Multidisciplinary assessment of congenital insensitivity to pain syndrome.

BACKGROUND: Congenital insensitivity to pain and anhidrosis (CIPA) is a rare clinical condition characterized by the absence of normal subjective and objective responses to noxious stimuli in patients with intact central and peripheral nervous systems. CASE PRESENTATIONS: Two patients with CIPA are reported. The first patient was a 13-year-old girl who presented to our hospital with multiple joint destructions secondary to osteomyelitis. The second patient was a 10-year-old boy who presented with multiple hand lesions and right leg osteomyelitis. Our patients were treated with multiple debridements and intravenous antibiotics according to our hospital protocol. CONCLUSION: Early recognition of the disease is important. The treatment for this condition is focused more on the prevention of bone injuries and joint infection, as opposed to a cure. There are no standard techniques or guidelines available to treat this rare disease. Overall, effective CIPA treatment is built around family education and patient training.

Thrombomodulin and GFC levels in Legg-Calve-Perthes disease.

Legg-Calve-Perthes disease (LCPD) is a self-limited microvascular disorder leading to the occlusion of the femoral blood supply, which results in bone necrosis. Endothelial injury and hemostatic alterations may play a role in the microvascular compromise and decreased blood flow, which occur during the course of LCPD. Global fibrinolytic capacity (GFC) is a novel assay reflecting the overall fibrinolysis response resulting from the dynamic interactions of numerous stimulatory and inhibitory fibrinolytic molecules. Circulating soluble thrombomodulin (TM) reflects endothelial activation and/or injury. It is a cofactor in the clinically important protein C natural anticoagulant system. Beyond the coagulation pathway it is shown to have effects on biological events, especially inflammation. The aim of this study was to determine GFC and TM levels in LCPD patients. The study included 77 children in two groups. Group I consisted of 42 patients with LCPD and Group II (control) comprised 35 healthy children. Median (interquartile ratios) GFC and TM levels were significantly higher in the LCPD patients (Group I) (p<0.0001 and p=0.049, respectively). Circulating high levels of soluble TM may be associated with ongoing endothelial injury or ongoing inflammation during the disease course. Along with increased overall fibrinolytic response, increased TM may be a compensatory reaction to thrombosis. Further investigations are needed to elucidate the endothelial, anticoagulant, and fibrinolytic kinetics associated with the microvascular compromise and self-limiting nature of LCPD.

Evaluation of risk factors in developmental dysplasia of the hip: results of infantile hip ultrasonography.

In this study, risk factors of developmental dysplasia of the hip (DDH) were evaluated. History, clinical examination and risk factors for DDH of the babies were recorded. The hips were evaluated with ultrasonography. Infantile hip ultrasonography is one of the best methods for screening of DDH. Ultrasonography is easy, repeatable and provides visualization of the cartilage part of the hip joint. Graf's method of infantile hip ultrasonography was used to evaluate the hip in this study. Both hips of 371 babies and 32 unilateral hips of 32 babies were included in the study. In 403 babies, 14 (3.4%) had DDH. There were 5 type IIB, 7 type IIC, 1 type D, and 1 type IV hips. Physiological immaturity was present in 81 hips (19% of babies). According to risk factor analysis, the only risk factor in unilateral analysis was presence of oligohydramnios (odd ratio-OR: 11.8, confidence interval-CI: 2.7-52.7). In correlation analysis, there was a correlation between female gender and swaddling. There was overall increase in DDH in female babies who were swaddled compared to those who were not. The results of this study showed that the most important risk factor was oligohydramnios for DDH. Swaddling and female gender increased the risk of the disease, but further studies in larger series are necessary for the confirmation of these results.

Interruption of the blood supply of femoral head: an experimental study on the pathogenesis of Legg-Calve-Perthes Disease.

