There has been no national register of infants born with a cleft lip and/or palate in
Ireland. The compilation of such a register for the purposes of clinical audit and strategic
planning has only recently (2000) been made possible with the appointment of a Cleft
Co-ordinator in the Eastern Regional Health Authority area.
Consequent on the lack of
record-keeping, any figure given of the number of babies born with a cleft lip and/or palate
in Ireland can only be an approximation. The figure of (‘approximately’) 75 children quoted
in the report ((Eastern Health Board. Development of Oral & Maxillofacial Surgery Services
in the Eastern Health Board Region. February 1999)) of a review into the development of oral
and maxillofacial surgery services in the Eastern Health Board region (February 1999) is
probably based on the often given estimation of 1:700. Based on the (provisional) number of
live births in 1998 of 53,551 (Source: Central Statistics Office), this figure calculates
correctly.
However, as can be seen from the varied research reports into the incidence of cleft
throughout the world (and reported here), there would seem to be quite a variance in
reported figures. Ratios have gone as high as 1:481 and as low as 1:1000. Most reported
studies we have examined indicate a figure of around 1:700 or higher. The figures from
Denmark where records have been kept since 1937 consistently show a higher ratio. The
average for the years 1976-81 was 1:529, with a high of 1:481 in 1981. Analysis of the
various research reports would seem to suggest that 1:700 may be a conservative figure. Many
figures (one exception being Denmark) are based on voluntary disclosure and not accurate
record-keeping. Based on the Danish figure of 1:529, this would give an Irish total of 121
such births in 2006 (1:700 = 92).
A fair conclusion would be that the figure of 92 per annum may be conservative, and it could
theoretically be as much as 30% higher, particularly allowing for year to year variation.
American figures indicate that the incidence of cleft lip/palate varies between
different races; among Asians it is 1.7 per 1000 births, while among African-Americans it is
approximately 1 per 2500 births. The risk of isolated cleft palate seems to be the same
across all racial groups. ((March of Dimes Birth Defects Foundation.
The incidence of cleft palate occuring alone is about 1 in 2000 births. More than 70% of
babies with cleft lip also have cleft palate. ((REF:2))
Combined cleft lip and palate
(CLP) represents approximately 50% of incidents, cleft palate alone c.30%, and cleft lip
alone c.20%. ((Young, Greg, M.D. (1998).
Cleft Lip and Palate. [Online] 28 January 1998.
Available from
http://www2.utmb.edu/otoref/Grnds/Cleft-lip-palate-9801/Cleft-lip-palate-9801.htm [verified
26 Mar 2001])) (These ratios are at slight variance with figures reported from Denmark and
the UK and referred to below. Other reports have also given slightly variant
figures).
According to Dr. John Mulliken, Director of the Craniofacial Centre, Children’s
Hospital, Boston, Massachusetts, less than 10% of cleft lips are bilateral. ((Mulliken, John
B., M.D. (2000). Repair of bilateral complete cleft lip and nasal deformity – state of the
art. [Online] American Cleft Palate-Craniofacial Association Journal 37:342-347 2000.
Available at http://cpcj.allenpress.com/ [verified 26 March 2001]))
Where a child is born
with a cleft, there is a 5% chance of the next born also having a cleft – that is a 1 in 20
chance. If a second child is born with a cleft, the likelihood increases further for any
subsequent child.
The chances of a parent who was born with a cleft having a cleft baby
is approximately seven in one hundred.
Cleft lip and palate are what are known as
congenital or birth defects, and are the fourth most common birth defect, being the most
common of the head and neck region.
Clefts of the lip and combined lip and palate are
twice as common in males. Isolated cleft palates are twice as common in females. This may be
explained by the fact that the secondary palate closes one week later in females.
((REF:4))
According to the UK Craniofacial Anomalies Register (CARE), patients with cleft
lip only account for 22% of total cases, with cleft lip and palate 34%, and cleft palate
only 44%. The sex distribution is reported as 59% male and 40% female, with 1%
unknown.Statistics from Denmark, where there is a compulsory reporting code, indicate that
the reported incidence of cleft lip and/or palate is higher than the widely accepted figure
of 1:700. Danish figures indicated a rise from 1:667 to 1:529 between 1942 and 1981 (Jensen
et al 1988). ((Jensen, B. L. et al, (1998). Cleft lip and palate in Denmark, 1976-1981.
Epidemiology, variability, and early somatic development. Cleft Palate Journal, 25:258-269))
It is thought that the Danish figure may be due to better reporting, decreased neonatal
mortality, increased levels of environmental teratogens and increased frequency of marriage
and childbirth in cleft patients because of better care. ((UK Craniofacial Anomalies
Register (CARE). Available at http://www.cfsgb.org.uk/care/ [accessed 25 June 2000])) The
sex distribution among Danish cases according to the Jensen report was 61% male and 39%
female. Cleft lip accounted for 34% of cases, cleft lip and palate 39% and cleft palate 27%.
The ratios of CL, CLP and CP among the sexes mirrors the ratios reported above.
According
to statistical data from Brian Sommerlad, ((Sommerlad, Brian C. (1994) Management of cleft
lip and palate. Current Paediatrics, 4:189-195)) Consultant Plastic Surgeon, North East
Thames Cleft Service, unilateral cleft lip and palate accounted for 24.1% of clefts among
522 patients (excluding submucous cleft palate), bilateral cleft lip and palate 9.4%,
isolated cleft palate 41%, and cleft lip 25.5%. These figures further highlight the
discrepencies in the reported statistical data from different sources.
During the period
1975-1992, 616 children (367 boys, 249 girls) with cleft lip and palate were born in greater
Stockholm, Sweden (pop. 1.3m). ((Milerad, J., et al. (1997) Duarte, R., Leal, M.J. (1999).
Associated Malformations in Infants with Cleft Lip and Palate: A Prospective,
Population-based Study. Pediatrics, August 1997, 100(2): 180-186.)) The number varied from
20 in 1977 to 51 in 1992. Related to the number of live births, this gave a range of
1.2/1000 (1 per 833) to 2/100 (1 per 500) live births and a mean incidence of 1.7/1000 (1
per 588) live births.
Of the total 616 infants, 163 (26%) had cleft lip, 239 cases (39%)
had isolated cleft palate, and 214 cases (35%) had both cleft lip and palate. Of these 214
infants with complete clefts, 143 were unilateral and 71 were bilateral cleft lip and
palate.