Embed Size (px)
Citation preview
Data Tables
Table 1: Prevalence of Birth Defects, Texas, 1998-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 191 2.98 2.56 - 3.40Spina bifida without anencephaly 259 4.04 3.55 - 4.53Encephalocele 62 0.97 0.74 - 1.24Microcephaly 383 5.97 5.37 - 6.57Holoprosencephaly 79 1.23 0.98 - 1.54Hydrocephaly 498 7.77 7.08 - 8.45
EYE AND EARAnophthalmia 19 0.30 0.18 - 0.46Microphthalmia 163 2.54 2.15 - 2.93Cataract 75 1.17 0.92 - 1.47Aniridia 6 0.09 0.03 - 0.20Anotia or microtia 159 2.48 2.09 - 2.86
CARDIAC AND CIRCULATORYCommon truncus 43 0.67 0.49 - 0.90Transposition of the great vessels 312 4.86 4.33 - 5.40Tetralogy of Fallot 186 2.90 2.48 - 3.32Ventricular septal defect 2483 38.72 37.19 - 40.24Atrial septal defect 2503 39.03 37.50 - 40.56Endocardial cushion defect 213 3.32 2.88 - 3.77Pulmonary valve atresia or stenosis 400 6.24 5.63 - 6.85Tricuspid valve atresia or stenosis 128 2.00 1.65 - 2.34Ebstein anomaly 43 0.67 0.49 - 0.90Aortic valve stenosis 157 2.45 2.07 - 2.83Hypoplastic left heart syndrome 124 1.93 1.59 - 2.27Patent ductus arteriosus 2748 42.85 41.25 - 44.45Coarctation of the aorta 285 4.44 3.93 - 4.96
RESPIRATORYChoanal atresia or stenosis 72 1.12 0.88 - 1.41Agenesis, aplasia, or hypoplasia of the lung 301 4.69 4.16 - 5.22
ORAL CLEFTSCleft palate alone (without cleft lip) 369 5.75 5.17 - 6.34Cleft lip with or without cleft palate 667 10.40 9.61 - 11.19
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia 132 2.06 1.71 - 2.41Pyloric stenosis 1139 17.76 16.73 - 18.79Stenosis or atresia of small intestine 193 3.01 2.58 - 3.43Stenosis or atresia of large intestine, rectum, or anal canal 303 4.72 4.19 - 5.26Hirschsprung disease 84 1.31 1.04 - 1.62Biliary atresia 37 0.58 0.41 - 0.80
GENITOURINARYHypospadias or epispadias 1726 26.91 25.64 - 28.18Renal agenesis or dysgenesis 332 5.18 4.62 - 5.73Obstructive genitourinary defect 1217 18.98 17.91 - 20.04Bladder exstrophy 9 0.14 0.06 - 0.27
MUSCULOSKELETALCongenital hip dislocation 335 5.22 4.66 - 5.78Reduction defects of the upper limbs 247 3.85 3.37 - 4.33Reduction defects of the lower limbs 127 1.98 1.64 - 2.32Craniosynostosis 226 3.52 3.06 - 3.98Diaphragmatic hernia 179 2.79 2.38 - 3.20Omphalocele 141 2.20 1.84 - 2.56Gastroschisis 245 3.82 3.34 - 4.30
CHROMOSOMALTrisomy 21 (Down syndrome) 664 10.35 9.57 - 11.14Trisomy 13 (Patau syndrome) 79 1.23 0.98 - 1.54Trisomy 18 (Edwards syndrome) 136 2.12 1.76 - 2.48
OTHERFetal alcohol syndrome or other alcohol related birth defects 16 0.25 0.14 - 0.41
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 13 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEM
Anencephaly 1996
45 3.94 2.88 - 5.28
1997
65 3.49 2.69 - 4.45
1998
75 2.57 2.02 - 3.22
1999
116 3.32 2.72 - 3.93
Spina bifida without anencephaly 1996
64 5.61 4.32 - 7.16
1997
88 4.72 3.79 - 5.82
1998
119 4.07 3.34 - 4.80
1999
140 4.01 3.35 - 4.67
Encephalocele 1996
18 1.58 0.94 - 2.49
1997
18 0.97 0.57 - 1.53
1998
31 1.06 0.72 - 1.51
1999
31 0.89 0.60 - 1.26
Microcephaly 1996
65 5.70 4.40 - 7.26
1997
112 6.01 4.90 - 7.12
1998
172 5.89 5.01 - 6.77
1999
211 6.04 5.23 - 6.86
Holoprosencephaly 1996
18 1.58 0.94 - 2.49
1997
28 1.50 1.00 - 2.17
1998
41 1.40 1.01 - 1.90
1999
38 1.09 0.77 - 1.49
Hydrocephaly 1996
76 6.66 5.25 - 8.34
1997
159 8.53 7.21 - 9.86
1998
210 7.19 6.22 - 8.16
1999
288 8.25 7.30 - 9.20
