196
against any profit being made by donors, arguing that such payments should be banned by law (subject only to the proper cost of recovering and processing tissue being recoverable by reputable suppliers of human tissue). As to indemnification of living donors, it would go no further than payments to cover out-of-pocket expenses, loss of income, and subsequent sickness. The publicity which the commission's activities attracted in the course of preparing and publishing its report did a lot in Australia to remedy the ignorance of the public and apathy of the medical profession towards this important subject. Of particular interest is its warning that the difficulties and distress experienced by medical staff in dealing with dying patients are likely to increase rather than diminish as medical advances add to the patient's prospect of survival, and the report concluded: "Careful instruction and precept in medical ethics and behaviour and related subjects are in the commission's views likely to benefit both the student and the community. The deans of medical faculties throughout Australia are invited to consider the adequacy of the present curricula." Notwithstanding similar evidence given to the Royal Commission on Medical Education) some years ago by several important bodies in Britain, that report contained no reference whatsoever to medical ethics.
Repotrt No. 7, Humnian Tissuie Tranisplants. Canberra, Australian Government Publishing Service, 1977. British Medical Journal, 1978, 1, 120. British MedicalyJtornal, 1976, 2, 1187. British Medical Journal, 1974, 2, 344. Royal Commission on Medical Education, 1965-8, Report, Cmnd 3569. London, HM1SO, 1968. Law Reform Commission,
2
5
Prevention and treatment of retinal detachment
28 JANUARY 1978
BRITISH MEDICAL JOURNAL
detachment is based on his principle of finding and closing the hole so that the retina may flatten. Success in retinal surgery depends, therefore, not only on surgical skill and judgment but also on expert ophthalmoscopy. Centres specialising in retinal detachment surgery now claim 80-90',, success rates in anatomical reattachment of the retina in this type of lesion. Furthermore, the fact that retinal holes are the precursors of retinal detachment allows a logical approach to prophylaxis. All patients coming to an ophthalmic clinic for the first time should have their pupils dilated so that the peripheral retina may be examined for flat retinal holes or weak areas. These may then be assessed for treatment either by photocoagulation or by cryotherapy. Certain groups should be examined with special care-those who have already had a retinal detachment in one eye, myopic and aphakic patients, and those who have suffered trauma to the eye. The skill required for identifying retinal holes is one of the main arguments against allowing opticians to be in charge of primary ophthalmic care. \WIith the growing use of photocoagulation for diabetic retinopathy, especially in the early proliferative phase,"5 the incidence of traction retinal detachment in diabetics should decline. At present, however, thcse lesions present major problems to the ophthalmologist and often require surgery, both to deal with the detachment and also to remove the source of traction by vitrectomy.' Combined vitrectomy and retinal detachment surgery has also proved to be an important advance in treating ocular trauma-much of which is due to injuries from car windscreens. Here prevention ought to be possible through legislation to make the wearing of seat belts compulsory and through the general use of laminated glass windscreens. B1ohringer, H R, ()phthalmolow'ica, 1956. 131, 331. Scheic, H B, Morse, P H, and Aminlari, A, Archives of ()phthlmtologv,, 1973, 89, 293. 3Boase, D), t al. Trasuacti(os of the ()phrhalmohnlt,ical Societies of the United Kin',gdom, 1978, 97, in press. Duke-Elder, S (ed), System of ()phthalmohwv£y, vol 10. Lonidon, Kimpnton,
2
1967.
