Surgical treatment of neuromuscular scoliosis: current techniques.

41 consecutive patients surgically treated at Our Department by posterior only instrumented fusion from January 1995 to January 2009 were reviewed. There were 20 females and 21 males with a mean age of 15.8 years (range, 10 to 38). Diagnosis was: cerebral palsy (13 cases), Duchenne muscular dystrophy (7), spinal amyotrophy (7), myelomeningocele (5), poliomyelitis (3), Friedreich's ataxia (2), Escobar syndrome (2), Steinert's disease (1), Charcot Marie Tooth disease (1). Main scoliosis Cobb angle averaged 94.05° (range, 34° to 165°), the curve was thoracic in 19 cases, thoracolumbar or lumbar in 22 cases. Kyphosis (T5-T12) averaged 42.86° (range, 7° to 90°), lordosis was 33.57°. The fusion was extended to the lumbar tract in 23 patients, to the sacrum in the other 18. Our results showed that, in patients with neuromuscular scoliosis, posterior instrumented fusion is a safe and effective procedure and is the treatment of choice for patients with limited respiratory function, as in Duchenne muscular dystrophy and spinal muscular atrophy. The surgery should be performed as early as possible, and the extension of the fusion to the sacrum should be avoided in patients with residual walking ability.

Surgical treatment for scoliosis associated with rare disease.

This is a retrospective study of 11 patients, 7 females and 4 males, treated at Our Department for an early onset scoliosis (EOS) associated with rare syndromes with growing spinal implants (Growing Rod or VEPTR-like) from 2006 to 2011. Mean follow-up was 24 months (range, 12 to 36). The mean age at surgery was 7. Patients were affected by Escobar's syndrome (1), scoliosis associated to congenital heart disease (1), Arnold Chiari type 1 (1), syringomyelia (1), NF 1 (2), Prader-Willi syndrome (1), trisomy 8 (1), arthrogryposis (2) and spondylo-rib dysplasia (1). Each patient was studied from the genetic point of view, and were performed: brain-spine MRI, pulmonary function tests, Cardio-US and abdominal US, neuropsychiatric and neurological evaluation, C0-C2 CT scan. After first implant and lengthening procedures (11), the correction of the thoracic curve averaged 50%. Unfortunately, a little loss of correction of the lumbar curve occurred during the follow up. There were 8 post-operative complications, that required revision surgery in 2 cases. Our results confirmed the effectiveness and safety of growing spinal implants in the treatment of early-onset scoliosis in rare syndromes.

Complications incidence in the treatment of early onset scoliosis with growing spinal implants.

Early onset scoliosis (EOS) surgery based on growing spinal implants can lead to several complications. Aim of the study was to identify strategies to prevent those complications. A retrospective review was conducted to identify all pediatric patients affected by EOS surgically treated with growing rod or Vertical Expandable Prosthetic Titanium Rib (VEPTR) at our division between 2006 and 2011. Nineteen consecutive patients (8 males, 11 females; mean age 6.8 years) were included. The scoliosis was: idiopathic in 7 cases, congenital in 5, associated with congenital heart disease in 2, with syringomyelia and Arnold Chiari syndrome in 1, with neurofibromatosis type 1 (NF1) in 1, with Prader Willi syndrome in 1, with trisomy 8 in 1, with arthrogryposis in 1. Instrumentation used was: growing rod in 9 patients (dual rod construct in 8 cases, single rod in 1), VEPTR in 10 (always rib to spine construct). At a mean follow-up of 28 months (range, 12 to 55) 12 mechanical complications occurred in 8 of 19 patients treated (42.1%). Among cases treated with growing rod (9) 6 complications occurred in 4 patients (44.4%): revision was performed in 4 cases due to proximal anchors migration, in 2 cases due to a rod breakage. Among cases treated with VEPTR (10) 6 complications occurred in 4 patients (40%): revision was performed in 4 cases due to rib fracture with anchors migration, in 1 case due to vertebral anchor migration and in 1 case due to proximal and distal anchor migration. So, in our series mechanical complications rate was 42.1%. Our strategy to prevent these complications is to use hooks as proximal anchors, to avoid single rod construct and to use a brace as external support until final surgery is performed. If it's possible, is better to substitute VEPTR with a dual Growing Rod implant when patient's age and anatomy permits this.

