Amiodarone pulmonary toxicity.

Amiodarone is an antiarrhythmic agent commonly used to treat supraventricular and ventricular arrhythmias. This drug is an iodinecontaining compound that tends to accumulate in several organs, including the lungs. It has been associated with a variety of adverse events. Of these events, the most serious is amiodarone pulmonary toxicity. Although the incidence of this complication has decreased with the use of lower doses of amiodarone, it can occur with any dose. Because amiodarone is widely used, all clinicians should be vigilant of this possibility. Pulmonary toxicity usually manifests as an acute or subacute pneumonitis, typically with diffuse infiltrates on chest x-ray and high-resolution computed tomography. Other, more localized, forms of pulmonary toxicity may occur, including pleural disease, migratory infiltrates, and single or multiple nodules. With early detection, the prognosis is good. Most patients diagnosed promptly respond well to the withdrawal of amiodarone and the administration of corticosteroids, which are usually given for four to 12 months. It is important that physicians be familiar with amiodarone treatment guidelines and follow published recommendations for the monitoring of pulmonary as well as extrapulmonary adverse effects.

Efficacy of inhaled steroids (beclomethasone dipropionate) for treatment of mild to moderately severe asthma in the emergency department: a randomized clinical trial.

STUDY OBJECTIVE: To examine the efficacy of an inhaled steroid, when added to a standard regimen of beta-agonist therapy, in the treatment of patients with mild to moderately severe asthma in the emergency department. METHODS: A convenience sample of adult patients with asthma (FEV1 % predicted 40% to 69%) presenting to the ED was randomly assigned in a double-blind fashion into 2 treatment groups. The first group received 2.5 mg nebulized salbutamol plus 1 mg (4 puffs) of beclomethasone dipropionate (BDP) at baseline, 30 minutes, and at 1, 2, and 4 hours, delivered by a metered-dose inhaler (MDI) attached to a spacer device (Vent-AH-aler, Glaxo). The second group was given the same salbutamol regimen plus MDI placebo through the Vent-AH-aler. The primary endpoint was improvement in FEV1 %predicted at 6 hours. RESULTS: Of 54 patients enrolled, 28 were assigned to the BDP group and 26 to the placebo group. Spirometry improved significantly in both groups over the 6 hours compared with baseline (ANOVA, P <.001). At 6 hours, the mean absolute improvement in FEV1 % predicted for BDP was 18% versus 17% for placebo (95% confidence interval for the absolute difference of 1% [-8% to 10%]). The proportion of patients in the BDP group who were hospitalized was 7% compared with 19% for patients in the placebo group (95% confidence interval for the difference of 12% [-6%, 30%]). CONCLUSION: In this group of patients with mild to moderately severe asthma, 5 mg BDP delivered by MDI during the initial 4 hours of an emergency visit was of no added benefit over standard therapy, as measured by improvement in FEV1 % predicted at 6 hours. However, a trend toward a difference in admission favoring BDP was observed. [Afilalo M, Guttman A, Colacone A, Dankoff J, Tselios C, Stern E, Wolkove N, Kreisman H: Efficacy of inhaled steroids (beclomethasone dipropionate) for treatment of mild to moderately severe asthma in the emergency department: A randomized clinical trial.

Meal-induced oxygen desaturation and dyspnea in chronic obstructive pulmonary disease.

OBJECTIVE: To study arterial oxygen saturation (SpO2) obtained by pulse oximetry and dyspnea during active eating (AE) and passive eating (PE) in patients with severe chronic obstructive pulmonary disease (COPD). DESIGN: Patients were studied on two consecutive days with AE and PE, which occurred in random order. SpO2 was recorded for 20 mins before and during eating, and dyspnea was recorded by the patient using a 10 cm visual analogue scale before and upon completion of eating. SETTING: Subjects were in-patients at an intermediate care facility who were hospitalized for pulmonary rehabilitation or for convalescence after an exacerbation of COPD. POPULATION STUDIED: Thirty-five patients with severe COPD (forced expiratory volume in 1 s [FEV1] less than 50% predicted, FEV1 to forced vital capacity ratio less than 65%) were studied. Mean age was 70.5 7.1 years. MAIN RESULTS: Mean SpO2 decreased significantly (P<0.05) from 91.7 3.4% to 90.1 4.0% during AE, and 91.7 3.2% to 90. 8 3.6% during PE. Mean lowest SpO2 was lower and percentage of time with SpO2 less than 90% was greater during eating compared with corresponding control periods during both AE and PE. Dyspnea increased significantly (P<0.05) from 1.4 1.2 to 3.3 2.3 cm during AE, and from 1.5 1.5 to 2.4 2.2 cm during PE. The increase in dyspnea was significantly greater during AE than PE. CONCLUSIONS: Eating is an activity that can adversely affect SpO2 and increase dyspnea in patients with severe COPD. Oxygen desaturation and particularly increased dyspnea may at least in part relate to the recruitment of upper extremity muscles during eating.

