IDIOPATHIC COXA MAGNA

1350 Discussion gene for F.c.p.

Assuming a single only about 1 in 20 of are heterozygotes: so, if the gene were population the in pathogenesis of chronic bronchitis and important the

other chest diseases a clear difference should have been found in their incidence between the parents of children with F.c.p. (all of whom were heterozygotes) and the parents of the controls (only 1 in 20 of whom were heterozygotes). In fact, no significant difference was found. The incidence of chronic bronchitis in both groups is similar to that reported for the country as a whole-i.e., 17% in men aged 40-60, and 8% in women (College of General Practitioners 1961). It would perhaps be higher in our groups if corrections were made for age, but this might well be because all the parents lived in

to thank the staff of Great Ormond Street, particuDr. A. P. Norman, for access to their patients; the board of governors of St. George’s Hospital for a small grant towards expenses; and Dr. L. G. Blair for arranging for patients to be X-rayed.

We should like

larly

REFERENCES

Anderson, C. M., Freeman, M. (1960) Arch. Dis. Childh. 35, 581. Allan, J., Hubbard, L. (1962) Med. J. Aust. 1, 965. Bernard, E., Israel, L., Debris, M. (1960) Pr. méd. 68, 1691. Doll, R., Buch, J. (1950) Ann. Eugen., Lond. 15, 135. Fletcher, C. M., Elmes, P. C., Fairbairn, A. S., Wood, C. H. (1959) Brit. med. J. ii, 257. Karlish, A. J., Tärnoky, A. L. (1960) Lancet, ii, 514. Koch, E. (1959) Dtsch. med. Wschr. ii, 1773. McKendrick, T. (1962) Lancet, i, 183. Muir, D., Batten, J., Simon, G. (1962) ibid. i, 181. College of General Practitioners (1961) Brit. med. J. ii, 973. Peterson, E. M. (1959) J. Amer. med. Ass. 171, 1. Simon, G. (1956) Principles of Chest X-ray Diagnosis. London. Wood, J. A., Fishman, A. P., Reemtsma, K., Barker, H. G., Di Sant’Agnese, P. A. (1959) New Engl. J. Med. 260, 951. — —

towns.

IDIOPATHIC COXA MAGNA and complicated bronchitis were evenly distributed in all groups-so there was no evidence for an J. R. PEARSON M.B. Birm., F.R.C.S. association between heterozygosity for F.c.p. and the OFFICER, THE ROBERT JONES AND AGNES HUNT development of incapacitating chest infection or emphy- RESIDENT SURGICAL ORTHOPÆDIC HOSPITAL, OSWESTRY, SHROPSHIRE sema in patients with chronic bronchitis. The similar IDIOPATHIC coxa magna is a condition which is shown by P.E.F. readings in the two groups confirmed this. No other chronic chest disease-such as asthma or X-ray to consist in progressive enlargement of the with thickening primary emphysema-was manifested in the F.c.p. upper femoral epiphysis, associated of the femoral neck heterozygotes by analysis of symptoms, P.E.F., or the (fig. 1). chest radiographs. Coxa magna was first It is interesting to consider what contribution heterozydescribed by Ferguson gotes for F.c.p. would make to the prevalence of chronic and Howarth in 1934 bronchitis, if the apparent increased incidence among when they were studyfathers of F.c.p. children is taken at its face value. This ing Perthes’ disease, increase is a little less than 4 in 45 (about 8%). The and the following year proportion of men in the general population who are they discussed 13 heterozygotes is about 5%. Therefore, men who have patients with this conbronchitis because of the F.c.p. gene would be 4 per 1000. dition. McMurray If the general prevalence of chronic bronchitis in middledescribed 6 patients in aged men is 16% (as in control fathers), this would suggest 1947, and 2 other cases that only 1 in 40 cases (about 21/2%) of chronic bronchitis were reported by in men is caused by the heterozygous state of the F.c.p. Adams in 1956.

Simple

gene. Patients Studied

Our results agree with those of Anderson et al. (1962), who found no difference in the incidence of chronic respiratory disease in 144 parents of children with F.c.p. and 144 matched controls. On the other hand, they conflict with the conclusions of Wood et al. (1959), Peterson (1959), Koch (1959), Bernard et al. (1960), and Karlish and Tarnoky (1960). No increase in the incidence of duodenal ulcer was found in the parents of children with F.c.p., and this again agrees with the findings of Anderson et al. (1962). The incidence of duodenal ulcer in both groups is close to that found at comparable ages by Doll and Buch (1950) in a North London population.

patients with idiopathic coxa magna 15

have been seen at the Robert Jones and Agnes Hunt Orthopsedic Hospital in the

Summary

Analysis of respiratory symptoms, peak expiratory-flow rate, and chest X-ray in 95 parents of children with established fibrocystic disease of the pancreas (mucoviscidosis), and 94 parents of children with coeliac disease, hiatus hernia, or tracheo-oesophageal fistula, revealed no significant difference in the incidence of chronic bronchitis or other chronic chest disease. No evidence of an increase in incidence of peptic ulcer was found. It was concluded that the heterozygous state of fibrocystic disease of the pancreas could not be implicated as an important cause of chronic chest disease or of peptic ulceration.