Experimental realization of optimal noise estimation for a general Pauli channel.

We present the experimental realization of the optimal estimation protocol for a Pauli noisy channel. The method is based on the generation of 2-qubit Bell states and the introduction of quantum noise in a controlled way on one of the state subsystems. The efficiency of the optimal estimation, achieved by a Bell measurement, is shown to outperform quantum process tomography.

Thoracoplasty in the surgical treatment of adolescent idiopathic scoliosis.

A consecutive series of 40 adolescents surgically treated between 1998-2001, by posterior spinal fusion and thoracoplasty were compared with a similar group of 40 adolescents treated in the same period by posterior only segmental fusion. Clinical and radiographic analysis was performed, including the SRS-30 questionnaire and Pulmonary Function Tests (PFT). Minimum five years follow-up was requested. No statistical differences were found between the two groups in PFT's both pre-operatively and at latest follow up. Our findings suggest that thoracoplasty did not adversely affected long-term PFT's in AIS patients treated by posterior spinal fusion alone.

Effects of exposure to air pollution on blood coagulation.

BACKGROUND: Consistent evidence has indicated that air pollution increases the risk of cardiovascular diseases. The underlying mechanisms linking air pollutants to increased cardiovascular risk are unclear. OBJECTIVES: We investigated the association between the pollution levels and changes in such global coagulation tests as the prothrombin time (PT) and the activated partial thromboplastin time (APTT) in 1218 normal subjects from the Lombardia Region, Italy. Plasma fibrinogen and naturally occurring anticoagulant proteins were also evaluated. METHODS: Hourly concentrations of particulate (PM10) and gaseous pollutants (CO, NO2, SO2, and O3) were obtained from 53 monitoring sites covering the study area. Generalized additive models were applied to compute standardized regression coefficients controlled for age, gender, body mass index, smoking, alcohol, hormone use, temperature, day of the year, and long-term trends. RESULTS: The PT became shorter with higher ambient air concentrations at the time of the study of PM10 (coefficient = -0.06; P < 0.05), CO (coefficient = -0.11; P < 0.001) and NO2 (coefficient =-0.06; P < 0.05). In the 30 days before blood sampling, the PT was also negatively associated with the average PM(10) (coefficient = -0.08; P < 0.05) and NO2 (coefficient = -0.08; P < 0.05). No association was found between the APTT and air pollutant levels. In addition, no consistent relations with air pollution were found for fibrinogen, antithrombin, protein C and protein S. CONCLUSIONS: This investigation shows that air pollution is associated with changes in the global coagulation function, suggesting a tendency towards hypercoagulability after short-term exposure to air pollution. Whether these changes contribute to trigger cardiovascular events remains to be established.

An innovative diagnostic procedure of vertebral deformities without exposure to X-rays.

The objective of the study was to compare standard manual X-ray measurements of vertebral deformities and values obtained from the Ortelius 800. 52 Patients (41 females and 11 males: mean age 20.35 years) with adolescent vertebral deformities, was studied. The patients were evaluated with standard radiographic views and the Ortelius 800. The parameters considered for the comparison were the angles of scoliosis and kyphosis and the values of global axial deformity, shoulder asymmetry and pelvic tilt. We also evaluated the modification of pelvic/shoulder angle after surgery (this parameter allows to evaluate vertebral rotation and can be derived only from the Ortelius 800). This study allowed us to conclude that there is a perfect agreement between measurements with the Ortelius 800 and those resulting from standard x-rays. The system has also proved to have the capability of quantifying changes in vertebral rotation.

Osteosarcoma of the pelvis.

AIMS: To describe the outcomes of a large number of patients with pelvic osteosarcoma, and to define the guidelines for appropriate treatment. METHODS: We reviewed 60 consecutive patients with primary pelvic high-grade osteosarcoma. The tumour involved the whole hemipelvis in 15 cases, while the most common location was the iliac wing in 29 cases (48.3%): 25 of these adjacent to or passing the sacroiliac joint. RESULTS: Thirty patients underwent surgery; there were 16 hindquarter amputations and 14 internal hemipelvectomies. All the patients who presented with metastasis died of their disease. In 18 cases wide margins were achieved, however, eight patients experienced local recurrence. Of the series, only eight patients are still alive. CONCLUSION: The use of intense chemotherapy and surgical wide margin, hardly seems to achieve local control, however, tumour necrosis was correlated with positive prognosis. When internal hemipelvectomy it is not safe enough, amputation must be considered, particularly for cases with sacrifice of the sciatic nerve roots or for older patients where a shorter surgical procedure can be less risky.

