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COBRA-VENOM CONJUNCTIVITIS
1026 incidence of side-effects highlighted in the literature surely the balance in favour of a long-acting injection. Like Dr. Marshall,8 I believe these long-acting corticosteroid
- tips
preparations to be the patients with hayfever.
treatment
of choice in selected
220 Knock
Road, Castlereagh, Belfast BT5 6QD.
W. P. MCMILLIN.
COBRA-VENOM CONJUNCTIVITIS SIR,-At this health centre we have seen a few instances of venom being thrown into the eyes by the black-necked or spitting cobra (Naja nigricollis). The spitting cobra can spray a fine jet of venom up to several feet at the face and eyes of the intruder. The effect on the eyes depends on the quantity sprayed on them. Pain is intense, and congestion and watering of the eyes profuse. We treated these patients with an eye-wash followed by 4% lignocaine eyedrops to relieve the pain and one of the eye-drop preparations or systemic antihistaminics. They recovered within two or three days. We have not seen any complications. Marampa Mines Health Centre, Sierra Leone Development Co. Ltd. P.O. Box 7, Freetown, Sierra Leone, West Africa.
A. K. SINHA.
The functional defect of the aortic valve associated with cystic necrosis is combined stenosis and regurgitation, both of only moderate degree. It was suggested in 19571a that valve disease places unusual hsmodynamic stress on the aorta, leading to " structural fatigue ", expressed as cystic medial necrosis. It was further thought that congenital weakness in the aorta, although it cannot be excluded, is unlikely. Now I am not so certain. A patient we reported (case 2) showed cystic medial changes in arteries where hasmodynamic stress should be less of a factor-i.e., the abdominal aorta, subclavian and mesenteric arteries, and especially the pulmonary artery. An innate aortic weakness with congenitally bicuspid aortic valve is suggested by the report of Gore,2 who, among 32 cases of dissecting aneurysm in men under 40, found bicuspid valve in 8 (and in a 9th coarctation and bicuspid valve). Other cases of aortic dissection and bicuspid valve have been reported 23-e.g., the dramatic case of a 16-year-old girl of pyknic build who had rupture of the aorta and was found to have bicuspid valve and no coarctation. 24 Recently I discussed the cases of a father and son with the same association as the 16-year-old girl. The findings in these cases arouse my suspicions that bicuspid aortic valve may be an expression of a developmental defect of the arterial tree which is also expressed in coarctation of the aorta in some, in cystic medial necrosis in others, or in various combinations of these three. discovered on an Army induction examination have aortic regurgitation. He died in his sleep at age 19. Examination 2 months before death showed dilatation of the ascending aorta. At necropsy, rupture of the aorta into the pericardium was found, together with bicuspid aortic valve and extensive cystic medial necrosis of the aorta. The father was first discovered to have aortic regurgitation at age 19. Despite this he led a vigorous life as a businessman and was an avid squash and tennis player until age 44, when he developed exertional dyspnosa, and the aortic valve was replaced with a Starr-Edwards prosthesis by Dr. Vincent L. Gott. At " operation, the ascending aorta was dilated but not aneurysmal ". There was no evidence of dissection. The aortic valve was bicuspid, with three " grape-sized " areas of calcification. The leaflets were large and saggy. Because of recurrence of aortic regurgitation and symptoms of left ventricular failure a second operation was performed 21 months after the first. At this time a transverse intimal tear was found on the posterior wall of the aorta about 12 inches above the annulus of the valve. A complete double lumen involved the ascending aorta and transverse arch. The patient did not survive the operation. At necropsy the dissection was found to extend into the iliac arteries. Lack of reaction or thrombosis suggested that dissection had occurred recently, perhaps during the preparation for cardiopulmonary bypass. This finding and the absence of painful episodes suggest that aortic dissection occurred in the interval between operations. Microscopical examination showed extensive cystic necrosis of the aortic