EYE AND EARAnophthalmia 199
63 0.26 0.05 - 0.77
1997
13 0.70 0.37 - 1.19
1998
9 0.31 0.14 - 0.58
1999
10 0.29 0.14 - 0.53
Microphthalmia 1996
22 1.93 1.21 - 2.92
1997
49 2.63 1.95 - 3.48
1998
78 2.67 2.11 - 3.33
1999
85 2.43 1.94 - 3.01
Cataract 1996
11 0.96 0.48 - 1.73
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 14 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
1997
21 1.13 0.70 - 1.72
1998
35 1.20 0.83 - 1.67
1999
40 1.15 0.82 - 1.56
Aniridia 1996
1 0.09 0.00 - 0.49
1997
1 0.05 0.00 - 0.30
1998
3 0.10 0.02 - 0.30
1999
3 0.09 0.02 - 0.25
Anotia or microtia 1996
32 2.80 1.92 - 3.96
1997
52 2.79 2.08 - 3.66
1998
65 2.22 1.72 - 2.84
1999
94 2.69 2.18 - 3.29
CARDIAC AND CIRCULATORYCommon truncus 199
63 0.26 0.05 - 0.77
1997
17 0.91 0.53 - 1.46
1998
26 0.89 0.58 - 1.30
1999
17 0.49 0.28 - 0.78
Transposition of the great vessels 1996
50 4.38 3.25 - 5.78
1997
96 5.15 4.17 - 6.29
1998
139 4.76 3.97 - 5.55
1999
173 4.95 4.22 - 5.69
Tetralogy of Fallot 1996
29 2.54 1.70 - 3.65
1997
63 3.38 2.60 - 4.33
1998
81 2.77 2.20 - 3.45
1999
105 3.01 2.43 - 3.58
Ventricular septal defect 1996
528 46.28 42.33 - 50.23
1997
814 43.68 40.68 - 46.68
1998
1116 38.20 35.95 - 40.44
1999
1367 39.15 37.08 - 41.23
Atrial septal defect 1996
473 41.46 37.72 - 45.20
1997
799 42.88 39.90 - 45.85
1998
1188 40.66 38.35 - 42.97
1999
1315 37.66 35.63 - 39.70
Endocardial cushion defect 1996
40 3.51 2.50 - 4.77
1997
66 3.54 2.74 - 4.51
1998
81 2.77 2.20 - 3.45
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 15 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
1999
132 3.78 3.14 - 4.43
Pulmonary valve atresia or stenosis 1996
66 5.79 4.47 - 7.36
1997
83 4.45 3.55 - 5.52
1998
189 6.47 5.55 - 7.39
1999
211 6.04 5.23 - 6.86
Tricuspid valve atresia or stenosis 1996
29 2.54 1.70 - 3.65
1997
43 2.31 1.67 - 3.11
1998
55 1.88 1.42 - 2.45
1999
73 2.09 1.64 - 2.63
Ebstein anomaly 1996
1 0.09 0.00 - 0.49
1997
12 0.64 0.33 - 1.12
1998
20 0.68 0.42 - 1.06
1999
23 0.66 0.42 - 0.99
Aortic valve stenosis 1996
25 2.19 1.42 - 3.23
1997
49 2.63 1.95 - 3.48
1998
72 2.46 1.93 - 3.10
1999
85 2.43 1.94 - 3.01
Hypoplastic left heart syndrome 1996
26 2.28 1.49 - 3.34
1997
45 2.41 1.76 - 3.23
1998
63 2.16 1.66 - 2.76
1999
61 1.75 1.34 - 2.24
Patent ductus arteriosus 1996
553 48.47 44.43 - 52.51
1997
1094 58.71 55.23 - 62.19
1998
1280 43.81 41.41 - 46.21
1999
1468 42.04 39.89 - 44.19
Coarctation of the aorta 1996
60 5.26 4.01 - 6.77
1997
90 4.83 3.88 - 5.94
1998
140 4.79 4.00 - 5.59
1999
145 4.15 3.48 - 4.83
RESPIRATORYChoanal atresia or stenosis 199
611 0.96 0.48 - 1.73
1997
27 1.45 0.95 - 2.11
1998
31 1.06 0.72 - 1.51
1999
41 1.17 0.84 - 1.59
Agenesis, aplasia, or hypoplasia of the lung 199 52 4.56 3.40 - 5.98
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 16 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
61997
90 4.83 3.88 - 5.94
1998
122 4.18 3.43 - 4.92
1999
179 5.13 4.38 - 5.88
ORAL CLEFTSCleft palate alone (without cleft lip) 199
664 5.61 4.32 - 7.16
1997
119 6.39 5.24 - 7.53
1998
163 5.58 4.72 - 6.44
1999
206 5.90 5.09 - 6.71
Cleft lip with or without cleft palate 1996
116 10.17 8.32 - 12.02
1997
234 12.56 10.95 - 14.17
1998
303 10.37 9.20 - 11.54
1999
364 10.43 9.35 - 11.50
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia 199
623 2.02 1.28 - 3.03
1997
49 2.63 1.95 - 3.48
1998
57 1.95 1.48 - 2.53
1999
