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British Journal of Orthodontics, Vol. 26, No. 2, 121-125, June 1999
© 1999 British Orthodontic Society

The Incidence of Cleft Lip and Palate Deformities in the South-east of Scotland (1971-1990)

Refereed Paper

T. H. Bellis, B.D.S., D.D.O., F.D.S.R.C.P.S., M.S.C.D., M.ORTH.

Orthodontic Department, Dorset County Hospital, Williams Avenue, Dorchester DT1 2JY, UK

B. Wohlgemuth

Postgraduate Dental Institute, University of Edinburgh, Edinburgh, UK


   Abstract
Top
 Abstract
Introduction
 Methods
 Results
 Conclusions
 References
 
This retrospective study reports the incidence of infants born with the cleft lip and palate anomaly within the Edinburgh Cleft Units catchment area, between 1 January, 1971, and 31 December, 1990. The importance of accurate data collection for local, regional, and national data bases is discussed with reference to the recent CSAG report on cleft lip and palate services in the UK.

Five-hundred-and-two cleft lip and palate patients were identified (291 males, 211 females). The incidence is reported as 1.4 per 1000 live births (1 in 711). Twenty-five per cent of clefts affected the primary palate, 45 per cent affected the secondary palate, and the remaining 30 per cent were clefts of both the primary and secondary palate. Overall, a higher percentage of males were affected (58 per cent males to 42 per cent females). Clefts of the secondary palate, however, were more common in females (56 per cent females to 44 per cent males).

Data presented in this study is similar to that previously reported from UK centers. It is suggested the accuracy of the UK cleft lip and palate date collection needs to be improved. Prospective data collection in a standardized format carried out on a national basis has to be a priority as recommended by the CSAG report.

Key words: Cleft Lip and Palate, Data Base, Epidemiology, Incidence


   Introduction
Top
 Abstract
 Introduction
Methods
 Results
 Conclusions
 References
 
It is now widely recognized that accurate record keeping and data collection is of paramount importance in the running of Health Services. This is particularly true in the planning and development of service provision and resource allocation, as well as clinical audit and research. The recording and documentation of cleft lip and palate deformities in the UK has historically been voluntary, and carried out by the Office of National Statistics (ONS), formally known as the Office of Population Censuses & Surveys (OPCS), in England and Wales, and in Scotland by the Information and Statistics Division NHS in Scotland. The Clinical Standards Advisory Group (CSAG) report into cleft lip and palate services in the UK has stated its concerns relating to the accuracy of this data.

Regional data bases as described by Greg et al. (1994Go), Luther and Cook (1994Go), and the Scottish Cleft Lip and Palate Group SCALP, have set the standard for data collection in the 1990s. CARE was initially set-up by the Craniofacial Society in 1989 as an additional voluntary register of cleft birth data nationally. This proved less reliable than the ONS data initially. However, in 1995, regional cleft co-ordinators were introduced to improve the system of collection personal communication. Registrations have subsequently improved dramatically. However, a few units still prefer not to inform this group of their cleft birth data.

National registration systems for cleft anomalies are not new. In Denmark, the government introduced compulsory recording of children with facial clefts in 1937 (Bixler et al., 1971Go). Norway established a medical register of births, including information on congenital malformations in 1967. A European initiative, EUROCAT, was established in 1979 European Registration of Congenital AnomaliesGo. This was supported by the European Commission with the aim of improving the methodology for population studies throughout the community. Initially, EUROCAT was set-up as a feasibility study to test the ability of pooling data across national boundaries. By 1991, as a result of its success, it is no longer supported as a research project, but as a fully funded service in its own right.

Studies in Denmark by Jensen et al. (1988Go) have demonstrated an increased incidence of cleft births between 1942 and 1981. Between 1938 and 1942 there were 1.5 per 1000 births, which increased to 1.89 per 1000)births between 1978 and 1981. Similar increases have been reported by Rintala (1986Go), and Srivastava and Bang (1990Go) in Finland and Kuwait, respectively. No such increases have been reported in UK studies. This demonstrates the importance of having base-line values easily available to determine any change in incidence.

Since 1989, the Edinburgh cleft unit has been participating in the Scottish Cleft Lip and Palate Project SCALP. This is a voluntary system for collecting standardized data on all cleft lip and/or palate infants born in Scotland. No accurate cleft data was available prior to this date in an easily accessible form.


