Refractory angina despite patent coronary artery bypass grafts: treatment with transmyocardial laser revascularization and scintigraphic evidence of improved myocardial perfusion

Erdheim-Chester disease is a rare sporadic systemic histiocytic disease of unknown aetiology that affects multiple organ systems. The case records of all patients with Erdheim-Chester disease who had been seen at the Mayo Clinic between 1975 and 1996 were reviewed to assess the neurological manifestations of the disease. Two of 10 patients had neurological involvement. A 42 year old woman developed central diabetes insipidus and a progressive cerebellar syndrome. Brain MRI showed a lesion in the left pons with patchy gadolinium enhancement and T2 weighted signal abnormalities extending into both cerebellar peduncles and the medulla. Biopsy of the brainstem mass showed a xanthogranulomatous lesion. The second patient was a 53 year old man with retroperitoneal fibrosis secondary to xanthogranulomatous infiltration. Although he had no neurological symptoms and a normal neurological examination, MRI of the head showed multiple uniformly enhancing extra-axial masses along the dura of both convexities and the falx, and a mass in the left orbital apex. Both patients had the characteristic radiographic and bone scan findings of Erdheim-Chester disease. Review of the literature disclosed a wide variety of neurological manifestations in Erdheim-Chester disease. The most frequent CNS manifestations are diabetes insipidus, cerebellar syndromes, orbital lesions, and extra-axial masses involving the dura.     

M cells in the rabbit palatine tonsil: the distribution, spatial arrangement and membrane subdomains as defined by confocal lectin histochemistry

We report a patient with eosinophilic granuloma localized to the left mandible who was subsequently shown to have Erdheim-Chester disease involving the lower extremities, omentum and lung. The diagnosis of eosinophilic granuloma was based on the presence of typical CD1a+ Langerhans' cell granulomas in a biopsy of mandible. The diagnosis of Erdheim-Chester disease was established on the basis of the pattern of radioisotopic uptake by long bones, seen on a technetium bone scan, and the presence of characteristic histopathological features in biopsies of lung and peritoneum. The pathological findings in lung were compatible with the abnormalities observed by tomodensitometry, but strikingly different from those seen in Langerhans' cell granulomatosis. The differences in the histological features of pulmonary involvement seen in the two diseases, and the possible relationship between Langerhans' cell granulomatosis and Erdheim-Chester disease, are discussed.     

A case of Erdheim-Chester disease with orbital involvement

The Erdheim-Chester disease is a rare idiopathic, systemic, histiocytic disorder. To our knowledge, ocular involvement has been reported in only 16 cases. We describe a 55-year-old man who had symmetrical exophthalmos and several skin nodules on the arms and trunk. A magnetic resonance imaging scan confirmed the presence of bilateral, intraconal, retrobulbar tumors. An examination of the histopathologic features of orbital and skin biopsy specimens revealed xanthogranulomatous infiltrate with Touton giant cells. Further systemic investigations showed bone and retroperitoneal involvement. Three years later, multiple eyelid xanthelasmas developed in the patient. These findings are consistent with the diagnosis of the Erdheim-Chester disease. The patient's condition is stable under therapy with low-dose corticosteroids. His survival is longer than usually described in the literature.     

Transmission of Leptospira interrogans serovar Balcanica infection among socially housed brushtail possums in New Zealand

We report a rare case of Erdheim-Chester disease (ECD) presenting as a progressive cerebellar syndrome and diabetes insipidus. On magnetic resonance imaging, a 7-mm extraaxial, enhancing mass was seen enveloping the right vertebral artery and was confirmed at autopsy to represent an adventitial xanthoma with lipid-laden, foamy histiocytes. The cerebellar syndrome most likely resulted from extensive histiocytic infiltration of the pons, particularly the basis pontis and middle cerebellar peduncles.     

