Surgical Treatment of Severe Early Onset Scoliosis (Idiopatic and Associated with Rare Diseases)

Tiziana Greggi*

Received: August 29, 2020   Published: September 10, 2020

DOI: 10.32474/SCSOAJ.2020.05.000222

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Abstract

Background: Early-onset scoliosis (EOS) is defined as a spinal deformity which occurs clinically before the age of 5 years. It can be divided into idiopathic, neuromuscular/syndromic, and congenital etiologies. Many patients may have congenital spinal malformations. Others have severe scoliosis, rapidly evolving , which occurs early as a phenotypic feature of rare diseases with major comorbidities or serious health problems. Treatment of non-idiopatic spine deformities in young children is very demanding. Rare syndromes are various clinical conditions, heterogeneous in terms of clinics but associated with spinal deformities in a large percentage of cases. Scoliosis is the most common early onset deformity, sometimes present at birth and often rapidly evolving.

Methods: The literature on the treatment of EOS was summarized, in addition to our experience.

Results: Conventional management tools, such as bracing and fusion, are not always effective. The natural syndromic scoliosis is highly dependent on the natural history of the underlying disorder. Scoliosis often involves the life of children early, creating situations of discomfort and isolation, making it difficult to treat the problem and sometimes opening the way to difficult and ineffective paths.

Conclusion: While lack of treatment has been shown to lead to increased mortality, extensive early definitive fusion may lead to thoracic insufficiency. There are a number of surgical techniques to treat severe and developmental spine deformities (eos) without the need for fusion to control growth and delay definitive surgery with fusion. Delaying definitive surgery and the use of growing instrumentation may provide benefit in maintaining pulmonary health.The genetic and molecular study of syndromic diseases associated with rapidly evolving scoliosis may lead to early diagnosis. Even the study of idiopathic scoliosis (certainly of a polygenic nature) will help to deal with the pre-symptomatic situation and better control the severity of the evolution.

Clinical relevance: Multidisciplinary collaboration with translational research will bring more specialized fields closer to new knowledge on on severe early onset scoliosisis associated with rare diseases.

Abstract| Introduction| The Treatment| Vertebral Fusion| Conclusion| References|