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. 1999 Mar 13;318(7185):733.

Congenital abdominal wall defects in the United Kingdom

Sources had different reporting patterns

Simon Clarke 1,2, Evelyn Dykes 1,2, Jean Chapple 1,2, Lenore Abramsky 1,2
PMCID: PMC1115157  PMID: 10074027

Editor—Stone et al have a very different view from ours of abdominal wall defects in our regions (former South East Thames and North West Thames).1 As they acknowledge, the cited rates for abdominal wall defects were derived from sources with different reporting patterns. In particular, the rates quoted for England and Wales were from data from the Office for National Statistics,2 which take no account of terminations for abnormalities diagnosed prenatally, whereas the Glasgow and northern England registers have tried to be comprehensive.

More appropriate north-south comparisons could have been achieved from the rates reported by individual regional registers such as those in the former South East Thames and North West Thames regions. These registers obtain data in a similar manner to the northern regional registers, with active data collection from multiple sources and inclusion of terminations and stillbirths. The table shows the data for abdominal wall defects from the South East Thames and North West Thames registers in 1992-6. These data confirm a higher incidence of abdominal wall defects in the south of England than that quoted by Stone et al. Consequently, the gradient hypothesis is not supported when comparable data are used. The apparent excess of exomphalos in Scotland does not distinguish between cases with isolated exomphalos and those with multiple defects or inherited syndromes, which may have completely different causes; it is therefore inappropriate to speculate on possible causal factors for all defects.

What Stone et al’s study does show, however, is the need for fully funded comprehensive regional registers for congenital malformations, which take account of the changes in perinatal practice and the frequent fragmentation of prenatal and postnatal tertiary services. The data derived from the Office for National Statistics are an exhaustive attempt to ascertain incidence on a national level but cannot be seen to be as efficient as data from regional registers.3 Until a national network of comparable registers is achieved, information derived for planning and public health issues will be seriously flawed at best and at worst misleading.

Table.

Incidence of abdominal wall defects according to former South East Thames and North West Thames registers, 1992-6

South East Thames
North West Thames
Both regions
Abdominal wall defect No of cases Incidence No of cases Incidence No of cases Incidence
Total No of births and terminations* 252 462 237 614 490 076
Exomphalos  63 2.5 108 4.55 171 3.5
Gastroschisis  67 2.8  49 2.06 116 2.37
Abdominal wall defects 130  5.15 157 6.61 287 5.86
Ratio of gastroschisis: exomphalos 1:0.9 1:2.2 1:1.47

Incidence per 10 000 total births *Includes live births, still births, and terminations. 

References

  • 1.Stone DH, Rimaz S, Gilmour WH. Prevalence of congenital anterior abdominal wall defects in the United Kingdom: comparison of regional registers. BMJ. 1998;317:1118–1119. doi: 10.1136/bmj.317.7166.1118. . (24 October.) [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 2.Tan KH, Kilby MD, Whittle MJ, Beattie BR, Booth IW, Botting BJ. Congenital anterior abdominal wall defects in England and Wales 1987-1993: retrospective analysis of OPCS data. BMJ. 1996;313:903–906. doi: 10.1136/bmj.313.7062.903. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 3.Chitty L, Isakaros J. Congenital anterior abdominal wall defects. BMJ. 1996;313:891–892. doi: 10.1136/bmj.313.7062.891. [DOI] [PMC free article] [PubMed] [Google Scholar]
BMJ. 1999 Mar 13;318(7185):733.

Analysis should be restricted to regional data

Mark Kilby 1,2,3, Anthony Lander 1,2,3, Ann Tonks 1,2,3, Mike Wyldes 1,2,3

Editor—Stone et al recently tested the hypothesis of a north-south variation in the UK prevalence of abdominal wall defects and confirmed the greater prevalence in the north of England and Scotland.1-1 We noted that the regional data used did not extend below the Mersey region in England. Many health regions now have congenital anomaly registers, including the West Midlands (table). Stone et al use several different time periods for comparing regional data, and an increasing prevalence of gastroschisis with time has been reported. A more complete assessment of variation in prevalence, however, may be noted using more recent data for all regions.

Table.

Prevalence of abdominal wall defects according to West Midlands congenital anomaly register, 1995-6

Abdominal wall defect No of cases Prevalence1-150 (95% CI)
Total No of births 135 420
Omphalocele      47 3.5 (2.5 to 4.5)
Gastroschisis      40 3.0 (2.0 to 3.9)
Both      87 6.4 (5.1 to 7.8)
1-150

Cases per 10 000 total births. 

Analysis of abdominal wall defects should be restricted to the use of regional data. National systems do not include terminations and fetal losses, which make up many of these cases, and in our experience comparisons with data from the Office for National Statistics have indicated low ascertainment levels in the National Congenital Malformation System.

We would choose to consider omphalocele and gastroschisis separately owing to their differing causes, and the classification of abdominal wall defects into either group must be reviewed. The majority of omphaloceles are lethal because of the association with major abnormalities of other systems. Gastroschisis is associated with low maternal age1-2,1-3 and social class.1-2,1-4

The West Midlands congenital anomaly register has recently completed a review of its data on congenital anterior abdominal wall defects. The register was set up in July 1994, modelled on the northern region Congenital Anomaly Survey, with an emphasis on notifications from multiple sources and prenatal diagnoses. The register runs in conjunction with the regional perinatal mortality survey, ensuring high ascertainment of anomalies resulting in termination, fetal loss, and infant death.

The prevalence rate for gastroschisis in 1995-6 in the West Midlands was higher than that reported by Stone et al. This implies either that the geographical trend does not extend to the West Midlands or that the prevalence of gastroschisis is continuing to increase.

Ascertainment levels of regional anomaly registers should be high with the availability of termination data and information from departments of paediatric surgery. Data on maternal age allow age standardised rates to be produced for gastroschisis. In this way any real differences in the north-south prevalence of abdominal wall defects could be identified.

References

  • 1-1.Stone DH, Rimaz S, Gilmour WH. Prevalence of congenital anterior abdominal wall defects in the United Kingdom: comparison of regional registers. BMJ. 1998;317:1118–1119. doi: 10.1136/bmj.317.7166.1118. . (24 October.) [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 1-2.Tan KH, Kilby MD, Whittle MJ, Beattie BR, Booth IW, Botting BJ. Congenital anterior abdominal wall defects in England and Wales 1987-93: retrospective analysis of OPCS data. BMJ. 1996;313:903–906. doi: 10.1136/bmj.313.7062.903. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 1-3.Roeper PJ, Harris J, Lee G, Neutra R. Secular rates and correlates for gastroschisis in California (1968-1977) Teratology. 1987;35:203–210. doi: 10.1002/tera.1420350206. [DOI] [PubMed] [Google Scholar]
  • 1-4.Hemminki K, Saloniemi I, Kyyronen P, Kekomaki M. Gastroschisis and omphalocele in Finland in the 1970s: prevalence at birth and its correlates. J Epidemiol Community Health. 1982;36:289–293. doi: 10.1136/jech.36.4.289. [DOI] [PMC free article] [PubMed] [Google Scholar]

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