INTRODUCTION: The etiology of LCPD still is not clear. Thrombosis has been accused in the pathogenesis of LCPD but it is not proven until now. The aim of this study is to evaluate the results of single episode of obstruction of blood supply to the femoral heads of dogs. MATERIAL AND METHOD: Blood supply femoral heads of 45 dogs was interrupted with embolisation with gel foam. The radiologic appearances, macroscopic and microscopic specimes of the hips were evaluated and compared with the human specimens of 15 LCPD patients obtained at the time of femoral osteotomies. RESULTS: After one infarct, we demonstrated changes in femoral heads of puppies showing close resemblance to the findings of LCPD in human. CONCLUSION: Obstruction of the femoral head caused by single artificial emboli caused changes in the femoral head similar to LCPD. The cause of the obstruction is obscure, intravascular and/or extravascular pathologies need specific attention, further studies focusing especially on the coagulation system are needed.

[Closed reduction in the treatment of developmental dysplasia of the hip]. / Gelisimsel kalça displazisi tedavisinde kapali redüksiyon.

One of the standard treatment methods for developmental dysplasia of the hip is closed reduction followed by immobilization in a hip spica cast. This treatment is performed before achievement of independent walking, under general anesthesia and with arthrographic control. Avoidance of forced reductions and immobilization of the hip with extreme positions is of utmost importance during the procedure. Development of avascular necrosis (AVN) of the femur head is the main parameter affecting the late outcome of the procedure. All modifiable factors should be influenced in favor of the patient to avoid AVN. This method appears to be satisfying with acceptable rates of AVN when applied by experienced pediatric orthopedic surgeons.

The effect of temporary hemiepiphyseal stapling on the growth plate: a radiologic and immunohistochemical study in rabbits.

The purpose of this study was to investigate the effect of temporary hemiepiphyseal stapling on the bone geometry and proliferative activity of the physis in immature rabbits. Proximal medial epiphyseal stapling of the right tibia was performed in 46 6-week-old New Zealand white rabbits. The rabbits were assigned randomly into two groups. In group 1, the staples were inserted extraperiosteally and the rabbits were killed at the end of 3 weeks. In group 2, the staples were fixed subperiosteally (group IIA) or extraperiosteally (group IIB), the staples were removed at the end of 3 weeks, and the rabbits were killed at the end of 6 weeks. The articular line-diaphysis angle (ALDA) was significantly increased with 3 weeks of stapling. After the removal of staples, while ALDA continued to worsen in group IIA, it improved in group IIB. Bone was observed to bridge the physis in group IIA. However, the proliferative activity of the physis continued. Temporary hemiepiphyseal stapling is a safe and effective method for control of physeal growth of long bones before skeletal maturity. However, it is of paramount importance not to disturb the periosteum during stapling.

Radiological outcome of proximal femoral varus osteotomy for the treatment of lateral pillar group-C Legg-Calvé-Perthes disease.

Results from 26 hips of 24 Legg-Calvé-Perthes disease patients were evaluated retrospectively. There were 22 male and two female patients in the study group. The mean age of the patients was 8.3 years and the mean follow-up was 13.03 years. All the patients were older than 6 years of age and all of them had lateral pillar group C hips. The patients were treated with intertrochanteric uniplanar varus osteotomy. All the patients were mature at the time of the last evaluation. Radiological end results were evaluated according to Stulberg's classification. Six hips healed with spherical congruency (Stulberg class 1 or 2), 19 with aspherical congruency (Stulberg class 3 or 4) and one with aspherical incongruency (Stulberg class 5). Six of 14 hips treated before 9 years of age healed with spherical congruency but all hips treated after 9 years of age healed with Stulberg class 3, 4 or 5 results. Trochanteric overgrowth was evident in 20 hips. The results of this radiological outcome study showed that Herring class C hips treated with proximal femoral varus osteotomy had a poor outcome, especially after 9 years of age in this group of patients.

[Treatment of simple bone cysts with methylprednisolone acetate in children]. / Cocukluk çagindaki basit kemik kistlerinin metilprednisolon asetat ile tedavisi.

