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The Schwartz-Jampel syndrome (SJS; chondrodystrophic myotonia; McK 255800) is a recessively inherited condition defined by myotonia, short stature, and bone dysplasia. Genetic linkage between SJS and chromosomal region 1q36-34 has been observed in several families, but the gene has not yet been identified. We studied the clinical and radiological features in 81 patients from the literature and 5 own patients trying to identify distinct subgroups. In addition, we tested genetic linkage to the SJS locus on chromosome 1 in one family with two affected sibs. We found that a group of patients have mild skeletal changes which may be secondary consequences of myotonia, while another group of patients appear to have primary bone dysplasia with myotonia. Within this latter group, there are differences in age of manifestation, clinical course and pattern of bone changes. We tentatively isolate three different types of SJS: type 1A, usually recognized in childhood, with moderate bone dysplasia, corresponding to the original descriptions of Schwartz, Jampel and Aberfeld; type 1B, similar to type 1A but recognizable at birth, with more pronounced bone dysplasia resembling Kniest dysplasia; and type 2, manifest at birth, with increased mortality and bone dysplasia resembling Pyle disease. Genetic analysis of the family with two sibs affected by SJS type␣2 showed evidence against linkage to chromosome 1p36-34. Conclusions SJS is clinically and radiologically heterogeneous. The causes of heterogeneity are not known yet but are likely to include both different mutations at the SJS locus on chromosome 1 and the presence of a second SJS locus. A tentative clinico-radiological classification can be useful for the characterization of patients and the development of genotype-phenotype correlations.

Fifty-one patients with unilateral severe (gliding angles 30 degrees-60 degrees) slipped capital femoral epiphysis (SCFE) treated by intertrochanteric corrective osteotomy were reexamined after 20-29 years (average 24 years) of follow-up; 55% of the patients showed neither radiographic signs of degenerative hip disease nor clinical symptoms, whereas 28% had moderate and 17% had severe osteoarthritis. These results are definitely superior to those reported in other series of patients with comparable slips treated by bed rest or in situ fixation only. Analysis of individual gliding angles and directions of the slips shows that results can probably be further improved by correct assessment of the gliding process to allow for best use of the potentials of intertrochanteric corrective osteotomies. Correction should also be performed as early as possible to allow for maximum remodeling.

Background: Local recurrence (LR) in osteosarcoma is associated with very poor prognosis. We sought to evaluate which factors correlate with LR in patients who achieved complete surgical remission with adequate margins. Patients and methods: We analyzed 1355 patients with previously untreated high-grade central osteosarcoma of the extremities, the shoulder and the pelvis registered in neoadjuvant Cooperative Osteosarcoma Study Group trials between 1986 and 2005. Seventy-six patients developed LR. Results: Median follow-up was 5.56 years. No participation in a study, pelvic tumor site, limb-sparing surgery, soft tissue infiltration beyond the periosteum, poor response to neoadjuvant chemotherapy, failure to complete the planned chemotherapy protocol and biopsy at a center other than the one performing the tumor resection were significantly associated with a higher LR rate. No differences were found for varying surgical margin widths. Surgical treatment at centers with small patient volume and additional surgery in the primary tumor area, other than biopsy and tumor resection, were significantly associated with a higher rate of ablative surgery. Conclusions: Patient enrollment in clinical trials and performing the biopsy at experienced institutions capable of undertaking the tumor resection without compromising the oncological and functional outcome should be pursued in the future

Summary From 1979 to 1990, 37 patients with extremity osteosarcomas, 22 of them males and 15 females, median age 19 years, received pre- and postoperative chemotherapy. The period of observation, calculated from after the primary operation, ranged from 1–114 months, median 25 months. After preoperative chemotherapy, 9 (24%) underwent a primary amputation, in 28 (76%) a limb salvage procedure was possible. 4/29 (14%) later developed local recurrences, metastases were diagnosed in 8/9 amputees and 5/28 after limb-sparing surgery. The five-year disease-free and overall survivals after amputation are 11% and 33%, respectively, compared to 68% and 75%, respectively (p =0.001 and 0.018, respectively, for long rank). Results of histologic assessment after preoperative chemotherapy are of significant prognostic impact. The earlier prognostic groups of 0%–49% necrosis versus 50%–100% necrosis were statistically no longer suitable for distinguishing useful prognostic groups. In this second analysis, patients with 0%–79% necrosis versus 80%–100% necrosis had 5-year disease free survivals of 81% versus 44% (p = 0.032) and 5-year overall survivals of 88% versus 56% (p = 0.07 log rank). In summary, 24% of our patients with extremity osteosarcomas, most of them with large primaries close to joints, had to undergo primary amputation and had only a 33% 5-yr-survival, whereas limb salvage procedures with pre and postoperative chemotherapy were associated with a 5-yr survival of 75% and thus had no adverse impact. Careful selection of patients for successful management of osteosarcomas is important; necrosis of 80% and more after preoperative chemotherapy is a prerequisite for a favourable outcome.

