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Arch Dis Child Fetal Neonatal Ed 90:F374-F379 doi:10.1136/adc.2003.047902
  • Original article

Prevalence of congenital anomalies in five British regions, 1991–99

Table 4

 Congenital anomaly subgroups by region, number, and prevalence per 10 000 registered births

Congenital anomaly†‡ Glasgow Northern North Thames West Wessex Oxford No. of cases resulting in a termination (%) Total
No. Rate¶ No. Rate No. Rate No. Rate No. Rate
AVSD, atrial ventricular septal defect.
*p<0.05; **p<0.01; ***p<0.001.
†Includes live births, stillbirths, fetal deaths from 20 weeks gestation, and terminations of pregnancy.
‡Some cases had more than one anomaly.
§‘Non-chromosomal’ includes the anomalies cited plus anophthalmos, microphthalmos, absence of auricle/ear, microtia, chondrodystrophy, osteodystrophies,
¶Per 10 000 registered births.
††Including five cases of submucous cleft palate.
‡‡Excluding 30 non-postcoded cleft palate cases, some of which would have resided in the study wards.
Non-chromosomal (total)§ 865 109.1 3084 99.6 1874 69.5 982 72.5 333 73.1 1811 (25.4) 7138***
Nervous system 181 22.8 639 20.6 499 18.5 260 19.2 80 17.6 1177 (70.9) 1659
Neural tube defects 136 17.2 484 15.6 332 12.3 171 12.6 54 11.9 957 (81.3) 1177***
Anencephalus and similar 55 6.9 220 7.1 153 5.7 85 6.3 27 5.9 483 (89.4) 540
Spina bifida 63 7.9 220 7.1 141 5.2 71 5.2 25 5.5 384 (73.8) 520**
Hydrocephaly 36 4.5 126 4.1 121 4.5 70 5.2 25 5.5 177 (46.8) 378
Congenital heart disease 275 34.7 1226 39.6 429 15.9 283 20.9 112 24.6 254 (10.9) 2323***
Anomalies of cardiac chambers 57 7.2 200 6.5 122 4.5 80 5.9 18 4.0 53 (11.1) 477**
Transposition of great vessels 45 5.7 122 3.9 76 2.8 43 3.2 14 3.1 18 (6.0) 300**
Malformations of cardiac septa 132 16.7 400 12.9 142 5.3 65 4.8 23 5.1 78 (10.3) 760***
AVSD 4 0.5 55 1.8 29 1.1 25 1.8 1 0.2 28 (24.8) 113**
Tetralogy of Fallot 31 3.9 96 3.1 26 1.0 20 1.5 9 2.0 12 (6.6) 181***
Malformations of valves 87 11.0 565 18.3 164 6.1 70 5.2 59 13.0 144 (15.2) 945***
Hypoplastic left heart 14 1.8 72 2.3 65 2.4 37 2.7 7 1.5 83 (42.6) 195
Malformation of great arteries and veins 133 16.8 290 9.4 145 5.4 111 8.2 50 11.0 53 (7.3) 728***
Coarctation of aorta 21 2.6 101 3.3 34 1.3 33 2.4 15 3.3 10 (4.9) 204***
Cleft lip with/without palate 77 9.7 220 7.1 237 8.8 108 8.0 36 7.9 56 (8.3) 678
Cleft palate 55†† 6.9 127 4.1 126 4.7 42‡‡ 3.1 12 2.6 21 (5.8) 361***
Digestive system 73 9.2 205 6.6 168 6.2 85 6.3 13 2.9 65 (17.8) 544**
Tracheo-oesophageal fistula, oesophageal atresia and stenosis 25 3.2 87 2.8 52 1.9 33 2.4 3 0.7 13 (6.5) 200*
Small intestine atresia 11 1.4 50 1.6 30 1.1 32 2.4 5 1.1 6 (4.7) 128*
Ano-rectal atresia and stenosis 37 4.7 68 2.2 86 3.2 20 1.5 5 1.1 46 (21.3) 216***
Internal urogenital system: ovaries, uterus, renal 131 16.5 379 12.2 642 23.8 320 23.6 38 8.3 460 (30.5) 1,507***
Bilateral renal agenesis 15 1.9 37 1.2 33 1.2 19 1.4 4 0.9 73 (67.6) 108
Cystic kidney disease 38 4.8 156 5.0 152 5.6 64 4.7 21 4.6 102 (23.8) 429
Limb reduction defects 60 7.6 127 4.1 104 3.9 46 3.4 19 4.2 59 (16.6) 355***
Miscellaneous 83 10.5 294 9.5 194 7.2 94 6.9 23 5.1 188 (27.3) 688***
Diaphragmatic hernia 33 4.2 102 3.3 64 2.4 32 2.4 7 1.5 69 (29.0) 238*
Omphalocele 23 2.9 69 2.2 78 2.9 29 2.1 8 1.8 93 (44.9) 207
Gastroschisis 25 3.2 129 4.2 54 2.0 31 2.3 8 1.8 32 (13.0) 247***
Multiples 133 16.8 332 10.7 164 6.1 158 11.7 36 7.9 308 (37.4) 823***
Chromosomal 229 28.9 1170 37.8 1045 38.7 579 42.7 178 39.1 1415 (44.2) 3201***
Down’s syndrome 143 18.0 522 16.9 584 21.6 281 20.7 109 23.9 716 (43.7) 1,639***
Trisomy 13 (Patau) 9 1.1 51 1.6 53 2.0 26 1.9 14 3.1 100 (65.4) 153
Trisomy 18 (Edwards) 29 3.7 114 3.7 135 5.0 68 5.0 25 5.5 244 (65.8) 371
Other chromosomal anomalies 50 6.3 493 15.9 273 10.1 203 15.0 30 6.6 358 (34.1) 1,049***
New dominant 16 2.0 48 1.6 34 1.3 18 1.3 6 1.3 49 (40.2) 122
Syndromes 35 4.4 103 3.3 149 5.5 85 6.3 11 2.4 96 (25.1) 383***
Total no. of registered births and termination of pregnancy 79258 309460 269796 135469 45538 3226/10844 (31.2) 839521

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