6rubh ~ourno/ qf~lastic Surgerv ( I990), 43,457462 C 1990 The Trustees of British Association of Plastic Surgeons 0007-1?26:9WOO43 0457;%10.00 Facial clefting in Kuwait and England: a comparative study S. SRlVASTAVAand R. L. BANG West Midlands Regional Plastic and Jaw Surgery Unit, Wordsley Hospital, Stourbridge, West Midlands, and Department of Surgery, University of Kuwait Summary-A retrospective study of all new cases of facial clefts seen between January 1985 and December 1987 was carried out at the Ibn Sina Hospital, Kuwait, and West Midlands Regional Plastic and Jaw Surgery Unit at Wordsley Hospital, Stourbridge, England; 234 arab children in Kuwait and 181 white Caucasian children in the West Midlandswerestudied. Despite vast differences in racial characteristics, environment and customs the pattern of clefting was very similar in the two groups. The incidence of clefts was calculated for the two regions and this demonstrated an increase in the incidence of cleft lip and palate (CL/P and CP) in the West Midlands since the last study was reported from this region in 1953. There have been several studies worldwide on the epidemiology of cleft lip and palate but it seems that no such study has been carried out among the arab races in modern times. Millard (1976) men- tions that among all the mummies excavated in Egypt only one showed evidence of cleft palate. The present study was undertaken to ascertain the incidence and pattern of clefting in the mixed arab population of Kuwait and compare it with the pattern of clefting found in the white Caucasian population of the West Midlands, England. The population of Kuwait (1.69 million in 1985) is composed of native Kuwaitis and expatriate workers of various nationalities, the majority of whom are arabs. As the Ibn Sina Hospital carries out nearly all the primary cleft repairs in the region, its records reflect the extent and pattern of cleft problem among the arab population there. The birth rate in Kuwait (33.7 per thousand) is among the highest in the world. The medical facilities are good and, due to the small distances involved, most patients are seen fairly early for treatment. In the West Midlands (population 5.18 million in 1986). the Regional Plastic and Jaw Surgery Unit deals with about 70% of the cleft lip and palate patients in the area and here again the records of the unit closely reflect the pattern of facial clefting among the white Caucasian population in the region. In both Kuwait and West Midlands, repair of the cleft lip is carried out at 3 months of age while the palate is repaired between 12 and 18 months of age. Materials and methods To ascertain the pattern of clefting, data from 3 years (1985 to 1987) were used, to reduce the possibility of random variations and provide a more representative sample. Calculation of the incidence of clefting was carried out on the basis of the total cleft cases and the live birth figures obtained for the year 1986. In Kuwait the total number of live birth figures were obtained from the Ministry of Public Health and these, together with the total number of new clefts repaired in 1986, were used to calculate an approximate incidence figure for that year. As clefts are not reported at birth in Kuwait, an assumption was made that the number of clefts repaired in a particular year must be approximately equal to the number of new cleft cases born that year as the population, birth rate and the number of new cleft cases presenting for treatment have not varied significantly during the past few years. In the West Midlands the number of live births and the number of clefts reported for 1986 were obtained from the Regional Health Authority and used to calculate the incidence rate of clefts in the region. A retrospective study of all the new cleft cases seen at Ibn Sina Hospital from 1985 to 1987 inclusive was carried out by examining their admission and operation records. Due to lack of photographic records, the decision as to the type and degree of cleft lip had to be based on the descriptions in the case notes. Only arab patients 457
Transcript
6rubh ~ourno/ qf~lastic Surgerv ( I990), 43,457462 C 1990 The Trustees of British Association of Plastic Surgeons
0007-1?26:9WOO43 0457;%10.00
Facial clefting in Kuwait and England: a comparative study
S. SRlVASTAVAand R. L. BANG
West Midlands Regional Plastic and Jaw Surgery Unit, Wordsley Hospital, Stourbridge, West Midlands, and
Department of Surgery, University of Kuwait
Summary-A retrospective study of all new cases of facial clefts seen between January 1985 and December 1987 was carried out at the Ibn Sina Hospital, Kuwait, and West Midlands Regional Plastic and Jaw Surgery Unit at Wordsley Hospital, Stourbridge, England; 234 arab children in Kuwait and 181 white Caucasian children in the West Midlandswerestudied. Despite vast differences in racial characteristics, environment and customs the pattern of clefting was very similar in the two groups. The incidence of clefts was calculated for the two regions and this demonstrated an increase in the incidence of cleft lip and palate (CL/P and CP) in the West Midlands since the last study was reported from this region in 1953.
