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The spectrum of mesenchymal tumors associated with rearrangements of the EWSR1 gene has been growing in recent years due to progress in molecular detection techniques. Originally identified as the gene involved in the pathogenesis of Ewing sarcoma, the EWSR1 gene is now known to be rearranged in diverse clinical and histopathological entities. The NFATC2 gene is one of the many translocation partners of EWSR1 in gene fusions in a morphologically typical, albeit rare, subgroup of mesenchymal tumors. Little is known about the clinical characteristics of tumors containing NFATC2 gene rearrangements since most of the few reports published describe molecular rather than clinical aspects. In the current study, we report three patients with tumors carrying the EWSR1-NFATC2 gene translocation, including one rare primary tumor of soft tissues. Another patient with a benign-appearing bone tumor with a unique FUS-NFATC2 gene translocation is described. In various mesenchymal tumors (e.g., myxoid/round cell liposarcoma, low-grade fibromyxoid sarcoma, or angiomatoid fibrous histiocytoma), the FUS gene, as a member of the TET family, may be alternatively rearranged instead of the EWSR1 gene without any noticeable influence on the microscopical appearance or clinical outcome. This fact seems not to apply to mesenchymal tumors with the involvement of the NFATC2 gene because both in our experience and according to the extensive literature review, they have different properties on the morphological and molecular level. Both ESWSR1-NFATC2 and FUS-NFATC2 fusion-carrying tumors do not show microscopical or clinical features of Ewing sarcoma.

This article reviews the methods recommended in the literature for treating sacral tumors, paying particular attention to the most common sacral tumors and those of uncertain malignancy. These recommendations for treatment are compared and analyzed in the light of the authors' own results in the treatment of 30 sacral tumors.

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.

In 12 boys with a unilateral Morbus Perthes' disease who were not surgically treated, growth and skeletal maturation were prospectively recorded from the time of diagnosis onward. There were no significant deviations from the norm at the time the condition was diagnosed. Most of the boys manifested a distinctly retarded growth rate in the first phase following diagnosis; this was followed by a phase of excessive compensatory growth. Skeletal maturation showed a similar course. The course of growth would appear to be an expression of a transitory systemic disturbance.

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.

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.

Hintergrund Die CT-gefuhrte Entfernung des Nidus eines Osteoidosteoms anstelle herkommlicher Verfahren der offenen Resektion bietet den Vorteil, die Lasion intraoperativ exakt zu lokalisieren und deren Entfernung korrekt zu kontrollieren. Zudem tritt anstelle eines oft grosen Eingriffs der Vorteil eines minimal-invasiven Vorgehens.

P rimary neurofibrosarcomas of the sacrum are rare and have been reported only sporadically in the literature1-3. Because of the anatomic location, sacral tumors are often diagnosed only when they have reached a large size involving neighboring structures, thus limiting the potential for a curative resection. The treatment of choice for aggressive sacral tumors is radical resection. Large sacral tumors require extensive surgery to achieve adequate margins, and reconstruction to provide stability between the spine and pelvis is a challenging problem. The complete removal of the sacrum leaves the lumbopelvic complex so unstable that, without reconstruction, a prolonged period of pain and rehabilitation is the usual result4-6. Descriptions of methods to reconstruct the pelvis after sacrectomy have included the use of autologous bone grafts, allografts, autoclaved parts of the resected sacrum, or metal prostheses7-17. We report the case of a patient with a large primary neurofibrosarcoma in the sacrum and describe the technique we used for extended total en bloc sacrectomy and primary reconstruction with use of structural tibial allografts. After five years of follow-up, the patient was disease-free and walking independently. Our patient was informed that data concerning the case would be submitted for publication. A thirty-five-year-old woman presented with a six-month history of progressive pain in the lumbosacral region radiating into both legs and episodes of bladder incontinence that had occurred during the latest three months. She had diminished sensation bilaterally in the S1 through S4 dermatomes and weakness in the left lower leg and foot. Signs of cauda equina compressions were present on the left side. An osteolytic lesion in the sacrum was seen on plain radiographs. Magnetic resonance imaging (Fig. 1) showed a large mass in the sacrum penetrating the anterior cortex, infiltrating into …

