Growth-Friendly Spine Surgery in Escobar Syndrome

Adam Margalit, Paul D. Sponseller, Richard E. McCarthy, Jeff B. Pawelek, Lynn McCullough, Lawrence I. Karlin, Eric D. Shirley, Richard M. Schwend, Amer F. Samdani, Behrooz A. Akbarnia

Research output: Contribution to journalArticlepeer-review

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<0.001). Mean pelvic obliquity improved from 16 degrees (range, 5 to 31 degrees) preoperatively to 4 degrees (range, 0 to 8 degrees) at final follow-up (P=0.005). There were no differences in the mean percentage of major curve correction between the idiopathic EOS and Escobar groups at the immediate postoperative visit (P=0.743) or final follow-up (P=0.511). There were no differences between the cohorts in T1-S1 height at initial presentation (P=0.129) or in growth per month (P=0.211).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.

Original languageEnglish (US)
Pages (from-to)E506-E513
JournalJournal of Pediatric Orthopaedics
Volume39
Issue number7
DOIs
StatePublished - Aug 1 2019

Keywords

  • Escobar syndrome
  • growth-friendly spine surgery
  • idiopathic early-onset scoliosis
  • spinal deformity

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Orthopedics and Sports Medicine

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