In the pediatric population, scoliosis is classified into congenital, syndromic, idiopathic, and neuromuscular in aetiology. Syndromic scoliosis represents a wide range of systemic anomalies associated with scoliosis. The primary challenge for a clinician is to think beyond the scoliotic curve, as the underlying pathology is multisystemic. The aim of this review is to identify the systemic anomalies, associated with syndromic scoliosis. MEDLINE, EMBASE, and CINAHL databases were searched, dating from 1990–2020, relevant to the purpose of our study. Keywords used: “scoliosis”+ “syndrome” + “genetic”. Retrospective, prospective studies were included. Case reports that had fewer than 4 patients were not included. Delineating 60 articles, we found a total of 41 syndromes to be associated with scoliosis. Thoracic region was the most common level of scoliosis curve, being noted in 28 syndromes. Mental retardation, seizures, and ataxia were the commonly noticed CNS anomalies. VSD, ASD, and TGA were the anomalies associated with CVS; Hypotonia, rib and vertebral malformations were the most identified neuromuscular anomalies; pulmonary hypoplasia, renal agenesis, and strabismus were other associations. A multidisciplinary approach, involving spinal surgeons, paediatricians, geneticists, anesthesiologists, and allied health professionals, is vital for the best care of patients with syndromic scoliosis. The location of the scoliotic curve reflects the associated anomalies, as thoracic curvature is more closely linked with cardiac anomalies, while lumbosacral curvature is seen to be often linked with genitourinary anomalies. We hope that this article provides a clear overview of the systemic associations in syndromic scoliosis and thus, facilitates and streamlines the management protocol.Abstract
Non-operative management of AIS can present practical and psychological challenges, as effective bracing requires a considerable investment of time in adolescence which is a formative point of physical and emotional development. The management team lacks input from the psychological team and thus, it would be prudent for the spinal teams to appreciate and deal with the psychosocial effects associated with bracing. We sought to investigate how bracing as a part of non-operative management of idiopathic scoliosis, is perceived among adolescents. We performed a search of CINAHL, Medline, AMED, PsychARTICLES, Psychology and Behavioral Sciences Collection and PsychINFO databases to identify qualitative research investigating the thoughts, feelings and experiences and attitudes of those undergoing bracing for AIS. Keywords used were (((“adoles∗” OR “young pe∗”) AND “idiopathic scoliosis”) AND “brac∗”) AND (“perce∗” OR “experience∗” OR “perspective∗” OR “attitude∗”). Ten research articles were identified using our search strategy. Only one article addressed our research question specifically. This reported that almost all adolescent patients experienced psychological difficulties during treatment and received most of their support from family and friends rather than health professionals. Our interpretation of the literature on this subject has yielded three recommendations for service providers. First, the policy ought to change to ensure that psycholological support is engrained within the treatment process; second, more information and advice must be given to patients and their families; and third, health professionals should appreciate and directly address in their consultations the psychological difficulties involved in brace wearing and the need for adequate support.Abstract
