Does rigid spinal orthosis carry more psychosocial implications than the flexible brace in AIS patients? A cross-sectional study.

BACKGROUND: A small body of data exists concerning psychosocial issues in adolescent idiopathic scoliosis (AIS) subjected to soft braces. No study was yet performed on the detailed psychosocial implications in AIS patients. OBJECTIVE: To compare the psychosocial implications of the flexible SpineCor with the Cheneau orthosis in AIS females. METHODS: Patients (aged 10-18 years) deliberately assigned to undergo SpineCor (SC group, 30 patients) or Cheneau brace (Ch group, 41 patients) intervention completed the Scoliosis Research Society-22 (SRS-22), the Spinal Appearance Questionnaire (SAQ), the Brace Questionnaire (BrQ) and the Pediatric Outcomes Data Collection Instrument (PODCI). RESULTS: Concerning the BrQ, study groups differ in regards to emotional functioning (p= 0.014), vitality (p= 0.022) and social functioning (p= 0.048), indicating better functioning in the Ch group. Considering the SAQ, the Ch group assesses body curve (p= 0.024) less critically. Regarding the PODCI, the Ch group scored higher in the Global Functioning Scale (p= 0.023), the Upper Extremity and Physical Function Scale (p= 0.000), the Transfer and Basic Mobility Scale (p= 0.088), the Pain/Comfort Scale (p= 0.009) and the Happiness Scale (p= 0.022). CONCLUSIONS: This study shows that patients treated with the rigid brace assess their vitality, physical function, emotional and social functioning better and are less critical towards body curve, in comparison to patients treated with the SpineCor.

Missense splice variant (g.20746A>G, p.Ile183Val) of interferon gamma receptor 1 (IFNGR1) coincidental with mycobacterial osteomyelitis - a screen of osteoarticular lesions.

Previously, dominant partial interferon-gamma receptor 1 (IFN-g-R1) susceptibility to environmental mycobacteria was found with IFNGR1 deletions or premature stop. Our aim was to search for IFNGR1 variants in patients with mycobacterial osteoarticular lesions. Biopsies from the patients were examined for acid-fast bacilli, inflammatory cell infiltration, and mycobacterial niacin. Mycobacterial rRNA was analyzed using a target-amplified rRNA probe test. Peripheral-blood-leukocyte genomic DNA was isolated from 19 patients using the QIAamp DNA Mini Kit, and all IFNGR1 exons were sequenced using an ABIPRISM 3130 device. After the discovery of an exon 5 variant, a Polish newborn population sample (n = 100) was assayed for the discovered variant. Splice sites and putative amino acid interactions were analyzed. All patients tested were positive for mycobacteria; one was heterozygous for the IFNGR1 exon 5 single-nucleotide-missense substitution (g.20746A>G, p.Ile183Val). No other variant was found. The splice analysis indicated the creation of an exonic splicing silencer, and alternatively, molecular graphics indicated that the p.Ile183Val might alter beta-strand packing (loss of van der Waals contacts; Val183/Pro205), possibly altering the IFN-g-R1/IFN-g-R2 interaction. The probability of non-deleterious variant was estimated as <10%. Heterozygous IFNGR1:p.Ile183Val (frequency 0.003%) was found to be coincidental with mycobacterial osteomyelitis. The small amount of variation detected in the patients with osteoarticular lesions indicates that screens should not yet be restricted: Intronic variants should be analyzed as well as the other genes affecting Type 1 T-helper-cell-mediated immunity.

[Patients functioning after surgical treatment for Perthes disease]. / Funkcjonowanie pacjentów po przebytej chorobie perthesa leczonych operacyjnie.

INTRODUCTION: Perthes disease appears in children and affects hip joints. The proximal head of the femur is damaged, which results in deformation of the femoral ball and loss of its spherical shape. Instant diagnosis and introduction of treatment is crucial for maintaining the femoral head in the hip socket, which facilitates recovery to its spherical shape. To assess the quality of life of patients who have undergone surgical treatment. The factors examined were: state of functioning and occurrence of pain in affected hip joint. MATERIAL AND METHODS: 61 participants underwent the examination. The duration and type of disease were established on the basis of a radiogram. The patients were treated with varus derotation osteotomy in the 2 and 3 degree of the Reinberg scale. A Modified Oxford Hip Score was used to assess the patients' quality of life. RESULTS: Subjective assessment of the quality of life after recovering from the disease was regarded as very good and good. CONCLUSION: According to the patients' own evaluation the disease and the treatment did not affect their everyday life.