75 2.15 1.69 - 2.69
Pyloric stenosis 1996
129 11.31 9.36 - 13.26
1997
380 20.39 18.34 - 22.44
1998
509 17.42 15.91 - 18.93
1999
630 18.04 16.63 - 19.45
Stenosis or atresia of small intestine 1996
40 3.51 2.50 - 4.77
1997
59 3.17 2.41 - 4.08
1998
102 3.49 2.81 - 4.17
1999
91 2.61 2.10 - 3.20
Stenosis or atresia of large intestine, rectum, or anal canal 1996
48 4.21 3.10 - 5.58
1997
87 4.67 3.74 - 5.76
1998
119 4.07 3.34 - 4.80
1999
184 5.27 4.51 - 6.03
Hirschsprung disease 1996
23 2.02 1.28 - 3.03
1997
17 0.91 0.53 - 1.46
1998
47 1.61 1.18 - 2.14
1999
37 1.06 0.75 - 1.46
Biliary atresia 1996
7 0.61 0.25 - 1.26
199 11 0.59 0.29 - 1.06
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 17 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
71998
13 0.44 0.24 - 0.76
1999
24 0.69 0.44 - 1.02
GENITOURINARYHypospadias or epispadias 199
6261 22.88 20.10 - 25.65
1997
469 25.17 22.89 - 27.45
1998
717 24.54 22.74 - 26.34
1999
1009 28.90 27.12 - 30.68
Renal agenesis or dysgenesis 1996
48 4.21 3.10 - 5.58
1997
100 5.37 4.37 - 6.53
1998
141 4.83 4.03 - 5.62
1999
191 5.47 4.69 - 6.25
Obstructive genitourinary defect 1996
178 15.60 13.31 - 17.89
1997
339 18.19 16.26 - 20.13
1998
536 18.34 16.79 - 19.90
1999
681 19.50 18.04 - 20.97
Bladder exstrophy 1996
2 0.18 0.02 - 0.63
1997
4 0.21 0.06 - 0.55
1998
6 0.21 0.08 - 0.45
1999
3 0.09 0.02 - 0.25
MUSCULOSKELETALCongenital hip dislocation 199
672 6.31 4.94 - 7.95
1997
100 5.37 4.37 - 6.53
1998
143 4.89 4.09 - 5.70
1999
192 5.50 4.72 - 6.28
Reduction defects of the upper limbs 1996
48 4.21 3.10 - 5.58
1997
82 4.40 3.50 - 5.46
1998
112 3.83 3.12 - 4.54
1999
135 3.87 3.21 - 4.52
Reduction defects of the lower limbs 1996
19 1.67 1.00 - 2.60
1997
25 1.34 0.87 - 1.98
1998
49 1.68 1.24 - 2.22
1999
78 2.23 1.77 - 2.79
Craniosynostosis 1996
31 2.72 1.85 - 3.86
1997
66 3.54 2.74 - 4.51
199 100 3.42 2.78 - 4.16
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 18 of 60
Table 2: Prevalence of Birth Defects by Year, Texas, 1996-1999
DEFECT YEAR‡ CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
81999
126 3.61 2.98 - 4.24
Diaphragmatic hernia 1996
28 2.45 1.63 - 3.55
1997
39 2.09 1.49 - 2.86
1998
68 2.33 1.81 - 2.95
1999
111 3.18 2.59 - 3.77
Omphalocele 1996
21 1.84 1.14 - 2.81
1997
48 2.58 1.90 - 3.42
1998
66 2.26 1.75 - 2.87
1999
75 2.15 1.69 - 2.69
Gastroschisis 1996
36 3.16 2.21 - 4.37
1997
71 3.81 2.98 - 4.81
1998
110 3.76 3.06 - 4.47
1999
135 3.87 3.21 - 4.52
CHROMOSOMALTrisomy 21 (Down syndrome) 199
6133 11.66 9.68 - 13.64
1997
246 13.20 11.55 - 14.85
1998
283 9.69 8.56 - 10.81
1999
381 10.91 9.82 - 12.01
Trisomy 13 (Patau syndrome) 1996
11 0.96 0.48 - 1.73
1997
21 1.13 0.70 - 1.72
1998
35 1.20 0.83 - 1.67
1999
44 1.26 0.92 - 1.69
Trisomy 18 (Edwards syndrome) 1996
36 3.16 2.21 - 4.37
1997
52 2.79 2.08 - 3.66
1998
57 1.95 1.48 - 2.53
1999
79 2.26 1.79 - 2.82
OTHERFetal alcohol syndrome or other alcohol related birth defects
1996
0 0.00 0.00 - 0.32
1997
3 0.16 0.03 - 0.47
1998
10 0.34 0.16 - 0.63
1999
6 0.17 0.06 - 0.37
‡ In 1996, the registry covered Regions 6 and 11 only. In 1997, the registry covered Regions 2, 3, 8, 9, 10, and 11. In 1998, data from three regions
are limited to half of the year (January 1-June 30, 1998, in Region 4; July 1-December 31, 1998, in Regions 5 and 6). In 1999, the registry covered
the entire state for the entire year.