Critics of high technology medicine who question its ability to add to the sum total of human happiness should perhaps look at the successes of treatment for retinal detachment. Detachment of the retina is a common problem for ophthalmologists, affecting as it does about one in every 20 000 of the population each year.' By far the most common type of detachment is that associated with retinal hole formation-rhegmatogenous detachment-and certain highrisk groups can be identified. These include shortsighted people and patients who have had their cataracts removed. People with myopia of over - 10 D run 45 times the normal risk of retinal detachment,' and among aphakic patients the incidence is about 2",,.2 This typc of detachment responds favourably to prophylactic treatment and to surgery. Exudative detachments occur as a secondary phenomenon associated, for example, with acute systemic hypertension and with ocular vascular or malignant tumours; their management depends on treating the precipitating condition. The third type of retinal detachment is associated with traction from fibrous tissue developing within the eye and the related vitreous stress. This type of detachment is common in patients with advanced diabetic retinopathy and may also be caused by direct trauma; again it is becoming increasingly amenable to treatment by modern techniques.:' The natural history of rhegmatogenous detachments is disastrous.4 The retina invariably becomes totally detached, and the eye loses all vision. It was Gonin5 who first realised the significance of the retinal hole, and all surgery of retinal
5Gonin, J, Le D&collc7ncwt de la RPrtin. L.ausanne, Librairc Pavot, 1934. fi
Cheng, H, et al, British
Mfedicalyo/Jonal, 1977, 1,
739.
Tumours of the nose Nasal obstruction is a symptom that leads most people to seek medical advice with little delay. Every practitioner should be able to examine the interior of the nose, and anterior rhinoscopy wi]l usually confirm clinicial suspicions. The most common causes of obstruction are deviation of the nasal septum, nasal polypi, and allergic rhinitis-all diagnosable readily enough from the history and the fact that the obstruction is usually (but not always) bilateral. On palpation a simple nasal polyp is soft and mobile, and does not bleed. Often its presence is obvious; but long-standing nasal polypi may present in the anterior nares, where the exposed surface can become thickened and vascular, making the polyp difficult to distinguish from a tumour without biopsy. In fact, innocent tumours are not common. Most of those that do occur are squamous papillomas, transitional cell papillomas, or haemangiomas; neurofibromas, keratoacanthomas, and chondromas are rarities, as are the intranasal mixed tumours (also known as pleomorphic salivary adenomas), which present special features. M\alignant tumours are rare: they comprise 0 3",, of all cancers in England but close to 1, in Africa.' Squamous carcinoma is seen most often; malignant
BRITISH MEDICAL JOURNAL
melanomas occur less frequently; and rare varieties include lymphosarcomas and other sarcomas, gliomas, plasmacytomas, and mucoepidermoid carcinomas. The most common malignant tumour seen in the nose is a late secondary extension of carcinoma of the maxillary sinuses, the treatment being that of the primary tumour. Both innocent and malignant neoplasms may cause a purulent nasal discharge. Pain or bleeding will also occur if a tumour of either type destroys bone by pressure absorption. In general, tumours confined to the nasal passages produce nasal obstruction and epistaxis rapidly while those that invade the nasal cavity secondarily (such as carcinomas of the paranasal sinuses) produce symptoms late in the course of the disease; often the diagnosis is not apparent until the tumour has penetrated the surrounding bony walls, causing swelling of the face, proptosis, and epistaxis. Swelling of the palate, pain, or loosening of the teeth may send the patient to a dental surgeon, and proptosis or unilateral epiphora to an ophthalmologist. Since the clinical differentiation between a simple and a malignant neoplasm of the nose may be difficult, all such cases should be referred promptly to a rhinologist, whose investigations are likely to include anterior and posterior rhinoscopy, x-ray examination of the nose and paranasal sinuses, and biopsy of the swelling-absolutely essential for accurate diagnosis. Simple tumours are removed intranasally under general anaesthesia, preferably augmented by instillation of a cocaine solution to control haemorrhage. If a haemangioma is present the base is cauterised to prevent recurrence. Squamous cell papillomas of the nasal vestibule are quite common; again the base of the tumour should be cauterised, otherwise a squamous carcinoma may develop at the site of repeated excisions. The pleomorphic adenoma or mixed tumour is rare and until the series recently reported by Compagno and Wong, no single investigator had been able to collect enough cases for evaluation. Compagno and Wong have studied 40 cases and have follow-up data on 34 of these for over seven years. Most of these neoplasms originate from the mucous membrane of the bony or cartilaginous septum but they may also occur on the lateral nasal wall. They are seen in all age groups but are most common in white people in the third to sixth decade. The symptoms are of nasal obstruction and a mass in the nasal cavity and the lesions are seen as polypoid, broad-based swellings. Local but adequate excision is the treatment of choice, and most patients in the American series were treated by local or wide excision in the form of septectomv, turbinectomy via a lateral rhinotomy, or a Caldwell Luc procedure. Thirty-one of the 34 patients followed up did not have a recurrence of the tumour. Those that did recur either had not been completely removed or recurred locally and were removed again without further recurrence. Squamous carcinoma of the skin of the nose may extend into the vestibule or nasal septum and may be treated by excision or radiotherapy. Basal-cell carcinoma may affect both the nose and the paranasal sinuses. Early lesions respond well to radiotherapy, but wide surgical excision is necessary if the tumour has reached the underlying bone. Malignant melanoma of the nasal passages is uncommon; bleeding occurs early in the disease and the tumour may metastasise to cervical lymph nodes. Wide surgical excision is essential-the response to radiotherapy is poor-and chemotherapy combined with surgery is the most successful form of treatment. linsell,
197
28 JANUARY 1978
C( A, and Martvn, R, Fist Africa
MAeddical Jo7()urnal, 1962, 39, 642.