Results of SpineCor dynamic bracing for idiopathic scoliosis.

BACKGROUND: Orthopedic bracing is used in conservative treatment of spinal curvatures. Apart from rigid braces, SpineCor dynamic braces with a flexible design have recently become available. The idea behind dynamic bracing is that derotational and correcting forces are transmitted via a system of corrective bands. The essence of this technique is maintenance of spine mobility while effecting a position in which all components of the three-plane deformity are corrected.The aim of this study is to evaluate early outcomes of SpineCor dynamic brace treatment for idiopathic scoliosis according to SRS methodology and criteria. MATERIAL AND METHODS: The study group included 50 patients who were using SpineCor braces due to idiopathic scoliosis. The indication for bracing was the finding of a >15° spinal curvature in skeletally immature patients (Risser grade 0-3). Correction or stabilization of the scoliosis (Cobb angle change of ±5°) were recognized as positive outcomes, while a negative outcome was defined as progression of the curve of more than 5° or to a value necessitating operative treatment. The study group was divided in to subgroups at enrollment, according to gender and degree of scoliosis. RESULTS: In the entire study group, correction was demonstrated in 24 patients (48%), stabilization in 14 (28%) and progression in spite of bracing occurred in 7 patients (14%). Five patients in the entire study group (10%) required operative treatment due to rapid curvature progression. CONCLUSIONS: SpineCor bracing leads to stabilization of scoliosis in the majority of the patients. Introducing the SpineCor brace in patients with a scoliosis angle over 20° and Risser grade 0-3 very effectively prevented curve progression.

The influence of the degree of spine curvature on the quality of life assessment among patients treated surgically with the Cotrel-Dubousset method.

UNLABELLED: Physical appearance and psychomotor ability rank high in the hierarchy of values accepted by children and teenagers. Therefore Idiopathic Scoliosis (IS), which is accompanied by the dysfunction of locomotive organ and often requires troublesome treatment, may lead to poor body image and frustration. Other common effects are a low self-esteem, identity problems and depressive symptoms. METHOD: Prospective examination was done in two independent groups of IS patients treated in one of Szczecin's clinic. The first group of 35 patients was examined twice: after being selected for surgery and 12 months after surgery. The second group of 52 patients was examined after a minimum of two years from operation. Research tools consisted of: personal data questionnaire, the assessment form of the clinical state and the Scoliosis Research Society (SRS)-22. The results were statistically analyzed using STATISTICA 5 software. Pearson's linear correlation coefficients and Spearman's rank correlation coefficients were calculated to determine the relationship between the pairs of variables. RESULTS/FINDINGS: Preoperatively, the Cobb angle of thoracic curves was found to significantly correlate only with the Physical Activity (rs = -0.41, p<0.02) and Self-Image (rs = - 0.51, p<0.002). Postoperatively, a positive correlation with statistical significance was observed only with correction values in thoracic section with total scores of the SRS-22 (p<0.03) in the 24-month period. CONCLUSIONS: 1. Clinical assessment appears insufficient to decide how advanced the disease is and what the treatment results are. 2. The degree of spinal curvature, especially in the postoperative period, have little effect on quality of patients' lives. 3. Studies with the use of the SRS-22 HRQL instrument suggest that other non-clinical factors may affect the quality of life assessment.

Familial occurrence of Caffey-Silverman syndrome.