There have been several studies worldwide on the epidemiology of cleft lip and palate but it seems that no such study has been carried out among the arab races in modern times. Millard (1976) men- tions that among all the mummies excavated in Egypt only one showed evidence of cleft palate. The present study was undertaken to ascertain the incidence and pattern of clefting in the mixed arab population of Kuwait and compare it with the pattern of clefting found in the white Caucasian population of the West Midlands, England.
The population of Kuwait (1.69 million in 1985) is composed of native Kuwaitis and expatriate workers of various nationalities, the majority of whom are arabs. As the Ibn Sina Hospital carries out nearly all the primary cleft repairs in the region, its records reflect the extent and pattern of cleft problem among the arab population there. The birth rate in Kuwait (33.7 per thousand) is among the highest in the world. The medical facilities are good and, due to the small distances involved, most patients are seen fairly early for treatment.
In the West Midlands (population 5.18 million in 1986). the Regional Plastic and Jaw Surgery Unit deals with about 70% of the cleft lip and palate patients in the area and here again the records of the unit closely reflect the pattern of facial clefting among the white Caucasian population in the region. In both Kuwait and West Midlands, repair of the cleft lip is carried out at 3 months of age while the palate is repaired between 12 and 18 months of age.
Materials and methods
To ascertain the pattern of clefting, data from 3 years (1985 to 1987) were used, to reduce the possibility of random variations and provide a more representative sample. Calculation of the incidence of clefting was carried out on the basis of the total cleft cases and the live birth figures obtained for the year 1986.
In Kuwait the total number of live birth figures were obtained from the Ministry of Public Health and these, together with the total number of new clefts repaired in 1986, were used to calculate an approximate incidence figure for that year. As clefts are not reported at birth in Kuwait, an assumption was made that the number of clefts repaired in a particular year must be approximately equal to the number of new cleft cases born that year as the population, birth rate and the number of new cleft cases presenting for treatment have not varied significantly during the past few years.
In the West Midlands the number of live births and the number of clefts reported for 1986 were obtained from the Regional Health Authority and used to calculate the incidence rate of clefts in the region. A retrospective study of all the new cleft cases seen at Ibn Sina Hospital from 1985 to 1987 inclusive was carried out by examining their admission and operation records. Due to lack of photographic records, the decision as to the type and degree of cleft lip had to be based on the descriptions in the case notes. Only arab patients
457
458 BRITISH JOURNAL OF PLASTIC SURGERY
were studied in detail, The clefts were classified according to the classification of Kernahan and Stark (1958).
Information on the sex of the patient, the severity and the side of presentation in unilateral cases, as well as other associated abnormalities were re- corded. Cleft lip and palate cases (CL/P) and isolated cleft palate (CP) were recorded separately. The last 50 patients in Kuwait were studied prospectively.
In the West Midlands a similar retrospective study of all new cleft cases among white Caucasian children from the years 1985-87 was carried out by examination of case notes and photographs.
Results
There were 53,786 live births in Kuwait during 1986. Approximately 80 new clefts were repaired during that year, giving an overall incidence figure of 1.48 clefts per 1,000 live births among all races.
In the West Midlands during the same year, 64,542 live births and 116 clefts (CL/P 70, CP 46) were reported. This gave an incidence figure of 1.79 clefts (CL/P 1.08, CP 0.71) per 1,000 live births.