A case of Gorham-Stout disease of the shoulder girdle and cervico-thoracic spine in a 65-year-old woman is described. The patient presented with progressive neurologic symptoms, pain, and deformities of the cervico-thoracic spine as well as of her left shoulder following a traumatic shoulder luxation. Since the patient had a history of uterine carcinoma, the current disease was clinically difficult to differentiate from osteolytic metastases. The results of the clinical, radiological and histopathologic examination leading to the diagnosis of Gorham-Stout disease are described. Neither attempted surgery nor radiotherapy produced clinical improvement and the patient died 1 year after the first clinical evaluation.

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.

Die Progredienz degenerativer Veranderungen des Huftgelenks nach Epiphyseolysis capitis femoris (ECF) korreliert im Wesentlichen mit dem Alter des Patienten beim Auftreten der Deformitat und dem Ausmas des Epiphysengleitens. Pathogenetisch durften hierbei die veranderten biomechanischen Verhaltnisse des Huftgelenks mit Deformation des koxalen Femurendes und der damit verbundenen Konfliktsituation zwischen Schenkelhalsmetaphyse und ventralem Acetabularrand sein. Beobachtungen des Spontanverlaufs oder nach unkorrigierter “In-situ-Fixation” der Epiphyse zeigen, namentlich bei Gleitwinkeln >30°, weitgehend ubereinstimmend die Entwicklung einer Koxarthrose 20 Jahre nach Epiphyseolysis capitis femoris. Die von G. Imhauser vorgeschlagene intertrochantare Korrekturosteotomie intendiert eine Wiederherstellung der Gelenkkongruenz zur Verminderung der praarthrotischen Deformitat und Senkung der Koxarthroseinzidenz. Zur Reorientierung der vornehmlich dorsal abgeglittenen Femurepiphyse erfolgt eine intertrochantare Flexionsosteotomie, welche die potentielle femoro-azetabulare Fehlkontaktierung reduziert. Auf der Grundlage von Langzeitbeobachtungen wurden Indikation und Resultate nach Imhauser-Osteotomie bei 51 Patienten mit chronischer einseitiger Epiphyseolysis capitis femoris aufgezeigt. Bei den von 1962–1972 operierten und klinisch nach Jerre und Merle d'Aubigne sowie radiologisch nach Bauer u. Kerschbaumer nachkontrollierten Patienten wiesen 55% ein gutes, 28% ein masiges und 17% ein schlecht einzustufendes Resultat nach einer durchschnittlichen Beobachtungszeit von 24 (20–29) Jahren auf. Bei geringen, vornehmlich technisch bedingten Problemstellungen erwies sich insbesondere auch die geringe Gefahrdung vonseiten der Femurkopfvaskularitat als gute therapeutische Option zur Korrektur mittlerer Abrutschgrade. In der Langzeitprognose, welche fur Patienten mit Epiphyseolysis capitis femoris von entscheidender Bedeutung ist, resultieren nach Imhauser-Osteotomie bei mehr als der Halfte der Patienten arthrosefreie Huftgelenke uber 20 Jahre nach Epiphyseolysis capitis femoris, was einer deutlichen Verbesserung der Prognose gegenuber dem “natural course” oder “In-situ-Fixation” entspricht und die Indikation dieser Korrektur des koxalen Femurendes bei Epiphyseolysis capitis femoris unterstreicht.