Evaluation of right ventricular systolic pressure during incremental exercise by Doppler echocardiography in adults with atrial septal defect.

STUDY OBJECTIVES: Pulmonary hypertension is the most important complication in patients with atrial septal defect (ASD), but its role in limiting exercise has not been examined. This study sought to evaluate exercise performance in adults with ASD and determine the contribution of elevated pulmonary artery pressure in limiting exercise capacity. DESIGN: We used Doppler echocardiography during exercise in 10 adults (aged 34 to 70 years) with isolated ASD (New York Heart Association class I, II) and an equal number of matched control subjects. Incremental exercise was performed on an electrically braked upright cycle ergometer. Expired gases and VE were measured breath-by-breath. Two-dimensional and Doppler echocardiographic images were obtained at rest prior to exercise to determine ASD size, stroke volume (SV), shunt ratio (Qp:Qs), right ventricular outflow tract (RVOT) size, and right ventricular systolic pressure at rest (RVSPr). Doppler echocardiography was repeated at peak exercise to measure right ventricular systolic pressure during exercise (RVSPex). RESULTS: Resting echocardiography revealed that RVOT was larger (21+/-4 vs 35+/-8 mm, mean+/-SD; p=0.0009) and RVSPr tended to be higher (17+/-8 vs 31+/-8 mm Hg; p=0.08) in ASD; however, left ventricular SV was not different (64+/-23 vs 58+/-23 mL; p>0.05), compared with control subjects. Despite normal resting left ventricular function, ASD patients had a significant reduction in maximum oxygen uptake (VO2max) (22.9+/-5.4 vs 17.3+/-4.2 mL/kg/min; p=0.005). RVSPex was higher (19+/-8 vs 51+/-10 mm Hg; p=0.001) and the mean RVSP-VO2 slope (1+/-2 vs 18+/-3 mm Hg/L/min; p=0.003) and intercept (17+/-4 vs 27+/-4 mm Hg; p=0.05) were higher in the ASD group. VO2max correlated inversely with both RVSPr (r=-0.69; p=0.007) and RVSPex (r=-0.67; p=0.01). CONCLUSION: These findings suggest that adults with ASD have reduced exercise performance, which may be associated with an abnormal increase in pulmonary artery pressure during exercise.

International differences in epidemiology of lung adenocarcinoma.

In Europe, the squamous cell carcinoma is the most frequent non-small cell lung cancer (NSCLC) subtype and until now, no increase in incidence of lung adenocarcinoma (ADC) has been described (except in the Netherlands), in contrast to North America where ADC predominates. Our aim was to compare the percentage of ADC in Montreal (MTL), Canada, with that in Strasbourg (STBG), France. We prospectively identified patients with NSCLC in MTL and in STBG over an 8-month period and described the distribution of NSCLC by sex, age, subtype and smoking history. A total of 172 patients in MTL and 166 in STBG were identified. The male/female ratio was significantly different in STBG (12:1) and in MTL (2:1). The percentage of ADC was significantly higher in MTL (40%) than in STBG (30%). This difference is partly due to the higher number of women with NSCLC in Montreal combined with the predominance of ADC in women. The proportion of ADC decreased with age in STBG, but was similar in each age category in MTL. In STBG, most women with NSCLC had never smoked (69%), in contrast to MTL where only 16% of women had never smoked. In conclusion, ADC is more frequent in MTL than in STBG. This is partly due to the higher number of women with NSCLC in MTL combined with the predominance of ADC in women. The greatest proportion of ADC subtype in the youngest cohorts of men in STBG suggests that ADC may be on the rise in this city.

The increasing incidence of lung adenocarcinoma: reality or artefact? A review of the epidemiology of lung adenocarcinoma.