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 19 of 60
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 20 of 60
Table 3: Prevalence of Birth Defects by Mother's Age, Texas, 1998-1999
DEFECTMOTHER'S
AGECASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly < 20 36 3.50 2.45 - 4.85
20-24 54 3.00 2.25 - 3.9125-29 51 2.94 2.19 - 3.8730-34 33 2.74 1.89 - 3.8535-39 13 2.40 1.28 - 4.1140 + 4 3.87 1.05 - 9.90
Spina bifida without anencephaly < 20 38 3.69 2.61 - 5.0720-24 68 3.77 2.93 - 4.7825-29 71 4.09 3.20 - 5.1630-34 48 3.99 2.94 - 5.2935-39 28 5.18 3.44 - 7.4840 + 6 5.80 2.13 - 12.63
Encephalocele < 20 9 0.88 0.40 - 1.6620-24 21 1.16 0.72 - 1.7825-29 15 0.86 0.48 - 1.4330-34 9 0.75 0.34 - 1.4235-39 8 1.48 0.64 - 2.9140 + 0 0.00 0.00 - 3.57
Microcephaly * (p=0.0007)
< 20 58 5.64 4.28 - 7.2920-24 127 7.05 5.82 - 8.2725-29 84 4.84 3.86 - 6.0030-34 60 4.99 3.81 - 6.4235-39 38 7.03 4.97 - 9.6440 + 16 15.47 8.84 - 25.12
Holoprosencephaly < 20 12 1.17 0.60 - 2.0420-24 19 1.05 0.63 - 1.6525-29 19 1.10 0.66 - 1.7130-34 14 1.16 0.64 - 1.9535-39 10 1.85 0.89 - 3.4040 + 5 4.83 1.57 - 11.28
Hydrocephaly < 20 80 7.78 6.17 - 9.6820-24 127 7.05 5.82 - 8.2725-29 131 7.55 6.26 - 8.8530-34 95 7.90 6.39 - 9.6635-39 55 10.17 7.66 - 13.2440 + 10 9.67 4.64 - 17.78
EYE AND EARAnophthalmia < 20 5 0.49 0.16 - 1.13
20-24 4 0.22 0.06 - 0.5725-29 6 0.35 0.13 - 0.7530-34 1 0.08 0.00 - 0.4635-39 3 0.55 0.11 - 1.6240 + 0 0.00 0.00 - 3.57
Microphthalmia * (p=0.0071)
< 20 33 3.21 2.21 - 4.5120-24 45 2.50 1.82 - 3.3425-29 32 1.85 1.26 - 2.6030-34 27 2.24 1.48 - 3.2735-39 17 3.14 1.83 - 5.0340 + 9 8.70 3.98 - 16.52
Cataract < 20 8 0.78 0.34 - 1.5320-24 22 1.22 0.76 - 1.8525-29 17 0.98 0.57 - 1.5730-34 18 1.50 0.89 - 2.3735-39 7 1.29 0.52 - 2.6740 + 3 2.90 0.60 - 8.48
Aniridia < 20 0 0.00 0.00 - 0.3620-24 3 0.17 0.03 - 0.4925-29 2 0.12 0.01 - 0.4230-34 1 0.08 0.00 - 0.4635-39 0 0.00 0.00 - 0.6840 + 0 0.00 0.00 - 3.57
Anotia or microtia < 20 23 2.24 1.42 - 3.3620-24 50 2.77 2.06 - 3.6625-29 43 2.48 1.79 - 3.3430-34 30 2.49 1.68 - 3.5635-39 11 2.03 1.02 - 3.64
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 21 of 60
Table 3: Prevalence of Birth Defects by Mother's Age, Texas, 1998-1999
DEFECTMOTHER'S
AGECASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
40 + 2 1.93 0.23 - 6.99CARDIAC AND CIRCULATORY
Common truncus < 20 4 0.39 0.11 - 1.0020-24 13 0.72 0.38 - 1.2325-29 11 0.63 0.32 - 1.1330-34 11 0.91 0.46 - 1.6435-39 4 0.74 0.20 - 1.8940 + 0 0.00 0.00 - 3.57
Transposition of the great vessels < 20 40 3.89 2.78 - 5.3020-24 81 4.49 3.57 - 5.5825-29 87 5.02 4.02 - 6.1930-34 66 5.49 4.24 - 6.9835-39 32 5.92 4.05 - 8.3540 + 6 5.80 2.13 - 12.63
Tetralogy of Fallot < 20 24 2.33 1.50 - 3.4720-24 55 3.05 2.30 - 3.9725-29 50 2.88 2.14 - 3.8030-34 35 2.91 2.03 - 4.0535-39 16 2.96 1.69 - 4.8040 + 6 5.80 2.13 - 12.63
Ventricular septal defect * (p<0.0001)
< 20 381 37.04 33.32 - 40.7620-24 660 36.61 33.82 - 39.4125-29 629 36.27 33.43 - 39.1030-34 462 38.41 34.91 - 41.9235-39 270 49.92 43.97 - 55.8840 + 81 78.31 62.19 - 97.34
Atrial septal defect * (p<0.0001)
< 20 408 39.67 35.82 - 43.5220-24 618 34.28 31.58 - 36.9925-29 657 37.88 34.99 - 40.7830-34 441 36.67 33.24 - 40.0935-39 290 53.62 47.45 - 59.7940 + 89 86.05 69.10 - 105.8