Transverse below-elbow forearm deficiency was treated in two adolescents by turning and transposing the radial stump on the ulna and later lengthening by 5 and 5.5 cm, respectively. This resulted in improved function without and with the use of prosthesis.

The CT-guided therapy of osteoid osteoma instead of older methods such as open resection has the advantage of exact localization of the nidus intraoperatively and exact documentation of its ablation. Another advantage is the less invasive approach.A total of 52 patients with osteoid osteoma were treated in our institution between 1996 and 2005 either by radiofrequency ablation (n=11) or by percutaneous resection under CT guidance (n=41). Their age was between 7 and 48 years, mean age was 22.3 years, and follow-up was 31.3 months.In all patients (n=52) the osteoid osteoma was successfully treated. In 50 patients the first treatment resulted in long-term success. In two patients the nidus was first missed; they were successfully treated with another operation using the same technique.The CT-guided operation of osteoid osteoma made the therapy much easier because of the exact localization and the less invasive approach. This technique can be used analogously to tumor biopsy. The advantage is the exact documentation of the biopsy path and the possibility to take specimens.

The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb.

Four patients with malignant tumors of the proximal toe phalanx who had closed contact or direct involvement of the metatarsophalangeal joints or direct infiltration of the metatarsal bone were treated. Treatment included ray resection and reconstruction either by free microvascular fibula transfer, intermetatarsal bony fusion, or soft-tissue stabilization. Foot function was analyzed by Novel pedobarography. The four patients with a follow-up between 21 months and 8 years show almost normal gait. All patients have remained relapse free. If adequate margins can be achieved, ray resection and appropriate reconstruction may be an alternative to amputation.

The progression of degenerative changes of the hip after slipped capital femoral epiphysis (SCFE) largely correlates with the patient's age at the time the deformity occurs and with the degree of the epiphyseal gliding. From the pathogenetic point of view, the altered biomechanical conditions of the hip joint with deformation of the proximal femur may result in an impingement of the femoral neck metaphysis against the anterior acetabular rim. Observations of the "natural course" or after "in situ fixation" of the epiphysis show the development of secondary hip arthritis at an average of 20 years after SCFE, specifically in slips with more than 30 degrees of epiphyseal gliding. The intertrochanteric osteotomy as introduced by G. Imhäuser aims at restoring joint congruity to reduce the prearthrotic deformity and thus to decrease the incidence of later hip arthritis. The reorientation of the predominantly posteriorly slipped femoral epiphysis is achieved by an intertrochanteric flexion osteotomy, which reduces the potential for femoroacetabular impingement. On the basis of a long-term evaluation, the indication for and results of an intertrochanteric osteotomy for chronic unilateral SCFE were presented. Of the 51 patients operated on between 1962 and 1972 and examined clinically and radiographically at an average follow-up time of 24 years (20-29 years) after osteotomy, 55% showed a hip free of degenerative changes, 28% had developed moderate degenerative changes, and 17% had advanced arthritis. Aside from a few technical errors, the correction at the intertrochanteric level for moderate slips proved to be a safe procedure regarding risk for femoral head necrosis. The long-term development after SCFE is most important for patients with SCFE. More than half of the patients have hip joints free of degenerative changes more than 20 years after intertrochanteric osteotomy according to G. Imhäuser, which compares favorably to the "natural course" or to "in situ fixation" and which supports the indication for this corrective procedure in SCFE.

In looking at the pathoanatomy of slipped capital epiphysis, it is important to appreciate that the articular cartilage ruptures at the level of the physis as the articular cartilage also covers part of the femoral neck metaphysis. As a consequence, smoothness is lost and already with mild slips the irregularities may injure the acetabular cartilage structures and may cause tears of the labrum. Principally therefore an open revision and restoration of the anatomy as perfectly as possible is needed. The technique for open reduction was developed by Dunn in 1964 and then refined by Ganz in 1997 applying new anatomical vascular studies. However, the long-term data on the "conservative" approach of only using transfixation to avoid further slipping show relatively good results and long-term studies of a more aggressive open approach will be needed to show evidence of further improvement. For the more severe cases, restoration of the correct anatomy by open reduction or improved axial relationships by more distant osteotomies have already been proven to give better results. Besides the extent of the slip, it is important to consider whether the slip is acute or chronic or acute and chronic. So far there are no data showing that the surgical treatment of slipped capital femoral epiphysis must be made on an emergency basis. However, prevention of further slips, e.g., by supine transport, is to be considered an emergency.

