Biomedical subjects
Publications and source records attributed to G U Exner.
The occurrence and development of the ossification nucleus of the femoral head on X-ray films were studied in 85 children with hip joint dysplasia/dislocation. Delayed development was found to be related to treatment modalities or the severity of the dysplasia. Delayed development of the ossification nucleus of the femoral head is probably of considerable prognostic importance.
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.
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.
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.
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 < 40 years; no pretreatment; interval diagnosis to chemotherapy < or = 3 weeks; no severe comorbidity. Chemotherapy: HD-methotrexate +/- doxorubicin +/- cisplatin +/- ifosfamide +/- BCD. Scheduled local therapy: Surgery. RESULTS: 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-size < 1/3 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 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.
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.
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.
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.
In 15 boys and eight girls with slipped capital femoral epiphysis (SCFE), height, weight, body proportions, and skeletal and sexual maturation were assessed prospectively. A significant preponderance of relatively long legs was found in boys and girls and of relatively high body weights in boys; otherwise, growth and maturation were not different from those of normal children. The time of occurrence of SCFE was most closely related to bone age and the peak of the growth spurt. If one considers growth and maturation the most sensitive indicators of a well-functioning endocrine system, there is no indication to assume in general an endocrine disorder as the cause of SCFE.
In a pilot study, hip development in 615 newborn children was assessed sonographically. Distinct dysplasia was found in 2.3%, and an intermediate "physiologically immature" hip development was noted in 13%. The number of controls and expenses would greatly increase if all physiologically immature hips were followed until they reached maturity. To reduce the number of unnecessary control and to facilitate assessment, we propose the use of "cut-off values" in a screening program for newborns.
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We report a 7-year 6-month-old boy with Legg-Calvé-Perthes (LCP) disease in whom a magnetic resonance imaging (MRI) was normal during the early symptomatic phase when the 99mTc bone scintigraphy showed segmental hypoperfusion of the femoral head suggestive of bone infarction. Only later in the disease did the MRI also show the typical changes of LCP. The follow-up on this patient with bilateral disease leaves no doubt about the diagnosis of LCP. This sequence of a positive scintigram before positive MRI findings may be the exception, but it is important to realize that MRI is not always the most sensitive way to diagnose or exclude LCP as suggested hitherto by the literature.
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.
A multicentric study was conducted by the European Musculoskeletal Oncology Society on 113 cases of resection for tumors of the major long bones and reconstruction with massive allografts, as intercalary diaphyseal or for arthrodesis. The follow-up was at least 12 months. There was a 14% incidence of infection, with a higher frequency (18.5%) in intercalary grafts as compared to arthrodeses (7%). Fracture of the graft occurred in 15.5% of the cases with a prevalence in the humerus (28.5%) as compared to the tibia (21.4%) and femur (14.7%). Fracture constantly occurred after osteosynthesis with screws, in 16% of the cases after osteosynthesis with a plate, and in 12.5% of the cases after osteosynthesis with an intramedullary nail. It was observed in 32% of the grafts preserved at -30 degrees, in 17% of those irradiated, and in 7% and 7.6% of those preserved at -180 degrees and -80 degrees, respectively. Delayed union and non-union was observed in 57% of the cases: 63% in intercalary grafts, 48% in arthrodeses. Final results after treatment of complications were excellent and good in 63% of the entire series. The results should, however, be evaluated after 3 years, as during this period of time important transformation phenomena of the graft occur. Complications are observed in 75% of the cases, but they may be reduced if planning is correct and surgery is accurate. Each complication may be dealt with and resolved without high risks for preservation of the limb.