- Email: [email protected]
CORONARY ARTERY ECTASIA
894
and those from lower socioeconomic groups.20 If 33-50% of newborns at risk get conjunctivitis, and 10-20% get pneumonitis, this gives some indication of the size of the problem. 10,18,19 Chlamydia trachomatis has also been isolated from Bartholin’s glands and the urethra. It has been proposed as a cause of the acute urethral syndrome2’ and has been linked with the Fitz-Hugh Curtis
syndrome.22 Several studies have suggested an association between cervical chlamydial infection, anti-chlamydial antibodies, and cervical dysplasia.23-25 Nevertheless, since isolation of the organism is associated with promiscuity, which in itself seems to be a risk factor for cervical dysplasia, the inferences are uncertain. Emphasis on early diagnosis and treatment is of paramount importance to reduce the prevalence of chlamydial infection and its complications; without this rates of ectopic pregnancy and infertility are bound to increase. Tetracycline is effective in the treatment of cervical chlamydia, but optimum dosage and duration of treatment are uncertain. Erythromycin may be used in those who are pregnant or sensitive to tetracycline, and appears to be equally effective. Since the symptomless nature of chlamydial infections frustrates the aims of early diagnosis and treatment, screening of high risk groups is perhaps worthy of greater consideration; this should have a considerable impact on the pool of symptomless carriers. Screening of patients who request termination of pregnancy may be of value in view of the dual risk factors of instrumentation and high sexual activity. The question of screening pregnant women to reduce neonatal infection has also been raised, but the cost would be considerable. Nevertheless, where the prevalence maternal infection is sufficiently high screening may prove cost-effective.26 But screening cannot take place without an expansion in diagnostic facilities, which at present appear patchy. Inevitably costs will mount rapidly, but the results appear worthwhile.
CORONARY ARTERY ECTASIA ATHEROSCLEROTIC vessels are usually narrow, and at subjects who have had a myocardial infarct have narrower coronary arteries than controls. This narrowing is primarily the result of lipid deposition and fibrosis in the intima but the media often shows focal scarring, or even necropsy
concentric atrophy of elastic or muscular tissue. Like the aorta, the coronary arteries tend to dilate progressively during life, but whether this is a natural ageing process or a to cardiac response hypertrophy is debatable.2,3 modest a of coronary dilatation should degree Theoretically, be beneficial in countering the occlusive tendency of atherosclerosis. In some patients the coronaries are diffusely, but irregularly, dilated and the process, now generally known as coronary artery ectasia,4 is clearly pathological. In three separate angiographic series the prevalence of coronary ectasia has been just over 1%, and a study of nearly 700 consecutive necropsies revealed at least focal areas of coronary artery dilatation in 1’4% of cases.By far the commonest presenting symptom in coronary ectasia is angina, and well over half of these patients have evidence of a previous myocardial infarct. The short-term prognosis in this condition is poor and survival rates are no better than in patients with severe triple vessel narrowing.4Extensive thrombosis, seen at necropsy, is a well recognised complication of ectasia and is generally attributed to reduced flow rates.2 However, in patients undergoing cardiac surgery coronary sinus flow seems to be within the normal ranger Ectatic coronary arteries show all the features of advanced atherosclerosis. One report showed that dilatation was least pronounced in areas where there was little medial damage4 Unusual inflammatory vascular disorders, such as polyarteritis nodosa8 and giant cell arteritis,9can occasionally produce focal coronary arterial aneurysms, but there is no evidence that they are the cause of diffuse ectasia. The histological changes in atherosclerosis were described by Virchowl° over a century ago, but the cellular pathology of this disease is still being unravelled. In the post-mortem room atherosclerotic arteries often feel rigid and are then assumed to be densely fibrotic and calcified (they are seldom examined histologically). When atheromatous material, obtained during femoral endarterectomy, was examined by cell culture techniques and by electron microscopy, smooth muscle was found to be the predominant cellular element, even in the fibrous cap of lesions."Furthermore, the extent of necrosis and calcification in these severely diseased vessels was rather less than previously thought. Armstrong and his colleagues’22 have studied the progression of atherosclerosis in the iliac arteries of monkeys receiving atherogenic diets for up to six years. Intimal thickening developed and there was also focal medial thinning. Detailed studies of the cross-sectional areas of diseased vessels showed that the intimal mass increased 2. Wilson J, Adams CWM, Brander WL. The antiocclusive effect of coronary dilatation with age Atherosclerosis 1978; 29: 503-07. 3. Massmann J, Oestreich S. Angiometric studies of human coronary artery sclerosis Atherosclerosis 1974; 20: 287-94. 4 Markis JE, Joffe CD, Cohn PF, Feen DJ, Herman MV, Gorlin R. Clinical significance of coronary arterial ectasia. Am J Cardiol 1976; 37: 217-22. 5. Swanton RH, Lea Thomas M, Coltart DJ, Jenkins BS, Webb-Peploe MM, Williams BT Coronary artery ectasia-a variant of occlusive coronary arteriosclerosis Br
7.