PURPOSE: Cooperative Ewing’s Sarcoma Study (CESS) 86 aimed at improving event-free survival (EFS) in patients with high-risk localized Ewing tumor of bone. PATIENTS AND METHODS: We analyzed 301 patients recruited from January 1986 to July 1991 (60% male; median age 15 years). Tumors of volume >100 mL and/or at central-axis sites qualified patients for “high risk” (HR, n = 241), and small extremity lesions for “standard risk” (SR, n = 52). Standard-risk patients received 12 courses of vincristine, cyclophosphamide, and doxorubicin alternating with actinomycin D (VACA); HR patients received ifosfamide instead of cyclophosphamide (VAIA). Tumor sites were pelvis (27%), other central axis (28%), femur (19%), or other extremity (26%). The initial tumor volume was RESULTS: On May 1, 1999 (median time under study, 133 months), the 10-year EFS was 0.52. Event-free survival did not differ between SR-VACA (0.52) and HR-VAIA (0.51, P = .92). Tumor volume of >200 mL (EFS, 0.36 v 0.63 for smaller tumors; P = .0001) and poor histologic response (EFS, 0.38 v 0.64 for good responders; P = .0007) had negative impacts on EFS. In multivariate analyses, small tumor volumes of CONCLUSION: Fifty-two percent of CESS 86 patients survived after risk-adapted therapy. High-risk patients seem to have benefited from intensified treatment that incorporated ifosfamide.

Glenoid dysplasia is a rare abnormality of the shoulder. We report glenoid dysplasia in two consecutive generations: a boy and his father. Both suffered recurrent shoulder dislocations, and radiological examination revealed bilateral glenoid dysplasia. Our cases confirm dominant inheritance of this osseous malformation.

We present a case of a 13 year old girl with swelling and pain of the right lower leg persisting for four months. The radiological and histological diagnosis is osteofibrous dysplasia. Osteofibrous dysplasia is a rare bone lesion predominantly affecting the cortex of the tibia. Almost all patients are younger than ten years at presentation. Swelling is a typical clinical manifestation. Radiologically and even histologically the distinction between the osteofibrous dysplasia and the more aggressive adamantinoma can be difficult. Other bone lesions potentially mimicking osteofibrous dysplasia include: fibrous dysplasia, osteoid osteoma, osteosarcoma, osteoblastoma and intracortical Brodie's abcess. The natural course of the disease is unpredictable. Lesions with typical radiological appearance are considered to be "no touch lesions". In equivocal cases block resection (with both a diagnostic and therapeutic purpose) should be performed.

Owing to twenty years of multicentric interdisciplinary cooperation, the COSS group has been able to collect data on a large group of osteosarcoma patients treated by neoadjuvant therapy. This paper reviews results achieved in patients with localized extremity tumors.Registration into a completed neoadjuvant COSS-Study. Histologically confirmed, primary, localized, high-grade, central osteosarcoma of an extremity; age40 years; no pretreatment; interval diagnosis to chemotherapyor = 3 weeks; no severe comorbidity. Chemotherapy: HD-methotrexate +/- doxorubicin +/- cisplatin +/- ifosfamide +/- BCD. Scheduled local therapy: Surgery.925 evaluable patients from 101 institutions. Median age 15 years, m:f 1.4:1. Primary site: femur 510, tibia 251, humerus 100, fibula 51, other 13. Tumor-size1/3 of the involved bone 616,or = 1/3 304. Definitive surgery in 903/925 cases, 443 limb salvage procedures. Good response (90% necrosis) in 469/806 (58.2%) evaluated tumors. Median follow-up for surviving patients: 5.42 years. Actuarial survival after 5 and 10 years: 72.5% (95%-CI 69.3-75.7) and 66.3% (62.5-70.0), relapse-free 62.1% (58.7-65.4) and 59.4% (55.8-63.0). 683/925 alive (601 first remission), 242 deceased (212 tumor progression, 30 other causes). 66.2% (97.3%) of all relapses within 2 (5) years. Prognosis correlates with tumor-size (vs.or = 1/3: 69.9% vs. 58.3% at 10 years) and -site (tibia: 74.2%, humerus: 54.5%) and -response (good vs. poor: 78.2% vs. 52.5%) (all p0.01). Actuarial 10-year survival by response grading I-VI according to Salzer-Kuntschik 80.9%, 82.8%, 71.1%, 60.7%, 47.7%, 27.3%. COSS-studies with preoperative 4-drug therapy more efficacious than less aggressive protocols. No impact of doxorubicin scheduling (sequential: rapid vs. 48 h-continuous infusion) or cisplatin scheduling (randomized: 5 h vs. 72 h-infusion) on prognosis detected.Intensive multiagent chemotherapy and delayed surgery for localized extremity osteosarcoma led to excellent oncologic results in the COSS-studies. Tumor-size, -site, and -response as well as the intensity of upfront chemotherapy correlated with outcome. Giving doxorubicin and cisplatin by continuous infusions did not result in discernible prognostic disadvantages.