OBJECTIVES: Steroid injections represent one of the simple treatment alternatives for simple bone cysts in pediatric age group. The aim of this study was to evaluate the results of steroid injections in the treatment of simple bone cysts. METHODS: Thirteen children (9 boys, 4 girls; mean age 9 years; range 4 to 14 years) with simple bone cysts were treated primarily with injection of methylprednisolone acetate with the use of the two-needle technique. The cysts were localized in the proximal humerus (n=7), femoral intertrochanteric region (n=4), and the proximal one-third of the femur (n=1). Four patients had fractures before treatment. Each patient received a maximum of three injections at six-week intervals, each consisting of 120-160 mg of methylprednisolone acetate. The patients were monitored by plain radiographs obtained in the sixth week, third month, sixth month, and at the end of a year followed by annual radiographic evaluations. Cyst healing was assessed according to the Neer classification. The mean follow-up was five years (range 1 to 11 years). RESULTS: Treatment with methylprednisolone acetate resulted in complete healing in five patients (38.5%) and healing with residual lesions in six patients (46.2%). One patient (7.7%) did not respond to steroid treatment and one patient developed recurrence. The results were satisfactory (84.6%) in patients with complete healing and healing with residual lesions, and unsatisfactory in two patients (15.4%). No procedure-related complications were encountered. CONCLUSION: Our results suggest that treatment with steroid injections offers satisfactory outcome in simple bone cysts in children, and thus, is worthy of consideration before more aggressive methods are to be applied.

The accuracy and reliability of estimation of lateral pillar height in determining the herring grade in Legg-Calvé-Perthes disease.

The amount of the preserved lateral pillar height (LPH) for assigning Herring grades is estimated during daily practice in Perthes disease. Despite this estimation, Herring classification has been reported to have a good interobserver agreement. The purpose of this study was to investigate whether the amount of preserved LPH has an effect on interobserver agreement. The results of surgeons' estimates were compared with objective measurement results. Good agreement was found among three experienced pediatric orthopaedic surgeons in 50 patients (1 vs. 2, kappa=0.527; 2 vs. 3, kappa=0.526; 1 vs. 3, kappa=0.539). Twenty of these cases had a ratio of the LPH scattered between 0.45 and 0.60 and 0.90 and 0.99 (transition between group B and C, and group A and B). When the borderline cases were evaluated separately, the interobserver reliability was poor (1 vs. 2, kappa=0.194; 2 vs. 3, kappa=0.256; 1 vs. 3, kappa=0.154), which may be explained by misperception of the preserved LPH. If the Herring classification is to be used as the prognostic indicator for deciding among the treatment alternatives, and LPH is the major determinant of the management, measurements should be used instead of estimates, especially in borderline cases.

Comparison between braced and non-braced Legg-Calvé-Perthes-disease patients: a radiological outcome study.

Twenty-three hips of 23 Legg-Calvé-Perthes-disease (LCPD) patients treated with abduction orthosis and 28 hips of 25 non-braced LCPD patients were evaluated radiologically retrospectively. The mean age of the brace group was 6.82 years and the mean follow-up was 12.30 years. The mean age and mean follow-up were 7.03 and 17.85 years for non-braced patients, respectively. The hips were evaluated according to lateral pillar classification. There were eight Herring A, 15 Herring B and five Herring C hips in the non-braced patients group; and there were five Herring A, 11 Herring-B and seven Herring C hips in the brace group. The groups were similar in terms of mean age, mean follow-up, sex distribution and lateral pillar grade. Patients' end results were evaluated according to Stulberg classification. All Herring A patients healed with Stulberg class 1 or 2, either braced or non-braced. Fifty-three percent of non-braced hips and 65% of the hips treated with brace healed with satisfactory radiological outcome. There were no significant differences between the groups in terms of end result. The overall results of this study suggest that Herring's lateral pillar classification is a useful system for the prediction of the long-term outcome of the disease and that there was no significant difference between the braced and non-braced patients in terms of radiological end result.

Lumbar pedicle morphology in the immature spine: a three-dimensional study using spiral computed tomography.