Merkel cell carcinoma of the auricle

Esophageal intramural pseudodiverticulosis is a very rare disease with unknown etiology, which especially affects male patients between 45 and 65 years. This disease is characterized by dilatation of the esophageal submucosal glands and their outlets. Stenosis caused by esophagitis due to intramural pseudodiverticulosis is found in most of the known patients. All patients presented with dysphagia, usually of long duration. The characteristic radiographic appearance is numerous intramural esophageal contrast-filled diverticulosis-like pouches--4 mm in depth.     

Intestinal lymphangiectasia: value of double-contrast radiographic study

Intestinal lymphangiectasia is a disorder presenting as enteric protein loss through the dilated lymphatics without mucosal ulceration. To determine the double-contrast radiographic features and to assess the significance of them, five patients with intestinal lymphangiectasia were examined using single- and double-contrast small intestinal studies. The double-contrast examinations demonstrated clearly the main radiographic findings of smooth nodular protrusions, thickening of the mucosal folds, with no evidence of mucosal ulceration. Compared with the single-contrast study, smooth nodular protrusions were seen more often and in more widespread segments, particularly in the duodenum, on double-contrast study. Thickening of the mucosal folds was revealed similarly by both methods. Double-contrast study appears to be worthwhile to demonstrate the characteristic radiographic findings of this disease.     

Erdheim-Chester disease: a primary macrophage cell disorder

Erdheim-Chester disease (ECD) is a rare focal or systemic infiltrative disorder resulting from xanthogranulomatous tissue deposition. Usually, bone marrow involvement affects long bone metaphyses symmetrically, but it spares the epiphyses. Retroperitoneal space, periaortic area, skin, and brain involvement have been described. Pulmonary involvement is frequent, occurring in 20% of cases. Reported histologic features in the lung include an infiltration of so-called lipid-laden macrophages and granulomatous lesions with fibrosis. Lung function outcome is unpredictable, but terminal respiratory failure is the most frequent cause of death. No effective treatment strategies have been described. We report a new case with lung and bone involvement occurring in a symptomatic woman. Histologic and electron microscopic analysis of the pulmonary infiltrate showed abnormal macrophages devoid of lipids forming nodular granulomas and rendering the previous hypothesis of this disease as a primary lipid storage disorder unlikely. These findings suggest that ECD histogenesis is instead based on a primary macrophage cell disease.     

X-ray characteristics of wrists in calcium pyrophosphate crystal deposition disease. Is pseudogout a major cause of scapholunate advanced collapse?

Deposition of calcium pyrophosphate dihydrate (CPPD) crystals has been considered to be a cause of scapholunate advanced collapse (SLAC) wrist. The aim of this study was to look at X-ray changes in wrist joints affected by CPPD crystal deposition disease and to determine whether crystal deposition is a cause of SLAC wrist. A total of 150 wrists of 78 patients with CPPD crystal deposition disease were examined. In our population of Japanese patients with CPPD crystal deposition disease, the incidence of SLAC wrist was very low, and no case of Stage III SLAC wrist was found. We therefore conclude that SLAC wrist is not a radiographic characteristic of CPPD crystal deposition disease and that pyrophosphate crystal deposition cannot be a major cause of SLAC wrist.     

The appropriateness of use of percutaneous transluminal coronary angioplasty in New York State

Between 1974 and 1989 we prospectively observed 530 patients with brucellosis. The findings for 62 patients (42 males and 20 females; mean age, 34.7 years) with 63 episodes in which the sacroiliac joint was involved (the most frequent osteoarticular location [11.7%]) were analyzed. Ten of the older patients (mean age, 55.3 years) had concomitant spondylitis. Systemic symptoms were usually important, and characteristic pain and findings of sacroiliitis were observed in approximately 75% of cases. Blood cultures were positive for Brucella melitensis for 44 patients. The most frequent radiographic findings were blurring of articular margins (42 cases) and widening of the sacroiliac space (20 cases). No radiographic anomalies were detected in 13 cases. Results of 99mTc and gallium-67 bone scans were abnormal in approximately 90% of cases (abnormalities were often mild). Overall, clinical, radiographic, and isotopic bone scan findings were sensitive and useful, although they were occasionally minimal or difficult to evaluate, making diagnostic findings confusing or misleading. Brucellar sacroiliitis is a mild disease associated with a good outcome similar to that observed for patients with uncomplicated brucellosis.     

