Title
Growth-Friendly Spine Surgery in Escobar Syndrome.
Document Type
Article
Publication Date
8-1-2019
Identifier
DOI: 10.1097/BPO.0000000000001315
Abstract
BACKGROUND: The aims of this study were to characterize the spinal deformity of patients with Escobar syndrome, describe results of growth-friendly treatments, and compare these results with those of an idiopathic early-onset scoliosis (EOS) cohort to determine whether the axial stiffness in Escobar syndrome limited correction.
METHODS: We used 2 multicenter databases to review the records of 8 patients with EOS associated with Escobar syndrome who had at least 2-year follow-up after initiation of growth-friendly treatment from 1990 to 2016. An idiopathic EOS cohort of 16 patients matched for age at surgery (±1 y), postoperative follow-up (±1 y), and initial curve magnitude (±10 degrees) was identified. A randomized 1:2 matching algorithm was applied (α=0.05).
RESULTS: In the Escobar group, spinal deformity involved 7 to 13 vertebrae and ranged from no vertebral anomalies in 3 patients to multiple segmentation defects in 6 patients. Mean age at first surgery was 5 years (range, 1.4 to 7.8 y) with a mean follow-up of 7.5 years (range, 4.0 to 10 y). Mean major curve improved from 76 degrees at initial presentation, to 43 degrees at first instrumentation, to 37 degrees at final follow-up (both P
CONCLUSIONS: Multiple congenital fusions and spinal curve deformity are common in Escobar syndrome. Despite large areas of congenital fusion, growth-friendly constructs facilitate spinal growth and improve curve correction. These results are comparable to those in idiopathic EOS.
LEVEL OF EVIDENCE: Level III-case-control study.
Journal Title
Journal of pediatric orthopedics
Volume
39
Issue
7
First Page
506
Last Page
506
MeSH Keywords
Abnormalities, Multiple; Case-Control Studies; Child; Child, Preschool; Female; Humans; Male; Malignant Hyperthermia; Orthopedic Procedures; Pediatrics; Retrospective Studies; Skin Abnormalities; Spinal Curvatures; Treatment Outcome
Keywords
Abnormalities, Multiple; Case-Control Studies; Child; Child, Preschool; Female; Humans; Male; Malignant Hyperthermia; Orthopedic Procedures; Pediatrics; Retrospective Studies; Skin Abnormalities; Spinal Curvatures; Treatment Outcome
Recommended Citation
Margalit, A., Sponseller, P. D., McCarthy, R. E., Pawelek, J. B., McCullough, L., Karlin, L. I., Shirley, E., Schwend, R. M., Samdani, A. F., Akbarnia, B. A., . Growth-Friendly Spine Surgery in Escobar Syndrome. Journal of pediatric orthopedics 39, 506-506 (2019).
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