   Methods
Top
 Abstract
 Introduction
 Methods
Results
 Conclusions
 References
 
A retrospective study was undertaken to identify all cleft lip and palate births within the regions of Lothian East, West, and Central, Borders, Fife, and the Highlands. The aim was to gather information from 1970 onwards specifically noting the cleft type, date of birth, presence of an associated syndrome, medical history where applicable, and details of surgical care received since birth. The cleft deformities were recorded using an anatomically descriptive classification as used in the CARE registration document. The classification therefore depicted the cleft types as follows:

The following list indicates the sources of information for this particular study:

  1. Plastic surgery records.
  2. Hospital operating lists, Edinburgh Sick Childrens Hospital.
  3. Edinburgh Dental Hospital patient records.
  4. Fife hospital patient records.
  5. Information and Statistics Division NHS in Scotland.
  6. Patients records at the Edinburgh Sick Childrens Hospital.
  7. Western General Hospital patient records.
  8. Leith Hospital patient records.

An initial register of 721 possible patients were identified. All case notes were searched and many cases were excluded on the following grounds:

  1. No cleft present.
  2. Birth location outside catchment area.
  3. Birth date not within the period 1 January, 1971, to 31 December, 1990.
  4. Submucous clefts (23 cases).

The resulting cohort comprised of 502 cleft lip and palate patients (291 males and 211 females).

A computer data base was formulated and a paper copy generated to record the data in a standardized format. A single operator was used to sift and record the data, and then enter the data into the computer data base. Live birth data was obtained from the Scottish Home and Health Birth Statistics, published annually. Statistical analysis was carried out using the Chi-squared analysis with 1 degree of freedom. Probability values of 0.05 or less were taken to be statistically significant.


   Results
Top
 Abstract
 Introduction
 Methods
 Results
Conclusions
 References
 
The overall incidence for cleft births over this 20-year period was 1.4 per 1000 or 1 in 711 live births. The incidence of cleft births varied from year to year, ranging from 1 in 1013 in 1973, to a high of 1 in 540 in 1987 Table 1.


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  		TABLE 1 Cleft births 1971-1990
 
Forty-three infants presented with Pierre Robin sequence and 92 with an associated syndrome. During the period of record collection, 12 post-natal deaths had been recorded from within the 502 cohort group (Table 2).


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  		TABLE 2 Cleft births identified with an associated syndrome, Pierre Robin Sequence and recorded post-natal deaths.
 
Comparison of the birth data for the first 10 years (1971-1980) with that of the second 10 years (1981-1990) show a remarkable overall consistency. While the cleft births have increased from 249 to 253 over this period, the overall birth rate dropped from 181,004 to 175,729. Thus, the incidence changed only slightly, increasing from 1.37 per 1000 to 1.4 per 1000 live births (1 in 727 to 1 in 695). The increase in cleft births between the first and the second 10-year period was not statistically significant Table 3.


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  		TABLE 3 Cleft births between 1971 and 1980, and 1981 and 1990
 
Twenty-five per cent of clefts affected the primary palate, while 45 per cent affected the secondary palate. The remaining 30 per cent affected both the primary and secondary palate. Left-sided cleft lip and palate defects (13 per cent ) were more common than right-sided clefts (6 per cent; Table 4). The distribution of cleft types is shown inTable 4 and pictorially presented in Figure 1.


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  		TABLE 4 Distribution of cleft types
 


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  		FIG. 1 Distribution of cleft birth types in South East Scotland 1971-1990

 
Fifty-eight per cent of clefts were males, while 42 per cent were females. In all categories males dominated, apart from the isolated cleft palate group, where females dominated by 56-44 per cent . Statistically, however, male dominance was significant in the cleft lip group, as well as the bilateral cleft lip and palate group (Table 5). In the cleft lip group only (Table 6), male dominance was statistically significant for left-sided clefts (Table 8), and highly significant in the male incomplete left sided cleft lip group (Table 7).