Lung adenocarcinoma is the most common cell type in females (smokers or non-smokers) and in non-smoking males. Its incidence has been increasing in younger cohorts of males and females until very recent years. Changes in classification and in pathological techniques account for some of this increase. In females and non-smoker males, the increase could be partly due to a detection bias in former studies. Nevertheless, successive cohorts over time seem more likely to develop adenocarcinoma and less likely to develop squamous cell carcinoma. These differences between birth cohorts suggest that the increasing incidence of adenocarcinoma is not only due to changes in pathological diagnosis. Geographical differences are also observed: in Europe, the squamous cell type still predominates and an increase in incidence of adenocarcinoma has only been reported in the Netherlands. In Asia, in the 1960s and 1970s, the proportion of adenocarcinoma was higher than in North America or Europe and seems to be increasing. To what extent these differences are due to differences. In establishing diagnosis remains unknown. Despite these biases in temporal and geographical trends detailed in this review, there has probably been a true increase in incidence of adenocarcinoma. An explanation for this should be sought in studies on detailed smoking history and passive smoking exposure, occupational exposure, diet and cooking, pollution and other environmental factors.

Treatment of small cell lung carcinoma in the elderly.

BACKGROUND: The number of elderly people with small cell lung carcinoma (SCLC) is increasing and currently nearly 25% are older than 70 years. Elderly patients may not tolerate intensive therapy and, therefore, often do not receive such treatment. Additionally, age may be an independent predictor for response and survival. We compared the investigation, staging procedure, and management of patients less than 60 years, 60 to 69, and older than 70 years who were diagnosed with SCLC between 1985 and 1991. We hypothesized that elderly patients were investigated and treated less aggressively, and that their outcome was poorer than that of younger patients with SCLC. METHODS: Information on weight loss, performance status, coexisting disease, staging investigations, and treatment was recorded. Treatment was categorized as optimal or suboptimal using predetermined criteria, and correlated with patient age. Toxicity grade, response to treatment, and survival were noted. RESULTS: There were no differences among the 3 age groups with respect to disease stage, and weight loss, although poorer performance status and comorbidity were more common in those patients older than 70 years. Elderly patients were investigated and treated less aggressively than the 2 younger patient groups. The oldest group received smaller chemotherapy dosage, fewer cycles, and had more dose reductions compared to the younger patients. Only 1 of 81 elderly patients was enrolled on an experimental protocol as compared with 19% and 28% of the younger patient groups. Furthermore, elderly patients had the highest frequency of supportive care alone. There was a significant relationship between advanced age and suboptimal treatment, with those older than 70 years having an odds ratio (OR) of 0.30 (95% confidence interval (CI) 0.15-0.61), for having received optimal treatment. Despite this, survival was similar for younger and older groups of patients (OR 0.89, CI 0.6-1.3). CONCLUSIONS: Elderly patients had poorer pre-treatment performance status, greater comorbidity, were more likely to have suboptimal therapy and were almost never entered into clinical trials. Despite this their survival did not differ from that of younger patients with SCLC. Randomized trials of treatment, with assessment of quality of life, are necessary to determine the effect of modified regimens for elderly patients with SCLC.

Comparison of response evaluation in small cell lung cancer using computerized tomography and chest radiography.

The percentage of patients achieving a complete response (CR) to therapy is often used as a measure of treatment efficacy in SCLC. Chest radiographs are difficult to evaluate following therapy and differences in reported response rates may be due to interobserver variation. CT scans of the thorax are more costly and are not as easily obtained as routine studies. We wanted to determine whether the measurability rate and interobserver agreement on response was superior when using CT scans, compared to chest radiographs alone. Fourteen radiologists with expertise in CT imaging of the thorax, reviewed 15 cases of SCLC pre- and post-chemotherapy (210 observations). In the first session, reviewers were required to measure or evaluate all possible lesions on the plain chest radiograph done at pre- and post-treatment. In the second session, accompanying CT scans were provided in order to make the same assessments. The number of instances in which neoplastic disease was measurable in two cross-sectional diameters on pretreatment films was 164 (79%) on chest radiographs and 202 (97%) on CT scans. Interobserver agreement was assessed by comparing tumour measurements, as well as reports of complete disappearance of tumour (CR) among the 15 observers. The rate of CR ranged from 0 to 87% with the use of the chest radiograph, and from 0 to 95% using the CT scan. Usually after viewing the CT scan, readers changed their opinion as to whether CR had actually occurred. For example, in one case, 87% of readers judged response to therapy as a CR based on chest radiograph; upon reevaluation with a CT scan this figure decreased to 15%. Agreement as to response was better on review of the CT scans, compared to the chest radiograph in all but two cases. It is therefore recommended that pre- and post-treatment CT scans, and not just chest radiographs, be used for assessment of response to therapy.