21. Stamm
WE, Wagner KF, Amsel R,
et
al. Causes of the
acute
urethral
syndrome
in
women. N Engl J Med 1980; 303: 409-15. 22. Hanssen PW, Svensson L, Westrom L, Mardh PA Isolation of Chlamydia trachomatis from the liver capsule in Fitz-Hugh-Curtis Syndrome. N Engl J Med 1982; 306: 113 23 Paavonen J, Vesterinen E, Meyer B, et al. Genital Chlamydia trachomatis infections in patients with cervical atypia Obstet Gynecol 1979; 54: 289-91 24. Carr M, Hanna L, Jawetz E. Chlamydiae, cervicitis and abnormal Papanicolaou smears. Obstet Gynecol 1979; 53: 27-30 25 Hare MJ, Taylor Robinson D, Cooper P. Evidence for an association between Chlamydia trachomatis and cervical intra epithelial neoplasia Br J Obstet Gynaecol
1982; 89: 489-92 26.
Schachter J, Grossman M. Chlamydial infections. Ann Rev Med 1981; 32: 45-61. G, Prineas RJ, Mitchell JRA. Myocardial infarction and the intrinsic calibre coronary arteries. Br Heart J 1967, 29: 548-52
1. Rose
of
Heart J 1978; 40: 393-400. B, Aranda JM, Embi A, Mullin FL, El-Sherif N, Lassara R. Coronary artery aneurysms Am J Med 1977; 62: 597-607. Daoud AS, Pankin D, Tulgan H, Florentin RA. Aneurysms of the coronary artery. Report often cases and review of literature. Am J Cardiol 1963, 11: 228-37 Przybojewski JZ. Polyarteritis nodosa in the adult. Report of a case with repeated myocardial infarction and a review of cardiac involvement. S Afr Med J 1981; 60:
6. Befeler
8.
512-18. ischaemia and occlusion in giant cell (temporal) arteritis Acta Med Scand 1980; 208: 257-63 10. Virchow R. Cellular pathology, 2nd ed, translated by F. Chance. New York Dover 9.
Paulley JW. Coronary Publication
Inc, 1971. R, Wight TN, Strandness E, Thiele B.
Human Atherosclerosis 1 Cell and characteristics of advanced lesions of the superficial femoral artery Am J Pathol 1984; 114: 79-93. 12 Armstrong ML, Heistad DD, Marcus ML, Megan MB, Piegors DJ. Structural and 11 Ross
constitution
hemodynamic responses of peripheral arteries of macaque monkeys to atherogenic diet. Arteriosclerosis
1985, 5: 336-46.
895
substantially but, because the overall size of the arteries also increased, there was no net reduction in the size of the lumen.
monkeys fed in this way do get arterial but there is at least one report that, in ageing occlusionsl3 monkeys, coronary arterial dilatation14 very similar to coronary artery ectasia develops spontaneously. Important insights into vasomotor responses in arterial disease have come from experiments in atheromatous monkeys. 12,15 Vascular resistance in the limbs of these animals was nearly a quarter above normal and there was evidence of impaired vasodilatation. In contrast, vasoconstrictor responses to noradrenaline and serotonin were not inhibited-in fact they were augmented in hypercholesterolaemic animals before the development of severe atheroma. 15 Shimokawa et al16 showed angiographically that coronary arterial spasm could be induced in atheromatous but not in normal miniature swine by intravenous or intracoronary injection of histamine or serotonin. Similarly, in patients with extensive coronary arterial disease coronary vascular resistance was substantially increased by immersion of the hand in ice water." Arterial spasm can also be provoked in atherosclerotic patients by intracoronary injection of ergonovine,18 and this effect has now been reported in two patients with undoubted coronary arterial ectasia.19 These findings indicate that severely diseased atheromatous vessels are not the firm pipe-stems that they seem to be at necropsy. Despite extensive intimal disease they contain enough smooth muscle to cause spasm and associated cardiovascular In due
course
symptoms.