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  		TABLE 5 Cleft types by sex
 

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  		TABLE 6 Cleft lip only
 

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  		TABLE 8 Cleft lip by side + sex
 

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  		TABLE 7 Cleft lip complete/incomplete + sex
 
Discussion

While retrospective studies such as this can easily be criticized for their inaccuracies, every attempt has been made to maintain consistency in this study. Several factors may affect the accuracy of regional data bases. These include:

  1. Cross-boundary flow as previously described by Knox and Braithwaite (1962Go).
  2. Fluidity of movement of personnel in and around her Majesty's armed forces bases (there are several large military bases within the Edinburgh catchment area).
  3. Quality and availability of record keeping.

The data presented in this study would seem to be consistent with previous studies reported in the UK literature (McMahon and McKeown, 1953Go; Knox and Braithwaite, 1962Go; Owens et al., 1985;Go Womersley and Stone, 1987;Go Coupland and Coupland, 1988Go; Srivastava and Bang, 1990;Go Greg et al., 1994Go; Fitzpatrick et al., 1994Go). In these studies, the incidence reported varies from a low of 1.12 per 1000 (Coupland et al., 1988Go) to a high of 1.79 per 1000 live births (Srivastava et al., 1990Go); Table 9.


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  		TABLE 9 Previously reported Cleft Lip and Palate incidence studies
 
The cleft incidence varies from year to year with quite dramatic swings. Table 1 shows the annual figures where the incidence varies from a low of 1 in 1013 births in 1973, to a high of 1 in 540 in 1987. These large swings, which are totally unpredictable, are consistent with previous findings (Knox et al., 1962;Go Greg et al., 1994Go).

Jensen et al. (1988Go), Rintala (1986Go), and Srivastava and Bang (1990Go) have previously reported an increase in cleft lip and palate incidence in recent years. No British authors have been able to show a similar trend. This study reports the incidence between 1971 and 1980, and 1981 and 1990, increased from 1.37 per 1000 to 1.4 per 1000 live births Table 3. The increase is small and not statistically significant.

High levels of clefts affecting the secondary palate were reported by Womersley and Stone in (1987Go) 52.2 per cent and again by Fitzpatrick et al. in 1994 53 per cent in the West of Scotland. Greg et al. (1994Go) also reported from Northern Ireland that clefts of the secondary palate were consistently high at 53 per cent . In this study, the clefts of the secondary palate occurred in 45 per cent of our sample.

Clefts of the secondary palate have been shown to have female dominance (Knox and Braithwaite, 1962;Go Fitzpatrick et al., 1994Go; Greg et al., 1994Go). This report can confirm these previous findings with females dominating by 56-44 per cent.

Unilateral cleft lip and palate clefts are more prevalent on the left side (Table 4, 65 left to 31 right), as has been previously reported by Knox and Braithwaite (1962Go), Jensen et al. (1988Go), and Greg et al. (1994Go). Unlike Greg et al. (1994Go), the right-sided clefts of the lip and palate were still dominated by males Table 4. There is one significant difference between this study and that reported by Greg et al. in 1994, this being that his unilateral cleft lip and palate group are separated into complete and incomplete clefts, which we have not carried out in this study.

A comparison of cleft data from recent publications has been tabulated in Table 9. Direct comparison between studies is often difficult due to differing classifications used to depict cleft types Vanderas, (1987Go). Thus, enhancing the argument for standardizing record collection for cleft patients.

Currently, data collected by ONS, on cleft births, only describes a cleft in two forms:

It is essential that a full classification of cleft type is recorded for each infant. Vandaras (1987), in his comprehensive review of the literature on cleft incidence, suggests that the cleft data collected should be separated by:

  1. Cleft type: cleft lip, cleft palate, and cleft lip and palate.
  2. Racial type.
  3. Sex.
  4. Geographical area.
  5. With and without associated malformations and syndromes.

In addition, live birth, still birth, and abortion statistics should also be reported.

The concern raised by the CSAG report regarding lack of completeness of recording the incidence of cleft lip and palate births, is a fundamental issue which needs to be addressed. The ONS data has been shown to be incomplete [Clinical Standards Advisory Group Report (CSAG Report) 1998Go]. The under reporting may well be due to the fact that this is a voluntary register, with several units choosing not to report the relevant information. Making a collection of cleft data mandatory may well improve its accuracy. However, Abyholm (1978Go) has reported that a compulsory national register alone is no guarantee of a comprehensive and accurate data collection system. In his article reviewing the registrations in Norway, he has shown an under-reporting of 14.46 per cent, when data from the national register was compared with the hospital operating statistics.