Caffey-Silverman syndrome, or infantile hyperostosis, is a rare condition of unclear etiology and pathogenesis affecting the skeletal system and the surrounding soft tissues. It is characterized by indurated swelling of soft tissues and cortical bone hyperostosis. The changes are usually multiple and affect such parts as the mandible, scapulae, ribs, clavicles, and forearm and shank bones. When long bones are affected, the lesions are typically limited to shafts, with the sparing of meta- and epiphyses. The prognosis is usually good and, in most patients, the changes resolve spontaneously after several months to over a year, leaving no permanent sequelae. Caffey-Silverman syndrome needs to be distinguished from osteitis, for which it is most often mistaken. We present a case of an early form of Caffey-Silverman syndrome. The course of disease in this form is usually severe, with multifocal lesions, and the typical self-limiting regression is not complete. In our patient, a systemic musculoskeletal condition was already suspected following the detection of skeletal defects in a prenatal US examination. Physical and radiological work-up in the first weeks of life revealed the typical signs of congenital Caffey-Silverman syndrome. Several years of follow-up showed gradual regression of the bone deformities with persistent lower-extremity bowing. Due to a positive family history for Caffey-Silverman syndrome, the patient's pedigree was prepared. On the basis of the history data, existing radiographs and in- and outpatient medical records, Caffey-Silverman syndrome was confirmed in 10 family members. It was established that the cases of Caffey-Silverman syndrome in the patient's family were characterized by diverse phenotypic expression and different times of onset.

Assessment of quality of life in patients with idiopathic scoliosis treated operatively.

BACKGROUND: A small percentage of patients with idiopathic scoliosis undergo surgery, which is only used in the case of the most extreme deformities of the spine and chest and progression of disease despite conservative treatment. The aim of this study was to assess the quality of life in patients with idiopathic scoliosis treated operatively and to examine the correlations between the quality of life and radiographic and socio-demographic indices. MATERIAL AND METHODS: wo independent groups of patients operated on at the Department of Pediatric Orthopedics of the Pomeranian Medical University in Szczecin were examined using a standardized SRS -22 HRQL instrument. Quality of life was assessed on three occasions in 35 patients from the first group. The second group included 52 patients at least two years post-surgery. RESULTS: The assessment of physical fitness revealed a decrease proportional to the length of time since surgery. Higher pain intensity was reported by patients whose surgery took place more than 2 years prior to the study. The analysis of mental health assessment results indicated a decrease proportional to the length of time since surgery. No statistically significant differences were found with respect to patients' self-assessment of their body image and treatment satisfaction. CONCLUSIONS: 1. Operative correction of scoliosis enhances patients' self-assessment of their body image and contributes to a high assessment score for treatment satisfaction. 2. No correlations were found between the overall quality of life and previous conservative treatment. 3. Deterioration of physical fitness, mental health impairment and increasing pain intensity were observed among patients residing in small towns and rural areas and those experiencing socioeconomic problems 4. The degree of scoliosis correction in the thoracic spine contributes to increasing life quality, especially in late assessment.

Conservative management of idiopathic scoliosis--guidelines based on SOSORT 2006 Consensus.

BACKGROUND: Idiopathic scoliosis, defined as a lateral curvature of the spine of above 10 degrees (Cobb angle), is seen in 2-3% of the growing age population, while curves above 20 degrees , requiring conservative treatment, are found in 0.3-0.5%. In our observation, both under-treatment of progressive curves and over-treatment of stable cases are common during conservative management of scoliosis. MATERIAL AND METHODS: A model of therapeutic management is presented based on the experience of Polish clinicians specialising in the treatment of scoliosis as well as the effects of work of a panel of experts of SOSORT (Society on Scoliosis Orthopaedic and Rehabilitation Treatment). The model comprises the indications for conservative treatment according to age, curve type and size and Risser grading. The aetiology, classifications, usefulness of the Lonstein and Carlson factor of progression and other methods of determining the probability of scoliosis progression, as well as the psychological aspects of conservative management are presented. RESULTS: Based on the knowledge of the natural history of idiopathic scoliosis, factors of progression and on the SOSORT experts' opinion, guidelines are proposed for clinicians treating children and adolescents with idiopathic scoliosis, including the timing and course of brace treatment and the types of exercises. CONCLUSIONS: Uniform practical guidelines developed by experts may represent an essential step towards establishing standards of conservative scoliosis care in our country.

Results of SpineCor dynamic bracing for idiopathic scoliosis.