Osteoid osteoma simulating an osteocartilaginous exostosis.

We describe a case of osteoid osteoma in the tibia of a 3-year-old patient who presented with a clinical and radiographic picture that suggested an exostosis. The formation of osteoid osteoma with a radiographic picture similar to that of osteophytes or exostosis has been previously documented only rarely. The authors hypothesize that the exostosis-like formation observed was actually the calcification of soft tissues that formed after the intense periosteal inflammatory reaction caused by the osteoid osteoma. As a result of its peculiar clinical and radiographic presentation, diagnosis of this lesion was delayed. Being located close to the medial growth plate of the tibia, it caused lengthening of the limb with a pronounced valgus deviation of the knee. An excisional biopsy provided histological evidence, clinical resolution and immediate pain relief, but incomplete resolution of the valgus deformity of the knee.

Long-term roentgenographic evaluation of proximal femur prosthesis after tumor resection.

The need for a better roentgenographic evaluation of implant behavior in long-term prosthetic replacement of the proximal femur was considered. The authors present the results of a group of 13 patients belonging to a wide range of ages, with more than ten-years follow-up after tumor resection, and replacement characterized by bipolar cup and non-cemented stem. A new X-ray film acquisition and evaluation method was devised. Results confirmed the good behavior of bipolar reconstructions in patients of different ages, whose sockets were still intact. In younger patients, the prevalent bipolar movement was in the outer bearing, thus limiting polyethylene particulate debris formation. However, debris formation did not significantly affect the bone/stem relationship as only one loose stem was observed in a bone submitted to radiotherapy.

Neoadjuvant chemotherapy for osteosarcoma of the extremities with metastases at presentation: recent experience at the Rizzoli Institute in 57 patients treated with cisplatin, doxorubicin, and a high dose of methotrexate and ifosfamide.

BACKGROUND: Effective adjuvant or neoadjuvant regimens of chemotherapy have dramatically improved the prognosis of patients with high-grade osteosarcoma of the extremity, localized at diagnosis. Currently, little is known about patients with metastatic disease at presentation. PATIENTS AND METHODS: From May 1995 to May 2000, 57 patients with osteosarcoma of the extremity, metastatic at presentation, were treated according to the following scheme: primary chemotherapy, restaging, simultaneous resection of primary tumor and metastatic lesions, and maintenance chemotherapy. RESULTS: Thirty-five patients achieved remission. At a follow-up ranging from 2 to 7 years, seven remained continuously free of disease, one died of chemotherapy-related toxicity and 27 patients relapsed. Twenty-one of the 22 patients who never achieved remission died as a result of the tumor, as well as 20 of the 27 who achieved remission but then relapsed. Of the remaining seven relapsing patients, six are alive with uncontrolled disease, while one is alive and free of disease 24 months after the last post-relapse treatment. Two-year event-free survival (EFS) and overall survival (OS) were 21% and 55%, respectively. These results are significantly poorer than those achieved in 128 contemporary patients with non-metastatic disease at presentation, treated with the same chemotherapy protocol (2-year EFS and OS of 75% and 94%, respectively). CONCLUSIONS: The results of our study confirm that the prognosis of patients with osteosarcoma of the extremity, metastatic at presentation, remains poor, despite the use of aggressive treatments.

The results of the surgical treatment of bone tumors using massive homoplastic grafts.