iMacbeth, R, 7Yorzal of Larynkgology, 1965, 79, 592. Willis, R A, Pathology of Tumnours, 3rd edn. London, Butterworths, 1960. Black, B K, and Smith, D E, Archives of Neurosurgery and Psychiatry, 1950, 64, 614. Wadsworth, P, Yoturntal of Laryngology, 1969, 83, 87. Harrison, D F N, Proceedisngs of thc Roval Societ Iof Mf edicinte, 1968, 61, 13. Compagno, J, and Wong, R T, Aineri'can Youtrnial of Clitlical Pathology, 1977, 68, 213.
Non-bacterial thrombotic endocarditis Non-bacterial thrombotic endocarditis is the term applied to bland vegetations of platelets and fibrin on a virtually normal heart valve. Sometimes there is minor degeneration of the collagen framework, but no appreciable inflammatory reaction occurs apart from an occasional macrophage infiltration.' 2 The condition normally comes to light as a coincidental postmortem finding-usually in about 05",, of consecutive necropsies-in patients who have died of other conditions, notably cancer; thus it used to be regarded as an agonal phenomenon and was called terminal endocarditis. Recently, however, it has become increasingly evident that non-bacterial thrombotic endocarditis is not always a silent, terminal condition. The sterile, rather friable vegetations have a tendency to embolise, and occasionally the systemic embolism may produce clinical syndromes due to infarction of the brain, kidney, or spleen. The endocardial lesion may be the dominant feature of an illness due to an adenocarcinoma of the pancreas, stomach, or lung that has remained completely silent. The condition should therefore always be suspected in a patient with known malignant disease who suddenly develops symptoms due to occlusion of one or more arteries. Sometimes there is also migratory thrombophlebitis-long known to complicate carcinoma of the pancreas, stomach, colon, and lung-and this association of intravascular clotting disorders suggests that the conditions are related to some disturbance of the coagulation mechanism. Among the clotting abnormalities that have been found are hyperfibrinogenaemia, hypofibrinogenaemia, thrombocytopenia, and decreased concentrations of factors V, VIII, and XIII.:3 Circulating fibrin degradation products have also been found. Many of these findings are compatible with disseminated intravascular coagulation, and some cases of nonbacterial thrombotic endocarditis may well be associated with this widespread disorder of clotting. Interestingly, non-bacterial thrombotic endocarditis has been reported in the adult type of respiratory distress syndrome precipitated by poisoning with the tricyclic antidepressant drug amitriptyline. X One factor in the pathogenesis of this type of respiratory distress syndrome appears to be the embolism of microthrombi from the peripheral circulation to the capillaries of the lungs. The factors that predispose to the development of microthrombi in the peripheral vasculature mav also operate in some cases of non-bacterial thrombotic endocarditis. Apart from its regional embolic features non-bacterial thrombotic endocarditis is usually silent clinically. Evidence of cardiac dysfunction is seldom present, as there is no valvular destruction. Coronary embolism, however, sometimes occurs. Fayemi and Deppisch 5 have recently reviewed six cases of non-bacterial thrombotic endocarditis with coronary embolism and myocardial infarction-part of a series of 65 cases of nonbacterial thrombotic endocarditis encountered during 10 years
against any profit being made by donors, arguing that such payments should be banned by law (subject only to the proper cost of recovering and processing tissue being recoverable by reputable suppliers of human tissue). As to indemnification of living donors, it would go no further than payments to cover out-of-pocket expenses, loss of income, and subsequent sickness. The publicity which the commission's activities attracted in the course of preparing and publishing its report did a lot in Australia to remedy the ignorance of the public and apathy of the medical profession towards this important subject. Of particular interest is its warning that the difficulties and distress experienced by medical staff in dealing with dying patients are likely to increase rather than diminish as medical advances add to the patient's prospect of survival, and the report concluded: "Careful instruction and precept in medical ethics and behaviour and related subjects are in the commission's views likely to benefit both the student and the community. The deans of medical faculties throughout Australia are invited to consider the adequacy of the present curricula." Notwithstanding similar evidence given to the Royal Commission on Medical Education) some years ago by several important bodies in Britain, that report contained no reference whatsoever to medical ethics.
