1/36. Florid osseous dysplasia of the mandible: report of a case.In 1986, a 60-year-old African American woman visited the Marquette University School of dentistry with a complaint of a vague, dull pain in her lower left quadrant. tooth No. 19 was extracted 10 years earlier because of extensive decay, and tooth No. 18 had received root canal therapy. A panoramic radiograph revealed the presence of ill-defined, multilocular, mixed (radiopaque-radiolucent) lesions present throughout the lower jaw. She was treated with antibiotics and scheduled for follow-up visits. When the symptoms persisted, tooth No. 18 was re-treated with root canal therapy and a representative biopsy was taken from the left mandibular area. The biopsy showed the presence of chronic osteomyelitis. The patient was treated with antibiotics and was scheduled for periodic check-up visits. In February 1995, she returned with the same symptoms in the left mandible. A panoramic radiograph showed persistence of the mixed radiopaque-radiolucent lesions throughout her mandible; however, the mass on the left side was more radiopaque and had assumed a "cotton wool" appearance. tooth No. 18 was extracted and a biopsy was taken from the area. After correlating the clinical behavior, radiographic appearance, and histopathologic features, a diagnosis of florid osseous dysplasia with osteitis was made. This case represents a classic example of the difficulty in diagnosing fibro-osseous lesions using radiographic interpretation alone and the need to correlate the clinical, radiographic, and histopathologic features to reach a diagnosis. Additionally, the present case clearly shows treatment problems of an otherwise self-limiting condition when secondary involvement with osteomyelitis is also present. A brief description of the conditions that were included in the differential diagnosis and their management is presented.- - - - - - - - - - ranking = 1keywords = canal (Clic here for more details about this article) |
2/36. Paget's disease of the bone: a case report.Paget's disease of bone is rare in asia. We report a case of Paget's disease in a 58-year-old Taiwanese man who was admitted with a 3-month history of bilateral numbness in the buttock region. Laboratory data disclosed an elevated serum alkaline phosphatase level (510 U/L). Plain radiographs of the lumbar spine showed generalized increased density at the third lumbar vertebra, associated with cortical thickening, loss of cortico-cancellous definition, and increased anteroposterior diameter. The T1-weighted magnetic resonance image of the lumbar spine showed diffuse, heterogeneous low signal intensity at the third lumbar vertebral body, pedicle, laminae, and spinal process; these areas showed mixed high and low signal intensity on the T2-weighted image. technetium-99m bone scan revealed abnormal uptake in the involved vertebra. Histologic examination of the third lumbar spinal process confirmed the diagnosis of Paget's disease of bone. The patient remained well during a follow-up period of 6 months.- - - - - - - - - - ranking = 8.4382836009711keywords = spinal (Clic here for more details about this article) |
3/36. vertebroplasty in focal Paget disease of the spine. Case report.Paget disease is an idiopathic metabolic disease of bone that may involve the axial and appendicular skeleton. In up to one third of patients there may be pagetoid involvement of the spine, which can cause back pain and vertebral collapse, with instability or myeloradiculopathy. Although medical therapy is the mainstay of treatment, decompressive surgery or stabilization may be required. The authors report on a case of localized Paget disease of the spine treated successfully by performing percutaneous vertebroplasty. They propose this procedure as a useful intervention that can be undertaken safely in patients with spinal Paget disease, in whom acquisition of a transpedicular biopsy sample is required as part of diagnosis.- - - - - - - - - - ranking = 4.2191418004856keywords = spinal (Clic here for more details about this article) |