It must be stressed, however, that in both countries these figures represent the cleft incidence amongst the total population as separate live birth figures for arab and white Caucasian children in their respective countries were unobtainable.
In Kuwait a total of 244 new cases were seen during the 3 years of the study. Records of 10 patients were unobtainable and they were accord- ingly left out of the study. Of the remaining 234 cases, there were 2 cases each of uncomplicated median cleft lip and transverse cleft of the mouth which were excluded; 230 patients had either cleft lip with or without cleft palate (CL/P 166 cases) or cleft palate alone (CP 64 cases).
At the West Midlands Plastic Surgery Unit, a total of 186 new cases were seen during the same period, of which 5 were left out of study due to incomplete records. Of the remainder, one had an uncomplicated median cleft of the upper lip and two had transverse oral clefts. 129 patients had CL/ P and 49 patients had isolated cleft palate (CP) (Table 1).
It can be seen that the relative percentage of the patients exhibiting CL/P and CP were very similar in the two countries as were the frequencies of unilateral and bilateral clefting (Table 1). The differences between left-sided and right-sided uni-
lateral clefting in arab and white Caucasian children are not statistically significant (Table 2).
Among the bilateral clefts the proportion of male and female children are similar in the arab and white Caucasian children, but the proportion of severe clefts among males (i.e. complete clefts of the lip and/or palate) is higher in the West Midlands group. This difference is statistically significant (p = .05) although the numbers are small (Table 3).
In the isolated cleft palate (CP) group a compar- ison of data from the two regions is shown in Table 4. The sex ratios in the two regions are similar and although arab children appeared to have a greater frequency of a more severe degree of cleft palate, the numbers are too small for a reasonable statistical analysis.
The frequency of associated problems among the CL/P and CP cases is given in Table 5, which shows a greater frequency of associated anomalies in the isolated cleft palate group. Five arab children had lower lip pits and three had intraoral bands needing division. One of the latter was associated with a bifid tongue and a very hypoplastic soft palate. The frequency of these two associated anomalies was negligible and nil respectively among the white Caucasian children.
Certain patterns of clefts were found to be uncommon. A complete cleft of the palate without any abnormality of the lip was found only twice among all the children studied, one of whom had
Table 1 Incidence and pattern of facial clefts
Kuwait England ( W. Midlands)
Population 1.69 million (1985) 5.18 million (1986)
Period studied 1985-87 1985-87 Overall birth rate 33.7 1,000 per 12.52 1,000 per
(1985) (1986) Overall incidence of 1.48per 1,OOOlive 1.79per 1,000 clefts births live births Race studied Arab White Caucasian Total clefts encountered 244 186 Total clefts studied 234 181 Transverse clefts 2 2 Median clefts Total CL/P and CP ;30 (100%)
England (W. Male 5 (13.88%) 21 (58.33”;) Midlands) 36 cases Female 2 (05.55S/,) 8 (22.224;) (1000/J
* This group includes cases of bilateral incomplete cleft lip without associated cleft palate
trisomy 21. Four arab and 5 white Caucasian children had complete palatal clefts associated with incomplete cleft lip. Slightly more common was the association of incomplete cleft of the lip associated with cleft soft palate (8 arab and 9 white Caucasian children). This has been shown to be a variant of CL/P (Ranta and Rintala, 1984).
Discussion
Determination of the true incidence of cleft lip and palate is difficult. In an ideal scenario all children born should be examined for these birth defects by a clinician with some training in plastic surgery to avoid missing the minor expressions of this prob- lem. However, this seldom if ever happens and an under-reporting of clefts at birth must be the rule
Table 4 Comparison of cleft palate cases
rather than the exception. If data from cleft clinics are used the incidence recorded is again low as some babies may not thrive, or move out of the area. It is customary to report the birth incidence of the clefts as N per 1,000 live births as it is realised that the frequency of birth defects among stillborn babies is much higher (Forfar, 1984; Holmes, 1987). However, some authors have reported their inci- dence figures using the total birth figures (McMohan and McKeown, 1953; Womersley and Stone, 1987).
