Pediatric Spine Foundation

Title: Spinal Deformity in Sotos Syndrome: First Results of Growth-friendly Spine Surgery Publication Summary

Authors: Bram P. Verhofste, MD, Michael P. Glotzbecker, MD, David S. Marks, MD, Craig M. Birch, MD, Anna M. McClung, BSN, RN, Pediatric Spine Study Group, John B. Emans, MD

Journal: J Pediatr Orthop

Date: October 01, 2020

Excerpt: This is the first study to evaluate the outcomes of GFI in children with SS and EOS. Compared with published data for outcomes of GFI in EOS, children with SS may have less major curve correction. Growth-friendly surgery remains an effective treatment method for EOS in patients with SS.

Keywords: Sotos syndrome, Sotos, early-onset scoliosis, growth-friendly spine surgery, growth-friendly instrumentation, spinal fusion, outcomes, adverse events, complications

Summary:

Purpose

This study explores the use of growth-friendly spine surgery treatments in children with Sotos Syndrome. Sotos syndrome (SS) is a genetic disorder that causes a lot of growth during childhood and developmental delays.

Results

  • In 13 patients with SS and EOS, an improvement of 36% was seen in major curves, from an average of 71 degrees to an average of 46 degrees with the use of growth-friendly surgery treatments.
  • Four patients (31%) had Spinal Fusion surgery at an average age of 11.9± 4.9 years after an average of 7.5 ± 2.1 years of treatment with GFI.
  • Average GFI treatment time was 5.7 ± 2.84 years with an average of 9 ± 4.0 lengthening surgeries.

Discussion Points

  • Because of the uncommon nature of Sotos Syndrome (between 1/10,000 – 1/50,000 of people have the diagnosis), more studies are needed to better understand surgical treatment for patients with SS.
  • 9 of the patients in this study had 26 issues, or about 2 for each patient.
  • Before this study, only one child with Sotos Syndrome had been treated with GFI. Because this surgical treatment helped to stop the spinal curves from getting bigger, GFI is an valuable option for treating EOS in patients with SS.