A few selected++ metric and numeric malformations of the foot (Fibular deficiency, vertical talus, coalition, syndactylism, macrodactylism, metatarsal malformation, cleft foot, overriding fifth toe) are discussed to demonstrate, that concepts for their treatment should be developed as early as possible in order to allow for bringing those structures that are developed in optimum use. Besides function cosmetic aspects of the foot also deserve being respected.

Sixty-six hips of 65 patients with moderate to severe slipped capital femoral epiphysis were treated by open reduction of the femoral head. In 60 hips this procedure was combined with a cuneiform subcapital wedge resection of the femoral neck according to the technique described by Dunn (14). Avascular necrosis occurred in seven cases, and chondrolysis was evident in eight hips. In the 48 hips with a follow-up time of more than 10 years (M = 20.6), the results were classified as good (normal hip) in 22, moderate in 16, and poor in 10 hips. Compared with the natural disease course, the long-term outcome appears to be improved by open reduction of the femoral head, with 60% of the hips free of degenerative changes and 19% with mild osteoarthrosis. Analysis of individual gliding angles and subcapital correction depending on the direction of the slip should lead to restoration of near-normal anatomic conditions or a normal hip function.

Osteomyelitis sclerosans Garré is still not completely understood. In spite of the fact that it is caused by bacterial infection, in most cases no bacterial growth can be discovered in culture. Furthermore, it remains open whether the chronic process is maintained by low-grade persistent infection or is maintained by itself after sterilization of the infection. Newer studies that may prove the presence of bacteria, e.g., by PCR, may elucidate the causes in the future. Sclerosing bone disease, sometimes multifocal, which present the same clinical, radiographic and histological features as chronic sclerosing osteomyelitis may be associated with diseases such as palmoplantar pustulosis, colitis ulcerosa, Crohn's disease, etc. and are reported as SAPHO (synovitis acne pustulosis hyperostosis osteitis) syndrome. In these diseases bacterial growth is almost always negative. However, temporary improvement under antibiotic therapy may be observed. A relationship between typical isolated chronic sclerosing osteomyelitis and SAPHO syndrome may exist, but the diseases should at present be differentiated. Neoplasms such as osteoid osteoma, Ewing sarcoma, or eosinophilic granuloma may simulate primary sclerosing osteomyelitis, and pathological examination of peripherally taken specimens may appear to be compatible with the diagnosis of sclerosing osteomyelitis. It should be appreciated that malignant tumors--even though this is the exception--can present a chronic, mild picture. Treatment of osteomyelitis sclerosans Garré needs be determined on an individual basis. A conservative approach with antibiotics usually leads only to temporary pain relief. The biopsy with opening of the medullary canal is sometimes sufficient for healing, while in extreme situations only segmental or even complete resection of the diseased bone case result in a permanent cure.

The purpose of this evaluation was the description of potentially misleading MR appearances of osteoid osteoma. The MR images of 10 patients with osteoid osteoma were retrospectively evaluated and compared to radiographic, intraoperative and histologic findings. 4 of the 10 abnormalities were located in the proximal femur, two in the lumbar spine, and one each in the tibial plateau, in the cervical spine, in the sacrum and in the first metacarpal. 8 of the 9 nidi visible on standard radiographs and/or CT scans were demonstrated on MR images. Edema was visible within the bone marrow in 3, within soft tissue in 2 and in both locations in 4 MR examinations. The soft tissue abnormalities were circumscribed in 3 patients and could be misdiagnosed as soft tissue tumors or an abscess in these patients. One of 5 osteoid osteomas located in the proximity of a joint mimicked septic arthritis. The MR appearance of osteoid osteoma may be misleading. However, false diagnoses usually can be avoided with a careful search of a nidus. MR imaging in osteoid osteoma is important for differential diagnosis.