Table 6 lists the incidence figures and the relative percentages of CL/P and CP given by, or calculated from the data given by, the authors from various parts of the world. The proportion of the two cleft types among the various Caucasian populations of the world are in broad agreement, with a few exceptions. Woolf et al. (1963) found the proportion of CL/P to be as high as 82.220,/, whilst McMohan and McKeown (1953) found it to be around 60%. The study of Womersley and Stone (1987) is exceptional because they found the proportion of CP cases higher than CL/P, a fact for which there seems to be no reasonable explanation. The incidence rates of the clefts reported varies mark- edly, with the highest incidence of 2.00 per 1,000 live births reported from Canada (Welch and Hunter, 1982). These authors were dealing with a mixed population. The figures from Japan and Nigeria should be considered separately as they involve completely different racial groups, one with
Kuwait 64 cases ( 100%)
Male Female
England ( W. Midlands 49 cases ClOO%i
_ Male Female
Submucous cleft palate
Cleft soft and posterior hard palate Bilateral incomplete cleft palate”
Total 19 (11.44%) 15 (23.43%) 15 (11.62%) 18 (36.73%)
the highest and the other with the lowest incidence of clefting.
No data are available to the best of our knowledge for the arab population. Kuwait is ideally suited for such a study because of its small size, high birth rate, centralisation of cleft care and good medical facilities. However, lack of cleft reporting at birth and absence of photographic records is a serious handicap. Due to the lack of reporting clefts at birth, a less than ideal method of calculation of incidence figure for clefts had to be used. Still it gives an idea of the incidence of clefts among the arab population, as clefts seen among the non-arab population amounted to 10% only. Further work is needed for a more accurate estimate of the incidence figure.
In the West Midlands we were fortunate to find a previous study carried out nearly 40 years ago by
McMohan and McKeown (1953) in Birmingham, the largest city in the region. They examined the records of all the births in the city and arrived at the incidence figure of 1.30 per 1,000 total births, which was in line with the cleft incidence figures for that time from Denmark (Fogh-Andersen, 1942). It is interesting that McMohan and Mc- Keown used the total birth figures because the incidence of congenital defects in stillborn babies is generally higher.
It can be argued that there can be no fair comparison between the composition of the popu- lations of the West Midlands in the 1940s and 1980s and hence the cleft incidence figures. However, it is well-known that the population of the West Midlands is composed of three main ethnic groups, namely, white Caucasians, Negroes and Asians, with other smaller ethnic groups being less numer-
Table 6 Incidence of cleft lip and palate in various parts of the world
Author and country
Incidence/thousand Year of live births (I.b.)/total study births (t.b.1 CL/P% CP%
Fogh-Andersen Denmark 193842 1.31 (lb.) 75 25 Bonaiti et al. France 1981 1.17 (Lb.) 74.16 25.83 Woolf et al. USA 1951~61 1.51 (1.b.j 82.22 17.77 Welch &Hunter Canada 1964-77 2.0 (1.b.) 68.04 31.96 Oliver Padilla Puerto Rico 1950-83 ~ 67.10 32.90 Iregbulem Nigeria 197680 0.37% (1.b.) 81.0 19.0 Natsume et al. Japan 1981 2.05% (lb.) 78.84 21.15 Knox & Braithwaite, UK 1949-60 1.42 (lb.) 67.3 32.7 Womersley & Stone, UK 1974-85 1.56 (tb.) 47.77 52.22 McMohan Jr McKeown, UK 1940-50 1.30 (t-b.) 60 40
Our study 1985-1987 Kuwait 1985-87 1.48 (lb.) (1986) 72.17 27.82 England (West Midlands) 1985-87 1.79 (lb.) (1986) 72.4 27.52
Note : The proportion of CL/P and CP has in many cases been calculated from the figures given by the authors
FACIAL CLEFTING IN KUWAIT AND ENGLAND: A COMPARATIVE STUDY 461
ous. Clefts among the Negro population are rarely seen, while clefts among Asian children accounted for only 10% of all the cleft cases seen in the year 1986. This leads to the conclusion that the incidence figure of 1.79 clefts per 1,000 (all races) is an underestimate of the cleft incidence in the Cauca- sian population. Even so, the rise in the incidence rate of clefts from 1.30 per 1,000 total births to 1.79 per 1,000 live births is statistically significant (p <O.Ol). If only the clefts from the year 1986 are considered, the increase applies equally to the CL/ P and CP group as in that year the proportions of the CL/P and CP were found to be similar to that reported by McMohan and McKeown (1953). However, when the clefts from all three years are considered, the frequency of CL/P rises to 72.17%.