PERTHES’ DISEASE OSTEOCHONDRITIS of the femoral head was described 1910 by Legg in the USA, Calve in France, and Perthes himself in Germany. This apparent infarct of the proximal femoral epiphysis is more common in boys, and usually presents between the age of 5 and 10 years. It may be that the epiphysis is vulnerable to transient ischaemia in this age-group, although factors precipitating such ischaemia have not been identified. Short children and those with delayed skeletal maturation are more commonly affected, but there is no evidence of a genetic predisposition.’ Whilst the initial pain and stiffness of the hip found in most cases of Perthes’ disease settle with two or three weeks’ bedrest, the subsequent radiographic changes suggest that healing of the infarcted epiphysis occurs slowly over the next two or three years. The changes of sclerosis, fragmentation, and collapse of the femoral head are usually present when the child is first
independently in
13. Bond
MG, Bullock BC, Bellinger DA, colony of non human primates with
Hamm TE. Myocardial infarction in coronary artery atherosclerosis Am J
large Pathol
a
1980; 101: 675-92. 14 Uno H, Poff B. Coronary arterial ectasia, 15
16
17
a predominant type of coronary sclerosis in aged rhesus monkeys (Macaca mulatta). Am J Pathol 1983; 111: 315-22. Heistad DD, Armstrong ML, Marcus ML, Piegors DJ, Mark AL. Augmented responses to vasoconstrictor stimuli in hypercholesterolemic and atherosclerotic monkeys. Circ Res 1984; 54: 711-18. Shimokawa H, Tomoike H, Nabeyama S, et al. Coronary artery spasm induced in atherosclerotic miniature swine. Science 1983; 221: 560-62 Mudge GH, Grossman W, Mills RM, Lesch M, Braunwald E. Reflex increase in coronary vascular resistance in patients with ischaemic heart disease. N Engl J Med
1976, 295: 1333-37. 18 Schroeder JS, Bolen JL, Quint RA, et al. Provocation of coronary spasm with ergonovine maleate New test with results in 57 patients undergoing coronary arteriography. Am J Cardiol 1977; 40: 487-91 19 Bove AA, Vlietstra RE Spasm in ectatic coronary arteries. Mayo Clin Proc 1985; 60: 822-26 1 Wynne-Davies R, Gormley J. The aetiology of Perthes’ disease. J Bone Joint Surg(Br) 1978, 60: 6-14
and are slow to resolve. Healing is usually followed by residual deformity which may predispose to osteoarthritis of the hip. By radiographic criteria, Ratliff2 showed that only a third of patients treated by prolonged immobilisation on an abduction frame had a good result, yet despite a high incidence of osteoarthritis only one of sixteen patients had required hip surgery after forty years of follow-up. Perthes’ disease was formerly treated in a manner analogous to the methods used for tuberculosis of the hip: the child was immobilised on an abduction frame, often remaining in hospital for two or three years until the radiographic appearances suggested healing. Later experience suggested that weight-bearing on the affected hip was unimportant provided the femoral head was contained by the acetabulum. Good results were reported for plaster casts which kept the hip in abduction,3 and many different splints have also been used, most of them designed to allow weightbearing on the affected hip with the leg in a position of abduction. Use of a splint for eighteen months or more is difficult for both the child and his parents. Radiographic criteria of healing of the capital epiphysis have been described, but in practice it is difficult for the surgeon to decide when to discontinue the splint. Axer4 suggested that a varus-rotation osteotomy of the proximal femur would contain the femoral head and that the operation would lead to more rapid healing; this procedure has become popular but its long-term results are unknown and its indications are not entirely clear. CatterallS classified Perthes’ disease according to the degree of involvement of the capital epiphysis. If radiographs show that less than half of the capital epiphysis is affected the prognosis is good, and patients in Catterall’s group I require neither