At the Balgrist hospital 38 patients with osteosarcoma and 18 patients with Ewing sarcoma were treated from 1970 through 1992; a follow-up of at least three years is available for all patients. Disease-free survival in this period of time has increased from 20% (Ewing sarcoma) and 30% (osteosarcoma) to over 75%. While systemic tumor control was improved by systematic chemotherapy, local tumor control was improved by appropriate biopsy techniques, and amputations have been replaced by local resections, in most cases with reconstruction (arthrodesis, rotation plasty, endoprosthesis, allografts), which require further improvements. With respect to cure of the neoplastic disease, the currently available methods have been used to their maximum potential, and a steady state has been reached regarding survival. Further improvement of survival will therefore depend on the introduction of completely new modalities.

In twelve patients with local aggressive or malignant bone tumors allograft reconstruction after limb sparing procedures were performed using titanium implants for osteosynthesis. In eleven of these patients magnetic resonance imaging studies could be completed. Distortions of the images were limited to the vicinity of the implants and did not preclude detailed analysis of the gross anatomy and of fine articular structures involving and adjacent to the allografts used. Titanium implants therefore can be recommended whenever magnetic resonance imaging can be anticipated to be of importance for follow-up studies, such as after limb saving procedures for malignant musculoskeletal tumors. Aspects of high interest and importance in such cases include early recognition of local recurrence, bone fusion and healing of soft tissue structures, incorporation of allografts, and viability of cartilage.

Poliomyelitis, in spite of vaccination, is still a problem: in the third world as well as for the victims of the epidemics of the first half of this century, who now, becoming older, have growing difficulties to overcome their paralyses. They needed and still need orthoses for walking. How do they manage? And what can orthopedic technique offer them today?

Fibrous and osteofibrous dysplasia are benign lesions of the bone involving single bones as monostotic or multiple bones as polyostotic forms. When the patient is in the growth phase, the lesions, which are usually asymptomatic initially, may lead to deformities with typical bowing of the proximal femur ("shepherd's crook") and fractures. It is important to recognize osteofibrous dysplasia of the tibia and fibula, where any surgical measures, even a small biopsy, may activate the process and cause the lesion to grow, although it may spontaneously rest around puberty. Polyostotic fibrous dysplasia may rarely be associated with areas of cutaneous pigmentation and endocrine disturbances, especially precocious puberty (McCune-Albright syndrome).

We report on two patients with linear scleroderma (a form of localised scleroderma) and their orthopaedic problems such as shortening of the limbs and contractions of the joints. The disease is very rare but easy to diagnose by its typical lesions of the skin (band like lesions with ivory like induration and hyper- or hypopigmentation; a peripheral erythema appears as a lilac ring). In the orthopedic management it is very important to follow the patient carefully during the period of growth for good planning of the conservative (physiotherapy and orthosis) and operative therapy, which in one of our patients consisted in repeated corrections as partial arthrodesis of the foot and osteotomies of the leg.

The pre-operative diagnosis of osteoid osteoma of the first metacarpal bone was suggested by Magnetic Resonance Imaging in a 15 year old girl. As indicated in the literature, osteoid osteoma of the hand is relatively rare. The symptoms and the x-rays features of osteoid osteoma are essentially independent from the location consisting in rather unspecific pain and radiographically osteolytic nidus surrounded with sclerosis. In the few published cases with MR Imaging, the nidus shows high signal intensity surrounded by low signal intensity in the area corresponding to the radiographically visible sclerosis.

The reconstruction of large segmental defects after the resection of malignant bone tumors is usually done with modular or custom-made endoprostheses, so far, they appear to work well. From the experience with other indications for endoprostheses it must be admitted that failures will be a matter of time only. With the improved prognosis for patients with primary malignant bone tumors with regard to relapse-free survival and increased chances for permanent cure, the trend for reconstruction procedures should be directed to more 'biological' techniques. The reconstruction of osseous and osteocartilaginous defects with massive allografts is somewhere between the use of autologous bone and artificial replacement. From the experience with 14 allograft reconstructions in primary malignant bone tumors (osteosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, chondrosarcoma, lymphoma) or other aggressive lesions (aneurysmal bone cyst, recurring giant-cell tumor, solitary metastasis) of the humerus (one osteoligamentous graft in combination with an endoprosthesis, one intercalary graft), the femur (three intercalary grafts, two osteoarticular distal femurs, one combination with an endoprosthesis), and the proximal tibia (four osteocartilaginous, two intercalary grafts) in patients aged 10 to 64 years, we feel that this type of reconstruction allows for a reconstruction without sacrificing more bone and soft tissue than needed for the surgical margins. Fusion between the patient's bone and the allograft has been seen after 6 to 18 months. From this small series it is concluded that using allografts might allow for the preservation of joint structures that need to be resected for the implantation of an endoprosthesis, increasing the possibilities for salvage procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

