Extraspinal ossifications after implantation of vertical expandable prosthetic titanium ribs (VEPTRs).

INTRODUCTION Though developed for thoracic insufficiency syndrome, the spinal growth-stimulating potential and the ease of placement of vertical expandable titanium ribs (VEPTRs) has resulted in their widespread use for early-onset spine deformity. Observation of implant-related ossifications warrants further assessment, since they may be detrimental to the function-preserving non-fusion strategy. PATIENTS AND METHODS Radiographs (obtained pre and post index procedure, and at 4-year follow-up) and the records of 65 VEPTR patients from four paediatric spine centres were analysed. Ossifications were classified as type I (at anchor points), type II (along the central part) or type III (re-ossification after thoracostomy). RESULTS The average age at the index procedure was 6.5 years (min 1, max 13.7). The most prevalent spine problem was congenital scoliosis (37) with rib fusions (34), followed by neuromuscular and syndromic deformities (13 and 8, respectively). Idiopathic and secondary scoliosis (e.g. after thoracotomy) were less frequent (3 and 4, respectively). Forty-two of the 65 (65 %) patients showed ossifications, half of which were around the anchors. Forty-five percent (15/33) without pre-existing rib fusions developed a type II ossification along the implant. Re-ossifications of thoracostomies were less frequent (5/34, 15 %). The occurrence of ossifications was not associated with patient-specific factors. CONCLUSIONS Implant-related ossifications around VEPTR are common. In contrast to harmless bone formation around anchors, ossifications around the telescopic part and the rod section are troublesome in view of their possible negative impact on chest cage compliance and spinal mobility. This potential side effect needs to be considered during implant selection, particularly in patients with originally normal thoracic and spinal anatomy.

Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival

BACKGROUND: Restrictive lung defects are associated with higher mortality in patients with acquired chronic heart failure. We investigated the prevalence of abnormal lung function, its relation to severity of underlying cardiac defect, its surgical history, and its impact on outcome across the spectrum of adult congenital heart disease. METHODS AND RESULTS: A total of 1188 patients with adult congenital heart disease (age, 33.1+/-13.1 years) undergoing lung function testing between 2000 and 2009 were included. Patients were classified according to the severity of lung dysfunction based on predicted values of forced vital capacity. Lung function was normal in 53% of patients with adult congenital heart disease, mildly impaired in 17%, and moderately to severely impaired in the remainder (30%). Moderate to severe impairment of lung function related to complexity of underlying cardiac defect, enlarged cardiothoracic ratio, previous thoracotomy/ies, body mass index, scoliosis, and diaphragm palsy. Over a median follow-up period of 6.7 years, 106 patients died. Moderate to severe impairment of lung function was an independent predictor of survival in this cohort. Patients with reduced force vital capacity of at least moderate severity had a 1.6-fold increased risk of death compared with patients with normal lung function (P=0.04). CONCLUSIONS: A reduced forced vital capacity is prevalent in patients with adult congenital heart disease; its severity relates to the complexity of the underlying heart defect, surgical history, and scoliosis. Moderate to severe impairment of lung function is an independent predictor of mortality in contemporary patients with adult congenital heart disease.

Vascularised fibula grafts for early tibia reconstruction in infants with congenital pseudarthrosis

Congenital pseudarthrosis of the tibia (CPT) is caused by an ill-defined, segmental disturbance of periosteal bone formation leading to spontaneous bowing, followed by fracture and subsequent pseudarthrosis in the first 2 years of life. The results of conventional treatment modalities (e.g., bracing, internal and external fixation and bone grafting) are associated with high failure rates in terms of persisting pseudarthrosis, malunion and impaired growth. As a more promising alternative, a more aggressive approach, including wide resection of the affected bone, reconstruction with free vascularised fibula grafts from the healthy contralateral leg and stable external fixation at a very early stage has been suggested. Between 1995 and 2007, 10 children (age 12-31 months, median 20 months) suffering from CPT were treated at our institutions according to this principle. Two patients were treated before a fracture had occurred. The length of the fibula graft was 7-9cm. End-to-end anastomoses were performed at the level of the distal tibia stump. The follow-up was 80 months (median, range 12 months to 12 years). Radiologic examination at 6 weeks postoperatively showed normal bone density and structure of the transplanted fibula in all cases and osseous consolidation at 19 of the 20 graft/tibia junctions. One nonunion was sucessfully treated with bone grafting and plate osteosynthesis. Pin-tract infection occurred in three patients. Five children sustained graft fractures that were successfully treated with internal or external fixation. Two patients developed diminished growth of the affected limb or foot; all others had equal limb length and shoe size. At long-term follow-up, tibialisation of the transplant had occurred, and normal gait and physical activities were possible in all children. We conclude that in spite of a relatively high complication rate and the reluctance to perform free flap surgery in infants at this young age, the present concept may successfully prevent the imminent severe sequelae associated with CPT.