STUDY DESIGN: A cross-sectional study was conducted. OBJECTIVE: To investigate the pediatric pedicle morphology with the help of modern computed tomography technology. SUMMARY OF BACKGROUND DATA: The use of pedicular screws recently has gained popularity because of their ability to provide three-dimensional correction of spinal deformity. Extensive work has been published on the pedicle morphology of the adult and adolescent thoracolumbar spine. Less is known about the pedicle morphology of children. METHODS: A total of 21 patients ages 5 to 10 years underwent standard spiral computed tomography of the abdomen. The patients were grouped according to age: Group 1 (5 to 8 years of age) and Group 2 (9 to 10 years of age). Images were reformatted, and multiplanar reconstructions were used to attain images of lumbar pedicles on sagittal, coronal, and transverse planes. The measurements included the inner and outer pedicle diameters on the transverse plane, the pedicle angle on both the transverse and sagittal planes, and pedicle length. RESULTS: The smallest pedicle lengths were 24 mm for Group 1 and 25 mm for Group 2. When the average values were considered, the smallest lengths were at L5 and the longest at L3. The smallest diameter was at L1 (2.3 mm for Group 1 and 3 mm for Group 2), whereas L5 had the largest diameter (6.17 mm for Group 1 and 8.72 mm for Group 2). In the transverse plane, the pedicle angle increased from L1 to L5 in both groups. In the sagittal plane, the angulations followed an opposite trend. CONCLUSIONS: The inner transverse diameter of the lumbar pedicle, particularly in young children, is smaller than previously reported. Insertion of screws currently available commercially screws seems to be safe in the L4-L5 pedicles of children ages 5 to 8 years, and in the L3-L5 pedicles of older children. Custom-made screws might be considered for upper levels for safe application.

Bipositional MR imaging vs arthrography for the evaluation of femoral head sphericity and containment in Legg-Calvé-Perthes disease.

Eleven hips of 10 patients with Legg-Calvé-Perthes disease (LCPD) were examined by arthrography and magnetic resonance (MR) imaging. Arthrogram and MR images were obtained with the hips in neutral position and in 20 degrees flexion-abduction and internal rotation. Modified arthrographic index (AI) was used for the evaluation of sphericity and acetabulum head index (AHI) was used for the assessment of containment. According to AI, there is no significant difference between the arthrograms and coronal MR imaging, but the difference between the coronal and sagittal MR imaging was significant. There was no statistical difference between the arthrograms and MR measurements in terms of AHI. Anterior flattening of the femoral head and the effect of hip flexion in the containment at the sagittal plane could be demonstrated clearly in sagittal MR imaging. Even if there was significant flattening in coronal plane, the sphericity of the head was preserved in the sagittal plane. Bipositional MR imaging is comparable to arthrography for the demonstration of sphericity and containment of the femoral head in both coronal and sagittal planes in LCPD.

Thoracic paravertebral leiomyosarcoma: rare but it does occur.

STUDY DESIGN: Marginal resection of a paraspinal tumor in the thoracic vertebra was performed. OBJECTIVE: To document a very rare pathology for a paraspinal tumor. SUMMARY OF BACKGROUND DATA: Primary leiomyosarcoma of the spine or paravertebral space is extremely rare. A case of a patient who was operated on for a mass in the spinal canal and whose pathology was reported to be leiomyosarcoma is presented. METHODS: Marginal resection of the paravertebral mass was performed. RESULTS: The pathology of the tumor was reported as leiomyosarcoma. CONCLUSION: Leiomyosarcomas may develop at any site where smooth muscle cells are present. However, primary leiomyosarcoma of the spine or paravertebral space is extremely rare. Leiomyosarcoma, although rare, should be kept in mind as one of the possible diagnoses when a patient with a paraspinal tumor is presented.

Treatment of developmental dysplasia of the hip before walking: results of closed reduction and immobilization in hip spica cast.

We retrospectively evaluated 200 hips of 129 patients with the diagnosis of developmental dysplasia of the hip treated with closed reduction and hip spica cast. There were 153 female and 47 male hips in the group. The mean age of the patients at the time of the reduction were six (range. 2-13) months and mean follow-up was 51 (range: 16-240) months. All the patients were treated with closed reduction and were immobilized in hip spica cast. The mean time of immobilization in the cast was 102 (range: 45-190) days. Avascular necrosis was observed in 15% of the hips. Clinical and radiological end results of the patients were evaluated with modified McKay criteria and Severin classification. Eighty-two percent of the patients had satisfactory results according to modified McKay criteria and 76% of the patients according to Severin classification. The most important parameters affecting the end result were pre-reduction location of the hip, pre-operative acetabular index values and avascular necrosis. Based on the results of this study, treatment of developmental hip dysplasia with closed reduction and hip spica cast is a relatively safe and effective method.