BACKGROUND: Orthopaedic bracing is used in conservative treatment of spinal curvatures. Apart from rigid braces, SpineCor dynamic braces with a flexible design have recently become available. The idea behind dynamic bracing is that derotational and correcting forces are transmitted via a system of corrective bands. The essence of this technique is maintenance of spine mobility while effecting a position in which all components of the three-plane deformity are corrected. The aim of this study is to evaluate early outcomes of SpineCor dynamic brace treatment for idiopathic scoliosis according to SRS methodology and criteria. MATERIAL AND METHODS: The study group included 50 patients who were using SpineCor braces due to idiopathic scoliosis. The indication for bracing was the finding of a >15(5) spinal curvature in skeletally immature patients (Risser grade 0-3). Correction or stabilization of the scoliosis (Cobb angle change of +/- 5 degrees ) were recognised as positive outcomes, while a negative outcome was defined as progression of the curve of more than 5 degrees or to a value necessitating operative treatment. The study group was divided into subgroups at enrollment, according to gender and degree of scoliosis. RESULTS: In the entire study group, correction was demonstrated in 24 patients (48%), stabilization in 14 (28%) and progression in spite of bracing occurred in 7 patients (14%). Five patients in the entire study group (10%) required operative treatment due to rapid curvature progression. CONCLUSIONS: SpineCor bracing led to stabilization of scoliosis in the majority of the patients. Introducing the SpineCor brace in patients with a scoliosis angle over 20 degrees and Risser grade 0-3 very effectively prevented curve progression.

Recombinant coagulation factor VIIa--a novel haemostatic agent in scoliosis surgery?

Spinal fusion surgery in children and adolescents with idiopathic scoliosis is often associated with severe haemorrhage. Recombinant coagulation factor VIIa (rFVIIa) has previously been shown to be an effective haemostatic treatment for severe bleeding associated with a variety of coagulopathic and non-coagulopathic indications. The aim of this retrospective study was to assess the safety and haemostatic efficacy of rFVIIa in a series of 26 consecutive adolescent patients with scoliosis (22 females; mean age 16.6 years) undergoing correctional surgery. A second series of 26 consecutive patients (20 females; mean age 16.2 years) who received standard therapy during surgery, represented historical controls. Blood loss, transfusion requirements, duration of surgery, and peri-operative measurements of coagulation parameters were compared between the two groups. Intra-operative and combined intra-operative and post-operative blood losses were significantly smaller in the rFVIIa-treatment group than in the historical controls (P=0.003 and 0.032, respectively); rFVIIa-treated patients also demonstrated significantly reduced blood loss per vertebral segment fused (P=0.032) and per hour of surgery (P<0.001). Intra-operative requirements for packed red blood cells were also significantly lower in the treatment group (P=0.042). Patients in the treatment group demonstrated rapid and maintained reduction of prothrombin time and international normalised ratio; values among rFVIIa-treated patients remained significantly lower than those in the control group at all time points evaluated (P<0.001). There were no deaths and no adverse events. These results suggest that rFVIIa is a safe and effective haemostatic agent for use during spinal fusion surgery in adolescent patients with idiopathic scoliosis; however, further research and randomised, placebo-controlled trials are needed to confirm these findings.

The use of the Ilizarov technique in the treatment of upper limb deformity in patients with Ollier's disease.

Between 1997 and 2001 three children with Ollier's disease underwent treatment of five upper limb segments using the Ilizarov technique. Average length discrepancy was 8.4 cm in the arm and 4.5 cm in the forearm, but coexisting large angular deformities were the major problem in all the children. Full correction of the axial deviations was achieved in all children. Restoration of length was achieved in all arms, but residual forearm length discrepancy persisted. In four segments conversion of the abnormal cartilage into normal regenerate was observed on radiograms. The problems, obstacles, and complications are similar to those met in more usual lengthening procedures. The Ilizarov technique should be the treatment of choice in restoring the correct axis and length of a limb in patients with Ollier's disease.

[Treatment results of triplanar correction of idiopathic scoliosis in relation to curve pattern and degree of scoliosis]. / Wyniki leczenia skoliozy idiopatycznej metoda trojplaszczyznowej korekcji w zaleznosci od typu i wielkosci znieksztalcenia.

