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Dense bone island. A review of 107 patients.

The dense bone island is an asymptomatic radiopacity of unknown origin and is known under many synonyms when it occurs in the jaw. This article discusses 113 dense bone islands in 107 patients with a review of the literature and comparison with previous reports. The average age of the patient at discovery was 36 years with a women to men ratio of 2:1. The mandible was involved in 109 of the 113 DBIs, with only 4 in the maxilla. The most common site affected was the mandibular first molar region, the premolar region was the second most common site affected. Resorption occurred in 11 (9.7%) cases, the first permanent molar was the most commonly affected.

Adolescent↗

Decortication in the treatment of diffuse sclerosing osteomyelitis of the mandible. Retrospective analysis of 41 cases between 1969 and 1990.

Thirty-four patients with mandibular diffuse sclerosing osteomyelitis who had been treated by means of 61 decortications were evaluated retrospectively. Eighteen patients (53%) were free from symptoms on an average of 5.4 years after surgery. Of these, 12 had improved after their first operation. In the other six patients, decortication was performed two to four times before healing was clinically observable. Symptoms recurred in 75% of the cases within 12 months after surgery. Neither sex, location, extent, and chronicity of the disease nor the precise surgical technique used seemed to affect the outcome. The patients who exhibited improvement, however, were significantly older and more often edentulous than the patients in whom the symptoms recurred. Possible causes of failure were an insufficiently radical surgical procedure and retention of devitalized teeth in the decorticated area.

Adolescent↗

Hereditary hemorrhagic telangiectasia with florid osseous dysplasia. Report of a case with differential diagnostic considerations.

The clinical presentation of hereditary hemorrhagic telangiectasia with various manifestations has been well described as has florid osseous dysplasia. There have been no cases reported of the two pathologic entities in the same patient. We present a case with the simultaneous occurrence of hereditary hemorrhagic telangiectasia and florid osseous dysplasia with important considerations for differential diagnosis, and we discuss whether this case presents a potential syndrome.

Bone Diseases, Developmental↗

Congenitally osteosclerotic (oc/oc) mice are resistant to cure by transplantation of bone marrow or spleen cells from normal littermates.

The osteosclerotic mouse is a new, lethal recessive skeletal mutation which inherits osteopetrosis as an autosomal recessive. Affected mice are hypocalcemic and also have rickets. Osteoclasts are small and numerous with little evidence of function. This report examines the effects of bone marrow and spleen cell transplantation from normal littermates on survival and the radiographic appearance of the skeleton in 29 mutants. This procedure did not significantly prolong life or effect radiographic changes in the skeleton of most (27) recipients. However, two treated mutants lived for almost a year with radiographically normal skeletons. These variable responses to stem cell transplantation in an osteopetrotic mutant with rickets deserve further study.

Animals↗

Serum laminin P1 in idiopathic myelofibrosis and related diseases.

The serum concentration of laminin P1 antigen was determined in 32 patients with various myeloproliferative disorders, including 19 patients with idiopathic myelofibrosis. The serum concentration of the aminoterminal propeptide of type III procollagen (PIIINP) was measured concomitantly in 27 patients. Serial laminin measurements were carried out in 25 patients. The median serum laminin concentration in patients with acute disease, i.e. acute myelofibrosis and patients in a transforming disease phase was significantly higher (1.58 U/ml; range 1.15-2.07) as compared with patients with chronic disease (1.02 U/ml; range 0.75-1.76; p = 0.012) and healthy control subjects (1.13 U/ml; range 0.75-1.67; p = 0.00015). In individual patients serum laminin covariated closely with serum PIIINP, the leucocyte count and LDH. The median serum laminin concentration in patients with a huge spleen was significantly lower than in the patient group with a normal spleen size/previous splenectomy. Pronounced splenomegaly was particularly prevalent in patients with chronic disease implicating splenic enlargement as a significant extrahepatic site of laminin uptake/degradation in myeloproliferative disorders.

Adult↗

Mixed sclerosing bone dystrophy presenting with upper extremity deformities. A case report and review of the literature.

We present a detailed study of a 59-year-old white woman with mixed sclerosing bone dystrophy: the rare occurrence of two or more sclerosing bone dysplasias in a single subject. She exhibited features of osteopoikilosis, osteopathia striata and melorheostosis. The symptoms were primarily the result of the melorheostosis lesions which were distributed within the C6 sclerotome. This is an unusual case of mixed sclerosing bone dystrophy in that the upper extremity was the main site of involvement, instead of the usual symmetrical involvement of all limbs. The patient developed a recalcitrant lateral epicondylitis in the un-involved contralateral elbow that required surgical treatment.

Female↗

Long-term evaluation of discectomy of the temporomandibular joint.

A clinical and radiographic survey was conducted with 39 patients (39 joints) who had undergone discectomy of the temporomandibular joint between 1965 and 1974 and whose cases had been followed for an average of 20 years after surgery. There were 3 males and 36 females, ranging in age from 16 to 70 years (average, 33 years) at the time of surgery. Masticatory disorders, arthralgia, limitation of condylar movement, and trismus were the chief symptoms, and clicking and crepitus were present in six and two patients, respectively. Plain radiography showed osteoarthritic changes such as osteophyte formation or lipping on the condyles of eight joints. Arthrography showed disc perforations and peridiscal adhesions in 27 patients, and displacement of the disc in eight. All discs were totally extirpated and condylar shaves were performed in eight patients who had disorders such as osteophytes, exostoses, or lipping on the condyles. In follow-up examinations, 37 patients had no pain, but mild arthralgia was noted occasionally in two patients. Postoperatively, no patients experienced subjective masticatory disorders; 38 patients were able to open their mouth more than 35 mm, and crepitus was heard in only two joints. Plain radiography showed bony changes on the condyles and eminences of all joints. From this long-term follow-up study it is concluded that discectomy is a useful operation for treatment of severe disc pathology causing pain and functional disturbance in the temporomandibular joint.