prolonged splintage nor femoral osteotomy. Controversy remains about the treatment of more advanced disease with increased involvement of the femoral head and sequestrum formation. In a review of children treated by osteotomy,6 it was suggested that the presence of radiographic risk factors was a better indication for surgery than the patient’s status according to Catterall’s groups II-IV. Risk factors include subluxation and extrusion of the femoral head, calcification lateral to the epiphysis, a metaphyseal reaction, and a horizontal growth-plate. Whether treated by splintage or osteotomy, only a small proportion of children are left with persistent hip pain and stiffness after treatment. A poor outcome is usually the result of inadequate containment with impingement ofa segment of the femoral head on the lip of the acetabulum, the condition known as hinge abduction.7 Quain and Catterall8 have now described treatment of this condition by abduction-extension osteotomy of the proximal femur. Twenty-three patients were largely relieved of pain and lost the fixed adduction and flexion deformity. A bonus was that shortening of the leg was often improved. seen
2. Rathff AHC. Perthes’ disease
a study of sixteen patients followed up for forty years. J Bone Joint Surg (Br) 1977; 59: 248 3 Petrie JG, Bitenc I The abduction weight-bearing treatment in Legg-Perthes’ disease. J Bone Joint Surg (Br) 1971; 53: 54-62. 4 Axer A. Subtrochanteric osteotomy in the treatment of Perthes’ disease A preliminary report. J Bone Joint Surg (Br) 1965; 47: 489-99. 5. Catterall A. The natural history of Perthes’ disease J Bone Joint Surg (Br) 1971; 53:
37-53. 6
Lloyd-Roberts GC, Catterall A, Salaman PB. A controlled study ofthe indications for and the results of femoral osteotomy in Perthes’ disease. J Bone Joint Surg(Br) 1976; 58: 31-36
7. Grossbard GD. Hip pain
during adolescence after Perthes’ disease. J Bone Joint Surg (Br) 1981, 63: 572-74. 8. Quain S, Catterall A Hinge abduction of the hip. J Bone Joint Surg (Br) 1986; 68: 61-64
and those from lower socioeconomic groups.20 If 33-50% of newborns at risk get conjunctivitis, and 10-20% get pneumonitis, this gives some indication of the size of the problem. 10,18,19 Chlamydia trachomatis has also been isolated from Bartholin’s glands and the urethra. It has been proposed as a cause of the acute urethral syndrome2’ and has been linked with the Fitz-Hugh Curtis
syndrome.22 Several studies have suggested an association between cervical chlamydial infection, anti-chlamydial antibodies, and cervical dysplasia.23-25 Nevertheless, since isolation of the organism is associated with promiscuity, which in itself seems to be a risk factor for cervical dysplasia, the inferences are uncertain. Emphasis on early diagnosis and treatment is of paramount importance to reduce the prevalence of chlamydial infection and its complications; without this rates of ectopic pregnancy and infertility are bound to increase. Tetracycline is effective in the treatment of cervical chlamydia, but optimum dosage and duration of treatment are uncertain. Erythromycin may be used in those who are pregnant or sensitive to tetracycline, and appears to be equally effective. Since the symptomless nature of chlamydial infections frustrates the aims of early diagnosis and treatment, screening of high risk groups is perhaps worthy of greater consideration; this should have a considerable impact on the pool of symptomless carriers. Screening of patients who request termination of pregnancy may be of value in view of the dual risk factors of instrumentation and high sexual activity. The question of screening pregnant women to reduce neonatal infection has also been raised, but the cost would be considerable. Nevertheless, where the prevalence maternal infection is sufficiently high screening may prove cost-effective.26 But screening cannot take place without an expansion in diagnostic facilities, which at present appear patchy. Inevitably costs will mount rapidly, but the results appear worthwhile.