Repotrt No. 7, Humnian Tissuie Tranisplants. Canberra, Australian Government Publishing Service, 1977. British Medical Journal, 1978, 1, 120. British MedicalyJtornal, 1976, 2, 1187. British Medical Journal, 1974, 2, 344. Royal Commission on Medical Education, 1965-8, Report, Cmnd 3569. London, HM1SO, 1968. Law Reform Commission,
2
5
Prevention and treatment of retinal detachment
28 JANUARY 1978
BRITISH MEDICAL JOURNAL
detachment is based on his principle of finding and closing the hole so that the retina may flatten. Success in retinal surgery depends, therefore, not only on surgical skill and judgment but also on expert ophthalmoscopy. Centres specialising in retinal detachment surgery now claim 80-90',, success rates in anatomical reattachment of the retina in this type of lesion. Furthermore, the fact that retinal holes are the precursors of retinal detachment allows a logical approach to prophylaxis. All patients coming to an ophthalmic clinic for the first time should have their pupils dilated so that the peripheral retina may be examined for flat retinal holes or weak areas. These may then be assessed for treatment either by photocoagulation or by cryotherapy. Certain groups should be examined with special care-those who have already had a retinal detachment in one eye, myopic and aphakic patients, and those who have suffered trauma to the eye. The skill required for identifying retinal holes is one of the main arguments against allowing opticians to be in charge of primary ophthalmic care. \WIith the growing use of photocoagulation for diabetic retinopathy, especially in the early proliferative phase,"5 the incidence of traction retinal detachment in diabetics should decline. At present, however, thcse lesions present major problems to the ophthalmologist and often require surgery, both to deal with the detachment and also to remove the source of traction by vitrectomy.' Combined vitrectomy and retinal detachment surgery has also proved to be an important advance in treating ocular trauma-much of which is due to injuries from car windscreens. Here prevention ought to be possible through legislation to make the wearing of seat belts compulsory and through the general use of laminated glass windscreens. B1ohringer, H R, ()phthalmolow'ica, 1956. 131, 331. Scheic, H B, Morse, P H, and Aminlari, A, Archives of ()phthlmtologv,, 1973, 89, 293. 3Boase, D), t al. Trasuacti(os of the ()phrhalmohnlt,ical Societies of the United Kin',gdom, 1978, 97, in press. Duke-Elder, S (ed), System of ()phthalmohwv£y, vol 10. Lonidon, Kimpnton,
2
1967.
Critics of high technology medicine who question its ability to add to the sum total of human happiness should perhaps look at the successes of treatment for retinal detachment. Detachment of the retina is a common problem for ophthalmologists, affecting as it does about one in every 20 000 of the population each year.' By far the most common type of detachment is that associated with retinal hole formation-rhegmatogenous detachment-and certain highrisk groups can be identified. These include shortsighted people and patients who have had their cataracts removed. People with myopia of over - 10 D run 45 times the normal risk of retinal detachment,' and among aphakic patients the incidence is about 2",,.2 This typc of detachment responds favourably to prophylactic treatment and to surgery. Exudative detachments occur as a secondary phenomenon associated, for example, with acute systemic hypertension and with ocular vascular or malignant tumours; their management depends on treating the precipitating condition. The third type of retinal detachment is associated with traction from fibrous tissue developing within the eye and the related vitreous stress. This type of detachment is common in patients with advanced diabetic retinopathy and may also be caused by direct trauma; again it is becoming increasingly amenable to treatment by modern techniques.:' The natural history of rhegmatogenous detachments is disastrous.4 The retina invariably becomes totally detached, and the eye loses all vision. It was Gonin5 who first realised the significance of the retinal hole, and all surgery of retinal
5Gonin, J, Le D&collc7ncwt de la RPrtin. L.ausanne, Librairc Pavot, 1934. fi
Cheng, H, et al, British
Mfedicalyo/Jonal, 1977, 1,
739.