A similar increase in the incidence of clefting was observed by Fogh-Andersen (1961, 1964) in Denmark where he noted that the incidence of clefts had risen from 1.31 in the 1938-42 period to 1.82 between 1958 and 1962. A similar increase in the incidence of clefts has been demonstrated by Rintala (1986) in Finland where the incidence is nearly I .8 per 1,000 now. It would appear that the incidence of clefts has also been rising in the West Midlands.
The aim of this study was to document the patterns of clefting among the arab population of Kuwait and compare it with the patterns of clefting among the white Caucasian population of West Midlands. It was thought that significant differ- ences between the cleft patterns of the two regions would be demonstrable given the vast differences in the racial make-up of the populations, environ- mental conditions, climate and marriage customs. It was therefore surprising that the patterns of clefting among the arab population of Kuwait was found to be no different from the white Caucasian population of West Midlands. The associated anomalies also seem to occur with similar frequency in both groups, except for a greater frequency with which lower lip pits were encountered in the arab children.
Cleft lip and palate (CL/P) and isolated cleft palate (CP) are genetically different conditions (Fogh-Andersen. 1942) and ought to be studied separately. Only in rare circumstances do they occur in the same family, as seen in the Van der Woude syndrome and popliteal pterygium syn- drome (Gorlin and Cerevenka, 1974). Although several factors are thought to be relevant in the causation of non-syndromic cleft lip and palate and isolated cleft palate, the only factor of overriding
importance appears to be the genetic predilection. It appears that the pattern of occurrence of genes responsible for CL/P and CP is similar in the two races studied. However, further work is needed to confirm this point.
Acknowledgements
We gratefully acknowledge the help given in this study by the consultant staff at the Ibn Sina Hospital, Kuwait, and the West Midlands Regional Plastic and Jaw Surgery Unit. England.
In particular, we would like to thank Mr A. G. Huddart and Mrs K. M. Randle for their invaluable help in obtaining the statistics for the West Midlands, Dr D. Verma for the statistical information from Kuwait and Mr D. Richmond for his help with the statistical analysis.
References
Bonaiti, C., Briard, M. L., Feingold, J., Pavy, B., Psuame, J., Migne-Tulferaud, G. and Kaplan, J. (1982). An epidemiology and genetic study of facial clefting in France. I. Epidemiology and frequency in relatives. Journal ofMedical Genetics, 19, 8.
Fogh-Andersen, P. (1942). Inheritance of harelip and cleft palate, Dissertation, Domus Biologiae Hereditariae Humanae. Uni- versity of Copenhagen: Arnold Busck.
Fogh-Andersen, P. (1961). Inheritence patterns for cleft lip and palate. In Pruzansky, S. (Ed.) Congenital Anomaliesoj‘the Face and Associufed Strucfures. Illinois: Thomas Springfield.
Fogh-Andersen, P. (1964). Recent statistics of facial clefts- frequency, heredity, mortality. In Hotz. R. (Ed.) Earl> Treatment of Clefi Lip and Palate. Bern. Stuttgart: Hans Huber.
