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Involvement of the humerus in two generations with spondyloepiphyseal dysplasia.

Children with spondyloepiphyseal dysplasia present with a disproportionate short stature, platyspondyly, scoliosis, coxa vara, and clubfeet. Extraskeletal manifestations such as retinal detachment and deafness have been reported. The authors report two patients, a mother and her daughter, aged 35 and 6 years, with findings of pseudarthrosislike lesions in the middiaphysis of both humeri. The mother had minimal symptoms that resolved spontaneously, and the child had no symptoms related to these lesions. The radiographs of the mother show complete remodeling of the lesion. The pseudarthrosislike lesion of the humerus may be one of the manifestations of spondyloepiphyseal dysplasia congentia. In time, the bone remodels completely. Because this is a relatively new radiographic finding, the authors suggest performing a radiograph of the humeri in patients with spondyloepiphyseal dysplasia congenita at least once during childhood.

Adult↗

Computed tomography findings in patients with sacroiliac pain.

This retrospective study evaluated the diagnostic value of computed tomography in patients with sacroiliac pain. Computed tomography scans of the sacroiliac joints of 62 patients with sacroiliac joint pain were reviewed. The criteria to include the patient in the current study were pain relief after a local injection in the sacroiliac joint under computed tomography guidance, a physical examination consistent with a sacroiliac origin of the pain, and negative magnetic resonance imaging of the lumbar spine. A control group consisted of 50 patients of matched age who had computed tomography scans of the pelvis for a reason other than pelvic or back pain. Computed tomography scans showed one or more findings in 57.5% and 31% of the sacroiliac joints in the symptomatic and the control groups, respectively. The computed tomography scans were negative in 37 (42.5%) symptomatic sacroiliac joints with a positive sacroiliac joint injection test. The sensitivity of computed tomography was 57.5 % and its specificity was 69%. The finding of the current study suggests limited diagnostic value of computed tomography in sacroiliac joint disease because of its low sensitivity and specificity. With clinical suspicion of a sacroiliac origin of pain, intraarticular injection is currently the only means to confirm that diagnosis.

Adolescent↗

CT and MRI of massive osteolysis of Gorham.

Massive osteolysis of Gorham is a clinical, radiographic, and histologic entity characterized by histologically benign vascular proliferation involving bone associated with extensive osteolysis. We present CT and MRI findings of the disease and serial radiography following the progressing bone destruction.

Adult↗

A comparison of MR and CT in suspected sacroiliitis.

OBJECTIVE: A prospective study to compare the MR and CT images of patients with suspected sacroiliitis and to establish the optimal MR sequences to demonstrate the changes of sacroiliitis was conducted. MATERIALS AND METHODS: Thirty-nine patients and nine controls were imaged in the axial plane, with SE T1-, T2-weighted fast spin echo (T2), T1 with fat suppression (T1WFS), and fast short tau inversion recovery (fast STIR) sequences on a 1.5 T system. The sacroiliac joints of all patients were imaged with CT. The images were evaluated by two independent radiologists. Following the blinded reading, direct comparison of T1 and T1WFS, T2, and fast STIR of the CT positive group was made to determine the optimal MR sequences. RESULTS: The sensitivity and specificity of MR images for the detection of cortical erosions and subchondral sclerosis when compared to CT images were 100 and 94.3%, respectively; interobserver variation was low (k = 0.80). T1WFS and fast STIR images were superior to T1 and T2 images, respectively, in demonstrating the changes of sacroiliitis. CONCLUSION: MRI (T1WFS and fast STIR) can replace CT in cases with a strong clinical suspicion of sacroiliitis and equivocal or normal plain radiographs.

Adult↗

Osteosclerotic myeloma with spinal leptomeningitis and severe polyneuropathy: A case report.

Osteosclerotic myeloma is a rare plasma cell disorder often associated with a polyneuropathy. We describe a young patient with progressive polyneuropathy associated with vertebral bone marrow lesions and unusual meningeal infiltration at cauda level. The diagnosis of osteosclerotic myeloma was confirmed by bone marrow biopsy findings including the chromosome 13q14 deletion in 37% of the cells. Leptomeningeal involvement was demonstrated by spine magnetic resonance imaging with gadolinium administration and fat-suppression technique.

Adult↗

Healing of a radiolucent periradicular lesion with periradicular radiopacity.

Periradicular radiopacities that reveal the existence of sclerotic bone are less common than periradicular radiolucencies. Replacement of a radiolucent periradicular area by sclerotic bone after endodontic treatment is not very often reported and may be confused with condensing osteitis. An unusual case is presented of sclerotic bone formation after endodontic treatment of a first mandibular molar with initial diagnosis of apical periodontitis.

Adolescent↗

Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature.