Between 1988 and 1992 lengthenings axial, corrections and reconstructions have been performed by distraction on lower arms, upper and lower legs in 25 patients because of deviations in length due to either congenital or acquired (multiple cartilaginous exostoses, posttraumatic conditions, congenital pseudarthrosis deformations or defects (fibula-defect, amnion-band-complex, amputation). On the average elongation amounted to 4 cm for an average installation time of the fixator system of 22 weeks and a mean duration of 25 weeks for the adaptation to full strength. Problems arose in the form of soft tissue infections at the rod perforation sites, the necessity for secondary interventions because of early fractures and for corrections of the fixator respectively. Complications compromising the expected goal of treatment did not occur. Consideration of the biological principle of distraction is essential whereas the type of fixator-system is of only minor importance.

The biopsy is the key for the exact diagnosis of bone and soft tissue sarcomas. However the biopsy implicates risks that eventually may significantly interfere with the prognosis. The steps of the biopsy can be differentiated in tactical and technical aspects. Tactical aspects include laboratory examinations (e.g. blood count, immunological, chemical tests) in order to establish the prebiopsy differential diagnosis, basic imaging procedures (bone scan, magnetic resonance imaging) and to discuss with the pathologist questions as which region of the tumor might be most informative, how should the material be preserved and whether a frozen section might be useful and allow for initiation of therapy as early as possible. Furthermore the site of the biopsy has to be carefully chosen, the compartments that need being passed through or opened up during biopsy must be considered. The technical aspects refer to the approach and tissue handling during the biopsy itself. Fine needle aspiration allows only for a cytologic, core biopsies for a histologic examination (however only of a small sample), while the open biopsy provides more material for more analyses. Any biopsy (open biopsy as well as percutaneous puncture) contaminates the path with tumor cells and increases the risk of systemic seeding. In order to keep these risks as small as possible the most careful technique and tissue handling is mandatory. The biopsy path has to be completely ('en bloc') resected later for local tumor control.

Treatment of congenital or acquired disturbances of the radius or ulna always requires recognition of the complex biomechanics of the forearm. In particular trauma, seemingly restricted to one of the bones, should caution against concomitant lesions to the other bone. The typical example is unfortunately the often missed dislocation of the capitulum radii in Monteggia-type lesions. Additional examples are deranged growth of the distal epiphysis of the radius after a fracture of the ulna, in association with multiple osteochondromas and of the proximal epiphysis of the radius in presumptively congenital dislocation of the capitum radii. Lesions encountered in children have to be analyzed carefully with respect to dynamics of maldevelopment. Corrections that have implications for neighbouring articulations should be undertaken with the intent to fully exploit the potential for remodelling by forthcoming growth processes.

Six patients who had been treated for osteosarcoma of the lower extremity report their experience and how they mastered diagnosis and therapy as well as the time thereafter. They give information regarding especially the following points: psychological stress during different phases of their disease, aspects of the information they received during treatment, how they felt during therapy and how and where they have received support, wishes, etc.In order to master the disease as well as the sequelae they need sufficient time and opportunities to express their feelings to others and sufficient understandable informations about examinations, therapy and prognosis. There are large variations in how the individual patient responds to the threat by the disease. The whole period of time with repeated hospitalizations that extends over about one year in retrospect has been judged to be very burdening. Most of the patients feel to be strongly challenged during chemotherapy; physically, they are reduced, are oversensitive and need sensitivity and support by the persons taking care of them. The informations given by the patients allow us to improve treatment and support of patients in the future.

The chances to be cured from a primary malignant musculoskeletal tumor have improved dramatically during the last two decades with the introduction of adjuvant chemotherapy. The cure rate e.g. for osteosarcomas and Ewing sarcomas has improved in this period from about 20% to 75%. Furthermore instead of amputations for local tumor control local resections have become possible in most cases when the rules of oncologic surgery for such tumors beginning with the biopsy are strictly followed. With the improved long term survival of patients afflicted by musculoskeletal malignancies the demands upon reconstructions after large resections have significantly increased. Techniques actually available for different localizations of tumors of the femur and tibia with trends for 'more biologic' procedures with arthrodeses and massive allografts are demonstrated besides new endoprosthetic material.

