[Esthetic and functional improvement in proximal femoral focal deficiency with lacking hip joint by rotation plasty Winkelmann type BIII]. / Rotationsumkehrplastik Winkelmann BIII bei proximalem Femurdefekt ohne Hüftgelenkanlage.

BACKGROUND: 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. PATIENTS AND METHODS: 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. CONCLUSION: 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.

The influence of tumor- and treatment-related factors on the development of local recurrence in osteosarcoma after adequate surgery. An analysis of 1355 patients treated on neoadjuvant Cooperative Osteosarcoma Study Group protocols.

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.

Allograft reconstruction for bone sarcoma of the tibia in the growing child.

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.

Additional muscle in idiopathic club foot.

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.

Free vascularised osteocutaneous filet flap for covering, that permitted sensitive terminal weight-bearing by a thigh stump after transfemoral amputation.

We covered a transfemoral amputation stump with a modified free filet flap including the calcaneus. The flap survived, resulting in lengthening of the femur, improved distal weight-bearing, preserved sensitivity of the stump, and the design of a prosthesis with terminal loading of the femur rather than an ischial contained prosthesis.

Localized Ewing tumor of bone: final results of the cooperative Ewing's Sarcoma Study CESS 86.

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 <100 mL in 33% of cases and > or =100 mL in 67%. Local therapy was surgery (23%), surgery plus radiotherapy (49%), or radiotherapy alone (28%). Event-free survival rates were estimated by Kaplan-Meier analyses, comparisons were done by log-rank test, and risk factors were analyzed by Cox models. 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 <200 mL, good histologic response, and VAIA chemotherapy augured for fair outcome. Six of 301 patients (2%) died under treatment, and four patients (1.3%) developed second malignancies. 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.

Uncemented short-length diaphyseal segmental replacement prosthesis fixation--finite element analysis and long-term results.

INTRODUCTION: Extensively porous coated segmental replacement prostheses with intramedullary cementless fixation to bone over the whole length of stem often exhibit resorption of the surrounding bone due to stress-shielding. This makes them particularly susceptible to aseptic loosening. STUDY: A finite element analysis of the state of loading of a short-length fixation in a new prosthetic stem design has shown a definite advantage over long-length fixation. The stress pattern within the bone surrounding the prosthesis confirmed that shortening of the ongrowth area in length increases the stress values at the resection level significantly. This stem (Endlock) has been used for diaphyseal anchorage in the treatment of tumors in combination with an artificial joint of proven design in order to reduce stress shielding. RESULTS: No Endlock stem fractures or aseptic loosenings were observed at recent follow-up. The early clinical results comply with the theoretical assumptions. CONCLUSIONS: A short-length fixation system based on intramedullary anchorage of segmental replacement endoprostheses would possibly support physiologic adaptive processes more than fixation over the full length of the stem.

Metaphyseal peg in geroderma osteodysplasticum: a new genetic bone marker and a specific finding?

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, agedependent alteration of bone shape and hence a new genetic bone marker apparently specific to GO.

Bone densitometry using computed tomography. Part I: selective determination of trabecular bone density and other bone mineral parameters. Normal values in children and adults.

Gamma-ray computed tomography (gamma-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.

Bone densitometry using computed tomography. Part II: increased trabecular bone density in children with chronic renal failure.

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.

Missed chronic anterior Monteggia lesion. Closed reduction by gradual lengthening and angulation of the ulna.

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.

Fractures about the knee: growth disturbances and problems of stability at long-term follow-up.

The aim of this study was to collect information about the incidence of leg-length discrepancy, axis deviation and instability resulting from fractures about the knee in childhood. We reviewed 43 patients with a clinical and radiological examination after a mean follow-up period of 13 years. The fractures were divided into four subgroups, each one presenting typical problems. Growth disturbance (shortening and axis deviation) making secondary procedures necessary was seen in 5 out of 14 (36%) distal femoral epiphyseal fractures. Complex instability was observed in 3 out of 7 (43%) proximal tibial epiphyseal fractures. In the tibial spine fractures anatomical reduction often did not prevent moderate cruciate ligament insufficiency; and in the metaphyseal fractures 2 of the 7 cases showed leg-lenghtening. In conclusion, the Salter classification seems to be of little prognostic value at the knee as Type-II fractures are frequently followed by asymmetric growth arrest. Open reduction is not reliable in avoiding this complication. Associated ligament injuries are not rare and deserve more attention.

Foot function after ray resection for malignant tumors of the phalanges and metatarsals.

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.

Transposition of the radius upon the ulna in transverse forearm deficiency to facilitate later lengthening: a report of two cases.

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.

Prevention of secondary coxarthrosis in slipped capital femoral epiphysis: a long-term follow-up study after corrective intertrochanteric osteotomy.

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.

Reconstruction of the first and second metatarsals with free microvascular fibular bone graft after resection of a Ewing sarcoma.

We report the case of a 12-year-old girl with a Ewing sarcoma originating from the first metatarsal of one foot. Because the girl and her parents refused amputation of the foot, we resorted to complete local resection and to reconstruction of the first and second metatarsals with a free microvascular fibular bone graft. At 3 1/2 years after surgery, the patient continues to walk without a limp, and the mechanical function of the forefoot has been largely restored, although considerable loss of function of the big toe is apparent. Details of the surgical technique and tumor management are described.

Slipped capital femoral epiphysis: a long-term follow-up study after open reduction of the femoral head combined with subcapital wedge resection.

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.

Congenital deformities of the foot.

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.

Osteomyelitis sclerosans Garré.

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 use of massive bone allografts for intercalary reconstruction and arthrodeses after tumor resection. A multicentric European study.

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.