Background. The King classification enables the surgeon to assess the curve pattern of scoliosis which determines the choice of operative technique as well as the extent of spinal fusion. The final outcome of operative treatment depends on the curve pattern and the degree of scoliosis. Material and methods. The aim of the study was to assess the relation between the correction of the deformity and the curve pattern. In this retrospective study, 97 patients were included who had undergone scoliosis surgery between 1999 and 2001, and for whom the follow-up period was at least 36 months. 12 patients were classified as type I according to the King classification, 26 as type II, 49 as type III, and 5 each as type IV and V. The mean Cobb angle in the whole group was 58 degrees in the thoracic region and 38 degrees in the lumbar region, while in the sagittal plane there was kyphosis of the thoraco-lumbar region of 3 degrees . Results. Postoperative values were as follows: thoracic region - 30.2 degrees , lumbar region - 27.6 degrees and thoraco-lumbar region - lordosis of 4 degrees . Conclusions. The Cotrel-Dubousset method makes it possible to achieve an average correction of 65% of the original deformity, the best result being achieved for type IV and V. Operative treatment should be applied to patients with scoliosis of an angle below 60 degrees , which enables compensation of trunk transposition.

[The use of recombinant coagulation factor VIIa in patients undergoing surgical correction of scoliosis with the C-D method]. / Zastosowani rekombinowanego czynnika krzepniecia VIIa u pacjentow z operacyjna korekcja skoliozy metoda C-D.

Background. Excessive intraoperative bleeding during scoliosis correction extends surgery time, and may increase the risk of complications during surgery and the postoperative period. Recombinant factor VIIa (rVIIa) is widely used to control hemorrhage in patients with hemophilia. Recent reports have examined the use of rVIIa for intraoperative bleeding in patients without coagulopathies. The aim of our study was to evaluate the influence of rVIIa on selected coagulation parameters and blood loss in patients undergoing surgical correction of scoliosis. Material and methods. 62 patients undergoing surgical correction of scoliosis were included in the study. The treatment group consisted of 29 patients who received rVIIa (20 g/kg). The coagulation parameters were evaluated at 15 minutes, 2 hours, 4 hours and 12 hours after the start of surgery. The control group consisted of 33 patients who underwent the same surgery without pharmacological blood loss control. Results. A single administration of rVIIa resulted in a reduction of prothrombin (PT) time and APTT. The effect was observed until the 4th hour of surgery, after which time the value of coagulation parameters increased and reached preoperative values after 12 hours. The volume of blood loss in the treatment group was 1046 ml, compared to 1393 ml in the control group. Conclusions. The use of small doses of rVIIa in healthy patients resulted in rapid thrombin activation at places of tissue damage caused by surgery. During extensive scoliosis surgery, the effect of rVIIa on coagulation process led to a reduction of the time spent in surgery.

Diagnosis of syringomyelia and Chiari malformations in patients with scoliosis.

Background. Syryngomyelia was first described by Olliver in 1824. Burwell and Williams reported a 50% incidence of scoliosis in patients with syringomyelia. Malformations of the central nervous system among patients with scoliosis are very difficult to diagnose on the basis of clinical and radiological examination. MRI examination is a useful tool in diagnosing neurogenic malformations. The aim of this study is to evaluate the prevalence of neurogenic malformations in a group of patients with scoliosis, and the evaluation of shared clinical symptoms in this group. Material and methods. Clinical, radiological and MRI assessment was performed on a group of 323 patients referred to the Clinic due to scoliosis with a Cobb angle > 20. Results. Four patients were diagnosed with syringomyelia, 1 had a Chiari malformation type I and 1 female patient aged 14 was diagnosed with tethered cord. Conclusions. In the group, the prevalence of neurogenic malformation was 1.9%. MRI examination in patients with scoliosis seems to be justified in the following cases: clinical findings suggests a neurogenic background of spine deformation; scoliosis with onset before 8 years of age; prior to the planned surgical correction of scoliosis.

Remodelling and congruency of the hip joint in children with Perthes' disease treated with varus-derotation subtrochanteric osteotomy.