media. Experienced observers who saw both the father and son, and others including myself who examined the father, concluded that neither had skeletal features indicative of the Marfan syndrome. (Dolichostenomelia is often largely in the eye of the beholder; evaluation of skeletal features of the Marfan syndrome is highly subjective. Some who were convinced that the father and son had the Marfan syndrome described the father as " tall and thin with long thin fingers and high arched palate ". Another described him as a " short, mesomorphic male ".) The son was 180 cm. tall, the father 170 cm. Ectopia lentis was detected in neither. I conclude this was not the Marfan syndrome but rather bicuspid aortic valve with Erdheim’s cystic medial necrosis. The
ASSOCIATION OF CONGENITAL BICUSPID AORTIC VALVE AND ERDHEIM’S CYSTIC MEDIAL NECROSIS
SiR,—The association of congenital bicuspid aortic valve with coarctation of the aorta is well recognised. Abbott 9 found bicuspid aortic valve in 47 of 200 casesof coarctation, and others 10have found an even higher frequency. The aortic valve is often bicuspid in cases of a rare anomaly-complete interruption of the aortic arch. Roberts et all found such in all 3 of their cases and in at least 14 of 52 previously reported cases. Because of the poor engineering design as compared with a tricuspid valve (as pointed out by Leonardo da Vinci over 500 years ago,12 although he focused mainly on quadri- cuspid valves), bicuspid aortic valves 13,14 are frequently complicated by fibrotic and calcific changes (and, as Osler 15 pointed out in 1886, bacterial endocarditis). Much of the calcific aortic stenosis of older people may originate in a bicuspid valve 16; however, only 2 of 21 showed bicuspid valve in the series of Roberts et al." Several of the reported cases of associated aortic-valve disease and Erdheim’s cystic medial necrosis have had congenitally bicuspid valve. An association between aortic-valve disease and cystic medial necrosis of the aorta was pointed out in 195718 and has been documented several times since then.19-21 Marshall, B. Br. med. J. 1970, i, 302. Abbott, M. E. Am. Heart J. 1928, 3, 392, 574. Reifenstein, G. H., Levine, S. A., Gross, R. E. ibid. 1947, 33, 146 Roberts, W. C., Morrow, A. G., Braunwald, E. Circulation, 1962, 26, 39. 12. O’Malley, C. D., Saunders, C. M. Leonardo da Vinci on the Human Body. New York, 1952. 13. Tranchesi, J., Carral, R., De Amorin, I., Perraloza, D. Am. Heart J. 1954, 47, 664. 14. Roberts, W. C. Am. J. Cardiol. 1970, 26, 72. 15. Osler, W. Trans. Ass. Am. Physns, 1886, 2, 185. 16. Bacon, A. P. C., Matthews, M. B. Q. Jl Med. 1959, 28, 545. 17. Roberts, W. C., Perloff, J. K., Constantino, T. Am. J. Cardiol. 1971, 27, 497. 18. McKusick, V. A., Logue, R. B., Bahnson, H. T. Circulation, 1956, 46, 188. 19. Heath, D., Edwards, G. E., Smith, L. A. Proc. Mayo Clin. 1958, 33, 8. 9. 10. 11.
228. 20. 21.
Read, R. C., Wolf, P. New Engl. J. Med. 1964, 271, 816. Ditchek, T., Bookstein, J. J. Circulation, 1965, 31, 127.
son was
at age 17 to
Incidentally, I know of no report of familial occurrence bicuspid aortic valve. Familial aggregation should occur now and then by chance alone, because the frequency of congenitally bicuspid valve is placed between 1% and 2%." of
22. Gore, I. Archs Path. 1953, 55, 1. 23. Layman, T. E., Wang, Y. Med. Clins N. Am. 1968, 52, 1145. 24. Huntington, R. W., Jr., Hirst, A. E., Jr. Am. J. clin. Path. 1967, 48,
44.
- tips
preparations to be the patients with hayfever.
treatment
of choice in selected
220 Knock
Road, Castlereagh, Belfast BT5 6QD.
W. P. MCMILLIN.
COBRA-VENOM CONJUNCTIVITIS SIR,-At this health centre we have seen a few instances of venom being thrown into the eyes by the black-necked or spitting cobra (Naja nigricollis). The spitting cobra can spray a fine jet of venom up to several feet at the face and eyes of the intruder. The effect on the eyes depends on the quantity sprayed on them. Pain is intense, and congestion and watering of the eyes profuse. We treated these patients with an eye-wash followed by 4% lignocaine eyedrops to relieve the pain and one of the eye-drop preparations or systemic antihistaminics. They recovered within two or three days. We have not seen any complications. Marampa Mines Health Centre, Sierra Leone Development Co. Ltd. P.O. Box 7, Freetown, Sierra Leone, West Africa.