Fogh-Andersen, P. (1967). Genetic and nongenetic factors in the aetiology of facial clefts. Scandinaoian Journul of Plastic und Reconstructice Surgery. I. 22.
Forfar. J. 0. (1984). Demography, vital statistics and pattern of disease in childhood. In Forfar. J. 0. and Arneil, G. C’. (Eds) Textbook yf‘paediatrics, Third Edition. Edinburgh: Churchill Livingstone.
Go&, R. J. and Cerevenka, J. (1974). Syndromes of facial cleftinn. Scandinavian Journal of’ Plastic and Recon.wuctir:e Surgei+. 8, 13.
Holmes, L. B. (1987). Congenital malformations. In Behrman, R. E.. Vaughan, V. C. and Nelson. W. E. (Eds) h’&on:r Textbook qf Paediotrics. Thirteenth Edition. Philadelphia: W. B. Saunders Co.
Iregbulem, L. M. (1982). The incidence of cleft hp and palate in Nigeria. Cleft Palate Journal. 19. 201.
Kernahan, D. A. and Stark, R. B. (1958). A new classification for cleft lip and palate. Plastic and Reconstructive Surgery. 22,435.
Knox, G. and Braithwaite, F. (1963). Cleft lip and palate in Northumberland and Durham. Architses qf’ Di.wa.te in C-hi/d- hood, 38.66.
McMohan, B. and McKeown, T. (1953). The incidence of harelip and cleft palate related to birth rank and maternal age. American Journal of Human Genetics, 5. 116.
Millard, D. R. Jr. ( 1976). Ctef Crnfi : The Ero!ution of Its Sur.er~. Volume I: The unilateral deformity. First EdItion. Boston: Little Brown and Company, pp 57-58.
Natsume, N., Suzuki, T. and Kawai, T. (1987). The prevalence of
462 BRITISH JOURNAL OF PLASTIC SURGERY
cleft lip and palate in the Japanese: their birth prevalence in 40,304 infants born during 1982. Oral Surgery, 63,421.
Oliver-Padilla, G. and Martinez Gonzalez, V. (1986). Cleft lip and palate in Puerto Rico: a thirty-three year study. Clef? Palate Journal, 23,48.
Ranta, R. and Riitala, A. (1984). Separate clefts of the lip and the palate: a variant of cleft lip and palate. Scandinavian Journal of Plastic and Reconstructive Surgery. 18,233.
Rintala, A. E. (1986). Epidemiology of orofacial clefts in Finland: review. Annals of Plastic Surgery, 17,456.
Welch, J. and Hunter, A. G. W. (1982). An epidemiological study of facial clefting in Manitoba. Journal of Medical Genetics, 17, 127.
Womersley, J. and Stone, D. H. (1987). Epidemiology of facial clefts. Archives of Disease in Childhood, 62, 7 17.
Woolf, C. M., Woolf, R. M. and Broadhent, T. R. (1963). A genetic study of cleft lip and palate in Utah. American Journal of Human Genetics, 15,209.
The Authors
Shekhar Srivastava, FRCS(Eng), FRCS(Fd), Locum Consultant Plastic Surgeon, West Norwich Hospital, Norwich; formerly Registrar in Plastic Surgery, West Midlands Regional Plastic and Jaw Surgery Unit, Wordsley Hospital, Stourbridge, and Senior Registrar in Plastic Surgery and Bums, Ibn Sina Hospital, Kuwait.
Rameshwar L. Bang, FRCS(Ed), FICS, Consultant Plastic Surgeon, Assistant Professor and Head of Plastic Surgery Unit, Department of Surgery, Faculty of Medicine, University of Kuwait.
Requests for reprints to: Mr S. Srivastava, Locum Consultant Plastic Surgeon, West Norwich Hospital, Bowthorpe Road, Norwich, Norfolk NR2 3TU.
Paper received 23 August 1988. Accepted 30 October 1989 after revision.