9Endocardial cushion defect * (p<0.0001)
< 20 30 2.92 1.97 - 4.1620-24 37 2.05 1.45 - 2.8325-29 50 2.88 2.14 - 3.8030-34 52 4.32 3.23 - 5.6735-39 32 5.92 4.05 - 8.3540 + 12 11.60 5.99 - 20.27
Pulmonary valve atresia or stenosis * (p=0.0197)
< 20 68 6.61 5.13 - 8.3820-24 108 5.99 4.86 - 7.1225-29 90 5.19 4.17 - 6.3830-34 74 6.15 4.83 - 7.7235-39 47 8.69 6.39 - 11.5640 + 13 12.57 6.69 - 21.49
Tricuspid valve atresia or stenosis < 20 23 2.24 1.42 - 3.3620-24 34 1.89 1.31 - 2.6425-29 27 1.56 1.03 - 2.2730-34 27 2.24 1.48 - 3.2735-39 10 1.85 0.89 - 3.4040 + 7 6.77 2.72 - 13.94
Ebstein anomaly < 20 6 0.58 0.21 - 1.2720-24 11 0.61 0.30 - 1.0925-29 13 0.75 0.40 - 1.2830-34 9 0.75 0.34 - 1.4235-39 2 0.37 0.04 - 1.3440 + 2 1.93 0.23 - 6.99
Aortic valve stenosis < 20 20 1.94 1.19 - 3.0020-24 38 2.11 1.49 - 2.8925-29 48 2.77 2.04 - 3.6730-34 30 2.49 1.68 - 3.5635-39 20 3.70 2.26 - 5.7140 + 1 0.97 0.02 - 5.39
Hypoplastic left heart syndrome < 20 20 1.94 1.19 - 3.0020-24 28 1.55 1.03 - 2.2425-29 34 1.96 1.36 - 2.7430-34 24 2.00 1.28 - 2.9735-39 13 2.40 1.28 - 4.11
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 22 of 60
Table 3: Prevalence of Birth Defects by Mother's Age, Texas, 1998-1999
DEFECTMOTHER'S
AGECASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
40 + 5 4.83 1.57 - 11.28Patent ductus arteriosus * (p<0.0001)
< 20 418 40.64 36.75 - 44.5420-24 685 38.00 35.15 - 40.8525-29 708 40.82 37.82 - 43.8330-34 509 42.32 38.64 - 46.0035-39 317 58.61 52.16 - 65.0640 + 111 107.32 87.35 - 127.2
8Coarctation of the aorta < 20 44 4.28 3.11 - 5.74
20-24 69 3.83 2.98 - 4.8425-29 84 4.84 3.86 - 6.0030-34 60 4.99 3.81 - 6.4235-39 24 4.44 2.84 - 6.6040 + 4 3.87 1.05 - 9.90
RESPIRATORYChoanal atresia or stenosis * (p=0.0274)
< 20 6 0.58 0.21 - 1.2720-24 21 1.16 0.72 - 1.7825-29 18 1.04 0.62 - 1.6430-34 12 1.00 0.52 - 1.7435-39 10 1.85 0.89 - 3.4040 + 5 4.83 1.57 - 11.28
Agenesis, aplasia, or hypoplasia of the lung < 20 49 4.76 3.52 - 6.3020-24 73 4.05 3.17 - 5.0925-29 98 5.65 4.59 - 6.8930-34 52 4.32 3.23 - 5.6735-39 24 4.44 2.84 - 6.6040 + 5 4.83 1.57 - 11.28
ORAL CLEFTSCleft palate alone (without cleft lip) < 20 57 5.54 4.20 - 7.18
20-24 90 4.99 4.01 - 6.1425-29 101 5.82 4.69 - 6.9630-34 74 6.15 4.83 - 7.7235-39 39 7.21 5.13 - 9.8640 + 8 7.73 3.34 - 15.24
Cleft lip with or without cleft palate * (p=0.0099)
< 20 98 9.53 7.74 - 11.6120-24 216 11.98 10.38 - 13.5825-29 164 9.46 8.01 - 10.9030-34 107 8.90 7.21 - 10.5835-39 63 11.65 8.95 - 14.9040 + 19 18.37 11.06 - 28.69
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia *(p=0.0006)
< 20 24 2.33 1.50 - 3.4720-24 27 1.50 0.99 - 2.1825-29 26 1.50 0.98 - 2.2030-34 27 2.24 1.48 - 3.2735-39 20 3.70 2.26 - 5.7140 + 8 7.73 3.34 - 15.24
Pyloric stenosis * (p=0.0001)
< 20 238 23.14 20.20 - 26.0820-24 336 18.64 16.65 - 20.6325-29 276 15.91 14.04 - 17.7930-34 196 16.30 14.02 - 18.5835-39 79 14.61 11.56 - 18.2040 + 14 13.54 7.40 - 22.71
Stenosis or atresia of small intestine < 20 37 3.60 2.53 - 4.9620-24 57 3.16 2.39 - 4.1025-29 37 2.13 1.50 - 2.9430-34 36 2.99 2.10 - 4.1435-39 21 3.88 2.40 - 5.9440 + 5 4.83 1.57 - 11.28
