4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced >= 6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following. Conclusions: This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.
Details
- Title: Subtitle
- Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure
- Creators
- Laura L. Bellaire - University of Wisconsin–MadisonChong Zhang - University of UtahJohn T. Smith - University of UtahJohn A. Heflin - University of UtahJoshua Klatt - University of UtahDavid Roye - Columbia UniversityPaul Sponseller - Johns Hopkins UniversityAmer Samdani - Shriners Hospitals for Children - ErieGraham T. Fedorak - University of UtahPediatric Spine Study Group
- Contributors
- Josh Holt (Contributor) - University of Iowa, Orthopedics and RehabilitationStuart Weinstein (Contributor) - University of Iowa, Orthopedics and Rehabilitation
- Resource Type
- Journal article
- Publication Details
- Journal of pediatric orthopaedics, Vol.41(3), pp.E204-E210
- Publisher
- Lippincott Williams & Wilkins
- DOI
- 10.1097/BPO.0000000000001730
- PMID
- 33370003
- ISSN
- 0271-6798
- eISSN
- 1539-2570
- Number of pages
- 7
- Language
- English
- Date published
- 03/01/2021
- Academic Unit
- Stead Family Department of Pediatrics; Orthopedics and Rehabilitation
- Record Identifier
- 9984303980602771
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