Introduction and purpose: The relationship between congenital heart disease and the increased prevalence of scoliosis is well known, although the same cannot be said about the etiology of scoliosis in these patients. Although thoracotomy is often associated to scoliosis, median sternotomy has so far not been identified as an etiological agent. he purpose of the study is to determine if patients with congenital heart disease who are subjected to a median sternotomy show a higher prevalence of spine deformities. Materials and methods: A retrospective review is made of patients operated on for congenital heart disease through median sternotomy before the age of 8, assessing the development of spine deformities. Simple chest radiographs of 128 patients were studied once they reached skeletal maturity and it was observed that they presented no spinal or costal deformities before surgery. Results: The prevalence of scoliosis was 34.3%; 16 of these patients (12.5%) had curves of more than 20° and 33 (25.8%) had thoracic kyphosis of less than 20°. Patients operated on before the age of 18 months had a significantly higher risk to develop scoliosis as compared with those treated later (odds ratio: 3.48; p=0.016). The development of scoliosis was not related with the type of cardiac malformation present. Conclusions: There is a high prevalence of scoliosis in patients subjected to a median sternotomy for a congenital heart pathology. The prevalence of scoliosis increases in patients operated on at younger ages

Although previous lnks have been made between congenital heart disease (CHD) and scoliosis, the molecular mechanisms involved in this association are poorly understood. During development, it appears that embryos exhibiting spine deformations resulting in scoliosis also suffer from an array of cardiac defects. Additionally, idiopathic scoliosis in patients with CHD is thought to be a response to a physiological phenomenon such as an enlarged size or abnormal thrust of the heart. Despite the fact that molecular omics data have been accumulated that are relevant to these two independent phenotypes, there appears to be a gap in the literature of over two decades on this matter and no clear correlations of the omics data have been provided. To identify genes involved in CHD and scoliosis, we have performed an analysis of genomic annotations, functional genomics data and text mining, and derived an inferred network of 123 human genes and 175 known gene interactions. Of these, 20 genes are unique to CHD, 11 to scoliosis and 5 genes are common to both abnormalities. These genes are known to be involved in molecular signaling cascades that affect the development of the musculo-skeletal system in humans and have been associated with disorders such as the Marfan or CHARGE syndromes. Our analysis sets the basis upon which investigations of this association can be performed at the molecular level, in order to both further understand the pathology and, in the future, develop suitable therapies for CHD/idiopathic scoliosis patients

There is believed to be a correlation between congenital idiopathic scoliosis and congenital heart disease (CHD). Clinical and cardiological data was recorded for 3538 adolescents suffering from CHD. Data collected included the type of scoliosis; the direction of the curve; the Cobb angle; the number of curves and the presence or not of previous corrective cardiac surgery. Over 30% of the study group were found to suffer from scoliosis and a positive correlation with specific syndromes was also identified. The mean age of the patients was 34.0 +/− 14.0 years. The maximum Cobb angle was 107 degrees while the median was 7.6 degreees. Scoliosis was present in 37/188 (19.7%) was Eisenmenger syndrome (with R-L shunt) and 60/158= 38% with complex cardiac anatomy. There were also 20/103= 19.4% patients with univentricular (Fontan) circulation. Scoliosis was not necessarily related to previous corrective cardiac surgery, contrary to the current assumption in the literature. The hypothesis of common genetic pathway defects expressed both in cardiovascular and musculoskeletal organogenesis was raised and the TGF-beta pathway involvement is speculated

We present a novel use for an adult proximal humeral locking plate. In our case an 18-year-old female with cerebral palsy sustained a peri-prosthetic fracture of a blade plate previously inserted for a femoral osteotomy. Treatment was revision using a long proximal humeral locking plate. She had a successful outcome. We present the history and operative management. The female had a history of quadriplegic cerebral palsy, asthma, diabetes mellitus and congenital heart disease. She had a gastrostomy tube for enteral feeding. She was on nutritional supplements, baclofen, Omeprazole and movicol. She is looked after by her parents and requires a wheelchair for mobility. She is unable to communicate. Surgical History: Right adductor tenotomy, aged 11. Femoral Derotation Osteotomy & Dega Acetabular Osteotomy, aged 13. Right distal hamstring and knee capsule release, aged 14. Admitted to A&E (aged 18); unwitnessed fall. Painful, swollen, deformed thigh with crepitus. Xrays demonstrated peri- prosthetic fracture below blade plate. No specific equipment available to revise. Decision made to use PHILOS (Synthes, UK). GA, antibiotics, supine on table. Lateral approach. Plate removed after excising overgrown bone. Reduced and held. 10hole PHILOS applied. Near anatomical reduction. Secure fixation with locking screws proximally away from blade plate defect. Blood loss 800ml. 5 days in hospital. Sequential fracture clinic review. Wound healed well. Fracture healed on Xray at 11 months and discharged. To our knowledge this is the first reported use of a PHILOS plate for this specific fracture. The complexity of this case and underlying neurological disorder deemed long blade plate revision unsuitable. Fracture rates after femoral derotation osteotomies rare. 5/157 and 1/58 in the two largest studies to date. Conservative measures were the main recommendation. We have demonstrated a straightforward method for revision fixation with an excellent outcome. It would be recommended as an alternative to other surgeons in this position

STUDY DESIGN. Retrospective review of outcome of submuscular rod placement without apical fusion for the treatment of scoliotic deformities in children with severe co morbidities (ASA IV). METHODS. 6 children with progressive scoliosis (2 severe cerebral palsy, 2 congenital cyanotic heart disease, 1 Worster Drought syndrome, 1 Leigh's disease), underwent a serial and limited exposure of the lower and then the upper end of the spine, and insertion of pedicle screws, hooks and clamps. Two submuscularly rods were connected and distracted. Mean age was 13 years old, the mean preoperatively Cobb angle was 87° and the mean postoperatively Cobb angle was 62°. The mean operation time was 120 min and the peri-operative blood loss was 410 ml. Mean follow up is 15 months. RESULTS. There were no adverse peri-operative events. We anticipated late implant failure and revisions but to date only one construct has failed and revised without difficulties (clamps to pedicle screws). 1 patient complicated with increased spasms 1 year postoperatively. The benefits from this procedure were. a) the decreased peri-operative:. blood loss. operation time. respiratory stress. b) the faster patients recovery. c) the significant improvement regarding pain relief and sitting positioning. CONCLUSION. We believe that this procedure can be offered as a safe and viable alternative in selected patients who present with predicted high peri-operative mortality. To the best of our knowledge this technique is not described before