A comparison of albuterol administered by metered dose inhaler (and holding chamber) or wet nebulizer in acute asthma.

BACKGROUND: Comparative studies of albuterol by wet nebulizer or metered dose inhaler have tested fixed doses of medications. We compared the dose-response relationship to albuterol by wet nebulization or metered dose inhaler in acute asthma. METHODS: Randomized, double-blind, placebo-controlled trial. Patients with acute asthma received either albuterol, 0.4 mg, by metered dose inhaler (and holding chamber) or albuterol, 2.5 mg, by wet nebulizer every 30 min until maximal bronchodilation. Forty patients (forced expiratory volume in 1 s [FEV1]: 1.15 +/- 0.43 L; 36 +/- 12 percent predicted) received metered dose inhaler and 40 others (FEV1: 1.08 +/- 0.52 L; 35 +/- 16 percent predicted) received wet nebulization. RESULTS: Twenty-six patients (65 percent) receiving metered dose inhaler and 30 (75 percent) receiving wet nebulization achieved maximal bronchodilation after two doses. Almost all reached maximal bronchodilation by four doses. The FEV1 improved by 0.72 +/- 0.49 L for metered dose inhaler and 0.68 +/- 0.61 L for wet nebulizer (p = 0.71). A significant linear relationship was seen in both groups (metered dose inhaler r = 0.94; wet nebulizer r = 0.98) between the log dose of albuterol and change in FEV1. About 1/6 the wet nebulizer dose of albuterol was needed to achieve similar response to the metered dose inhaler. CONCLUSIONS: Albuterol by metered dose inhaler provided similar bronchodilation to that achieved by wet nebulization in patients with acute asthma. The cumulative dose-response technique is applicable in the emergency department setting and is helpful in comparing the relative utility of various bronchodilator regimens.

Pulmonary toxicity of antineoplastic therapy.

Pulmonary toxicity is often encountered in patients receiving antineoplastic therapy. It may produce a wide variety of clinicopathologic syndromes. As new agents are introduced and their adverse effects recognized, clinicians should be ever vigilant about the possibility of pulmonary toxicity.

Uses and misuses of oxygen in hospitalized patients.

PURPOSE: To document the use/misuse of oxygen therapy as compared with antibiotics in internal medicine inpatients. To determine whether the same care was being taken in the prescription and administration of both forms of therapy. PATIENTS: Ninety-six eligible patients at a university teaching hospital were entered into a study examining the use of oxygen. They were compared with 60 patients for whom antibiotics were prescribed. MATERIAL AND METHODS: Three hundred thirty consecutive newly admitted patients were screened for the presence of either (1) oxygen apparatus at the bedside, (2) physician orders for oxygen, or (3) oxygen orders noted in nursing kardex. Those meeting one of these three criteria were observed on up to four occasions over 48 hours following study entry. Similarly, patients were screened for medical orders or nursing medication kardex notation for antibiotics and were also observed for proper prescription and administration of medication. Procedural errors, determined by kardex audit and direct patient observation, were compared for the individuals with specific oxygen orders and those receiving antibiotics. RESULTS: Oxygen delivery apparatus was found in the room in 17 of 96 patients without it ever having been ordered by the physician or noted in the nursing kardex. In 27 of 96 patients, oxygen was noted in the nursing kardex and administered to patients without a physician order. There were no cases of antibiotic therapy without a physician order. Observations of 47 patients with specific orders for oxygen revealed the following errors: (1) physician order incorrectly transcribed to nursing kardex (16%), (2) flow meter off (34%), (3) oxygen delivery apparatus improperly worn (57%), (4) wrong fractional inspired oxygen concentration (58%). Improper transcription of antibiotic orders occurred on only one occasion (2%), and antibiotics were improperly administered in 5%. Arterial blood gas determinations preceded oxygen orders in 61%, whereas microbiologic cultures preceded antibiotic orders in 87% of the patients. CONCLUSIONS: We conclude that oxygen therapy is neither prescribed nor administered with the same attention that is given to other drugs such as antibiotics. Oxygen prescription and/or delivery is associated with significantly greater error than that seen with antibiotics. Education of medical personnel should stress more prudent prescription and use of oxygen in hospitalized patients.