Pulmonary tuberculosis in five young infants with nursery exposure: clinical, radiographic and CT findings

Clinical, radiographic (n = 5) and CT findings (n = 4) of five Korean infants ranging in age from 2 to 3 months with confirmed tuberculosis were retrospectively analysed. All of the patients were symptomatic, anergic to tuberculin, and had a positive culture of Mycobacterium tuberculosis in gastric aspirates. The probable source of infection was the hospital in which they were born. CT scans demonstrated hilar and mediastinal lymph node enlargement with central low attenuation and peripheral enhancement in all cases. CT may be useful in diagnosis by demonstrating characteristic adenopathy and disseminated disease in young infants.     

Relation between radiographic mottle for double and single emulsions

The radiographic density fluctuations produced by using dual screen-film systems are designated as the radiographic mottle. The density fluctuation of the radiographic mottle for the double emulsions at a density of the double emulsions consists of those for the front and back emulsions on a radiograph. However, the relation between the Wiener spectra of the radiographic mottle for the double and single emulsions had not been studied. Hence we compared the Wiener spectra of the radiographic mottle for the double emulsions with the sum of those for the front and back emulsions on the same radiographs and with the sum of those for the emulsions at the same densities. At all densities of more than 0.62 for lower spatial frequencies (< or = 1 mm-1), the Wiener spectral values of the radiographic mottle for the double emulsions were greater than the sum of those for the front and back emulsions for both comparisons on the same radiographs and at the same densities. In order to investigate the reason of the above phenomena, we separated the Wiener spectral values of the radiographic mottle for various densities into those of the three factors, i.e., quantum mottle, structure mottle, and film granularity, and performed the same comparisons as the radiographic mottle. Also, to explain the results for the three factors, we obtained the Wiener spectral values of the spatial fluctuations of the light exposure or the fluorescence intensity and the gradients of the characteristic curves of the film for the double and single emulsions of the x-ray film. As a result of the investigation, we found that the phenomena on the radiographic mottle were caused by that (1) on the same radiographs the squares of the gradients of the characteristic curves for the double emulsions were about 5.3 times as great as those for the single emulsion at densities of more than 0.62 of the double emulsions, and (2) at the same density of more than 0.62 those were more than about 2.2 times as great as those for the single emulsion.     

Right mandible swelling of unknown origin

Infantile cortical hyperostosis (ICH), or Caffey's disease, has a low prevalence, is not easily recognized clinically, and is seldom reported in the primary care literature. A case of infantile cortical hyperostosis of the right mandible of a newborn is reported. Multiple radiographic tests, as well as bone biopsy, were necessary to establish a definitive diagnosis. The most striking features of this disease are presented, including the clinical and physical presentation, radiographic characteristics, and pathology.     

Prospective screening of dyspeptic patients by Helicobacter pylori serology

A 24-year-old Chinese woman presented with cough, chest pain, weight loss, low grade fever and bronchial breath sounds on auscultation. The diagnosis of chronic eosinophilic pneumonia was made on characteristic systemic and pulmonary clinical manifestations, blood eosinophilia and the striking chest radiographic appearance. This rare, idiopathic but benign condition responds well to corticosteroid treatment and the long term prognosis is excellent. The typical chest radiographic pattern of 'photographic negative of pulmonary oedema' in this condition is emphasised.     

Relationship of mineral status and intake to periodontal disease

A periodontal index (PI), based on visual and radiographic data, was used to evaluate the severity of periodontal disease in a group of 80 dental patients. The average PI was 3.1 +/- 1.8 SD (0=no detectable lesions; 8=most severe lesions). The PI was directly related to patient age (r=0.73; P is less than 0.01). The serum concentration of calcium copper, iron, magnesium, manganese, and zinc was measured. Regression of PI on the serum constituents demonstrated that serum copper was linearly related (r-0.64; P is less than 0.001) to the PI. The other serum parameters were not correlated to the PI. Dietary protein, calcium, and vitamin A were slightly related to the PI, based on a 24-hr dietary recall. Dietary trace elements were not related to the PI. These results indicate that the serum copper concentration, in some way is related to the severity of periodontal disease. Possibly the observation is linked to the role of copper in the inflammatory response since inflammation is a characteristic of periodontal disease.     