Proximal femoral focal deficiency (PFFD) types II and III (Pappas) have no femoral or only abortive femoral head development. Winkelmann published a case of one patient treated with a modification of rotation plasty by creating a new articulation after 180° rotation of the lower leg and insertion of the lateral tibial plateau into the deficient acetabulum. This article reports on three consecutive patients treated with this technique to validate this procedure and to consider this operation in selected patients.At the time of rotation plasty the patients (2 boys and 1 girl) were aged between 5 and 10 years. The observation period is between 5 and 9 years after surgery. All patients used an orthoprosthesis with full contact in the foot region and a shaft connecting the thigh. A hip basket and any additional walking support were not necessary (e.g. canes). They were all highly satisfied and would choose the same treatment again.Rotation plasty is a technically highly demanding procedure, especially in situations such as PFFD where the anatomy is distorted and structures such as blood vessels and nerves are too short. However, it is an option to improve the orthoprosthetic fitting functionally and esthetically.

Two consecutive cases of chronic dislocation of the head of the radius after missed Bado type-I Monteggia lesions are presented. Reduction was successfully achieved in both patients after ulnar corticotomy, gradual lengthening and angulation of the ulna using an external fixator. Open reduction or reconstruction of the radio-ulnar capitellar joint was not undertaken. The age at injury was seven years in the older and two years in the younger patient. The time from injury to treatment was five years in the older and three months in the younger child. At follow-up, nine years after completion of treatment in the older and eight months in the younger patient, both show satisfactory movement, function of the forearm and reduction of the head of the radius. This technique may be considered in missed Monteggia lesions before open procedures on the radio-ulnar capitellar joint are undertaken.

Three cases with destructive bone lesions of the distal end of the ulna caused by different pathologic entities (Ewing's sarcoma, osteosarcoma, rheumatoid pseudotumoral synovitis) are presented, all with similar clinical and comparable x-ray and magnetic resonance imaging features. Although the distal end of the ulna may be resected without significant functional impairment, careful evaluation of treatment strategies compatible with oncologic standards is warranted.

BACKGROUND 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. PATIENTS AND METHODS INCLUSION CRITERIA Registration into a completed neoadjuvant COSS-Study. Histologically confirmed, primary, localized, high-grade, central osteosarcoma of an extremity; age 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 ( 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 p < 0.01). 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. CONCLUSIONS 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 selectec 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.

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 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.

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.

Summary 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 chon-drolysis 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 longterm 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 for a normal hip function.

During the dissection of seven club feet of foetuses, aborted between the 25th and 37th week of gestation, an additional muscle bundle was found in a right foot. This muscle arose from both heads of the gastrocnemius muscle, continued downwards onto the posterior surface of the soleus, crossed the calcaneal tendon and assumed a position on the lateral side of the calcaneal tendon. The muscle's tendon was fixed just laterally to the calcaneal tendon in the calcaneal tuberosity with some tiny fibres branching off into the superior fibular retinaculum.

The standard treatment of acute distal femoral fractures consists of open anatomic reconstruction and fixation. Internal fixation may be accomplished with angular plates, condylar screw fixation, antegrade or retrograde nailing. Inveterated condylar fractures are rarely seen in our society and present challenging management problems. A 64-year-old man presented with a pseudarthrotic dislocated multifragmentary supracondylar fracture and a knee flexion contracture two years following the accident, mobilized only with a wheelchair. Because of the severly osteoporotic bone, degenerative articular changes and the flexion contracture, we chose resection of the fragments and implantation of a reconstruction system (MUTARS) instead of osteosynthesis. Eleven weeks postoperatively, the patient was able to walk without crutches. Nine months after surgery, he has flexion up to 90° and full extension, and has regained an active life.

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 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.