CORONARY ARTERY ECTASIA ATHEROSCLEROTIC vessels are usually narrow, and at subjects who have had a myocardial infarct have narrower coronary arteries than controls. This narrowing is primarily the result of lipid deposition and fibrosis in the intima but the media often shows focal scarring, or even necropsy
concentric atrophy of elastic or muscular tissue. Like the aorta, the coronary arteries tend to dilate progressively during life, but whether this is a natural ageing process or a to cardiac response hypertrophy is debatable.2,3 modest a of coronary dilatation should degree Theoretically, be beneficial in countering the occlusive tendency of atherosclerosis. In some patients the coronaries are diffusely, but irregularly, dilated and the process, now generally known as coronary artery ectasia,4 is clearly pathological. In three separate angiographic series the prevalence of coronary ectasia has been just over 1%, and a study of nearly 700 consecutive necropsies revealed at least focal areas of coronary artery dilatation in 1’4% of cases.By far the commonest presenting symptom in coronary ectasia is angina, and well over half of these patients have evidence of a previous myocardial infarct. The short-term prognosis in this condition is poor and survival rates are no better than in patients with severe triple vessel narrowing.4Extensive thrombosis, seen at necropsy, is a well recognised complication of ectasia and is generally attributed to reduced flow rates.2 However, in patients undergoing cardiac surgery coronary sinus flow seems to be within the normal ranger Ectatic coronary arteries show all the features of advanced atherosclerosis. One report showed that dilatation was least pronounced in areas where there was little medial damage4 Unusual inflammatory vascular disorders, such as polyarteritis nodosa8 and giant cell arteritis,9can occasionally produce focal coronary arterial aneurysms, but there is no evidence that they are the cause of diffuse ectasia. The histological changes in atherosclerosis were described by Virchowl° over a century ago, but the cellular pathology of this disease is still being unravelled. In the post-mortem room atherosclerotic arteries often feel rigid and are then assumed to be densely fibrotic and calcified (they are seldom examined histologically). When atheromatous material, obtained during femoral endarterectomy, was examined by cell culture techniques and by electron microscopy, smooth muscle was found to be the predominant cellular element, even in the fibrous cap of lesions."Furthermore, the extent of necrosis and calcification in these severely diseased vessels was rather less than previously thought. Armstrong and his colleagues’22 have studied the progression of atherosclerosis in the iliac arteries of monkeys receiving atherogenic diets for up to six years. Intimal thickening developed and there was also focal medial thinning. Detailed studies of the cross-sectional areas of diseased vessels showed that the intimal mass increased 2. Wilson J, Adams CWM, Brander WL. The antiocclusive effect of coronary dilatation with age Atherosclerosis 1978; 29: 503-07. 3. Massmann J, Oestreich S. Angiometric studies of human coronary artery sclerosis Atherosclerosis 1974; 20: 287-94. 4 Markis JE, Joffe CD, Cohn PF, Feen DJ, Herman MV, Gorlin R. Clinical significance of coronary arterial ectasia. Am J Cardiol 1976; 37: 217-22. 5. Swanton RH, Lea Thomas M, Coltart DJ, Jenkins BS, Webb-Peploe MM, Williams BT Coronary artery ectasia-a variant of occlusive coronary arteriosclerosis Br
7.
21. Stamm
WE, Wagner KF, Amsel R,
et
al. Causes of the
acute
urethral
syndrome
in
women. N Engl J Med 1980; 303: 409-15. 22. Hanssen PW, Svensson L, Westrom L, Mardh PA Isolation of Chlamydia trachomatis from the liver capsule in Fitz-Hugh-Curtis Syndrome. N Engl J Med 1982; 306: 113 23 Paavonen J, Vesterinen E, Meyer B, et al. Genital Chlamydia trachomatis infections in patients with cervical atypia Obstet Gynecol 1979; 54: 289-91 24. Carr M, Hanna L, Jawetz E. Chlamydiae, cervicitis and abnormal Papanicolaou smears. Obstet Gynecol 1979; 53: 27-30 25 Hare MJ, Taylor Robinson D, Cooper P. Evidence for an association between Chlamydia trachomatis and cervical intra epithelial neoplasia Br J Obstet Gynaecol
1982; 89: 489-92 26.