Florid periosteal reaction and focal fibrocartilaginous dysplasia

Focal fibrocartilaginous dysplasia (FFCD) is a rare condition causing tibia vara in childhood. It is characterized by progressive tibia vara in young children with a characteristic radiographic lesion. This paper is thought to be the first to describe FFCD exhibiting florid periosteal reaction at the time of presentation with a subtle faint osteolytic lesion in the diametaphysis of the proximal tibia.     

Primary ileal adenocarcinoma simulating Crohn's disease

An unusual case of primary adenocarcinoma of the terminal ileum with a clinical and radiographic appearance simulating Crohn's disease is presented. This example further supports the concept that terminal ileal disease should not immediately be equated with regional enteritis.     

Severe pulmonary hypertension: a feature of Swyer-James syndrome?

Swyer-James syndrome, unilateral hyperlucent lung with air entrapment, generally occurs after severe infections during childhood. It is usually diagnosed by its characteristic chest radiographic image film or computed tomography, in patients who are almost asymptomatic. We report a case of Swyer-James syndrome, diagnosed from the study of severe pulmonary hypertension and with a fatal outcome.     

Vascularized fibular graft for reconstruction of the wrist after excision of giant cell tumor

Seven patients with a giant-cell tumor involving the distal end of the radius were treated with en bloc resection and reconstruction with a free vascularized fibular graft. Two patients with stage 2 disease of Enneking's surgical staging and grade 2 of Campanacci's radiographic grading system were reconstructed with an articular fibular head graft. Five patients with stage 3 and grade 3 disease underwent wrist arthrodesis using fibular shaft transfer. There was radiographic evidence of bone union at the host-graft junctions in all cases. No local recurrence was seen in any of the patients at the most recent follow-up examinations. There were six good and one excellent functional results. Wrist arthroplasty using a vascularized fibula head graft is the best procedure for a stage 2 or grade 2 giant-cell tumor of the distal end of the radius. In cases of stage 3 or grade 3 disease, wrist arthrodesis using a vascularized fibular shaft graft is indicated.     

The use of dental radiographs to estimate the probability of cavitation of carious interproximal lesions. Part II: Two patient scenarios

Diagnosis of a cavitated interproximal lesion is often not confirmed until the tooth is treated. Before treatment, clinicaians and patients are dealing with probabilities which can be estimated from clinical evidence, radiographic evidence, and information found in the dental literature. The probability of a diagnostic test result being correct is affected by the threshold of the test and the pretest probability of the disease in question, which varies with the prevalence of the disease. We illustrate how clinicians can inform themselves and their patients of the probability of a radiographic lesion being truly cavitated based on graphs that summarize the currently available evidence. Dentists who use these graphs can more accurately inform their patients who can then choose the course of care that best meets their individual needs.     

The utility of monitoring carcinoembyronic antigen during systemic therapy for advanced colorectal cancer

To determine if pre-treatment serum carcinoembryonic antigen (CEA) levels or changes in CEA values during treatment have prognostic value, we reviewed five prior fluorouracil/leucovorin-based trials and identified 125 colorectal cancer patients with no prior chemotherapy for metastatic disease in whom CEA values were available. Although pre-treatment serum CEA values did not predict for clinical response or time to progression, serial monitoring of CEA appeared to be useful in patients with an elevated pre-treatment CEA, particularly when a decrease in CEA occurred in concert with radiographic evidence of disease response. The CEA nadir was a strong prognostic variable with respect to time to disease progression. A consistent rise in CEA values over the minimum value signals the need for radiographic re-assessment of the patient's disease status to rule out disease progression.