Two patients with plasma cell dyscrasias, manifested by osteosclerotic bone lesions and small amounts of M protein, and a complicating multi-system disorder are described. Their features of severe sensory-motor polyneuropathy, organomegaly, endocrine dysfunction, anasarca, elevated CSF protein, and skin hyperpigmentation are similar to a clinical syndrome reported primarily in Japanese men. Two previously unrecognized findings--hyperprolactinemia and an unusual radiographic abnormality of fluffy, spiculated bony proliferation--may facilitate recognition of the syndrome. The relationship of these various manifestations to the plasma cell dyscrasia is unknown, but a number of possibilities are discussed.

Adult↗

Revascularized intercalary bone allografts with short-term immunosuppression with cyclosporine in the canine.

To study the healing process of vascularized intercalary bone allograft after withdrawal of immunosuppressive drugs, allotransplantation of the tibia diaphysis with a vascular pedicle was performed in eight adult mongrel dogs (group 2) and assessments were made both during administration and after discontinuation of cyclosporin A. As controls, similar grafts with the vascular pedicles were removed and reimplanted back to the same animals (five dogs, group 1). Allotransplantation of frozen stored bone without a vascular pedicle (10 dogs, groups 3A and 3B) were also compared. No union occurred in most cases of frozen stored bone allotransplant because the transplanted bone was resorbed, leading to loosening and subsequent failure of osteosynthesis with the plate and screws used. Under cyclosporin A immunosuppression, bony union (i.e., when trabeculae were seen crossing the graft-recipient junction with obliteration of the junction line) occurred at almost similar time intervals in all dogs of group 2 (bone allotransplant with a vascular pedicle) by 3 months postoperatively, which was similar to those of group 1. No systemic side effects of cyclosporin A were observed. Cyclosporin A was discontinued 3 months following graft implantation. The bone graft became avascular within a week following withdrawal of cyclosporin A. However, bone union was maintained, and the transplanted bone never showed bone resorption, sclerosis, or fracture on serial radiographs up to the time the animals were sacrificed, between 5 and 14 months later. Histology at sacrifice showed that the transplanted allografts were being replaced at both ends by fresh bone derived from the transplantation bed. We conclude on the basis of the results of this study that solid bony union can be obtained in allotransplanted bone with a vascular pedicle if cyclosporin A is given for a brief period. After cyclosporin A is withdrawn, although the bone becomes nonviable secondary to rejection occurring in the blood vessels, its skeletal structure remains intact, enabling it to maintain its structural support while awaiting replacement by bony ingrowth from both ends of the graft.

Animals↗

Bone scans in condensing osteitis of the clavicle.

Two additional cases of condensing osteitis of the clavicle are reported. Technetium Tc 99m phosphate bone scans were positive in both cases. Clinical and roentgenographic manifestations may allow nonoperative diagnoses of this entity which mimics metastatic disease on bone scans.

Bone Neoplasms↗

The management of patients with back pain and idiopathic vertebral sclerosis.

Twenty-two patients with severe back pain associated with radiologic evidence of vertebral sclerosis are reported. There were 19 women and 3 men; the average age was 42 years. Duration of pain averaged 4.3 years. The characteristic radiologic findings were diffuse sclerosis of the anterior inferior portion of the vertebral body and narrowing of the adjacent disc space. There was no extension of the sclerosis beyond the vertebral body, no paravertebral mass, and no loss of vertebral height. These radiologic characteristics, in conjunction with other clinical findings, should permit a diagnosis without the need for biopsy.

Adult↗

Multiple lateral meningoceles, distinctive facies and skeletal anomalies: a new case of Lehman syndrome.

We describe a 19-year-old boy who presented with facial dysmorphism, multiple lateral meningoceles, skeletal abnormalities and normal intelligence. Neurofibromatosis and Marfan syndrome were excluded. Electron microscopy of the skin showed non-specific abnormalities suggesting a connective tissue disorder. The features of this boy closely resemble those in a mother and daughter with Lehman syndrome.

Abnormalities, Multiple↗

Raine dysplasia: a Brazilian case with a mild radiological involvement.

We report a preterm male infant, the first child of a young consanguineous couple, whose physical examination revealed craniofacial disproportion with microcephaly, wide fontanelles, exophthalmos, low nasal root and hypoplastic nose, long philtrum, small mouth, high arched and narrow palate, micrognathia, dysplastic, low-set and rounded ears, short neck and, arthrogryposis. Postmortem findings included hypoplastic lungs. Radiological examinations showed mild and localized increased of bone density in the cranial vault and skull base and facial bones and undermodelled in the long bones. The above findings are characteristics of Raine dysplasia but the case reported here presents a mild bone involvement with only a localized bone sclerosis and absence of prenatal fractures. We discuss the possibility that this case represents an allelic mutation of the Raine gene. The consanguinity of the parents reinforces the hypothesis of autosomal recessive inheritance for this entity.

Abnormalities, Multiple↗