Fatigue (stress) fractures are due to increased repetitive stresses. The onset of clinical symptoms and the pain characteristic usually are vague; furthermore the radiographic picture shows lesions of different age and therefore may mimic a pattern typical of bone tumors. While for malignant bone tumors early diagnosis, biopsy and initiation of the appropriate treatment is of importance, the biopsy of a fatigue fracture is not only unnecessary, but also may interfere with the healing process. The value of magnetic resonance imaging in the differential diagnosis between tumors and fatigue fractures has therefore been analysed. Five consecutive cases of fatigue fractures have been evaluated from the onset of symptoms for a mean of 19 month (range 3-45 month) of follow-up. The signal pattern with low intensity in the area of the lesion in T1- and T2-weighted images seen in 2 cases has lead to observe the process under immobilization of the affected limb, while because of an increase of the signal intensity from T1- to T2-weighted images as seen in almost all malignant bone tumors biopsy has been performed in 2 cases. In 1 cases no biopsy but close-meshed clinical examinations were performed although there was an increase of the signal. From these observations we conclude that the MRI in fatigue fractures may either show a 'chronic' type of reaction with low signal intensity allowing for the caution observation while in cases with the rather 'acute' reaction with increased signal biopsy usually cannot be avoided in order not to miss a malignant tumor.

Typically an osteochondrosis dissecans occurs in the region of a convex articular surface, the most frequently localisation observed being the medial femoral condyle. Only few cases of an osteochondrosis dissecans in concave articular surfaces have been reported; these involved the tibial plateau, the distal tibia and the proximal surface of the navicular bone. Two more cases with an osteochondrosis dissecans in the proximal and distal tibial epiphysis are reported and additionally a hitherto unreported localisation in the glenoid fossa of the scapula. True osteochondrosis must be differentiated from ossification variants.

The typical radiologic finding of a "non-ossifying fibroma" of the distal femoral metaphysis was seen in a 17-year-old female patient with pain in the distal femur. The rapid development of this lesion was confirmed on the basis of earlier radiographs performed to clarify other complaints. Since the lesion had developed in no more than 34 months, with pronounced scintigraphic and MRI signal intensity, particularly in the late phase, and pain had prompted the patient to seek examination, the cause was assumed to be a neoplasia rather than a growth disorder. Histopathological examination revealed a proliferative fibrohistiocytic lesion which was histologically different from a malignant fibrous histiocytoma. The finding indicates that in the differential diagnosis between non-ossifying fibroma and benign fibrous histiocytoma in cases with identical localization, pain and the intensity of the scintigraphic signal may well be the key to further action: since benign fibrous histiocytomas may be regarded as genuine neoplasias, the question of malignancy should also be clarified beyond doubt.

Complete removal of the tumour without leaving microscopic disease is vital in the management of bone sarcomas. Magnetic resonance imaging now seems to be the best method of depicting the changes produced by the tumour within the bone and surrounding soft tissues. In order to define its reliability, five cases of primary bone sarcomas (Ewing's sarcoma, osteosarcoma, chondrosarcoma) are described where radiographs, bone scans and magnetic resonance images are directly compared to the pathological findings after resection. We conclude that surgical margins should be redefined with respect to the extent and borders of the tumour as depicted by magnetic resonance imaging. This will allow improvement in salvage procedures, but further experience is needed so as not to jeopardise the prognosis by incomplete removal of the tumour.

Gamma-ray computed tomography (γ-ray CT), using a special purpose scanner, enables in-vivo quantitative analysis of bone mineralization. Trabecular bone density (TBD), the relative amount of compact bone (bone density, BD) and the total absorption (TA) for a cross-section of the radius are determined from measurements of local linear absorption coefficients. A preliminary study of normal children (n = 49) and adults (n = 34) indicated that TBD is independent of age and sex in the age range 4 to 40 years. DB remains constant throughout childhood but increases after puberty in both women and men. TA is higher for adults than for children, and also higher for men than for women. A correlation between TA and parameters relating to body size indicates a relationship between body weight and bone mass.

The method of gamma-ray computed tomography (gamma-ray CT) bone densitometry described in the preceding article provides selective determination of trabecular bone density (TBD), the relative amount of compact bone (bone density, BD), and the total absorption (TA) within a bone cross section. Seven of nine children with chronic renal failure (CRF), and selected only on the basis of their serum creatinine value (greater than 5 mg/100 ml), had increased TBD values above the normal range, whereas the other bone mineral parameters were normal. Radiographic signs of secondary hyperparathyroidism (subperiosteal erosions, cysts) were reported in the five patients with the highest TBD values, whereas the subjective diagnosis of osteosclerosis reported in three of these five and in one other patient correlated less well with the TBD increases. However, this is the first report of an objective, non-invasive documentation of the radiological finding of osteosclerosis in CRF. It also explains why methods for bone mineral measurements used previously, such as a photon absorptiometry which provides only a parameter equivalent to TA, failed to reveal increases in bone mineral content in renal osteodystrophy even when signs of osteosclerosis were present. Thus, gamma-ray CT helps to document objectively the degree of osteosclerosis and its location.