A. K. SINHA.
The functional defect of the aortic valve associated with cystic necrosis is combined stenosis and regurgitation, both of only moderate degree. It was suggested in 19571a that valve disease places unusual hsmodynamic stress on the aorta, leading to " structural fatigue ", expressed as cystic medial necrosis. It was further thought that congenital weakness in the aorta, although it cannot be excluded, is unlikely. Now I am not so certain. A patient we reported (case 2) showed cystic medial changes in arteries where hasmodynamic stress should be less of a factor-i.e., the abdominal aorta, subclavian and mesenteric arteries, and especially the pulmonary artery. An innate aortic weakness with congenitally bicuspid aortic valve is suggested by the report of Gore,2 who, among 32 cases of dissecting aneurysm in men under 40, found bicuspid valve in 8 (and in a 9th coarctation and bicuspid valve). Other cases of aortic dissection and bicuspid valve have been reported 23-e.g., the dramatic case of a 16-year-old girl of pyknic build who had rupture of the aorta and was found to have bicuspid valve and no coarctation. 24 Recently I discussed the cases of a father and son with the same association as the 16-year-old girl. The findings in these cases arouse my suspicions that bicuspid aortic valve may be an expression of a developmental defect of the arterial tree which is also expressed in coarctation of the aorta in some, in cystic medial necrosis in others, or in various combinations of these three. discovered on an Army induction examination have aortic regurgitation. He died in his sleep at age 19. Examination 2 months before death showed dilatation of the ascending aorta. At necropsy, rupture of the aorta into the pericardium was found, together with bicuspid aortic valve and extensive cystic medial necrosis of the aorta. The father was first discovered to have aortic regurgitation at age 19. Despite this he led a vigorous life as a businessman and was an avid squash and tennis player until age 44, when he developed exertional dyspnosa, and the aortic valve was replaced with a Starr-Edwards prosthesis by Dr. Vincent L. Gott. At " operation, the ascending aorta was dilated but not aneurysmal ". There was no evidence of dissection. The aortic valve was bicuspid, with three " grape-sized " areas of calcification. The leaflets were large and saggy. Because of recurrence of aortic regurgitation and symptoms of left ventricular failure a second operation was performed 21 months after the first. At this time a transverse intimal tear was found on the posterior wall of the aorta about 12 inches above the annulus of the valve. A complete double lumen involved the ascending aorta and transverse arch. The patient did not survive the operation. At necropsy the dissection was found to extend into the iliac arteries. Lack of reaction or thrombosis suggested that dissection had occurred recently, perhaps during the preparation for cardiopulmonary bypass. This finding and the absence of painful episodes suggest that aortic dissection occurred in the interval between operations. Microscopical examination showed extensive cystic necrosis of the aortic media. Experienced observers who saw both the father and son, and others including myself who examined the father, concluded that neither had skeletal features indicative of the Marfan syndrome. (Dolichostenomelia is often largely in the eye of the beholder; evaluation of skeletal features of the Marfan syndrome is highly subjective. Some who were convinced that the father and son had the Marfan syndrome described the father as " tall and thin with long thin fingers and high arched palate ". Another described him as a " short, mesomorphic male ".) The son was 180 cm. tall, the father 170 cm. Ectopia lentis was detected in neither. I conclude this was not the Marfan syndrome but rather bicuspid aortic valve with Erdheim’s cystic medial necrosis. The
ASSOCIATION OF CONGENITAL BICUSPID AORTIC VALVE AND ERDHEIM’S CYSTIC MEDIAL NECROSIS
SiR,—The association of congenital bicuspid aortic valve with coarctation of the aorta is well recognised. Abbott 9 found bicuspid aortic valve in 47 of 200 casesof coarctation, and others 10have found an even higher frequency. The aortic valve is often bicuspid in cases of a rare anomaly-complete interruption of the aortic arch. Roberts et all found such in all 3 of their cases and in at least 14 of 52 previously reported cases. Because of the poor engineering design as compared with a tricuspid valve (as pointed out by Leonardo da Vinci over 500 years ago,12 although he focused mainly on quadri- cuspid valves), bicuspid aortic valves 13,14 are frequently complicated by fibrotic and calcific changes (and, as Osler 15 pointed out in 1886, bacterial endocarditis). Much of the calcific aortic stenosis of older people may originate in a bicuspid valve 16; however, only 2 of 21 showed bicuspid valve in the series of Roberts et al." Several of the reported cases of associated aortic-valve disease and Erdheim’s cystic medial necrosis have had congenitally bicuspid valve. An association between aortic-valve disease and cystic medial necrosis of the aorta was pointed out in 195718 and has been documented several times since then.19-21 Marshall, B. Br. med. J. 1970, i, 302. Abbott, M. E. Am. Heart J. 1928, 3, 392, 574. Reifenstein, G. H., Levine, S. A., Gross, R. E. ibid. 1947, 33, 146 Roberts, W. C., Morrow, A. G., Braunwald, E. Circulation, 1962, 26, 39. 12. O’Malley, C. D., Saunders, C. M. Leonardo da Vinci on the Human Body. New York, 1952. 13. Tranchesi, J., Carral, R., De Amorin, I., Perraloza, D. Am. Heart J. 1954, 47, 664. 14. Roberts, W. C. Am. J. Cardiol. 1970, 26, 72. 15. Osler, W. Trans. Ass. Am. Physns, 1886, 2, 185. 16. Bacon, A. P. C., Matthews, M. B. Q. Jl Med. 1959, 28, 545. 17. Roberts, W. C., Perloff, J. K., Constantino, T. Am. J. Cardiol. 1971, 27, 497. 18. McKusick, V. A., Logue, R. B., Bahnson, H. T. Circulation, 1956, 46, 188. 19. Heath, D., Edwards, G. E., Smith, L. A. Proc. Mayo Clin. 1958, 33, 8. 9. 10. 11.
228. 20. 21.
Read, R. C., Wolf, P. New Engl. J. Med. 1964, 271, 816. Ditchek, T., Bookstein, J. J. Circulation, 1965, 31, 127.
son was
at age 17 to
Incidentally, I know of no report of familial occurrence bicuspid aortic valve. Familial aggregation should occur now and then by chance alone, because the frequency of congenitally bicuspid valve is placed between 1% and 2%." of
22. Gore, I. Archs Path. 1953, 55, 1. 23. Layman, T. E., Wang, Y. Med. Clins N. Am. 1968, 52, 1145. 24. Huntington, R. W., Jr., Hirst, A. E., Jr. Am. J. clin. Path. 1967, 48,
44.