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Growth-Friendly Spine Surgery in Escobar Syndrome.

Adam Margalit¹, Paul D Sponseller¹, Richard E McCarthy²

¹Department of Orthopaedic Surgery, The Johns Hopkins Hospital, Baltimore, MD.

Journal of Pediatric Orthopedics

|January 11, 2019

Summary

Growth-friendly scoliosis treatments are effective for Escobar syndrome, showing comparable curve correction and spinal growth to idiopathic early-onset scoliosis. These findings suggest axial stiffness does not limit treatment outcomes in Escobar syndrome.

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Area of Science:

Orthopedics
Pediatric Spine Surgery
Genetics

Background:

Escobar syndrome is associated with spinal deformities, including congenital vertebral anomalies and scoliosis.
Early-onset scoliosis (EOS) in Escobar syndrome presents unique challenges due to potential axial stiffness from vertebral fusions.

Purpose of the Study:

To characterize spinal deformity in Escobar syndrome.
To evaluate the efficacy of growth-friendly treatments for scoliosis in Escobar syndrome.
To compare outcomes with idiopathic EOS to assess the impact of axial stiffness.

Main Methods:

Retrospective review of 8 patients with Escobar syndrome and EOS from multicenter databases (1990-2016).
Comparison with a matched cohort of 16 patients with idiopathic EOS.
Analysis of spinal deformity, curve correction, pelvic obliquity, and spinal growth.

Main Results:

Spinal deformity in Escobar syndrome involved 7-13 vertebrae, with vertebral anomalies in most patients.
Growth-friendly treatments significantly improved major curve (76° to 37°) and pelvic obliquity (16° to 4°).
No significant differences in curve correction or spinal growth were observed between Escobar syndrome and idiopathic EOS cohorts.

Conclusions:

Growth-friendly spinal constructs are effective for managing scoliosis in Escobar syndrome.
Comparable outcomes to idiopathic EOS suggest axial stiffness does not impede correction or growth.
These findings support the use of growth-friendly techniques in Escobar syndrome patients with spinal deformities.