We reviewed our experience as concerns 588 patients treated by resection for bone tumor and reconstructed by massive bank bone implant. The period taken into consideration goes from 1984 to 2001. The mean age of the patients was 24 years with a minimum of 1 and a maximum of 77 years: in 186 cases (31.6%) age was lower by 14 years. Most of the patients were referred for a diagnosis of malignancy (85%), and of these, 70% had undergone antiblastic therapy postoperatively according to the modalities of the protocols. Of the cases that were not treated by chemotherapy there are 16 that had a homoplastic graft after failure of a previous one. In a high number of cases (189) surgery included reconstruction of an intercalar segment of a long bone. Of these, 106 were in the femur, 69 in the tibia, and 14 in the humerus. One joint reconstruction was carried out in 128 cases of which 52 in the knee, 49 in the shoulder, 10 in the elbow, 11 in the wrist, 3 in the ankle, and 3 in the metatarsals. Fusion, which was one of the most frequent indications in the eighties, is used less today; overall it was carried out in 91 cases of which 65 in the knee, 14 in the hip, 9 in the ankle and 3 in the wrist. This type of implant may also be used in the pelves, and this took place in 56 cases using different procedures of reconstruction. The most current method of reconstruction for a joint is composite prosthesis, and this was used in 124 cases of which 67 around a knee, 53 in the hip, 4 in the shoulder. Finally, in 78 cases a vascularized fibula transplant was associated with an intercalar graft, fusion, or osteoarticular graft to increase the mechanical capacity of the implant together with a more rapid integration of the implant, particularly at the osteotomic lines. Despite the excellent results demonstrated in the first 3 years of follow-up, infection, resorption, delay in consolidation and fracture led to 18% failures of the implant. Massive homoplastic graft is still a good means of reconstruction that allows for excellent functional results, but despite this intense research activity is required to improve its mechanical hold in time together with better and complete integration.

Knee resection arthrodesis with allograft: a long-term follow-up study.

A consecutive series of 57 patients treated by knee resection arthrodesis for malignant or aggressive tumor around the knee was reviewed. Infection was present only after repeated surgery for other complications, delayed union or non-union occurred in 50% of the cases that could be evaluated, but were still easy to manage. Fracture incidence was higher than expected (32.6%) even occurring after 10 years; this was difficult to deal with and it often led to failure. The best possible method of fixation is still being debated, but locked nail and allograft cementation is often advised. Several satisfactory functional results were however achieved when surgery was performed in young patients; final results can be less satisfactory when there is leg length discrepancy and poor acceptance on the part of the patient. In recent years this type of surgery has been limited to younger male patients (10 to 14 years of age) in whom extra-articular knee resection was required or when most of the quadriceps muscle must be removed.

A computer assisted approach for monitoring and planning biological reconstructions in orthopaedic oncology: preliminary results and future perspectives.

The evaluation of the mechanical strength of a reconstructed bone, and its evolution during the follow-up is, at present, almost impossible. This information, however, may prove extremely useful in clinical practice, both in the surgical planning and in the management of the rehabilitation therapy. In this work, a non-invasive technique, based on the use of finite element modelling, is presented that allows the simulation of the mechanical behaviour of a skeletal reconstructions starting from Computed Tomography data. This method was applied to study the evolution, during the first year of follow-up, of the strength of the reconstructed femur of a ten year-old child if a short, slow, level walk were allowed. The preliminary results indicate that the window sculpted by the surgeon to allow the anastomosis of the vascular pedicle acts as a stress concentrator and that the reduced bone mineral density, induced in the child's bone by the absence of load during the post-operative period, increases the stress level in the proximal femur.

Neoadjuvant chemotherapy for Ewing's tumour of bone: recent experience at the Rizzoli Orthopaedic Institute.

The results achieved in 157 patients with non-metastatic Ewing's sarcoma of the bone treated at a single institution between 1991 and 1997 according to a new protocol (REN-3) are reported. Induction chemotherapy consisted of two cycles of 'VAC': vincristine (V), doxorubicin (A), cyclophosphamide (C) alternated with one cycle of 'VIAc': V, ifosfamide (I), actinomycin (Ac). After local treatment, patients received three more cycles of VAC, two of VIAc, three cycles of I plus etoposide (E) and two cycles with V, C and Ac. Local treatment was surgery in 53% of patients, surgery+radiotherapy in 25% and radiotherapy only in 22%. With a follow-up ranging between 4 and 10 years (mean: 7 years), 110 patients (70%) remained continuously event-free, 2 patients died of toxicity and 45 patients relapsed: 33 due to metastases and 12 due to local recurrence always associated with metastases. The 5-year event-free survival (EFS) and overall survival (OS) were 71.0 and 76.5% respectively. These results are significantly better that the ones achieved in our previous three studies in which a three-drug VAC regimen (REA-1), and 4-drug VACAc regimen (REA-2 and REN-1) was used, and in our most recent study (REN-2) which was based on a six-drug regimen as in the present study, but where I and Ac were used only after the local treatment. However, since REN-3 surgery was used in a significantly larger number of patients, we cannot say whether the better outcome of this study was due to the use of a six-drug regimen with an early delivery of ifosfamide and actinomycin, or to the wider use of surgery as local treatment or both.