Kernspintomogramm (MRI) und Computertomogramm (CT) sind geeignete Methoden zur objektiven Erfassung der Zentrierung nach offener oder geschlossener Reposition einer Huftgelenkluxation und konnen in besonderen Situationen die Arthrographie ersetzen. Bis zur ausreichenden Ossifikation der Huftkopfkerne, d. h. im 1. Lebensjahr, ist dem MRI der Vorzug zu geben. Vom 2. Lebensjahr an ist fur die Beurteilung des Repositionsergebnisses das CT gleichwertig. Beim alteren Kind kann das MRI aber auch indiziert sein fur die Beurteilung der knorpeligen Huftstrukturen oder zur Diagnose einer Femurkopfnekrose. In hoherem Alter konnen durch eine aufwendige CT-Untersuchung die knochernen Verhaltnisse auch dreidimensional dargestellt werden, z. B. zur Planung komplexer Korrekturosteotomien.

We describe two sibs with geroderma osteodysplasticum (GO) who, in addition to the known clinical and radiologic manifestations of the disorder, presented a metaphyseal peg indenting the epiphysis of the long bones, particularly at the knees. The peg was visible only at the age of 4 to 5 years but was invisible in infancy and following physeal closure. This may explain why this anomaly was not described in previous reports of 23 patients in 11 families with GO. The metaphyseal peg is an abnormality of bone development so far unknown to us. We speculate that it represents a primary, age-dependent alteration of bone shape and hence a new genetic bone marker apparently specific to GO. © 1996 Wiley-Liss, Inc.

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 The purpose of this study was to analyze whether hips treated for developmental dysplasia of the hip (DDH) during infancy, which were clinically and radiologically fully normalized by walking age, may become dysplastic again during later growth. Materials and methods A total of 150 patients were randomly selected out of a collective of 386 patients treated for DDH at the Department of Orthopaedics at the University of Zurich between 1993 and 2004. Treatment was started at birth and continued for 6 months. All patients had clinically and radiographically normal hips by walking age. The patients did not suffer from other diseases, in particular, neurological or neuromuscular diseases. Results We detected four female subjects among the 150 patients who had been successfully treated for DDH, who had developed dysplastic hips in early adolescence, necessitating acetabular surgery. Conclusion The successful treatment of DDH in infancy does not ensure normal hip development; therefore, follow up into maturity may be recommended. Level of evidence Level IV.

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

In 11 children (aged 4 weeks to 8 months) with congenital dysplasia of the hip and complete dislocations of the femoral head, magnetic resonance imaging was performed after closed reduction. Gradient echo sequences allowing for a short examination time are well suited for imaging of the articular cartilage and documentation of the position of the femoral head. The use of magnetic resonance imaging decreases the risk that incompletely reduced or redislocated hips after closed or open reduction are retained in this position. Magnetic resonance imaging can be recommended for postreduction assessment of congenital dislocation of the hip in infants and may significantly reduce the need for arthrography.

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.

Purpose. Rotationplasty has proven its efficacy in the treatment of malignant bone tumors of the lower extremity in predominantly young patients. To our knowledge this procedure has not been reported in patients over 60 years before.Materials and Methods. 3 patients over 60 years with an A1-rotationplasty because of a sarcoma were included in this study. Complications and functional results were recorded. In one patient an electromyography was done.Results. Despite electromyography showing good adaptation of the muscles to the altered function, the functional results of these three patients were limited. two out of three patients needed a cane for walking distances over 200 meters. No secondary amputation was necessary.Discussion. Our study demonstrates that rotationplasty is an alternative to an above-knee amputation in older patients but with poorer functional results in comparison to younger patients. However, limb-salvage surgery should be preferred whenever possible.

Fritz Heftia, Gernot Jundtb, Miklos D. Cserhatic, Michel Dutoitd, Gerhard U. Exnere, Andre Kaelinf, Klaus Siebenrockg, Arthur von Hochstetterh a Chefarzt, Kinderorthopadische Universitatsklinik, Universitatskinderspital beider Basel, Basel, b Leitender Arzt, Institut fur Pathologie der Universitat, Basel, c FMH Orthopadische Chirurgie und Traumatologie, Zurich, d Medecin-chef, Hopital orthopedique de la Suisse romande, Lausanne, e Orthopadische Universitatsklinik Balgrist, Zurich, f Medecin-chef, Service d’orthopedie pediatrique, Hopital des enfants, Geneve, g Chefarzt, Klinik und Poliklinik fur orthopadische Chirurgie, Inselspital, Bern, h Pathologie Institut Enge, Zurich

