The first operation for congenital pyloric stenosis in Malta was carried out in 1949. Up to that time it was thought that this disease did not exist locally. However, an increasing number of cases have been diagnosed since then: presumably this is due to more accurate diagnosis and to the fact that doctors have become more aware of its existence. The purpose of this paper is to review the incidence of congenital pyloric stenosis in Malta as well as to compare this incidence to that of other countries.peer-reviewe

Cachia, E. A.

Fenech, F. F.

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J. med. Genet. (1966). 3, 49. Incidence of Congenital Pyloric Stenosis in Malta E. A. CACHIA and F. F. FENECH From St Luke's Hospital, Malta The first operation for congenital pyloric stenosis in Malta was carried out in 1949. Up to that time it was thought that this disease did not exist locally. However, an increasing number of cases have been diagnosed since then: presumably this is due to more accurate diagnosis and to the fact that doctors have become more aware of its exis-tence. The purpose of this paper is to review the incidence of congenital pyloric stenosis in Malta as well as to compare this incidence to that of other countries. TABLE I INCIDENCE OF CONGENITAL PYLORIC STENOSIS BETWEEN 1952-1964 Year No. of Live Births No. of Cases % Annual Incidence 1952 8,501 8 0·049 1953 8,302 4 0·048 1954 8,287 6 0·072 1955 7,899 3 0·038 1956 7,794 5 0·064 1957 8,117 II 0·135 1958 7,872 5 0·063 1959 7,825 6 0·076 1960 7,847 7 0·089 1961 7,674 3 0·039 1962 7,506 8 o·ro6 1963 6,672 II 0·164 1964 6,359 12 o·r88 Totals 100,655 89 Clinical Material Between 1952 and 1964, 89 cases of congenital pyloric stenosis were seen at St Luke's Hospital. Ramstedt's operation was carried out on all the cases. Table I shows the number of cases per year in relation to the number of live births. Of these patients, 50 were under the care of one of us (E.A.C.), and Table II shows the birth order of these 50 patients. The inci-dence in the first-born and second-born child was practically equal, both groups making up 64 % of the series. Cases in later-born children did occur and one of the children affected was the twelfth child. Of the 89 patients, 65 were male and 24 were female. There were 2 cases in a family with 3 children, the first two Received May 17, 1965. - MG4 49 born were affected and both patients were male. The youngest child operated on was IO days old and the oldest was 4 months. TABLE II BIRTH ORDER OF 50 PATIENTS Birth Order I St 2nd 3rd 4th 5th 6th 7th 8th 9th 10th II th 12th No. of Patients 17 IS 7 2 2 2 In Malta, there are no records of the proportion of first-born in the country as a whole, so the births that took place between 1960 and 1964 in the maternity unit of this hospital were accordingly analysed. We believe that this is fairly representative of the whole island as the admissions to our hospital are uns'elected. Stillbirths were excluded. Table III shows the number of first-born and later-born occurring in the last five years of the period under review. The percentage of first-born is 25 ·55 %. TABLE III ADMISSIONS TO THE MATERNITY SECTION OF FIRST-AND LATER-BORN Year No. of Live Births First-born Later-born 1960 2 ,214 589 1,625 1961 2,242 446 1,796 1962 2,112 508 r ,604 1963 2,043 506 1,537 1964 1,988 660 1,328 Discussion The incidence of pyloric stenosis is affected by such factors as sex, birth order, heredity, and race. The increasing tendency for the disease to affect I 50 Cachia and Fenech the first-born children and males has been amply confirmed by this study. McKeown and MacMahon (1955) found that the offspring of parents, one of whom had recovered from pyloric stenosis, were more liable to develop pyloric stenosis when the mother was the affected parent. This has further been confirmed by Carter (1961). Carter and Savage (1951) in their study on 4 first-cousin twins found that identical twins seemed to be more affected than fraternal twins of like sex, and that the incidence was higher than in the general population. Moreover, children of men and women who recovered from pyloric stenosis had an increased liability of developing the disease (Carter and Powell, 1954; McKeown and MacMahon, 1955). Carter (1961) states, 'relatives of female index patients have considerably higher risk of being affected. For any index patient the risk of being affected is rather higher in female than in male relatives'. As more survivors of infantile pyloric stenosis begin to have their own families, the number of particularly susceptible newborn seems likely to increase (Lancet, 1956), though Wallgren (1960) has reported that the incidence in Gothenburg has actually gone down from 0"4 to 0·199%. Racial factors also play an important role. In the United States, the incidence in the Negro is significantly lower than in the white population (Laron and Horne, 1957). In Europe most of the evidence suggests that the disease is more common in the Northern European countries such as Sweden and the United Kingdom. In Italy, it is reported to be rare (Cocchi, 1950). Wallgren (1941) .quoted Eckhaus from Turkey who demon-strated the rarity of pyloric stenosis in that country. Laron (1955) reported the lower incidence in Israel as compared to that in Sweden. Infantile pyloric stenosis is unknown in Malaya (Field, 1951) and it has not been reported in the Australian Aborigine (Watson, 1956). In Malta, the incidence over a 13-year period is 0·088% ± o·II4. This incidence is higher than that of Israel and pre-sumably higher than that of Italy and Turkey. Of the 89 patients, 7 had typical English surnames. At the start of its association with Britain in 1800, Malta's population was 90,000; it has now increased to just over 300,000. In these 160 years, marriages between the Maltese and British servicemen were not infrequent. We believe that this genetic mix-up of the Maltese population could well account for the higher incidence of congenital pyloric stenosis in Malta as compared to that of neighbouring Mediterranean countries. TABLE IV INCIDENCE OF PYLORIC STENOSIS IN DIFFERENT COUNTRIES Country Year Author % Incidence England 1946 Davison 0·28 Israel 1955 Laron 0·5 Italy 1950 Cocchi Very rare Malaya 1951 Field Unknown Malta 1965 Present study 0 ·088 ± 0 ·045 Scotland 1956 McLean 0·3 Sweden 1941 Wallgren 0'4 Sweden 1960 Wallgren 0·199 Turkey 1941 Eckhaus Very rare U.S.A. White 1957 Laron and Horne 0·12 ± 0 ·04 Negro 1957 Laron and Horne 0 ·046 ± 0 ·044 Summary This paper reviews the incidence of C©ngenital pyloric stenosis in Malta. There were 89 patients who presented during the 13-year period, giving an annual incidence of 0·088%. This is below that found in the United Kingdom and Sweden and higher than that of some Mediterranean countries. REFERENCES Carter, C . 0. (1961). The inheritance of congenital pyloric stenosis. Brit. nzed. Bull., 17, 251. --, and Powell, B. W. (1954) . Two-generation pyloric stenosis. Lancet, I, 746. --, and Savage, T. R. (1951). Pyloric stenosis in four first cousins . Arch. Dis. Childh., 26, 50 . Cocchi, C. (1950). Manuale di Pediatria, 2nd ed., ed. G. Frontali, p. 95r. Minerva Medica, Torino. Davison, G . (1946). The incidence of congenital pyloric stenosis. Arch. Dis. Childh., 21, rr3. Eckhaus, cited by Wallgren (1941). Field, C. E . (1951). Congenital hypertrophic pyloric stenosis. Lancet, z, 452. Lancet (1956). Infantile pyloric stenosis. I, 732. Laron, Z. (1955). Incidence of hypertrophic pyloric stenosis in various Jewish communities in Israel. Acta paediat. ( Uppsala), 44, 40. --, and Horne, L. M. (1957). The incidence of, infanti le pyloric stenosis. Amer. J. Dis. Child., 94, 15I. McKeown, T., and MacMahon, B. (1955). Infantile hypertrophic pyloric stenosis in parent and child. Arch. Dis. Child., 30, 497. McLean, M. M. (1956). The incidence of infantile pyloric stenosis in the north-east of Scotland. ibid., 31, 48r. Wallgren, A . (1941). Incidence of hypertrophic pyloric stenosis . Amer.J. Dis. Child., 62, 75r. - - (1960) Is the rate of hypertrophic pyloric stenosis declining? Acta paediat. (Uppsala), 49, 530. Watson, J . (1956). Infantile pyloric stenosis. Lancet, z, 517. COPYRIGHT © 1966 JOURNAL OF MEDICAL GENETICS ALL RIGHTS OF REPRODUCTION OF THIS REPRINT ARE RESERVED IN ALL COUNTRIES OF THE 'WORLD II 

Incidence of congenital pyloric stenosis in Malta

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Incidence of congenital pyloric stenosis in Malta

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