Allograft arthrodesis treatment of bone tumors: a two-center study.

The current study consists of an outcome review of a consecutive series of 92 patients with knee arthrodesis using an allograft, done for malignant or aggressive tumors in two centers on different continents during a period of 18 years (mean followup, 5 +/- 4 years). The data were compiled by creating a computerized file using the information provided by both centers. Seventy-five of the patients (81%) had high-grade nonmetastatic tumors (Stage II), mostly osteosarcoma. In addition seven (8%) had metastases at outset (Stage III) and the remaining 10 (11%) had benign disease, mostly giant cell tumor or revision procedures for failed allograft or total joint replacement. Seventy-two patients (78%) had distal femoral lesions (78%) whereas the proximal tibia was the site of the tumor in 20 patients (22%). The average age of the patients was 23 +/- 16 years; 51 were males and 41 were females. Tumor complications were a major problem for patients in the series. Thirty-four percent of the patients died, 47% had metastases develop, and 9% had a local recurrence. Allograft complications included an infection rate of 20%, a fracture rate of 25%, and a nonunion rate of 44%. Repeat surgery was required for more than 50% of the patients with 26 requiring one additional operation, 11 requiring two, and 10 requiring three or more operations. Nineteen of the patients required an amputation (20%), only four of which were for recurrent tumor. When these data were compared with data for a control series of 880 patients with allografts other than allograft arthrodeses, the complications were greater and the outcome less successful, suggesting that other approaches should be considered unless there are special indications for this procedure.

Knee arthrodesis with a temporary spacer performed in malignant tumor around the knee.

The issue of utilizing a temporary cement spacer after a knee resection arthrodesis of a highly malignant bone tumor is discussed. In our experience, this is the first type of reconstruction performed in the early days of the limb salvage. Now, after more than 12 years of follow-up based on the results of 84 patients, the usefulness and mechanical performance of this type of reconstruction are considered. Of these patients, 45 died of disease. Wound complications were present in 31% of the patients and led to amputation in 7 and healing failure in 6. Mechanical complications occurred in 39%, but radiographic analysis of the implant showed 32 cases (38%) with an inadequate spacer. Only 9 patients are still walking on the original reconstruction, 6 of them uncomplicated (the remaining 3 had rod migrations and shortening). The survival curve showed no statistical difference between the two groups (adequate vs inadequate spacer). In 33 patients who are still alive and retain their limb, the final functional results were excellent in 6, good in 8, fair in 14, and poor in 5. This type of reconstruction can still be considered in developing countries, or when a definitive arthrodesis is planned where there is a lack of a bone source (children, no bone bank). In our institution, reconstruction with cement is still suggested for patients with a large tumor around the knee, when the quadriceps is completely sacrificed, and the patient prognosis is markedly unfavorable.

Fracture of the lesser trochanter.

Isolated fracture or detachment of the lesser trochanter is an infrequent occurrence. In most cases the event is part of a complex of fractures involving the femoral neck and the greater trochanter. Isolated fracture caused by direct trauma is rare because of the anatomical location of the lesser trochanter which is protected anteriorly and posteriorly by large muscular masses, superiorly by the head and the neck of the femur, laterally by the femur itself, and medially by the ilio- and ischio-public branches of the pelvis. Trauma is usually indirect, caused by sudden traction by the iliopsoas muscle on the femoral tendinous insertion. The highest frequency is observed in young patients who are still growing where there is an imbalance between muscular strength and resistance of the osteochondral plate of the tendinous insertion. Particularly affected are adolescent athletes of male sex aged from 13 to 17 years. Similar overloading of traction in an adult would probably produce only muscular stretching. Diagnosis is based on radiographic ascertainment, obtained with the thigh in extra-rotation, supported by rather typical clinical findings such as acute pain in the inguinal region and in Scarpa's triangle, limping, passive movement of the hip in all directions with pain in maximum extension and relief when seated. Nonsurgical treatment with the limb resting in flexion, further confirmed by the case presented in this study, still remains the treatment of choice in most cases, obtaining excellent functional results. The authors believe that it was of interest to report this clinical case because of the rareness of the pathology observed and because of the specific features of its etiology.