Adolescent↗

Progress of brown tumors in patients with chronic renal insufficiency undergoing dialysis.

In a group of 73 patients, affected by chronic renal insufficiency, whose skeleton was periodically checked, three cases of brown tumors were found. These cases demonstrated a variety of locations that were involved, especially the pelvis, ribs and mandible. During therapeutic treatment the brown tumors presented a different behavior; while some lesions tended toward complete sclerosis, others increased in size and in both instances new lesions appeared. Parathyroidectomy, carried out in two patients, determined a definitive sclerosis of these lesions. Brown tumors also correlate with high PTH levels and with lesions from osteitis fibrosa.

Adolescent↗

Sarcoidosis of the spinal cord with extensive vertebral involvement: a case report.

We report on a patient with systemic sarcoidosis who was presented with myelopathy and backache. Plain spinal films were normal, CT scan showed sclerotic lesions within the vertebrae. MRI showed more extensive involvement of the spine with multiple vertebral lesions which were hypointense on both T1W1 and T2W1 and did not enhance with gadolinium. MRI also showed high signal lesions within the cervical and lumbar spinal cord on T2-weighted images (T2W1) which were isointense on T1-weighted images (T1W1) and did not enhance. Vertebral biopsy results were consistent with the diagnosis of sarcoidosis. MRI is very sensitive in detecting sarcoidosis of bone but non-specific and other types of sclerotic or lytic bone lesions (notably metastases) need to be excluded.

Adult↗

Massive rotator cuff tears. Screening by routine radiographs.

To clarify their usefulness in screening massive rotator cuff tear, routine radiographs of the shoulder were compared in three groups: (1) shoulders with a massive rotator cuff tear (MRCT), (2) shoulders with a small full-thickness rotator cuff tear (SRCT), and (3) normal control shoulders. Two different statistical methods were employed to assess the gradation (order) of correlation in 83 shoulders (22 were MRCTs, 31 with SRCTs, and 30 controls). Superior migration of the humerus and deformity of the greater tuberosity showed by far the more significant contribution to MRCT than did other abnormalities. When the shoulders were screened for these two abnormalities, the sensitivity was 78% (21/27) and specificity was 98% (55/56).

Acromioclavicular Joint↗

Primary amyloidosis of the sternum. Imaging findings.

A rare case of focal primary amyloidosis is presented with CT and MR correlation. Imaging findings demonstrate an expansile, mixed sclerotic and lytic lesion in the sternum, with MR signal characteristics similar to those previously described for diffuse primary amyloidosis and hemodialysis-related amyloidosis.

Adult↗

Bilateral visual loss in craniodiaphysial dysplasia.

PURPOSE: To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. DESIGN: Observational case report. METHODS: A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. RESULTS: The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. CONCLUSIONS: CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.

Blindness↗

Osteopathia striata-cranial sclerosis: otorhinolaryngologic clinical presentation and radiologic findings.

BACKGROUND: Osteopathia striata with cranial sclerosis (OS-CS) is a rare bone dysplasia that presents with variable symptoms mainly due to the associated CS. METHODS: The otorhinolaryngologic clinical presentation and temporal bone computed tomography (CT) findings of a new case of OS-CS are presented. Differential diagnosis is also discussed. RESULTS: Our study confirms the typical appearance of the temporal bone reported in previous CT studies and the utility of CT for evaluating the various etiologies of conductive-mixed hearing loss present in OS-CS. Radiographs of the femur and pelvis revealed the typical striations of OS-CS and allowed us to diagnose this rare form of generalized skeletal dysplasia involving the skull base. CONCLUSIONS: To our knowledge, this is the third reported case of OS-CS in which temporal bone CT evaluation corroborated the smallness of the middle ear cavities, abnormal ossicular fixation, and bone sclerosis of the mastoid cells. Other radiographic studies regarding the whole skeleton may, in combination, be very helpful in the differential diagnosis between the different forms of generalized skeletal diseases with bone sclerosis involving the skull base.

Child↗

Cone beam computed tomographic findings in refractory chronic suppurative osteomyelitis of the mandible.

AIMS: To report the imaging features of osteomyelitis of the mandible in various two-dimensional multiplanar and three-dimensional reformations using cone beam computed tomography (CBCT). METHODS: The images were 12-bit DICOM files acquired with a 10cm field of view and voxel resolution of 0.4mm. Two-dimensional multiplanar reformatted reconstructions included coronal, "panoramic" (variable thickness), and serial cross-sections. Three-dimensional reconstructions included surface renderings. Images were presented to referring oral and maxillofacial surgeons in "real time" immediately after acquisition. RESULTS: The features of mandibular osteomyelitis seen on CBCT included: a peripheral sclerotic rim, cortical layering (involucrum), central loss of trabecular pattern with internal round radiolucent resorptive tracts, minimal jaw expansion, and reduction of the alveolar cortex. Sequestra were occasionally evident. The history and presentation of each case on CBCT were consistent with osteomyelitis of the mandible; however, the clinical differential diagnosis in each case had included malignancy. The definitive diagnosis was confirmed by histological examination of biopsy specimens. Two of the three cases were patients who had been treated with bisphosphonates. CONCLUSION: CBCT facilitated comprehensive and dynamic imaging of the jaws based on surgical consultation, rather than inflexible imaging protocols. CBCT images guided operative planning.

Aged↗