Schachter J, Grossman M. Chlamydial infections. Ann Rev Med 1981; 32: 45-61. G, Prineas RJ, Mitchell JRA. Myocardial infarction and the intrinsic calibre coronary arteries. Br Heart J 1967, 29: 548-52
1. Rose
of
Heart J 1978; 40: 393-400. B, Aranda JM, Embi A, Mullin FL, El-Sherif N, Lassara R. Coronary artery aneurysms Am J Med 1977; 62: 597-607. Daoud AS, Pankin D, Tulgan H, Florentin RA. Aneurysms of the coronary artery. Report often cases and review of literature. Am J Cardiol 1963, 11: 228-37 Przybojewski JZ. Polyarteritis nodosa in the adult. Report of a case with repeated myocardial infarction and a review of cardiac involvement. S Afr Med J 1981; 60:
6. Befeler
8.
512-18. ischaemia and occlusion in giant cell (temporal) arteritis Acta Med Scand 1980; 208: 257-63 10. Virchow R. Cellular pathology, 2nd ed, translated by F. Chance. New York Dover 9.
Paulley JW. Coronary Publication
Inc, 1971. R, Wight TN, Strandness E, Thiele B.
Human Atherosclerosis 1 Cell and characteristics of advanced lesions of the superficial femoral artery Am J Pathol 1984; 114: 79-93. 12 Armstrong ML, Heistad DD, Marcus ML, Megan MB, Piegors DJ. Structural and 11 Ross
constitution
hemodynamic responses of peripheral arteries of macaque monkeys to atherogenic diet. Arteriosclerosis
1985, 5: 336-46.
895
substantially but, because the overall size of the arteries also increased, there was no net reduction in the size of the lumen.
monkeys fed in this way do get arterial but there is at least one report that, in ageing occlusionsl3 monkeys, coronary arterial dilatation14 very similar to coronary artery ectasia develops spontaneously. Important insights into vasomotor responses in arterial disease have come from experiments in atheromatous monkeys. 12,15 Vascular resistance in the limbs of these animals was nearly a quarter above normal and there was evidence of impaired vasodilatation. In contrast, vasoconstrictor responses to noradrenaline and serotonin were not inhibited-in fact they were augmented in hypercholesterolaemic animals before the development of severe atheroma. 15 Shimokawa et al16 showed angiographically that coronary arterial spasm could be induced in atheromatous but not in normal miniature swine by intravenous or intracoronary injection of histamine or serotonin. Similarly, in patients with extensive coronary arterial disease coronary vascular resistance was substantially increased by immersion of the hand in ice water." Arterial spasm can also be provoked in atherosclerotic patients by intracoronary injection of ergonovine,18 and this effect has now been reported in two patients with undoubted coronary arterial ectasia.19 These findings indicate that severely diseased atheromatous vessels are not the firm pipe-stems that they seem to be at necropsy. Despite extensive intimal disease they contain enough smooth muscle to cause spasm and associated cardiovascular In due
course
symptoms.
PERTHES’ DISEASE OSTEOCHONDRITIS of the femoral head was described 1910 by Legg in the USA, Calve in France, and Perthes himself in Germany. This apparent infarct of the proximal femoral epiphysis is more common in boys, and usually presents between the age of 5 and 10 years. It may be that the epiphysis is vulnerable to transient ischaemia in this age-group, although factors precipitating such ischaemia have not been identified. Short children and those with delayed skeletal maturation are more commonly affected, but there is no evidence of a genetic predisposition.’ Whilst the initial pain and stiffness of the hip found in most cases of Perthes’ disease settle with two or three weeks’ bedrest, the subsequent radiographic changes suggest that healing of the infarcted epiphysis occurs slowly over the next two or three years. The changes of sclerosis, fragmentation, and collapse of the femoral head are usually present when the child is first
independently in
13. Bond
MG, Bullock BC, Bellinger DA, colony of non human primates with
Hamm TE. Myocardial infarction in coronary artery atherosclerosis Am J
large Pathol
a
1980; 101: 675-92. 14 Uno H, Poff B. Coronary arterial ectasia, 15
16
17
a predominant type of coronary sclerosis in aged rhesus monkeys (Macaca mulatta). Am J Pathol 1983; 111: 315-22. Heistad DD, Armstrong ML, Marcus ML, Piegors DJ, Mark AL. Augmented responses to vasoconstrictor stimuli in hypercholesterolemic and atherosclerotic monkeys. Circ Res 1984; 54: 711-18. Shimokawa H, Tomoike H, Nabeyama S, et al. Coronary artery spasm induced in atherosclerotic miniature swine. Science 1983; 221: 560-62 Mudge GH, Grossman W, Mills RM, Lesch M, Braunwald E. Reflex increase in coronary vascular resistance in patients with ischaemic heart disease. N Engl J Med