4/36. Treatment of idiopathic hyperphosphatasia with intensive bisphosphonate therapy.In a family with IH, a rare high turnover bone disease, two older siblings were wheelchair-bound with severe skeletal deformity by age 15. Their youngest affected sibling was treated intensively with intravenous bisphosphonates for 3 years. The treatment was well tolerated and prevented the development of deformity and disability. INTRODUCTION: Idiopathic hyperphosphatasia (IH, also known as juvenile Paget's disease) is a rare genetic bone disease characterized by very high bone turnover and progressive bony deformity. Inhibitors of bone resorption have been used to suppress bone turnover in the short term, but there is no published data on long-term efficacy. MATERIALS AND methods: An 11-year-old girl with IH, who had two severely affected older siblings, presented with progressive deformity and deafness and long bone fractures. Conventional pediatric doses of pamidronate had failed to prevent clinical deterioration or suppress bone turnover completely. Intensive bisphosphonate therapy (frequent 5-mg ibandronate infusions) was given to try and arrest progression of the skeletal disease. growth and development, pure tone audiometry, biochemistry, radiology, densitometry (DXA), and bone histology were monitored. RESULTS: A total of 45 mg ibandronate was given over 3 years until skeletal maturity was reached (20, 15, and 10 mg for years 1-3, respectively). Ibandronate treatment was well tolerated, and biochemical markers of bone turnover suppressed to within the age-appropriate normal range There was some progression of her thoracic kyphosis, but she had no further fractures and remained mobile and active at an age when her siblings had become wheelchair-bound. A significant recovery of hearing (p < 0.01) was documented, particularly at low frequencies. Radiographs showed improvement in spinal osteoporosis and cortical bone dimensions and arrest of progressive acetabular protrusion. Areal bone density increased substantially (lumbar spine z-score from -2.2 to 1.8). tetracycline-labeled bone biopsy specimens were taken before and after 18 months of intensive treatment. The second biopsy showed suppression of bone turnover and a doubling of trabecular thickness, with no mineralization defect, and no osteopetrosis. CONCLUSIONS: Intensive bisphosphonate treatment prevented the development of deformity and disability and improved hearing in this child with IH. The dose of bisphosphonate, which is substantially greater than is usually used in pediatric bone disease, had no adverse effects, in particular on bone mineralization.- - - - - - - - - - ranking = 4.2191418004856keywords = spinal (Clic here for more details about this article) |
5/36. Spinal manifestations of Paget's disease.This paper reviews the syndromes which affect the spinal column as a result of Paget's disease. These are back pain, spinal canal stenosis, paraplegia or paraparesis, sarcomatous transformation, intradiscal invasion, and extramedullary haemopoesis.- - - - - - - - - - ranking = 8.9382836009711keywords = spinal, canal (Clic here for more details about this article) |
6/36. MRI of Paget disease with temporal bone involvement presenting with sensorineural hearing loss.temporal bone involvement in Paget disease can result in hearing loss by a variety of mechanisms. One such mechanism is compression of neural structures within the internal auditory canal. This report describes such a case. magnetic resonance imaging characteristics are discussed, including the appearance of Paget disease following Gd-DTPA administration.- - - - - - - - - - ranking = 0.5keywords = canal (Clic here for more details about this article) |
7/36. Paget's disease of bone affecting a single vertebra: clinical, radiologic, and histopathologic correlations.We report a 67-year-old man who presented with a 3-month history of progressively increasing pain in the lumbar spine. His past medical history was unremarkable, and physical examination disclosed local tenderness over the lower spine. No neurologic dysfunction was identified. Routine laboratory evaluation including alkaline phosphatase activity was normal. An x-ray film of the lumbar spine showed enlargement and increased density of L-5 vertebra. A whole-body bone scan revealed markedly increased uptake at the L-5 level. To further evaluate the nature of the disorder and the cause of his pain, a computed tomography (CT) scan was obtained. It disclosed multiple lucent areas with some sclerotic changes mainly affecting the vertebral body of L-5. No spinal stenosis was found. Subsequently, a bone biopsy of L-5 was performed that showed typical findings consistent with Paget's disease. The patient was treated with etidronate (200 mg b.i.d. for 6 months) followed by salmon calcitonin (50 IU 3 times/week s.c. for 6 months). The pain declined gradually in severity and the patient became symptom free after 12 months of treatment. A repeat x-ray film, obtained at that time, showed no significant change. However, a bone scan showed almost complete normalization. The present case illustrates that a high index of suspicion is required when only a single vertebra is affected by Paget's disease, especially, when alkaline phosphatase activity is normal. It may present with severe pain without evidence of neurologic dysfunction. CT scan may be a useful adjunct in establishing the diagnosis and elucidating the cause of pain.- - - - - - - - - - ranking = 4.2191418004856keywords = spinal (Clic here for more details about this article) |
