- Email: [email protected]
Spontaneous bilateral pneumothoraces from synovial cell sarcoma
Respiratory Medichle (1992) 86, 357-360
Letters to the Editor Dear Editor
Special treatment for serious asthma We have a difficult asthma patient who has been taken by helicopter seven to eight times to central hospitals for respirator treatment. She is a farmers wife of 38, a non-smoker with small infection problems. Although she is allergic to grass, birch, cows etc, her disease behaves like an 'intrinsic asthma'. F o r more than a year she has had severe bronchospasms with 0.25-2 h intervals, and abundant mucus production. Conventional asthma treatment has been unsatisfactory. Steroids locally or systemic at times in very high doses, have been of limited help. Beta-agonists were used in rising doses, but because ofmuscle spasms and twitchings they can only now be used in small doses. The dosage of theophylline must be limited because of nausea. In a very difficult period we had to give her ether (diethylether) injections intravenously. We gave cyclosporin treatment during 1 year combined with methotrexate for 5 months. The benefits from this immunosuppressive treatment seemed so marginal that we stopped the treatment. To avoid mucous plugging of the airways we started to give her acute episodes o f overhydration by fast infusions of fluid via her subcutaneous 'vene port' which ends in the right atrium. During and 0.75-1.5 h after an infusion of 500-1000 ml Ringer acetate she expectorates a lot of very viscid mucus. We have tried to boost ciliary function by adding a small dose of terbutalin to the fluid. We believe that these infusions have kept her clear of helicopter trips and respirator treatment for more than a year now. We began to give her meperidine through her 'vene port' because she needed quick relief of coughing. We have found meperidine to be a very good anti-asthmatic medication for her: bronchospasms become milder or stop completely after injections of 75-200 mg, and peak expiratory flow rises from approximately 75 1min- ~ before injections to 200-3001 min- ~ after injection. She needs the injections so quickly and so often that she has to do them herself. Euphoric effects seems to be insignificant in her case. She never injects meperidine when not indicated for bronchospasms or severe cough. Respiration does not seem to become slower than 10-12 min- t even after repeated meperidine injections. For more than 1 year we thus have treated this patient with pressurized infusions of fluid one or two 0954-6111/92/040357 + 04 $03.00/0
times daily to avoid mucous plugging of airways and intracardial injections of meperidine 20-30 times in 24 h to stop bronchospasms. The regimen is tough for her, but we feel sure that it has saved her life. She is able to stay at home, and can in short periods be at ease together with her family. The anti-asthmatic effect of meperidine must be well known from respirator treatment. It seems to fit well with the role that the nervous system is thought to play in the production of bronchospasms. Can the fluid infusions work by influencing secretion of atrial natriuretic peptide? Or can overhydration give serous secretion in the mucosal glands so that plugs of viscid mucus in the glands are loosened, plugs which via sticky threads have anchored the clots of mucus on the epithelial surface? A. OYRI
Internal Medicine and Nephrology Musegata 66 4010 Stavanger Norway
Dear Editor
Spontaneous bilateral pneumothoraees from synoviai cell sarcoma I was interested to read the report of a case of bilateral spontaneous pneumothoraces from synovial cell sarcoma by O'Leary and colleagues (1). I saw a very similar case in 1974 at the Reading Central Chest Clinic, Berkshire. The patient was a previously healthy 16-year-old schoolgirl who presented to the surgical outpatient department with a mass in the right thigh which proved on biopsy to be a highly vascular synoviosarcoma. Her chest X-ray at that time was normal. She was treated with intermittent courses of radiotherapy and chemotherapy. Six months later her chest X-ray showed enlargement of the left hilum and an opacity behind the right third rib. Three months later a small, asymptomatic right-sided pneumothorax was noted, and 5 months afterwards (in April 1975) she attended a routine outpatients appointment, after having been swimming all morning; her chest X-ray showed bilateral pneumothoraces, approximately 70% on the right side and 20% on the left. She came to thoracotomy shortly afterwards when a necrotic leaking © 1992 Bailli,~re Tindall
358
Letters to the Editor
area in the right upper lobe was noted; partial pleurectomy was performed. Ten weeks later a left thoracotomy was carried out; torrential bleeding from the pulmonary artery, which was surrounded with necrotic tumour tissue, necessitated a pneumonectomy. She went slowly downhill and died 3 months later, at home. It was particularly interesting, from the physiological point of view, that at the time when she attended with bilateral pneumothoraces she was completely asymptomatic, and had noticed only mild dyspnoea earlier that same day when engaging in strenuous physical exertion. Incidentally, the patient reported by Singh and colleagues (2) and cited by O'Leary (1), is not the first report of such a case; a patient with bilateral pneumothoraces due to metastatic lung involvement 18 months after amputation of the right arm for synovial-cell sarcoma was included in the series reported by Dines and his colleagues in 1973 (3). N.J.C. SNELL Host Defence Unit National Heart and Lung Institute Emmanuel Kaye Building Manresa Road London SW3 6LR, U.K.