1976, 295: 1333-37. 18 Schroeder JS, Bolen JL, Quint RA, et al. Provocation of coronary spasm with ergonovine maleate New test with results in 57 patients undergoing coronary arteriography. Am J Cardiol 1977; 40: 487-91 19 Bove AA, Vlietstra RE Spasm in ectatic coronary arteries. Mayo Clin Proc 1985; 60: 822-26 1 Wynne-Davies R, Gormley J. The aetiology of Perthes’ disease. J Bone Joint Surg(Br) 1978, 60: 6-14
and are slow to resolve. Healing is usually followed by residual deformity which may predispose to osteoarthritis of the hip. By radiographic criteria, Ratliff2 showed that only a third of patients treated by prolonged immobilisation on an abduction frame had a good result, yet despite a high incidence of osteoarthritis only one of sixteen patients had required hip surgery after forty years of follow-up. Perthes’ disease was formerly treated in a manner analogous to the methods used for tuberculosis of the hip: the child was immobilised on an abduction frame, often remaining in hospital for two or three years until the radiographic appearances suggested healing. Later experience suggested that weight-bearing on the affected hip was unimportant provided the femoral head was contained by the acetabulum. Good results were reported for plaster casts which kept the hip in abduction,3 and many different splints have also been used, most of them designed to allow weightbearing on the affected hip with the leg in a position of abduction. Use of a splint for eighteen months or more is difficult for both the child and his parents. Radiographic criteria of healing of the capital epiphysis have been described, but in practice it is difficult for the surgeon to decide when to discontinue the splint. Axer4 suggested that a varus-rotation osteotomy of the proximal femur would contain the femoral head and that the operation would lead to more rapid healing; this procedure has become popular but its long-term results are unknown and its indications are not entirely clear. CatterallS classified Perthes’ disease according to the degree of involvement of the capital epiphysis. If radiographs show that less than half of the capital epiphysis is affected the prognosis is good, and patients in Catterall’s group I require neither prolonged splintage nor femoral osteotomy. Controversy remains about the treatment of more advanced disease with increased involvement of the femoral head and sequestrum formation. In a review of children treated by osteotomy,6 it was suggested that the presence of radiographic risk factors was a better indication for surgery than the patient’s status according to Catterall’s groups II-IV. Risk factors include subluxation and extrusion of the femoral head, calcification lateral to the epiphysis, a metaphyseal reaction, and a horizontal growth-plate. Whether treated by splintage or osteotomy, only a small proportion of children are left with persistent hip pain and stiffness after treatment. A poor outcome is usually the result of inadequate containment with impingement ofa segment of the femoral head on the lip of the acetabulum, the condition known as hinge abduction.7 Quain and Catterall8 have now described treatment of this condition by abduction-extension osteotomy of the proximal femur. Twenty-three patients were largely relieved of pain and lost the fixed adduction and flexion deformity. A bonus was that shortening of the leg was often improved. seen
2. Rathff AHC. Perthes’ disease
a study of sixteen patients followed up for forty years. J Bone Joint Surg (Br) 1977; 59: 248 3 Petrie JG, Bitenc I The abduction weight-bearing treatment in Legg-Perthes’ disease. J Bone Joint Surg (Br) 1971; 53: 54-62. 4 Axer A. Subtrochanteric osteotomy in the treatment of Perthes’ disease A preliminary report. J Bone Joint Surg (Br) 1965; 47: 489-99. 5. Catterall A. The natural history of Perthes’ disease J Bone Joint Surg (Br) 1971; 53:
37-53. 6
Lloyd-Roberts GC, Catterall A, Salaman PB. A controlled study ofthe indications for and the results of femoral osteotomy in Perthes’ disease. J Bone Joint Surg(Br) 1976; 58: 31-36
7. Grossbard GD. Hip pain
during adolescence after Perthes’ disease. J Bone Joint Surg (Br) 1981, 63: 572-74. 8. Quain S, Catterall A Hinge abduction of the hip. J Bone Joint Surg (Br) 1986; 68: 61-64