Predictive factors of disease-free survival for non-metastatic osteosarcoma of the extremity: an analysis of 300 patients treated at the Rizzoli Institute.

BACKGROUND: To identify predictive factors of disease-free survival (DFS) in patients with non-metastatic osteosarcoma of the extremity, treated with primary chemotherapy and delayed surgery. PATIENTS AND METHODS: The relationship between patient-related and treatment-related factors and prognosis was evaluated in 300 patients treated from 1986 to 1992 according to chemotherapy protocols based on high-dose methotrexate, cisplatin and doxorubicin, with the addition of ifosfamide in the post-operative phase. Univariate and multivariate analyses of prognostic factors for disease-free survival were performed. RESULTS: With a median follow-up of 9.2 (4.4-12) years, eight-year DFS was 59% (95% confidence interval (95% CI): 54-64.9). Univariate analyses showed that tumor volume > or = 150 ml (P = 0.002), histologic subtype (P = 0.028), age > 12 years (P = 0.044), high serum lactate dehydrogenase (P = 0.044) and alkaline phosphatase (P = 0.064) levels adversely affected DFS. Gender of patients and site of tumor did not influence DFS. No differences in DFS were found among the three chemotherapy protocols, whereas the use of limb-sparing surgery vs. amputation or rotation plasty (P = 0.006) and a good histologic response to primary chemotherapy (P = 0.014) positively correlated with DFS. After multivariate analyses, tumor volume > or = 150 ml (P = 0.028), age > 12 years (P = 0.051), and histologic subtype (P = 0.052) retained prognostic significance. CONCLUSIONS: In patients with non-metastatic osteosarcoma of the extremity treated with neoadjuvant chemotherapy, the disease-free survival is significantly influenced by tumor volume, age, and histologic subtype.

Relationship between dose-intensity of treatment and outcome for patients with osteosarcoma of the extremity treated with neoadjuvant chemotherapy.

One hundred and forty-four patients with osteosarcoma of the extremity treated with neoadjuvant chemotherapy at the authors' institution between 1986 and 1989 were retrospectively analyzed to evaluate the relationship between the dose-intensity of chemotherapy actually received (RDI) and the prognosis. Preoperative chemotherapy consisted of high-dose methotrexate i.v., cisplatin i.a., and doxorubicin i.v. After surgery "good responder" patients (90% or more tumor necrosis) had a 31-weeks of chemotherapy with the same drugs, while "poor responder" patients (less than 90% tumor necrosis) received a 40 weeks treatment with ifosfamide and etoposide added to the three drugs used preoperatively. Due to delays and dose-reductions, only 17 patients (12%) received the treatment exactly as scheduled by the protocol, 66 (46%) received a dose-intensity between 90 and 99%, and 61 (42%) a dose-intensity between 63 and 89%. At a follow-up ranging between 10 and 13 years, 97 patients (67%) remained continuously free of disease, 45 relapsed, and two died of doxorubicin-induced cardiopathy. The continuous disease-free survival (CDFS) was not related to patients' gender and age, tumor histology, site and size, serum value of alkaline phosphatase, type of surgery and histologic response to chemotherapy. According to the RDI, CDFS resulted significantly higher for those 81 patients who received 90% or more of the scheduled dose-intensity than for those 61 who had less than 90% of the scheduled dose-intensity (76.5% v.s. 57.3%; p<0.02). These results seem to suggest that in neoadjuvant treatment of osteosarcoma the dose-intensity of chemotherapy is crucial for outcome, therefore every effort should be made to avoid reductions of doses and/or delays in performing the cycles of chemotherapy.