Long-term postthoracotomy pain.

We used a pain questionnaire to evaluate the prevalence and functional significance of long-term postthoracotomy pain. Data on 56 patients who were at least 2 months postsurgery were analyzed. Thirty patients (54 percent) with a median follow-up of 19.5 months had persistent pain; 26 others were pain free at a median of 30.5 months postthoracotomy. Pain was reported in 24 of 44 patients (55 percent) who were more than one year after surgery, 13 of 29 patients (45 percent) more than two years, six of 16 (38 percent) more than three years, and three of ten patients (30 percent) greater than four years postthoracotomy. Pain intensity was low, but 13 patients stated that pain "slightly" or "moderately" interfered with their lives. Five of 56 patients had sufficiently severe chronic pain to require either daily analgesic use, nerve blocks, relaxation therapy, acupuncture, or referral to a pain clinic. We conclude that long-term chest wall pain is common postthoracotomy. It is generally not severe, but a small proportion of patients may experience persistent, moderately disabling pain.

Effect of caffeine on histamine bronchoprovocation in asthma.

It was recently reported that caffeine may reduce the clinical symptoms of asthma and may prevent the clinical manifestations of this disease. The effect of caffeine on histamine responsiveness is unknown. The effect of caffeine (5 mg/kg) and placebo on histamine responsiveness (the provocation concentration causing a 20% fall in FEV1, PC20) was studied in 10 subjects with mild asthma (prechallenge FEV1 84% of predicted value). The PC20 for histamine bronchoprovocation after caffeine ingestion was 2.65 (95% confidence limits 0.99, 7.10) mg/ml. After placebo the PC20 was 1.89 (0.96, 3.71) mg/ml. It is concluded that caffeine in a dose equivalent to about three cups of coffee has a very small effect, if any, on histamine bronchoprovocation in those with mild asthma. Specific instructions about not having drinks containing caffeine before histamine challenge are therefore not necessary.

Small cell lung cancer presenting as a solitary pulmonary nodule.

Small cell lung cancer (SCLC) rarely presents radiographically as a solitary pulmonary nodule (SPN). Twenty-five patients with this feature were identified among 408 individuals with SCLC at McGill University (Montreal, Quebec) from 1979 through 1984. Of these, 15 (60%) were confirmed on pathologic review as SCLC (ten intermediate cell, four oat cell, one indeterminate). Pathologic review of a control group comprising 24 other limited-disease patients who were long-term survivors (greater than 20 months) confirmed 20 (84%) as SCLC (eight intermediate cell, 12 oat cell). Ten of the 15 patients with SPN were resected whereas five had chemotherapy and/or radiotherapy as primary treatment. Postoperative chemotherapy was administered to most of the resected patients. The median survival of the 15 patients with SPN was 24 months, a significantly longer survival than the other patients with SCLC. This improved prognosis in patients with SPN may be due to smaller initial tumor burden or to a fundamental biologic difference between SPN and other forms of SCLC.

Sensation of inspired volumes and pressures in professional wind instrument players.

Previous studies have failed to show consistent differences in pulmonary function between wind instrument musicians and normal controls. In this study, respiratory sensation was assessed in 13 professional wind instrument players and 13 age-matched controls. Psychophysical techniques were used to assess magnitude estimation and reproduction of lung volumes and inspiratory pressures. The exponent for volume magnitude estimation was not different in musicians and controls (1.17 +/- 0.11 vs. 1.16 +/- 0.11), but volume reproduction was more accurate in musicians. The mean exponent for pressure magnitude estimation was 1.34 +/- 0.14 and 1.06 +/- 0.09 (P = 0.057) in musicians and controls, respectively. There was no difference between groups for absolute or constant error for pressure reproduction. Professional wind instrument players appear to have some inherent or acquired differences in respiratory perception and ventilatory neuromuscular control compared with other normal subjects.

Continuous nebulization of albuterol (salbutamol) in acute asthma.