Tumours of the nose Nasal obstruction is a symptom that leads most people to seek medical advice with little delay. Every practitioner should be able to examine the interior of the nose, and anterior rhinoscopy wi]l usually confirm clinicial suspicions. The most common causes of obstruction are deviation of the nasal septum, nasal polypi, and allergic rhinitis-all diagnosable readily enough from the history and the fact that the obstruction is usually (but not always) bilateral. On palpation a simple nasal polyp is soft and mobile, and does not bleed. Often its presence is obvious; but long-standing nasal polypi may present in the anterior nares, where the exposed surface can become thickened and vascular, making the polyp difficult to distinguish from a tumour without biopsy. In fact, innocent tumours are not common. Most of those that do occur are squamous papillomas, transitional cell papillomas, or haemangiomas; neurofibromas, keratoacanthomas, and chondromas are rarities, as are the intranasal mixed tumours (also known as pleomorphic salivary adenomas), which present special features. M\alignant tumours are rare: they comprise 0 3",, of all cancers in England but close to 1, in Africa.' Squamous carcinoma is seen most often; malignant
BRITISH MEDICAL JOURNAL
melanomas occur less frequently; and rare varieties include lymphosarcomas and other sarcomas, gliomas, plasmacytomas, and mucoepidermoid carcinomas. The most common malignant tumour seen in the nose is a late secondary extension of carcinoma of the maxillary sinuses, the treatment being that of the primary tumour. Both innocent and malignant neoplasms may cause a purulent nasal discharge. Pain or bleeding will also occur if a tumour of either type destroys bone by pressure absorption. In general, tumours confined to the nasal passages produce nasal obstruction and epistaxis rapidly while those that invade the nasal cavity secondarily (such as carcinomas of the paranasal sinuses) produce symptoms late in the course of the disease; often the diagnosis is not apparent until the tumour has penetrated the surrounding bony walls, causing swelling of the face, proptosis, and epistaxis. Swelling of the palate, pain, or loosening of the teeth may send the patient to a dental surgeon, and proptosis or unilateral epiphora to an ophthalmologist. Since the clinical differentiation between a simple and a malignant neoplasm of the nose may be difficult, all such cases should be referred promptly to a rhinologist, whose investigations are likely to include anterior and posterior rhinoscopy, x-ray examination of the nose and paranasal sinuses, and biopsy of the swelling-absolutely essential for accurate diagnosis. Simple tumours are removed intranasally under general anaesthesia, preferably augmented by instillation of a cocaine solution to control haemorrhage. If a haemangioma is present the base is cauterised to prevent recurrence. Squamous cell papillomas of the nasal vestibule are quite common; again the base of the tumour should be cauterised, otherwise a squamous carcinoma may develop at the site of repeated excisions. The pleomorphic adenoma or mixed tumour is rare and until the series recently reported by Compagno and Wong, no single investigator had been able to collect enough cases for evaluation. Compagno and Wong have studied 40 cases and have follow-up data on 34 of these for over seven years. Most of these neoplasms originate from the mucous membrane of the bony or cartilaginous septum but they may also occur on the lateral nasal wall. They are seen in all age groups but are most common in white people in the third to sixth decade. The symptoms are of nasal obstruction and a mass in the nasal cavity and the lesions are seen as polypoid, broad-based swellings. Local but adequate excision is the treatment of choice, and most patients in the American series were treated by local or wide excision in the form of septectomv, turbinectomy via a lateral rhinotomy, or a Caldwell Luc procedure. Thirty-one of the 34 patients followed up did not have a recurrence of the tumour. Those that did recur either had not been completely removed or recurred locally and were removed again without further recurrence. Squamous carcinoma of the skin of the nose may extend into the vestibule or nasal septum and may be treated by excision or radiotherapy. Basal-cell carcinoma may affect both the nose and the paranasal sinuses. Early lesions respond well to radiotherapy, but wide surgical excision is necessary if the tumour has reached the underlying bone. Malignant melanoma of the nasal passages is uncommon; bleeding occurs early in the disease and the tumour may metastasise to cervical lymph nodes. Wide surgical excision is essential-the response to radiotherapy is poor-and chemotherapy combined with surgery is the most successful form of treatment. linsell,
197
28 JANUARY 1978
C( A, and Martvn, R, Fist Africa
MAeddical Jo7()urnal, 1962, 39, 642.
