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

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