A major problem in lengthening a short femur in proximal focal deficiency of the femur (PFFD) is the development of contractures and dislocation of the hip and knee joint. The knee joint is particularly prone to dislocation because of the cruciate ligament insufficiency associated with PFFD. Axis deviations also need specific attention.In four patients (age 2.5-11 years) with PFFD (PAPPAS class III in one patient and VII in three patients), five femoral lengthenings with mechanical axis corrections were performed by the callotasis technique using a hybrid fixation system (Monotube/Triax) connecting the femur and the tibia with a fixed hinged knee joint to protect the knee against contracture and dislocation.All patients retained their hip and knee function. Hip flexion contracture during lengthening may make inclusion of the hip joint into the fixation system necessary, but was not carried out in the four patients presented.Four consecutive lengthenings of the femur with focal deficiency were carried out under protection by a hinged knee bridging external fixator. A dislocation could be prevented in all patients. We consider that such a system could be used during these lengthening procedures.

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.

Background:: Successful treatment of allograft infections by the temporary implantation of an antibiotic-loaded polymethylmethacrylate cement spacer depends on the diffusion of antibiotics out of the cement and inhibition of bacterial growth in the surrounding tissue. We investigated with an in vitro model how long antibiotics are released by the cement and if gentamicin-resistant coagulase-negative staphylococci (CNS) are inhibited by vancomycin mixed with the gentamicin-loaded cement. Materials and Methods:: Four formulations of antibiotic-loaded cement disks, i.e. gentamicin, tobramycin, vancomycin and tobramycin combined with vancomycin, respectively, were used to test the inhibition of eight isolates of Staphylococcus epidermidis and two reference strains of Staphylococcus aureus by an agar diffusion test on Mueller-Hinton (MH) agar similar to the routine laboratory disk diffusion method. Moreover, cement spacer cylinders loaded with gentamicin alone or combined with vancomycin were submerged in MH agar for weeks and the capacity to inhibit five different isolates of S. epidermidis was measured. Results:: The size of the inhibition zones around the antibiotic-loaded cement disks correlated with the minimal inhibitory concentration (MIC) of the antibiotics against the tested strains. All five strains of S. epidermidis were inhibited by vancomycin-loaded cement spacers for at least 30 days. However, two gentamicin-resistant S. epidermidis strains with MICs of 4 mg/l and 16 mg/l could not be inhibited longer than 3 days by the gentamicin-loaded cement spacer. Conclusion:: The in vitro data suggest that antibiotic-loaded cement spacers inhibit susceptible bacteria for 4-6 weeks. The addition of vancomycin to commercial aminoglycoside-loaded cements might be helpful in allograft infections in tumor patients to inhibit a broad range of bacteria including gentamicin-resistant CNS very commonly found in such infections

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.

Conservative treatment of clubfoot deformities with braces and bandages is traditionally several hundred years old. The clubfoot braces of Venel, Scarpa, and Schulthess, as well as the redressing bandage of Cheselden, are reviewed as typical examples. The treatment of clubfoot deformities was initiated in the Balgrist Hospital for Orthopedic Surgery by W. Schulthess. Recently, in Balgrist, we developed a brace that retains and redresses the foot. This brace is described, and early clinical results are reported.

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.

Three cases with destructive bone lesions of the distal end of the ulna caused by different pathologic entities (Ewing's sarcoma, osteosarcoma, rheumatoid pseudotumoral synovitis) are presented, all with similar clinical and comparable x-ray and magnetic resonance imaging features. Although the distal end of the ulna may be resected without significant functional impairment, careful evaluation of treatment strategies compatible with oncologic standards is warranted.

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.