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PubMed2000

Diffuse chronic sclerosing osteomyelitis and the synovitis, acne, pustolosis, hyperostosis, osteitis (SAPHO) syndrome in two sisters.

Two sisters with diffuse chronic sclerosing osteomyelitis of the mandible and the humerus and the synovitis, acne, pustolosis, hyperostosis and osteitis syndrome (SAPHO syndrome) are presented. The diagnoses of diffuse chronic sclerosing osteomyelitis at the age of 12 years and 27 years, respectively, were based on typical medical history, clinical symptoms and radiographic, histologic and scintigraphic findings. Because skin lesions and scintigraphic enhancement of the sternoclavicular joints with hyperostosis were present, a SAPHO syndrome was diagnosed in both sisters. Microbiological cultures of biopsy specimens revealed coagulase-negative Staphylococcus aureus at the humerus and Haemophilus parainfluenzae, Streptococcus, Actinomyces and Veilonella species at the mandible. Repeated operative procedures, including decortications, resection and reconstruction, and multiple histologic and microbiologic studies were performed over a period of up to 20 years. Since HLA typing yielded identical gene loci, we suggest that hereditary and autoimmune factors may play a role in the pathogenesis of these cases.

Acquired Hyperostosis Syndrome↗
PubMed1976

Histopathologic and roentgenologic studies on the effects of irradiation on the human mandibles.

The present study deals with the pathologic change of the mandible following radiation therapy for oral cancer, histopathologically and roentgenologically. The results are summarized as follows: 1) As the resorptive change of the cortex and the trabeculae, irregular and bizarre resorption and enlargement of the lacunae were observed, 2) The grade of injury of the osteocytes was described by the percentage of the number of the lacunae, which were empty and in which the nuclei of the osteocytes could not be seen. Consequently these results correspond considerably well with the clinical aspects. 3) Osteophytes in the spongy and compact bone were observed, and also narrowing and plugging of the Haversian canal and the canal of Volkmann with mineral were seen as the sclerotic change. 4) Thickening of the tunica intima, obstruction, disarrangement of the elastic fibers and distruption of the vessels were observed in the inferior alveolar artery, the arterioles, and also occlusion, which gave various appearances, in the vessels within the Haversian canal and the canal of Volkmann was seen. 5) The following four groups, based upon the radiographic change, were classified as: Group I, irregular osteolytic change; Group II, marked osteosclerotic change; Group III, irregular mixture of osteolytic and osteosclerotic change; and Group IV, no remarkable change.

Adult↗
PubMedDate not supplied

Case report: natural development of osteosarcoma from precancerous lesion.

We encountered a very rare case that suggested the natural development of osteosarcoma from a precancerous lesion. The patient presented with a huge osteosarcoma in the distal femur on the initial consultation to our hospital. He had undergone X-ray examination twice previously, due to a knee injury. The findings of the lesion detected by the first X-ray examination were similar to a fibrous cortical defect (FCD), differing from those of an osteosarcoma lesion detected by second and last X-ray examinations. We retrospectively estimated the growth rate of the FCD-like and osteosarcoma lesions and found that FCD-like lesion was not osteosarcoma, but might have been a precancerous lesion. We also speculated that this osteosarcoma lesion might have appeared 18 months before the patient consulted our hospital.

Accidental Falls↗
PubMed2000

Osteopathia striata with cranial sclerosis: literature reappraisal argues for X-linked inheritance.

Osteopathia striata with cranial sclerosis (OS-CS) is characterized by linear striations of tubular bones and fan-shaped configurations of the ilia. Although referred in literature as an autosomal dominant disorder the clinical pattern of partial involvement as well as the reported family observations plead for X-linked inheritance with mild striated bone affections in carrier women and severe syndromic morbidity and high mortality in the males. Sporadic affected males are probably somatic mosaics. There is no proven father-son transmission. Symptomatic osteopathia striata (OS) is characteristic in X-linked focal dermal hypoplasia Goltz-Gorlin.

Adolescent↗
PubMed2000

Rapidly destructive hip disease: a case report and review of the literature.

Rapidly destructive hip disease (RDHD) is an arthropathy of unknown cause that involves rapid and total deterioration of both the acetabular and femoral aspects of the hip joint. We report a case in which radiographs taken 6 weeks apart vividly and poignantly demonstrate the rapidity of the disease. Because there is little in the orthopedic literature regarding RDHD, we would like orthopedic surgeons to be aware of the condition and the importance of repeat radiographs for patients with continued severe hip pain without an apparent cause.

Acetabulum↗
PubMed2000

[POEMS syndrome disclosed by ophthalmological manifestation].

POEMS syndrome is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. Papilledema is a common finding but ophthalmological complaints are rare. We report a case of POEMS syndrome in a 42-year old man which was revealed by oscillopsia and blurred vision. An osteosclerotic myeloma of the fifth lef rib was found, but there was no improvement after removal of the rib. The patient died two months later from pulmonary embolism.

Adult↗
PubMed2000

Concomitant sickle cell disease and skeletal fluorosis.

Skeletal fluorosis typically manifests as a diffuse increase in bone density, whereas avascular necrosis of the epiphyses and diaphyseal marrow are the main skeletal manifestations of sickle cell disease. The diagnostic and therapeutic challenges raised when both disorders are present are illustrated by two cases in Senegalese patients from an area characterized by high fluoride contents in the water and soil. Both had SS sickle cell disease. Skeletal fluorosis was diagnosed during evaluation for avascular necrosis in one patient and in the wake of septic arthritis in the other. Femoral head necrosis is difficult to identify in a patient with skeletal fluorosis. The bone lesions due to sickle cell disease and those due to fluorosis can mimic other bone diseases, most notably metastases. The combination of sickle cell disease and fluorosis results in significant medullary canal narrowing due to cortical thickening and to accumulation of necrotic bone. When performing hip replacement surgery, careful reaming of the medullary canal may reduce the risk of iatrogenic femoral fracture and inappropriate stem placement.

Adult↗