Stenosis or atresia of large intestine, rectum, or anal canal < 20 54 5.25 3.94 - 6.8520-24 86 4.77 3.82 - 5.8925-29 77 4.44 3.50 - 5.5530-34 51 4.24 3.16 - 5.5835-39 30 5.55 3.74 - 7.9240 + 5 4.83 1.57 - 11.28
Hirschsprung disease < 20 7 0.68 0.27 - 1.4020-24 30 1.66 1.12 - 2.3825-29 21 1.21 0.75 - 1.85
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 23 of 60
Table 3: Prevalence of Birth Defects by Mother's Age, Texas, 1998-1999
DEFECTMOTHER'S
AGECASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
30-34 14 1.16 0.64 - 1.9535-39 10 1.85 0.89 - 3.4040 + 2 1.93 0.23 - 6.99
Biliary atresia < 20 10 0.97 0.47 - 1.7920-24 11 0.61 0.30 - 1.0925-29 9 0.52 0.24 - 0.9930-34 6 0.50 0.18 - 1.0935-39 1 0.18 0.00 - 1.0340 + 0 0.00 0.00 - 3.57
GENITOURINARYHypospadias or epispadias * (p<0.0001)
< 20 245 23.82 20.84 - 26.8020-24 404 22.41 20.23 - 24.6025-29 487 28.08 25.59 - 30.5730-34 345 28.69 25.66 - 31.7135-39 200 36.98 31.85 - 42.1040 + 45 43.51 31.73 - 58.22
Renal agenesis or dysgenesis < 20 61 5.93 4.54 - 7.6220-24 88 4.88 3.92 - 6.0125-29 88 5.07 4.07 - 6.2530-34 66 5.49 4.24 - 6.9835-39 24 4.44 2.84 - 6.6040 + 5 4.83 1.57 - 11.28
Obstructive genitourinary defect * (p=0.0002)
< 20 196 19.06 16.39 - 21.7220-24 301 16.70 14.81 - 18.5825-29 310 17.87 15.88 - 19.8630-34 241 20.04 17.51 - 22.5735-39 136 25.15 20.92 - 29.3740 + 33 31.91 21.96 - 44.81
Bladder exstrophy < 20 0 0.00 0.00 - 0.3620-24 3 0.17 0.03 - 0.4925-29 3 0.17 0.04 - 0.5130-34 3 0.25 0.05 - 0.7335-39 0 0.00 0.00 - 0.6840 + 0 0.00 0.00 - 3.57
MUSCULOSKELETALCongenital hip dislocation < 20 43 4.18 3.03 - 5.63
20-24 87 4.83 3.87 - 5.9525-29 95 5.48 4.43 - 6.7030-34 66 5.49 4.24 - 6.9835-39 37 6.84 4.82 - 9.4340 + 7 6.77 2.72 - 13.94
Reduction defects of the upper limbs < 20 44 4.28 3.11 - 5.7420-24 62 3.44 2.64 - 4.4125-29 66 3.81 2.94 - 4.8430-34 43 3.58 2.59 - 4.8235-39 27 4.99 3.29 - 7.2640 + 5 4.83 1.57 - 11.28
Reduction defects of the lower limbs < 20 25 2.43 1.57 - 3.5920-24 31 1.72 1.17 - 2.4425-29 41 2.36 1.70 - 3.2130-34 21 1.75 1.08 - 2.6735-39 6 1.11 0.41 - 2.4140 + 3 2.90 0.60 - 8.48
Craniosynostosis * (p<0.0001)
< 20 28 2.72 1.81 - 3.9320-24 34 1.89 1.31 - 2.6425-29 63 3.63 2.79 - 4.6530-34 59 4.91 3.73 - 6.3335-39 35 6.47 4.51 - 9.0040 + 7 6.77 2.72 - 13.94
Diaphragmatic hernia < 20 25 2.43 1.57 - 3.5920-24 46 2.55 1.87 - 3.4025-29 57 3.29 2.49 - 4.2630-34 31 2.58 1.75 - 3.6635-39 16 2.96 1.69 - 4.8040 + 4 3.87 1.05 - 9.90
Omphalocele * (p=0.0158)
< 20 21 2.04 1.26 - 3.1220-24 34 1.89 1.31 - 2.6425-29 32 1.85 1.26 - 2.60
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 24 of 60
Table 3: Prevalence of Birth Defects by Mother's Age, Texas, 1998-1999
DEFECTMOTHER'S
AGECASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
30-34 26 2.16 1.41 - 3.1735-39 21 3.88 2.40 - 5.9440 + 7 6.77 2.72 - 13.94
Gastroschisis * (p<0.0001)
< 20 106 10.31 8.34 - 12.2720-24 90 4.99 4.01 - 6.1425-29 29 1.67 1.12 - 2.4030-34 17 1.41 0.82 - 2.2635-39 3 0.55 0.11 - 1.6240 + 0 0.00 0.00 - 3.57
CHROMOSOMALTrisomy 21 (Down syndrome) * (p<0.0001)
< 20 70 6.81 5.31 - 8.6020-24 116 6.43 5.26 - 7.6125-29 106 6.11 4.95 - 7.2830-34 117 9.73 7.97 - 11.4935-39 157 29.03 24.49 - 33.5740 + 98 94.75 76.92 - 115.4
7Trisomy 13 (Patau syndrome) * (p<0.0001)