We studied the safety and efficacy of albuterol (salbutamol) delivered by continuous nebulization (CN) in the initial emergency department treatment of asthma. In a randomized fashion 21 patients received 5 mg of albuterol by bolus nebulization (BN) at time 0 and again 60 minutes later. Twenty-one others received albuterol (0.2 mg/ml) by CN using a calibrated nebulizer with a known output of 25 ml/h. Thus, each patient had received 10 mg of albuterol over two hours. FEV1, blood pressure (BP), heart rate (HR), respiratory rate (RR), and hand tremor were recorded at 30-minute intervals. The FEV1 was 1.48 +/- 0.64 L prior to BN and increased to a maximum of 2.20 +/- 0.94 L (p less than 0.05) 90 minutes later. The FEV1 prior to CN was 1.13 +/- 0.51 L and improved to 2.20 +/- 1.02 L (p less than 0.05) at 120 minutes. The FEV1 did not differ significantly between regimens over the 2-hour period. Both modes of therapy were well tolerated. There was a slight but significant increase in HR at 30 and 90 minutes in the BN group when compared with CN. There was no significant difference in BP, RR, or tremor between the groups. Thus, albuterol by CN was found to be equally effective as the same medication by BN in the early treatment of asthma in patients seen in the emergency department.

The relationship between pulmonary function and dyspnea in obstructive lung disease.

Increased importance is now being placed on evaluating dyspnea in patients with obstructive lung disease (OLD). We measured breathlessness at rest, using a Borg scale dyspnea index (BSDI) before and after bronchodilator albuterol [salbutamol] 200 micrograms) in 93 patients with OLD drawn from a larger population undergoing routine spirometry. The median BSDI declined from 3 to 1 before and after bronchodilator, suggesting improvement in dyspnea. However, there was no correlation between initial or postbronchodilator spirometry and BSDI. The change in FEV1 similarly did not correlate with the change in BSDI (r = 0.05). A large bronchodilator response was usually associated with improvement in dyspnea, but the converse was not observed. Thus, of ten patients with an improvement in BSDI of more than two categories, six had a change in FEV1 of 0.1 L or less after bronchodilator. Analyzing a subgroup of 65 dyspneic patients with an initial BSDI of 2 or more revealed the following response groups: those with either a bronchodilator or dyspnea response alone, both together, or neither. Twenty-eight patients (43 percent) responded both subjectively and objectively. Eleven (17 percent) had a bronchodilator response only, 17 (26 percent) had a dyspnea response only, while nine (14 percent) had neither measurable response. We conclude that dyspnea is poorly correlated with results of routine spirometry in patients with OLD. The use of dyspnea ratings may yield information about bronchodilator responsiveness not appreciated by spirometry alone.

Coordination of eating, drinking and breathing in adults.

The coordination of breathing and swallowing was studied in seven normal males at rest, as well as during eating and drinking. Ventilation was monitored using respiratory inductive plethysmography and swallowing was recorded by submental electromyogram. Swallowing occurred almost exclusively in expiration. Two hundred and seventy-one swallows were seen and only two occurred in inspiration. The mean expiratory duration (Te) of breaths containing a swallow was significantly greater than the mean Te of all breaths during the same period. There was no change in mean tidal volume (VT), inspiratory duration (Ti), expiratory duration (Te), mean inspiratory flow (VT/Ti) or minute ventilation (VE) between the periods of normal breathing, eating and drinking. However, breathing became more irregular during eating and drinking. The mean coefficient of variation of VT, Te, and VT/Ti was significantly greater during eating and drinking than at rest. The coefficient of variation for VT was 22 + 3 percent, 36 +/- 5 percent, and 41 +/- 5 percent during the initial period of resting breathing, eating and drinking. For VT/Ti it was 24 +/- 6 percent, 43 +/- 14 percent and 44 +/- 8 percent during resting breathing, eating and drinking. We conclude that swallowing is almost exclusively an expiratory activity. This may play a protective role in preventing aspiration. Although the level of ventilation is maintained constant during eating and drinking, the pattern of breathing becomes increasingly irregular. This may contribute to dyspnea during meals in some patients with lung disease.

Hemothorax and hemopericardium in a patient with Bean's blue rubber bleb nevus syndrome.

A patient with Bean's blue rubber bleb nevus syndrome presented with a hemothorax, and 18 years earlier had presented with "idiopathic" hemopercardium and tamponade. Typical hemangiomas were found in the pleura and skin. This is the first report of intrathoracic bleeding with this disorder.