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The treatment of progressive coxa vara in children with bone softening disorders.

Fractures of the proximal femur frequently occur in children with osteogenesis imperfecta or fibrous dyplasia and may lead to progressive coxa vara and a "shepherds crook" deformity. We have found existing methods of fixation of the femoral neck in small children to be unsatisfactory. We have devised a method of fixation using a small signal arm nail. With a mean follow-up of 9.3 years there was a successful outcome in all 11 hips treated.

Bone Nails↗

Excisional biopsy for bone tumours.

Compartmental muscular resections without open biopsy is a common procedure for soft tissue tumours suspected of malignancy. In bone tumours, where the diagnosis is supposed to be sarcoma, an excisional biopsy is seldom possible without severe recontructive problems and it may be unnecessarily mutilating should the tumour be benign. For the fibula, the clavicula, metatarsal and metacarpal bones, the distal third of ulna and the proximal third of radius, however, excisional biopsy as the primary procedure should be taken into account. The resulting loss of function is minor and can well be accepted even if the tumour turns out to be benign. On the other hand, if it is malignant as supposed, the radical excisional biopsy saves the patient from amputation. When incisional biopsy is used instead of excisional biopsy the definite surgery has to be made much wider and will often be mutilating. A case of chondrosarcoma illustrates the advantage and the disadvantage of this principle as well as an unusual reconstruction.

Adult↗

Chest wall tumors.

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Bone Neoplasms↗

Bone disease in long-term dialysis.

We reviewed the clinical features, laboratory findings, and skeletal abnormalities of six patients who have been on dialysis therapy for 12 to 20 years (average, 16.7 years). Hemodialysis has been the major therapeutic modality in five of the six individuals. Ages of the patients ranged from 28 to 67 years (mean, 44.5). Bone biopsy specimens were available for five of the patients. In three of these, the predominant changes in bone were those of osteitis fibrosa. In two patients with severe osteitis fibrosa, there was a decrease in height. Two patients had predominantly osteomalacia, but in one of these, the condition was noted before the initiation of 13 years of chronic ambulatory peritoneal dialysis, supplanted 2 years ago by hemodialysis. Total parathyroidectomies with implants of parathyroid tissue in the forearm were performed in four of the six patients. All four had, and continue to have, markedly elevated levels of parathyroid hormone (PTH). Symptoms and signs of amyloidosis of bone have occurred in all six patients, including carpal tunnel syndrome in two, bone cysts in three, and presumptive tendon involvement of the shoulder in one patient. The pathogenesis of hyperparathyroidism is described briefly, and appropriate treatment is summarized.

Adult↗

Renal osteodystrophy in dialysis patients: diagnosis and treatment.

This article reviews the clinical, biological, radiological, and pathological procedures and their respective indications for the practical diagnosis of the following various histological patterns of renal osteodystrophy: osteitis fibrosa due to parathyroid hormone (PTH) hypersecretion: osteomalacia or rickets due to native vitamin D deficiency and/or aluminum overload; and adynamic bone disease (ABD) due to aluminum overload and/or PTH secretion oversuppression. Our advice regarding bone biopsy is to restrict it to patients with symptoms and hypercalcemia, especially those who have been previously exposed to aluminum. In other cases, we propose relying merely on the determination of the plasma concentrations of calcium, protide, phosphate, bicarbonate, intact PTH, aluminum, 25(OH)D3, and alkaline phosphatase (total and bony if hepatic disease is associated) to choose the appropriate treatment. Because of the danger of the desferrioxamine treatment necessary to chelate and remove aluminum, the suspicion of aluminic bone disease (osteomalacia or ABD) will always be confirmed by a bone biopsy. In the case of nonaluminic osteomalacia, correction of the vitamin D deficiency by native vitamin D or 25(OH)D3, and of the calcium deficiency and acidosis by alkaline salts of calcium and if necessary sodium bicarbonate are sufficient to cure the disease. In the case of nonaluminic ABD, the stimulation of PTH secretion by the discontinuation of 1alpha hydroxylated vitamin D and the induction of a negative calcium balance during dialysis by decreasing the calcium concentration in the dialysate will allow an increase of the CaCO3 dose to correct for hyperphosphatemia without inducing hypercalcemia. For hyperparathyroidism, i.e., plasma intact PTH levels greater than two- or four-fold the upper limit of normal levels (according to the absence or presence of previous aluminum exposure), the treatment will consist in increasing the CaCO3 dose to correct for hyperphosphatemia together with a decrease of the calcium concentration in the dialysate if the dose of CaCO3 is so high that it induces hypercalcemia. When the hyperphosphatemia has been corrected and there is still a low or normal corrected plasma calcium level, 1alpha(OH)D3 in an oral bolus 2 or 3 times a week should be given at the minimal dose of 1 microg. When the PTH level stays above 400 pg while hypercalcemia occurs and hyperphosphatemia persists, surgical subtotal parathyroidectomy is recommended or the injection of calcitriol into the big nodular hyperplastic parathyroid glands under sonography control in high surgical risk patients. Special recommendations are given for children.

Adult↗

[Bone tumors and tumor-like changes in the neonatal period and in infancy].

Bone tumours and tumour-like lesions occurring in newborns and infants are described presenting 5 own cases and a literature review. Age specific features in diagnosis, therapy and prognosis are discussed. Due to the rarity of these diseases a centralized registry at a national level is recommended.

Bone Cysts↗

Benign fibro-osseous diseases of the maxillofacial bones. A review and differential diagnosis.

Benign fibro-osseous lesions (BFOLs) of the maxillofacial bones represent a diverse group of pathologic conditions that includes developmental lesions, reactive or dysplastic diseases, and neoplasms. Owing to substantial overlap of the histopathologic findings, subclassification of BFOLs may be problematic. Moreover, this is not merely an academic exercise because the therapeutic management of BFOLs varies depending on the actual disease process. To further complicate matters, a number of other disease processes demonstrate clinical, radiographic, and microscopic features that bear resemblance to those encountered in recognized fibro-osseous conditions. The objective of this article is to review the most current clinicopathologic, radiographic, and molecular studies of BFOLs to aid the surgical pathologist in the recognition and diagnosis of this diverse group of maxillofacial lesions. A discussion of the various disease processes that occasionally may be confused with BFOLs also is included.

Adult↗

[Origin and treatment of bone cysts].

Osteocyst is shown to be the resulting issue of a number of pathological processes in bones, its etiology could be determined only if tissues typical for some lesions are revealed in cystic walls. At the CITO department of bone pathology for adults the author observed 49 patients having cysts of the proximal femoral portion. Pathological fractures were noted in 16 of 49 patients. It is the author's opinion that a carefully selected anamnesis and clinico-roentgenological investigation are of great importance in establishing the diagnosis of an isolated osteocyst. The differential diagnosis is made between this lesion and tumors, inflammatory process, Recklinghausen disease, etc. All patients under investigation were treated surgically. The results of treatment are described.

Adolescent↗