< 20 8 0.78 0.34 - 1.5320-24 13 0.72 0.38 - 1.2325-29 16 0.92 0.53 - 1.5030-34 13 1.08 0.58 - 1.8535-39 20 3.70 2.26 - 5.7140 + 9 8.70 3.98 - 16.52
Trisomy 18 (Edwards syndrome) * (p<0.0001)
< 20 9 0.88 0.40 - 1.6620-24 18 1.00 0.59 - 1.5825-29 28 1.61 1.07 - 2.3330-34 21 1.75 1.08 - 2.6735-39 34 6.29 4.35 - 8.7840 + 26 25.14 16.42 - 36.83
OTHERFetal alcohol syndrome or other alcohol related birth defects
< 20 1 0.10 0.00 - 0.54
20-24 0 0.00 0.00 - 0.2025-29 7 0.40 0.16 - 0.8330-34 2 0.17 0.02 - 0.6035-39 5 0.92 0.30 - 2.1640 + 1 0.97 0.02 - 5.39
† cases per 10,000 live births
* statistically significant by Poisson regression (p<0.05)
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 25 of 60
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 26 of 60
Table 4: Prevalence of Birth Defects by Mother's Race/Ethnicity, Texas, 1998-1999
DEFECTMOTHER’S
RACE/ETHNICITYCASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEM
Anencephaly * (p<0.0001)
White 71 2.75 2.15 - 3.47African American 6 0.84 0.31 - 1.84
Hispanic 105 3.59 2.91 - 4.28Spina bifida without anencephaly White 91 3.53 2.84 - 4.33
African American 24 3.38 2.17 - 5.03Hispanic 138 4.72 3.94 - 5.51
Encephalocele White 21 0.81 0.50 - 1.24African American 9 1.27 0.58 - 2.41
Hispanic 27 0.92 0.61 - 1.34Microcephaly * (p<0.0001)
White 123 4.76 3.92 - 5.61African American 68 9.58 7.44 - 12.14
Hispanic 179 6.13 5.23 - 7.02Holoprosencephaly * (p=0.0059)
White 19 0.74 0.44 - 1.15African American 9 1.27 0.58 - 2.41
Hispanic 49 1.68 1.24 - 2.22Hydrocephaly * (p=0.0376)
White 198 7.67 6.60 - 8.74African American 74 10.42 8.18 - 13.08
Hispanic 214 7.33 6.34 - 8.31EYE AND EAR
Anophthalmia White 5 0.19 0.06 - 0.45African American 2 0.28 0.03 - 1.02
Hispanic 12 0.41 0.21 - 0.72Microphthalmia White 68 2.63 2.05 - 3.34
African American 12 1.69 0.87 - 2.95Hispanic 80 2.74 2.17 - 3.41
Cataract White 31 1.20 0.82 - 1.70African American 11 1.55 0.77 - 2.77
Hispanic 33 1.13 0.78 - 1.59Aniridia White 5 0.19 0.06 - 0.45
African American 0 0.00 0.00 - 0.52Hispanic 1 0.03 0.00 - 0.19
Anotia or microtia * (p<0.0001)
White 43 1.67 1.21 - 2.24African American 5 0.70 0.23 - 1.64
Hispanic 107 3.66 2.97 - 4.36CARDIAC AND CIRCULATORY
Common truncus White 15 0.58 0.33 - 0.96African American 3 0.42 0.09 - 1.23
Hispanic 22 0.75 0.47 - 1.14Transposition of the great vessels White 142 5.50 4.60 - 6.41
African American 29 4.08 2.73 - 5.86Hispanic 135 4.62 3.84 - 5.40
Tetralogy of Fallot White 66 2.56 1.98 - 3.25African American 18 2.53 1.50 - 4.01
Hispanic 95 3.25 2.63 - 3.98Ventricular septal defect * (p<0.0001)
White 896 34.71 32.44 - 36.98African American 217 30.56 26.49 - 34.62
Hispanic 1319 45.15 42.71 - 47.59Atrial septal defect White 1014 39.28 36.86 - 41.70
African American 280 39.43 34.81 - 44.04Hispanic 1134 38.82 36.56 - 41.08
Endocardial cushion defect * (p=0.0364)
White 86 3.33 2.66 - 4.11African American 35 4.93 3.43 - 6.85
Hispanic 84 2.88 2.29 - 3.56Pulmonary valve atresia or stenosis White 153 5.93 4.99 - 6.87
African American 49 6.90 5.10 - 9.12Hispanic 183 6.26 5.36 - 7.17
Tricuspid valve atresia or stenosis White 44 1.70 1.24 - 2.29African American 22 3.10 1.94 - 4.69
Hispanic 59 2.02 1.54 - 2.61Ebstein anomaly * (p=0.0246)