Background. Dislocation of the femoral head to the front and to the side during the course of Perthes' disease is the main threat to the development of the hip. Containment of the femoral head into the acetabulum is the main goal of conservative and operative treatment. The spherical shape of the acetabulum is used as the modelling factor for the femoral head during the regeneration period, which enables the femoral head to rebuild its spherical shape. The aim of the study is to present the changes occurring within the hip joint during the course of Perthes' disease, before and after operative treatment with varus-derotation subtrochanteric osteotomy, based on objective radiological measurements. Material and methods. 53 patients, aged from 5 to 10 years, were treated operatively. The necrosis period was assessed on radiographic examination according to Reiberg and Catterall. All patients were treated with varus-derotation subtrochanteric osteotomy, decreasing the neck-shaft angle to an average value of 111 degrees . During the follow-up period of 6 years, a control examination was performed every 12 months. Measurements were compared with norms published in the literature. Various parameters were assessed in this study to assess the proximal end of the femur bone (neck-shaft angle, epiphyseal head index of Heyman and Herndon) and the position of the femoral head inside the acetabulum (centre-edge angle, distance angle, acetabular head index of Heyman-Herndon). Results. During the 6-year follow-up period, the value of the neck-shaft angle remained within the range of norms for the age and group, and for operated limbs the value was lower than the norm. The other assessed parameters showed substantial improvement, which led to the preservation of the correct shape of both the femoral head and acetabulum and improved the congruency of the joint. Conclusion. The value of parameters assessed post-operatively show a substantial improvement compared with preoperative measurements. Good results are obtained as a result of varus-derotation subtrochanteric osteotomy in patients with Perthes' disease type III and IV according to Catterall, and lead to improvement in the congruency of the joint.

Variability of the anteversion angle in children with Perthes' disease treated with varus-derotation subtrochanteric osteotomy.

Background. Despite extensive research, it is still unknown whether a 40% increase in the anteversion angle value is a primary or a secondary change in Perthes' disease. The aim of the study was to evaluate the variability of the anteversion angle in children with unilateral Perthes disease treated operatively with varus and derotation osteotomy. Material and methods. 53 patients between 5 and 10 years were included in this study. The stage and type of necrosis were classified using radiological examination according to the Reiberg and Cattarall classification. The value of anteversion was determined using clinical and ultrasound examination in addition to directly during surgery. After the operation, the anteversion angle was 11 degrees . During a 6-year follow-up period, ultrasound examination was performed every 12 months. Angle values obtained through direct measurements were compared with the norms published by Lanz and Wachsmuth, and ultrasound measurements were compared with the norms for the age groups. Results. 47 patients were found to be in stage III of the disease and 6 were in stage II. During the period up to 36 months after surgery, the value of the anteversion angle gradually reached the same value as in a limb considered as "healthy". During the following 36 months these values remained unchanged. Conclusions. An increased value of the anteversion angle in children with Perthes' disease is observed in a limb considered as "healthy". An increased value of the anteversion angle in children with Perthes' disease is a secondary deformation caused by the arrest of the physiological decrease of its value during development.

Assessment of Synvisc treatment in osteoarthritis.

Background. Viscosupplementation is a relatively new method for the treatment of osteoarthritis (OA). The main goal of this project was to assess the safety and clinical utility of Hylan G-F20 (Synvisc(R)) in the treatment of pain associated with osteoarthritis of the knee. The type and frequency of additional therapies used during Synvisc(R) treatment were also assessed. It was a prospective project designed for monitoring Synvisc(R)-prescribing habits in usual medical care.
Material and method. One hundred ninety knee joints in 187 patients were studied (OA symptoms were bilateral in 3 women). Synvisc(R) was indicated for the local treatment of pain in osteoarthritis of the knee. After the diagnosis, Synvisc(R) therapy was started at the recommended dose of 2 ml per intra-articular injection once
a week (at 1-week intervals), three injections in total. The data collected, including medical history, physical examination, radiographic examination and treatment efficacy (overall assessment performed at each visit), were recorded on case report forms designed to facilitate statistical analysis. The physicians completed visual Analogue Scale (VAS) for overall assessment of OA pain at each visit. The clinical outcome was recorded after the end of therapy. The patient data were collected by physicians taking part in this project only.
Results. In 156 cases (82.1%) either a substantial improvement or subsidence of symptoms was observed. In 34 cases (17.9%) the improvement was small or there was no change in the patient's condition. There were local adverse reactions in 2 patients (1.07%). There was no need to cease the treatment, to hospitalise a patient or to start any additional treatment. In 167 patients (89.3%) there was no need to start any non-pharmacological concomitant treatment.
Conclusions. In summary, Synvisc(R) viscosupplementation should be rated among the safest and most effective methods for the treatment of OA, for it alleviates OA-related pain, thus reducing the need for NSAIDs and steroid injections. The use of Synvisc(R) in OA patients alleviates pain regardless of sex and age, the effect being the most pronounced in patients with low- and medium-grade radiographic changes.