8/36. Surgical management of hydrocephalic dementia in Paget's disease of bone: the 6-year outcome of ventriculo-peritoneal shunting.Paget's disease of bone is a chronic progressive skeletal disorder usually occurring in the long bones and skull of older adults and elderly persons. In the skull, softening of the skull base may lead to basilar impression and consequently obstruction of the cerebrospinal fluid through the basilar cisterns, resulting ventricular enlargement in association with gait difficulties, incontinence and dementia: a syndrome resembling normal pressure hydrocephalus. The optimal management of hydrocephalus associated with Paget's disease of the skull is not well documented and is still debated. We report a patient with hydrocephalic dementia linked to Paget's disease of the skull who showed marked sustained improvement in her neurological condition after ventriculo-peritoneal shunt insertion. We have now followed this patient for 6 years. Our experience supports the view that ventricular shunting is the procedure of choice for treating hydrocephalus linked to the Paget's disease of bone and is best carried out in the early stages of the illness.- - - - - - - - - - ranking = 4.2191418004856keywords = spinal (Clic here for more details about this article) |
9/36. Spinal decompression and vertebroplasty in Paget's disease of the spine.Paget's disease is an osteometabolic disorder affecting in particular long bones. The spine is the second most commonly involved site in Paget's disease. This pathology can cause low back pain, spinal stenosis, myeloradiculopathy, and vertebral collapse. Medical therapy is the first choice for the treatment of Paget's disease of the spine; in case of failure, surgery remains a valid option. In the present article, we report a case of a patient with leg disability due to myelopathy caused by spinal Paget's disease treated with spinal decompression and vertebroplasty. To our knowledge, this is the first case report in which these procedures have been performed together in the same operation to treat spinal pathologies due to Paget's disease.- - - - - - - - - - ranking = 16.876567201942keywords = spinal (Clic here for more details about this article) |
10/36. Traumatic fracture in a healthy man: benign or pathologic?OBJECTIVE: To describe the challenge of determining the correct diagnosis in a healthy adult male patient with a recent femoral fracture and a history of multiple bone fractures. methods: We present clinical, radiologic, laboratory, and histopathologic details in a patient with a history of recurrent fractures associated with minimal trauma. Moreover, the various types of osteopetrosis are reviewed. RESULTS: A 34-year-old African American man was in his usual state of good health when he fell hard on concrete. Immediately after the fall, he was able to bear weight, although pain prompted him to seek medical care. Besides a personal history of multiple fractures, he had no other medical problems. He had never smoked, denied illicit drug use, and had no family history of bone disorders or recurrent fractures. Findings on physical examination were unremarkable. radiography disclosed an incomplete femoral fracture and osteosclerosis. Bone survey revealed diffuse, symmetric osteosclerosis of both the axial and the appendicular skeleton. The long bones showed areas of almost complete obliteration of the medullary canal, along with prominent hyperostosis. Additionally, a "bone-within-bone" appearance to the thickened endosteum was noted. A bone scan demonstrated numerous areas of symmetric radiotracer uptake. Laboratory analyses were unremarkable, including a complete blood cell count, electrolytes, serum protein electrophoresis, thyrotropin, and parathyroid hormone. Total alkaline phosphatase was mildly elevated at 162 U/L (normal range, 35 to 130). Seven needles were broken during attempts to perform a bone biopsy. Histologic examination showed normal bone marrow with "woven" bone and areas of primary spongiosa within mature osteoid. Autosomal dominant osteopetrosis type 2 was diagnosed on the basis of his clinical presentation and the radiologic and pathologic findings. CONCLUSION: The preliminary diagnosis for this patient's condition was Paget's disease, and determining the correct diagnosis of osteopetosis prevented the administration of inappropriate therapy. In addition, this case report reminds the clinician that genetic disease may manifest in adulthood.- - - - - - - - - - ranking = 0.5keywords = canal (Clic here for more details about this article) |
| | Next -> |