Aims

The aim of this study was to define the complications and long-term outcome following adolescent mid-shaft clavicular fracture.

The management of spinal deformity in children with univentricular cardiac pathology poses significant challenges to the surgical and anaesthetic teams. To date, only posterior instrumented fusion techniques have been used in these children and these are associated with a high rate of complications. We reviewed our experience of both growing rod instrumentation and posterior instrumented fusion in children with a univentricular circulation.

Six children underwent spinal corrective surgery, two with cavopulmonary shunts and four following completion of a Fontan procedure. Three underwent growing rod instrumentation, two had a posterior fusion and one had spinal growth arrest. There were no complications following surgery, and the children undergoing growing rod instrumentation were successfully lengthened. We noted a trend for greater blood loss and haemodynamic instability in those whose surgery was undertaken following completion of a Fontan procedure. At a median follow-up of 87.6 months (interquartile range (IQR) 62.9 to 96.5) the median correction of deformity was 24.2% (64.5° (IQR 46° to 80°) vs 50.5° (IQR 36° to 63°)).

We believe that early surgical intervention with growing rod instrumentation systems allows staged correction of the spinal deformity and reduces the haemodynamic insult to these physiologically compromised children. Due to the haemodynamic changes that occur with the completed Fontan circulation, the initial scoliosis surgery should ideally be undertaken when in the cavopulmonary shunt stage.

Cite this article: Bone Joint J 2014;96-B:94–9.

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

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

More than a million hip replacements are carried out each year worldwide, and the number of other artificial joints inserted is also rising, so that infections associated with arthroplasties have become more common. However, there is a paucity of literature on infections due to haematogenous seeding following dental procedures. We reviewed the published literature to establish the current knowledge on this problem and to determine the evidence for routine antibiotic prophylaxis prior to a dental procedure.

We found that antimicrobial prophylaxis before dental interventions in patients with artificial joints lacks evidence-based information and thus cannot be universally recommended.

It has been reported that there is an association between Perthes’ disease and poverty. We examined the demographic data of a group of 240 children (263 hips) who presented with Perthes’ disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applied and whether other clues to the aetiology of Perthes’ disease could be found. There were 197 boys and 43 girls; 39 (16.25%) had a family history of Perthes’ disease. Bone age in this series was heavily skewed towards the lower percentiles. The mean number of siblings was 1.9, with 31 (12.9%) being an only child. Maternal age at the birth of the first child showed no preponderance of older mothers. Maternal smoking during and after pregnancy was noted in 132 (55%), which compared with the 52% reported in the population of Greater Glasgow in general. Of the children in our series, 60 (25%) were in social class IV and V. However, this applies to more than half of the population of Greater Glasgow. There was no significant evidence of a preponderance of Perthes’ disease in the most deprived groups. The aetiology of Perthes’ disease is likely to be multifactorial and may include a genetic or deprivation influence resulting in delayed bone age.

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

An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3.5 per cent of cases and congenital heart disease in 2.5 per cent; and inguinal hernia was found in 7.4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children, all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors

Since this paper was submitted for publication three additional reports have appeared, two of which represent typical examples of the syndrome. Husson and Parkman (1961) reported the case of a female infant dying at the age of four months with chondroectodermal dysplasia, obliteration of the upper labiogingival sulcus, and congenital heart disease. The heart lesions included anomalous pulmonary venous return, single atrium and a persistent left superior vena cava. There was no known consanguinity. In reviewing the cardiac lesions described in chondroectodermal dysplasia they include another case with anomalous pulmonary venous return described by Darling, Rothney and Craig (1957), which also showed chondroectodermal dysplasia and polydactyly, though details of the extracardiac malformations are not given. Nabrady (1961) described a four-year-old Hungarian girl with ectodermal defects involving the teeth and nails but not the hair, polydactyly, distal shortening of the extremities producing dwarfing, and typical radiological appearances of the long bones. There was presumptive evidence of a septal defect of the heart. There was no consanguinity, but the mother was considered to show a "forme fruste" of the condition. A necropsy report by Meitner (1961) of a newly born premature infant with multiple congenital malformations of organs of ectodermal, mesodermal and endodermal origin is of interest because these malformations included extreme polydactyly of hands and feet, absence of nails, chondrodysplasia, and trilocular heart. In many respects, however, the case is atypical of chondroectodermal dysplasia

1. Two cases are reported showing the syndrome of chondrodysplasia, manual polydactyly, ectodermal dysplasia affecting the teeth and nails, and congenital heart disease. 2. Particulars of thirty-eight cases are tabulated, and the features of the syndrome are discussed. 3. The syndrome is regarded as showing an autosomal recessive mode of inheritance