Early-onset scoliosis: a narrative review

Overview

This narrative review, published in EFORT Open Reviews in 2022, summarizes current knowledge on early-onset scoliosis (EOS). EOS is defined as any spinal deformity present before the age of ten, regardless of cause. The authors stress that the deformity must be assessed together with the lungs and thorax, because curves that start early carry a higher risk of progression, cardiorespiratory problems and increased morbidity and mortality.

Causes and classification

EOS is grouped into four etiologies: idiopathic, neuromuscular, congenital (structural vertebral defects) and syndromic. A standardized classification combines age with etiology, major curve size, kyphosis and an optional modifier for curve progression. It shows good reliability, although parts of it are based on expert opinion and still need validation.

Why early onset matters

The spine, rib cage and lungs grow rapidly and in step during the first decade of life, and alveolar development is most active in early childhood. A progressive curve in this period can restrict the chest and lead to thoracic insufficiency syndrome, in which a small, stiff, distorted thorax cannot support normal breathing or lung growth. Its diagnosis relies on combined clinical, radiological and respiratory assessment. EOS also carries a heavy psychological burden, and repeated surgery in childhood is linked with emotional and behavioral problems, so the authors call for a holistic approach that includes mental health support.

Treatment options

Early spinal fusion has largely given way to growth-friendly treatment. Non-surgical options include serial elongation-derotation-flexion casting, which can correct curves in young children who are referred early, and bracing, whose role is still debated. Surgical techniques are distraction-based (traditional growing rods, rib-based devices and magnetically controlled growing rods), compression-based (vertebral body stapling and tethering) or guided-growth systems (Luque trolley and Shilla). Vertebral column or hemivertebra resection and convex hemiepiphysiodesis keep a role in selected congenital cases. All surgical methods carry substantial complication and unplanned revision rates, and growth-friendly techniques have not met initial expectations for correcting the deformity and improving lung function.

Future directions

The authors highlight the need for better tools to measure pulmonary outcomes, multicenter research through dedicated study groups and wider use of the EOS quality-of-life questionnaire to assess the impact of each treatment.