Hypokalemic rhabdomyolyisis in congenital tubular disorders: a case series and a systematic review

Hypokalemia is a recognized cause of rhabdomyolysis but very few reports document its association with inborn renal tubular disorders. We report our experience with hypokalemic rhabdomyolysis in 5 pediatric patients affected by inborn renal tubular disorders and the results of a careful review of the literature disclosing 9 further cases for a total of 14 patients (8 male and 6 female subjects, aged between 1.6 and 46, median 16 years). The inborn renal tubular disorders underlying rhabdomyolysis were classic distal renal tubular acidosis (n = 7), Gitelman syndrome (n = 5), classic Bartter syndrome (n = 1), and antenatal Bartter syndrome (n = 1). In 8 patients rhabdomyolysis followed an acute intestinal disease, an upper respiratory illness or the discontinuation of regular medication. Five patients experienced two or more episodes of rhabdomyolysis. In 10 patients the underlying renal tubular disorder was recognized concurrently with the episode of rhabdomyolysis or some weeks later. In conclusion some congenital renal tubular disorders predispose to hypokalemic rhabdomyolysis. Prevention of discontinuation of regular medication and electrolyte repair in the context of acute intercurrent illnesses might avoid the development of hypokalemic rhabdomyolysis.

Coronal plane segmental flexibility in thoracic adolescent idiopathic scoliosis assessed by fulcrum-bending radiographs

Knowledge about segmental flexibility in adolescent idiopathic scoliosis is crucial for a better biomechanical understanding, particularly for the development of fusionless, growth-guiding techniques. Currently, there is lack of data in this field. The objective of this study was, therefore, to compute segmental flexibility indices (standing angle minus corrected angle/standing angle). We compared segmental disc angles in 76 preoperative sets of standing and fulcrum-bending radiographs of thoracic curves (paired, two-tailed t tests, p < 0.05). The mean standing Cobb angle was 59.7 degrees (range 41.3 degrees -95 degrees ) and the flexibility index of the curve was 48.6\% (range 16.6-78.8\%). The disc angles showed symmetric periapical distribution with significant decrease (all p values <0.0001) for every cephalad (+) and caudad (-) level change. The periapical levels +1 and -1 wedged at 8.3 degrees and 8.7 degrees (range 3.5 degrees -14.8 degrees ), respectively. All angles were significantly smaller on the-bending views (p values <0.0001). We noted mean periapical flexibility indices of 46\% (+1), 49\% (-1), 57\% (+2) and 81\% (-2), which were significantly less (p < 0.001) than for the group of remote levels 105\% (+3), 149\% (-3), 231\% (+4) and 300\% (-4). The discal and bony wedging was 60 and 40\%, respectively, and mean values 35 degrees and 24 degrees (p < 0.0001). Their relationship with the Cobb angle showed a moderate correlation (r = 0.56 and 0.45). Functional, radiographic analysis of idiopathic thoracic scoliosis revealed significant, homogenous segmental tethering confined to four periapical levels. Future research will aim at in vivo segmental measurements in three planes under defined load to provide in-depth data for novel therapeutic strategies.

Scoliosis surgery: correction not correlated with instrumentation, quality of life not correlated with correction or instrumentation

The authors conducted a retrospective study on 24 consecutive adolescent scoliosis patients, 11 of whom were instrumented with hooks and 13 with hooks and screws (hybrid technique). The mean preoperative Cobb angle was 62.2 degrees (range: 48 degrees-96 degrees). The mean correction of the primary curve was 56.6% at followup after +/- 1.18 years ; there was no statistically significant difference between groups. Special attention was given to the postoperative quality of life (QOL) by means of the following scores: COMI patient self-assessment, SF-36, ODI, and VAS. Again, there was no statistical difference between groups but, interestingly, there was no correlation between QOL and degree of correction, after a follow-up period of +/- 2.1 years. Nevertheless, on the COMI patient self-assessment, there was a high level of satisfaction with treatment. Further studies should concentrate on how to achieve a high QOL, and abandon the best possible correction as a primary endpoint of success.

Right ventricular absolute myocardial blood flow in complex congenital heart disease

A consequence in patients with d-transposition of the great arteries (d-TGA) and tetralogy of Fallot (TOF) is right ventricular hypertrophy (RVH) and right ventricular failure. Myocardial contrast echocardiography (MCE) permits the determination of the myocardial microvascular density reflected by the relative myocardial blood volume (rBV; ml/ml). This study was conducted to elucidate the relationship between RVH and myocardial microvascular changes by quantitative MCE in patients with d-TGA and TOF.

Cerebrovascular accidents in adult patients with congenital heart disease

To investigate the prevalence and characteristics of cerebrovascular accidents (CVA) in a large population of adults with congenital heart disease (CHD).

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: complex congenital cardiac lesions

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmenger's syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: executive summary

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure, and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death.Medical aspects that need to be considered relate to the long-term and multisystemic effects of single-ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the understanding of the late outcomes, genetics, medical therapy and interventional approaches in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. The present executive summary is a brief overview of the new guidelines and includes the recommendations for interventions. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology, including sections on genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy and contraception risks, and follow-up requirements. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.