IntroductionA spinal deformity is almost never observed in children with Duchenne muscular dystrophy (DMD) who can walk [48], and therefore its onset is usually registered between the ages of 12 and 13 years and rarely before the age of 11.Numerous factors are responsible for the onset of the deformity. According to Gibson and Wilkins [22] the main cause of spinal deformity is the paralysis of the extensor muscles.Rideau [44], Siegel [48] and Dubousset [10,11] considered the inclination of the pelvis to be the major cause of the deformity -in particular the asymmetry of the scar retractions at level with the lower limbs. Ledoux [30] foAbstract Background. Surgical treatment of spinal deformities in Duchenne muscular dystrophy (DMD) is influenced by a number of factors which have proven to be a difficult challenge. Each case should be carefully evaluated, considering not only the natural history of the spinal deformity, but also the patient's general condition. These should be thoroughly assessed through clinical and radiographic investigations together with other medical specialists. Life expectancy should be determined according to the cardio-respiratory function, and both preoperative and postoperative quality of life should be taken into consideration, trying to imagine the functional status of each patient after surgery. Methods. From February 1985 to February 2000, 58 patients with spinal deformity in DMD were surgically treated. Of 25 patients that were operated on between 1985 and 1995, only 20 were followed-up after 5 years because 5 of them had died during this time. Therefore, the present study focuses on the results obtained in 20 cases. The 20 cases reviewed presented with a mean angular value of scoliosis equal to 48°(range 10-92°). Spinal fusion with our modified Luque technique [6] was performed in 19 cases, whereas CD instrumentation was applied in only one case. Results. At the 5 year follow-up (range 5.6-10 years), the age ranged from 18 to 24 years and averaged 20.4 years. The postoperative angular value of scoliosis averaged 22°(58%, range 0-43°), the mean correction at follow-up was 28°(range 0-60°), and the mean loss of correction was equal to 6°(range, 0-11°). Vital capacity showed a slow progression, slightly inferior to its natural evolution in untreated patients. The severest complication was the death that occurred in one of the patients. Conclusions. According to the present study, an early surgery (angular value lower than 35-40°) dramatically reduces the rate of risk factors associated with spinal deformities in DMD, and its advantages far exceed the disadvantages, above all in terms of quality of life.
From 1950 to 1994, 257 cases of benign bone tumors of the foot were treated at the Bone Tumor Center of the Rizzoli Institute. Aneurysmal bone cysts (ABC) and giant cell tumors (GCT) are rare and often they are misdiagnosed. To define the clinical and radiographic features useful for diagnosis, x-rays of 24 ABCs and 21 GCTs of the foot were reviewed. Adding our series to the cases reported in the literature, the only important clinical data that emerged was the average age of patients affected by ABC and GCT (15 and 27 years, respectively). ABCs localized in small tarsal bones were rare (6%), whereas 19% of GCTs were found in this site. The eccentric, round shape of a lytic lesion was more characteristic of GCT. An aggressive pattern of radiographic bone destruction was observed more frequently in GCT than in ABC (P = 0.01). Septation was seen in both tumors. Typical of ABC was a sharp and trabeculated margin, whereas GCT had an ill-defined edge with normal cancellous bone (P = 0.007). The growth of ABCs often expanded the cortex, while GCTs eroded, thinned, and broke the cortical bone (P = 0.001). A saucerized cortex was observed only in ABCs. A bony shelf was more characteristic of ABC than of GCT (P = 0.002). Pathologic fractures (67%) and joint involvement, evaluated by computed tomography and magnetic resonance imaging (83%), were more frequent in GCTs, whereas fluid levels were more frequently observed in ABCs (47%). The majority of the tumors were stage 2, but GCTs represented 70% of stage 3 aggressive lesions.
A 65-year-old male patient with an 8-year history of poliostotic Paget's disease complained of shoulder pain that started 6 months prior to admission. An extensive lytic area was identified in the right proximal humerus along with Paget's disease. There was cortical destruction and a soft tissue mass. Following an incisional biopsy, a diagnosis of grade 2 chondrosarcoma associated with Paget's disease was made. The histologic identification of chondrosarcoma associated with Paget's disease is rare. However, the presence of a calcified matrix in a destructive lesion associated with Paget's disease should alert the radiologist and the pathologist to the possibility of a chondromatous differentiation taking place in the sarcoma associated with Paget's disease. The histologic evaluation of the lesion will form the basis for the diagnosis.
Background: Loose bodies are frequently encountered during clinical activity and a common finding during knee arthroscopy. Usually, treatment consists of the removal of the loose bodies, which can be challenging even for experienced surgeons. The excision alone is not always the complete treatment, because loose bodies are generally secondary to other diseases that can cause persistent symptoms with the risk of new loose body formation. The aim of this narrative review is to show the clinical, imaging and arthroscopic evaluation of loose bodies in order to plan optimal treatment. Methods: A comprehensive search of PubMed was conducted to find the most recent and relevant studies investigating the aetiopathogenesis, the assessment tools and the therapeutic strategies for knee loose bodies and their related diseases. Results: When dealing with a loose body, the first issue is the evaluation of the intra-articular fragment (location, size, number, symptoms) and its aetiopathogenesis by identifying the underlying pathology (e.g., osteochondritis dissecans, osteoarthritis, chondral defect, tumour-like lesions, rheumatoid arthritis, etc.). In the case of symptomatic intra-articular loose bodies, treatment consists of fragment removal and the management of related diseases (e.g., lifestyle modification, physiotherapy, pharmacological and surgical treatment). Conclusion: Loose bodies are not separate entities and in addition to their pathological aspect, must be evaluated within the context of the underlying disease. Correct assessment and comprehensive management allow for relief of symptomatology and prevention of loose body formation by removal and treatment of the associated diseases.
Knee osteonecrosis is a debilitating progressive degenerative disease characterized by subchondral bone ischemia. It can lead to localized necrosis, tissue death, and progressive joint destruction. For this reason, it is essential to diagnose and treat this disease early to avoid subchondral collapse, chondral damage, and end-stage osteoarthritis, where the only solution is total knee arthroplasty. Three types of knee osteonecrosis have been documented in the literature: spontaneous or primitive, secondary, and post arthroscopy. Spontaneous osteonecrosis is the most common type studied in the literature. Secondary osteonecrosis of the knee is a rare disease and, unlike the spontaneous one, involves patients younger than 50 years. It presents a particular set of pathological, clinical, imaging, and progression features. The management of secondary osteonecrosis is determined by the stage of the disorder, the clinical manifestation, the size and location of the lesions, whether the involvement is unilateral or bilateral, the patient’s age, level of activity, general health, and life expectancy. This review aims to present the recent evidence on treatment options for secondary osteonecrosis of the knee, including conservative treatment, joint preserving surgery, and knee replacement.
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