The Group, using regional co-ordinators and standardized data collection forms, have recently shown that their collection of data has improved to such a degree that the number of clefts being reported is larger than that reported by the ONS data (CARE News Letter, March 1998)Go. It would therefore seem sensible to try and merge these two separate data collection systems and make use of their respective strengths, as well as making registration notification compulsory.


   Conclusions
Top
 Abstract
 Introduction
 Methods
 Results
 Conclusions
References
 
Data presented here clearly demonstrate a similar distribution of cleft type, number and sex, to previous reports published from other UK centres. Prospective data collection in a standardized format carried out on a National basis has to be a priority as recommended by the CSAG report.


   Acknowledgments
 
The authors would like to thank Mr A. C. H. Watson, Consultant Plastic Surgeon, for his help and encouragement with this project. Additional thanks and gratitude are passed on to Fife Health Board for their generous financial assistance, and the Medical Records Department, Edinburgh Sick Childrens Hospital and the Western General Hospital.


   References
Top
 Abstract
 Introduction
 Methods
 Results
 Conclusions
 References
 
Abyholm, F. E. (1978) Cleft Lip and Palate in Norway. I. Registration, incidence and early mortality of infants with cleft lip and palate,Scandinavian Journal of Plastic Reconstructive Surgery , 12,29 –34.

Bixler, D., Fogh-Andersen, P. and Conneally, P. M. (1971) Incidence of cleft lip and palate in the off-spring of cleft patients,Journal of Clinical Genetics , 2,155 –159.

CARE Newsletter—March (1998)

Clinical Standards Advisory Group Report (1998) Cleft Lip and/or Palate: (1998), Her Majesty's Stationery Officer, London.

Coupland, M. A. and Coupland, A. I. (1988) Seasonality, incidence and sex distribution of cleft lip and palate births in Trent region (1973)-(1982),Cleft Palate Journal , 25,33 –37.[Medline]

European Registration of Congenital Anomalies (EUROCAT) www.iph.fgov.be/eurocat/.

Fitzpatrick, D. R., Raine, P. A. M. and Boorman, J. G. (1994) Facial clefts in the West of Scotland in the period (1980)-(1984): epidemiology and genetic diagnosis,Journal of Medical Genetics , 31,126 –129.[Abstract]

Greg, T., Boyd, D. and Richardson, A. (1994) The incidence of cleft lip and palate in Northern Ireland from (1980)-(1990),British Journal of Orthodontics , 21,387 –392.[Abstract]

Jensen, B. L., Kreiborg, S., Dahl, E. and Fogh-Andersen, P. (1988) Cleft lip and palate in Denmark (1976)-(1981) epidemiology, variability and early somatic development,Cleft Palate Journal , 25, 258-269.

Knox, G. and Braithwaite, F. (1962) Cleft lips and palates in Northumberland and Durham,Archives of Disease in Childhood , 38,66 –70.

Luther, F. and Cook, P. H. (1994) The development of a regional cleft lip and palate data base: a preliminary report,British Journal of Orthodontics , 21,291 –295.[Abstract]

MacMahon, B. and McKeown, T. (1953) The incidence of harelip and cleft palate related to birth rank and maternal age,American Journal of Human Genetics , 5,176 –183.[Medline]

Owens, J. R., Jones, J. W. and Harris, F. (1985) Epidemiology of facial clefting,Archives of Diseases in Children , 60,521 –524.[Abstract]

Rank, B. K. and Thomson, J. A. (1960) Cleft lip and palate in Tasmania,Medical Journal of Australia , 2,681 –688.

Rintala, A. E. (1986) Epidemiology of orofacial clefts in Finland: a review,Annals of Plastic Surgeons , 17,456 –459.

Srivastava, S. and Bang, R. L. (1990) Facial clefting in Kuwait and England: a comparative study,British Journal of Plastic Surgery , 43,457 –462.[Medline]

Vanderas, A. P. (1987) Incidence of cleft lip, cleft palate and cleft lip and palate among races: a review,Cleft Palate Journal , 24,216 –225.[Medline]

Womersley, J. and Stone, D. H. (1987) Epidemiology of facial clefts,Archives of Disease in Childhood , 62,717 –720.[Abstract]




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