Twentythree boys with delayed adolescence (age 15.7 ± 2.0, bone age 12.4 ± 2.1 years) were studied. Their cortisol response to insulin was normal. After oral metyrapone (500 mg/m2 by mouth) one to three consecutive 12 h urine samples were collected for analysis of THS. Thirtyseven tests with 37 first, 21 second, and 11 third samples were carried out. The results could be divided into two main groups: 25 tests (group A) were subnormal in the first sample, 12 of them with a very weak (40 ± 8 μg/m2/12 h) and 13 with an insufficient (191 ± 16 μg/m2/12 h) THS response. Values in the second and third sample were higher, indicating a dealyed response. In 12 tests (group B), the results were normal (1016 ± 143 μg/m2/12 h) in the first and lower in the second and third samples. In three patients with repeated tests, there was improvement with increasing bone age. The THS-responses to metyrapone did not correlate with those of growth hormone, gonadotrophins, and TSH to stimuli. It is concluded that the THS-response to a single dose of metyrapone may be temporarily insufficient or delayed in delayed adolescence. We interpret this finding as showing transiently reduced or slow hypothalamic responsiveness.

125I-computed tomography (CT) for the bone mineral analysis of the radius was performed on 9 children with vitamin D resistant hypophosphatemic rickets (VDRR) treated with vitamin D in pharmacological doses. Trabecular bone density was increased in 1 patient and normal in all others. The cortical width of the diaphysis of the radius was decreased, and the cross sectional area of the bone increased, but the total amount of compat bone mineral in the cross section was normal. Measurements of the second metacarpal bone on X-rays of the hand revealed similar findings. Microradiographic and histological examinations of the fibula on 9 different patients also treated with vitamin D showed grossly abnormal mineralization of bone tissue with a perilacunar mineral deficit. It is concluded, that the amount of compact bone and trabecular bone is not decreased in children with VDRR. The normal mineral content determined by CT and the impaired mineralization of the bone material examined by microradiography indicate an over-abundance of incompletely mineralized bone.

Growth data, clinical symptoms and bone mineral parameters were analyzed in 20 children with coeliac disease in whom the diagnosis was established by biopsy at age 3-13 years. Small stature and bone age retardation (greater than 2 SD) were present in 65% and 60%, respectively. Typical clinical symptoms of coeliac disease as found in the younger child were present in many cases, but 3 were completely asymptomatic except for severe growth retardation. Metacarpal diameters and cortical thickness were significantly decreased for chronological age but in most cases normal for bone age. Quantitative bone mineral analysis of the radius by computed tomography revealed normal values for height and weight in the 4 cases investigated. It is concluded that coeliac disease should always be considered in the differential diagnosis of retarded growth and bone age. "Osteoporosis" may occur in coeliac disease, but does not necessarily accompany growth failure. The analysis of metacarpal diameters and cortical thickness in the search of "osteoporosis" may result in false interpretation if not correlated to height and weight.

The maturation of the iliac apophysis (Risser sign) and of the bones of the hand and wrist of 34 girls with scoliosis with 46 pairs of data were analyzed to get information upon their predictive value for further growth and development of the scoliotic curve. A strong correlation was found between the Risser sign and the maturation of the hand and wrist (r = 0.74, p less than 0.005). The prognostic value of the Risser sign (r = -0.57) for further height gain is superior to chronological age only (r = -0.52), the maturation of the hand and wrist (r = -0.62) however is superior to the Risser sign. It can be assumed that this also holds for their prognostic value for the progression of the scoliotic curve. We feel that in the clinician routine for girls with scoliosis the determination of the skeletal maturity from X-rays of the hand is sufficient and with its probably unavoidable irradiation risks to the gonads the determination of the Risser sign should be abandoned.

An increasingly severe hollow foot deformity is typical of Friedreich's hereditary ataxia and Charcot-Marie-Tooth's progressive muscular atrophy. However, on the basis of personal observations it has been found that in infancy, patients with either of these diseases may have an extreme pes valgoplanus. The retrospective analysis presented here is intended to draw attention to this possibility and to point out that these diseases should also be taken into consideration in the differential diagnosis of the cause of a pes valgoplanus.