Fig. I-Established

coxa

magna

showing enlargement

of the

femoral epiphysis and thickening of the femoral neck.

upper

1351 years 1945-62. These cases were discovered by reviewing all cases classified under the heading of " Perthes’ disease " tuberculosis of the hip-joint ", " irritable hip ". " 7 of the cases were diagnosed as transient synovitis of " the hip-joint ", 8 as tuberculosis of the hip, not proven ", and 2 as " ? Perthes’ disease ". Idiopathic coxa magna is evidently not readily recognised as an entity, and has in the past been confused with the synovial form of tuberculosis of the hip. The incidence of tuberculosis of the hip-joint during the period 1945-62 has fallen from 22 to 2, INCIDENCE OF TUBERCULOSIS OF THE HIP-JOINT AND IDIOPATHIC COXA MAGNA

while the incidence of idiopathic constant

coxa

magna has remained

(see table).

The youngest patient in the series was three years old, and the oldest thirteen. More than two-thirds were under eight. There were 8 boys and 7 girls.

Symptoms and Signs the presenting feature in 10 patients-in the region of the hip-joint in 7, the anterior region of the thigh in 2, and the knee in 1. The other 5 had a limp as the main symptom, and 2 of these had had a fall immediately followed by pain in the region of the Pain

was

hip-joint. 3 patients had a pyrexial illness before the onset of symptoms. In 14 there was restriction of movement of the hip-joint, varying from almost complete loss of movement to terminal restriction in all directions. In 1 patient who had had a limp for three months, the hip-joint showed a full range of movement, but there was radiographic evidence of a coxa magna. The erythrocyte-sedimentation rate was raised initially in 4 patients (12-21 mm. per hour) and fell to within normal limits within a month. In the remaining 11 patients it was normal. Biopsy of the synovial membrane of the hip-joint of 5 patients, and of the superficial inguinal lymph-nodes of 1; revealed no evidence of tuberculosis.

Radiological Appearances

patients the hip was radiol"gically normal on first examination, in 4 the head and neck of the femur were less dense when first seen, and 1 patient had a coxa magna. In the 10 patients the head and neck of the femur and the In 10

acetabulum became less dense after immobilisation for less than 1 month (fig. 2). There was no bone destruction or loss of joint space. Density of the femoral head returned to normal within six to eighteen months and was followed by a gradual enlargement of the femoral epiphysis. Treatment and Results

In the earlier years

patients

were

treated

by fixation

on

frame, and in the later years by immobilisation on Pugh’s traction. 1 patient in whom coxa magna was discovered a

initially was not immobilised. Immobilisation was more prolonged in the earlier years: thus 9 patients were immobilised on a frame for six to twenty-four months. The 5 later patients were immobilised on Pugh’s traction for a maximum of two months. 6 patients were given a full course of antituberculous drugs. The patients treated on Pugh’s traction regained a full range of movement of the hip-joint within two months. Among those treated by immobilisation on a frame, only 1 (immobilised for six months) regained a full range of movement: all the others had a persistent limitation-the last few degrees of either flexion or abduction. 2 patients of this group had been treated for two months in a plasterof-paris hip spica, and 1 in a partial weight-relieving calliper after leaving the frame. The follow-up period varies from one to fifteen years. All the patients treated by frame-fixation developed stiffness of the hip. 2 patients treated by Pugh’s traction developed terminal limitation of movement of the hipjoint three years after the onset of the condition. There was no radiographic evidence of osteoarthritis of the hip-joint in any of these patients. Discussion and Howarth Ferguson (1934) and McMurray (1947) have suggested that infection and trauma are predisposing causes of idiopathic coxa magna. In the present series there is little evidence to support these contentions. In the years 1945-53, idiopathic coxa magna in its early stages had been mistaken for the synovial form of tuberculosis of the hip, although this diagnosis should not have been made in the absence of positive histological and bacteriological proof. A coxa magna can occur at a late stage of synovial tuberculosis of the hip-joint. Ferguson and Howarth stressed the importance of simple immobilisation of the hip-joint in bed, and they thought that it was treatment in a plaster-of-paris hip spica that made the hip stiff. In the present series the more prolonged and rigid the fixation of the hip, the more likely seemed the development of stiffness. Early mobilisation of the affected hip-joint as soon as the irritable phase has subsided appears to be the ideal form of treatment.

Summary 15 patients with idiopathic enlargement of the upper femoral epiphysis are described. Early mobilisation of the affected hip-joint appears to be the best form of treatment. REFERENCES

Fig. 2-Decreased density in

sign

of

coxa

magna.

the

area

of the

hip

which is

an

early

Adams, J. P. (1956) S. med. J. 49, 604. Ferguson, A. B., Howarth, M. B. (1934) J. Bone Jt Surg. 16, 781. (1935) J. Amer. med. Ass. 104, 808. McMurray, B. (1947) Brit. J. Radiol. 20, 477. -

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