References I. O'Leary C, El Soussi M, Cowie J. Spontaneous bilateral pneumothoraces from synovial cell sarcoma. Respir Med 1991; 85: 533-534. 2. Singh H, Singh N, Kaur R. Bilateral spontaneous pneumothorax with pulmonary metastases from synovial cell sarcoma. BrJDis Chest 1977; 71:211-212. 3. Dines D, Cortese D, Brennan M, Hahn R, Payne W. Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax. Mayo Clin Proc 1973; 48: 541-544.
Dear Editor
Who needs referral to the hospital asthma specialist? Dr Bucknall's review (1) of referral to the hospital asthma specialist follows largely along the BTS guidelines and expands them to some degree. However, she does not tackle a very important area, whether referral should be obligatory or at least strongly suggested if the patient him/herself requests onward referral. With the advent of the Patients Charter this is put into more precise focus. However, it has always been my belief that if a patient consistently requests referral to a specialist for whatever condition, that should be
acquiesced to by the General Practitioner unless there are extremely good reasons why not. It may well be that the hospital consultant will say no more and do no more than the General Practitioner has already done, but the patients themselves will feel that 'no stone has been left unturned'. Our General Practitioner colleagues might feel that this a slur upon their ability, but this is in no way so. However hard one argues for control of the majority of asthma in General Practice there will always be patients who feel that they need to see a consultant and really will not be satisfied that all is being done that could be done until this happens. Recently a very sad case of a young girl was made known to me whose G P refused persistent requests from her father for referral on to hospital which culminated in the girl dying from acute severe asthma. The patient's reason for wishing to be referred may be many and varied, some of which were covered in Dr Bucknall's review but I do believe this is a fundamental point which needs to be addressed. J. G. AYRES Department of Respiratory Medicine East Birmingham Hospital Bordesley Green East Birmingham B9 5ST, U.K. 12 December 1991
Reference I. Bucknall CE. Who needs referral to the hospital asthma specialist? Respir Med 1991; 85: 453-455.
Dear Editor
Patient education, self management plans and peak flow measurement We note with interest the above brief review (1). In contrast to Dr Brewis we note a number of recent adult studies (2,3,4) all of which show an improvement in asthma control with an education programme. The different locations of these studies appear to make the results generalizable. A study of both adults and children (2) in a rural based primary care setting showed a clear benefit from both a symptom and peak flow based action plan. In the study by Mayo et al. (3) an action plan-based educational programme reduced significantly relapse to the Emergency Room and multiple readmissions to hospital. We feel that issues that need to be addressed in the future include: confirming Charlton's study regarding peak flow meters and their lack of use in a primary care
Letters to the Editor Dear Editor
Special treatment for serious asthma We have a difficult asthma patient who has been taken by helicopter seven to eight times to central hospitals for respirator treatment. She is a farmers wife of 38, a non-smoker with small infection problems. Although she is allergic to grass, birch, cows etc, her disease behaves like an 'intrinsic asthma'. F o r more than a year she has had severe bronchospasms with 0.25-2 h intervals, and abundant mucus production. Conventional asthma treatment has been unsatisfactory. Steroids locally or systemic at times in very high doses, have been of limited help. Beta-agonists were used in rising doses, but because ofmuscle spasms and twitchings they can only now be used in small doses. The dosage of theophylline must be limited because of nausea. In a very difficult period we had to give her ether (diethylether) injections intravenously. We gave cyclosporin treatment during 1 year combined with methotrexate for 5 months. The benefits from this immunosuppressive treatment seemed so marginal that we stopped the treatment. To avoid mucous plugging of the airways we started to give her acute episodes o f overhydration by fast infusions of fluid via her subcutaneous 'vene port' which ends in the right atrium. During and 0.75-1.5 h after an infusion of 500-1000 ml Ringer acetate she expectorates a lot of very viscid mucus. We have tried to boost ciliary function by adding a small dose of terbutalin to the fluid. We believe that these infusions have kept her clear of helicopter trips and respirator treatment for more than a year now. We began to give her meperidine through her 'vene port' because she needed quick relief of coughing. We have found meperidine to be a very good anti-asthmatic medication for her: bronchospasms become milder or stop completely after injections of 75-200 mg, and peak expiratory flow rises from approximately 75 1min- ~ before injections to 200-3001 min- ~ after injection. She needs the injections so quickly and so often that she has to do them herself. Euphoric effects seems to be insignificant in her case. She never injects meperidine when not indicated for bronchospasms or severe cough. Respiration does not seem to become slower than 10-12 min- t even after repeated meperidine injections. For more than 1 year we thus have treated this patient with pressurized infusions of fluid one or two 0954-6111/92/040357 + 04 $03.00/0