iMacbeth, R, 7Yorzal of Larynkgology, 1965, 79, 592. Willis, R A, Pathology of Tumnours, 3rd edn. London, Butterworths, 1960. Black, B K, and Smith, D E, Archives of Neurosurgery and Psychiatry, 1950, 64, 614. Wadsworth, P, Yoturntal of Laryngology, 1969, 83, 87. Harrison, D F N, Proceedisngs of thc Roval Societ Iof Mf edicinte, 1968, 61, 13. Compagno, J, and Wong, R T, Aineri'can Youtrnial of Clitlical Pathology, 1977, 68, 213.
Non-bacterial thrombotic endocarditis Non-bacterial thrombotic endocarditis is the term applied to bland vegetations of platelets and fibrin on a virtually normal heart valve. Sometimes there is minor degeneration of the collagen framework, but no appreciable inflammatory reaction occurs apart from an occasional macrophage infiltration.' 2 The condition normally comes to light as a coincidental postmortem finding-usually in about 05",, of consecutive necropsies-in patients who have died of other conditions, notably cancer; thus it used to be regarded as an agonal phenomenon and was called terminal endocarditis. Recently, however, it has become increasingly evident that non-bacterial thrombotic endocarditis is not always a silent, terminal condition. The sterile, rather friable vegetations have a tendency to embolise, and occasionally the systemic embolism may produce clinical syndromes due to infarction of the brain, kidney, or spleen. The endocardial lesion may be the dominant feature of an illness due to an adenocarcinoma of the pancreas, stomach, or lung that has remained completely silent. The condition should therefore always be suspected in a patient with known malignant disease who suddenly develops symptoms due to occlusion of one or more arteries. Sometimes there is also migratory thrombophlebitis-long known to complicate carcinoma of the pancreas, stomach, colon, and lung-and this association of intravascular clotting disorders suggests that the conditions are related to some disturbance of the coagulation mechanism. Among the clotting abnormalities that have been found are hyperfibrinogenaemia, hypofibrinogenaemia, thrombocytopenia, and decreased concentrations of factors V, VIII, and XIII.:3 Circulating fibrin degradation products have also been found. Many of these findings are compatible with disseminated intravascular coagulation, and some cases of nonbacterial thrombotic endocarditis may well be associated with this widespread disorder of clotting. Interestingly, non-bacterial thrombotic endocarditis has been reported in the adult type of respiratory distress syndrome precipitated by poisoning with the tricyclic antidepressant drug amitriptyline. X One factor in the pathogenesis of this type of respiratory distress syndrome appears to be the embolism of microthrombi from the peripheral circulation to the capillaries of the lungs. The factors that predispose to the development of microthrombi in the peripheral vasculature mav also operate in some cases of non-bacterial thrombotic endocarditis. Apart from its regional embolic features non-bacterial thrombotic endocarditis is usually silent clinically. Evidence of cardiac dysfunction is seldom present, as there is no valvular destruction. Coronary embolism, however, sometimes occurs. Fayemi and Deppisch 5 have recently reviewed six cases of non-bacterial thrombotic endocarditis with coronary embolism and myocardial infarction-part of a series of 65 cases of nonbacterial thrombotic endocarditis encountered during 10 years
