Abstract. 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. Purpose. Intracanal rib head penetration is a well-known entity in dystrophic scoliotic curves in neurofibromatosis type 1. There is potential for spinal cord injury if this is not recognised and managed appropriately. No current CT-based classification system is currently in use to quantify rib head penetration. This study aims to propose and evaluate a novel CT-based classification for rib head penetration primarily for neurofibromatosis but which can also be utilised in other conditions of rib head penetration. Materials and methods. The grading was developed as four grades: normal rib head (RH) position—Grade 0, subluxed ext-racanal RH position—Grade 1, RH at pedicle—Grade 2, intracanal RH—Grade 3. Grade 3 was further classified depending on the head position in the canal divided into thirds. Rib head penetration into proximal third (from ipsilateral side)—Grade 3A, into the middle third—Grade 3B and into the distal third—Grade 3C. Seventy-five axial CT images of Neurofibromatosis Type 1 patients in the paediatric age group were reviewed by a radiologist and a spinal surgeon independently to assess interobserver and intraobserver agreement of the novel CT classification. Agreement analysis was performed using the weighted Kappa statistic. Results. There was substantial interobserver correlation with mean Kappa score (k = 0.8, 95% CI 0.7–0.9) and near perfect intraobserver Kappa of 1.0 (95% CI 0.9–1.0) and 0.9 (95% CI 0.9–1.0) for the two readers. Conclusion. The novel CT-based classification quantifies rib head penetration which aids in management planning