times daily to avoid mucous plugging of airways and intracardial injections of meperidine 20-30 times in 24 h to stop bronchospasms. The regimen is tough for her, but we feel sure that it has saved her life. She is able to stay at home, and can in short periods be at ease together with her family. The anti-asthmatic effect of meperidine must be well known from respirator treatment. It seems to fit well with the role that the nervous system is thought to play in the production of bronchospasms. Can the fluid infusions work by influencing secretion of atrial natriuretic peptide? Or can overhydration give serous secretion in the mucosal glands so that plugs of viscid mucus in the glands are loosened, plugs which via sticky threads have anchored the clots of mucus on the epithelial surface? A. OYRI
Internal Medicine and Nephrology Musegata 66 4010 Stavanger Norway
Dear Editor
Spontaneous bilateral pneumothoraees from synoviai cell sarcoma I was interested to read the report of a case of bilateral spontaneous pneumothoraces from synovial cell sarcoma by O'Leary and colleagues (1). I saw a very similar case in 1974 at the Reading Central Chest Clinic, Berkshire. The patient was a previously healthy 16-year-old schoolgirl who presented to the surgical outpatient department with a mass in the right thigh which proved on biopsy to be a highly vascular synoviosarcoma. Her chest X-ray at that time was normal. She was treated with intermittent courses of radiotherapy and chemotherapy. Six months later her chest X-ray showed enlargement of the left hilum and an opacity behind the right third rib. Three months later a small, asymptomatic right-sided pneumothorax was noted, and 5 months afterwards (in April 1975) she attended a routine outpatients appointment, after having been swimming all morning; her chest X-ray showed bilateral pneumothoraces, approximately 70% on the right side and 20% on the left. She came to thoracotomy shortly afterwards when a necrotic leaking © 1992 Bailli,~re Tindall
358
Letters to the Editor
area in the right upper lobe was noted; partial pleurectomy was performed. Ten weeks later a left thoracotomy was carried out; torrential bleeding from the pulmonary artery, which was surrounded with necrotic tumour tissue, necessitated a pneumonectomy. She went slowly downhill and died 3 months later, at home. It was particularly interesting, from the physiological point of view, that at the time when she attended with bilateral pneumothoraces she was completely asymptomatic, and had noticed only mild dyspnoea earlier that same day when engaging in strenuous physical exertion. Incidentally, the patient reported by Singh and colleagues (2) and cited by O'Leary (1), is not the first report of such a case; a patient with bilateral pneumothoraces due to metastatic lung involvement 18 months after amputation of the right arm for synovial-cell sarcoma was included in the series reported by Dines and his colleagues in 1973 (3). N.J.C. SNELL Host Defence Unit National Heart and Lung Institute Emmanuel Kaye Building Manresa Road London SW3 6LR, U.K.
References I. O'Leary C, El Soussi M, Cowie J. Spontaneous bilateral pneumothoraces from synovial cell sarcoma. Respir Med 1991; 85: 533-534. 2. Singh H, Singh N, Kaur R. Bilateral spontaneous pneumothorax with pulmonary metastases from synovial cell sarcoma. BrJDis Chest 1977; 71:211-212. 3. Dines D, Cortese D, Brennan M, Hahn R, Payne W. Malignant pulmonary neoplasms predisposing to spontaneous pneumothorax. Mayo Clin Proc 1973; 48: 541-544.
Dear Editor
Who needs referral to the hospital asthma specialist? Dr Bucknall's review (1) of referral to the hospital asthma specialist follows largely along the BTS guidelines and expands them to some degree. However, she does not tackle a very important area, whether referral should be obligatory or at least strongly suggested if the patient him/herself requests onward referral. With the advent of the Patients Charter this is put into more precise focus. However, it has always been my belief that if a patient consistently requests referral to a specialist for whatever condition, that should be
acquiesced to by the General Practitioner unless there are extremely good reasons why not. It may well be that the hospital consultant will say no more and do no more than the General Practitioner has already done, but the patients themselves will feel that 'no stone has been left unturned'. Our General Practitioner colleagues might feel that this a slur upon their ability, but this is in no way so. However hard one argues for control of the majority of asthma in General Practice there will always be patients who feel that they need to see a consultant and really will not be satisfied that all is being done that could be done until this happens. Recently a very sad case of a young girl was made known to me whose G P refused persistent requests from her father for referral on to hospital which culminated in the girl dying from acute severe asthma. The patient's reason for wishing to be referred may be many and varied, some of which were covered in Dr Bucknall's review but I do believe this is a fundamental point which needs to be addressed. J. G. AYRES Department of Respiratory Medicine East Birmingham Hospital Bordesley Green East Birmingham B9 5ST, U.K. 12 December 1991
Reference I. Bucknall CE. Who needs referral to the hospital asthma specialist? Respir Med 1991; 85: 453-455.
Dear Editor
Patient education, self management plans and peak flow measurement We note with interest the above brief review (1). In contrast to Dr Brewis we note a number of recent adult studies (2,3,4) all of which show an improvement in asthma control with an education programme. The different locations of these studies appear to make the results generalizable. A study of both adults and children (2) in a rural based primary care setting showed a clear benefit from both a symptom and peak flow based action plan. In the study by Mayo et al. (3) an action plan-based educational programme reduced significantly relapse to the Emergency Room and multiple readmissions to hospital. We feel that issues that need to be addressed in the future include: confirming Charlton's study regarding peak flow meters and their lack of use in a primary care