White 13 0.50 0.27 - 0.86African American 1 0.14 0.00 - 0.78
Hispanic 26 0.89 0.58 - 1.30Aortic valve stenosis * (p=0.0073)
White 74 2.87 2.25 - 3.60African American 7 0.99 0.40 - 2.03
Hispanic 75 2.57 2.02 - 3.22Hypoplastic left heart syndrome White 57 2.21 1.67 - 2.86
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 27 of 60
Table 4: Prevalence of Birth Defects by Mother's Race/Ethnicity, Texas, 1998-1999
DEFECTMOTHER’S
RACE/ETHNICITYCASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
African American 13 1.83 0.97 - 3.13Hispanic 49 1.68 1.24 - 2.22
Patent ductus arteriosus * (p<0.0001)
White 997 38.62 36.23 - 41.02African American 291 40.98 36.27 - 45.68
Hispanic 1380 47.24 44.75 - 49.73Coarctation of the aorta * (p=0.0476)
White 134 5.19 4.31 - 6.07African American 23 3.24 2.05 - 4.86
Hispanic 122 4.18 3.44 - 4.92RESPIRATORY
Choanal atresia or stenosis White 30 1.16 0.78 - 1.66African American 7 0.99 0.40 - 2.03
Hispanic 33 1.13 0.78 - 1.59Agenesis, aplasia, or hypoplasia of the lung * (p=0.0448)
White 104 4.03 3.25 - 4.80African American 30 4.22 2.85 - 6.03
Hispanic 159 5.44 4.60 - 6.29ORAL CLEFTS
Cleft palate alone (without cleft lip) * (p=0.0245)
White 170 6.59 5.60 - 7.58African American 29 4.08 2.73 - 5.86
Hispanic 158 5.41 4.57 - 6.25Cleft lip with or without cleft palate White 266 10.30 9.07 - 11.54
African American 57 8.03 6.08 - 10.40Hispanic 322 11.02 9.82 - 12.23
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia White 62 2.40 1.84 - 3.08
African American 13 1.83 0.97 - 3.13Hispanic 53 1.81 1.36 - 2.37
Pyloric stenosis * (p<0.0001)
White 505 19.56 17.86 - 21.27African American 44 6.20 4.50 - 8.32
Hispanic 579 19.82 18.20 - 21.43Stenosis or atresia of small intestine White 73 2.83 2.22 - 3.56
African American 28 3.94 2.62 - 5.70Hispanic 87 2.98 2.39 - 3.67
Stenosis or atresia of large intestine, rectum, or anal canal
White 111 4.30 3.50 - 5.10African American 45 6.34 4.62 - 8.48
Hispanic 140 4.79 4.00 - 5.59Hirschsprung disease * (p<0.0001)
White 45 1.74 1.27 - 2.33African American 18 2.53 1.50 - 4.01
Hispanic 20 0.68 0.42 - 1.06Biliary atresia * (p=0.0002)
White 5 0.19 0.06 - 0.45African American 11 1.55 0.77 - 2.77
Hispanic 18 0.62 0.37 - 0.97GENITOURINARY
Hypospadias or epispadias * (p<0.0001)
White 928 35.95 33.64 - 38.26African American 233 32.81 28.60 - 37.02
Hispanic 521 17.83 16.30 - 19.37Renal agenesis or dysgenesis White 134 5.19 4.31 - 6.07
African American 45 6.34 4.62 - 8.48Hispanic 148 5.07 4.25 - 5.88
Obstructive genitourinary defect White 511 19.80 18.08 - 21.51African American 132 18.59 15.42 - 21.76
Hispanic 545 18.66 17.09 - 20.22Bladder exstrophy * (p=0.0096)
White 8 0.31 0.13 - 0.61African American 0 0.00 0.00 - 0.52
Hispanic 1 0.03 0.00 - 0.19MUSCULOSKELETAL
Congenital hip dislocation White 149 5.77 4.85 - 6.70African American 25 3.52 2.28 - 5.20
Hispanic 156 5.34 4.50 - 6.18Reduction defects of the upper limbs White 87 3.37 2.70 - 4.16
African American 23 3.24 2.05 - 4.86Hispanic 130 4.45 3.68 - 5.21
Reduction defects of the lower limbs White 44 1.70 1.24 - 2.29African American 21 2.96 1.83 - 4.52
Hispanic 59 2.02 1.54 - 2.61Craniosynostosis * (p<0.0001)
White 132 5.11 4.24 - 5.99African American 6 0.84 0.31 - 1.84
Hispanic 84 2.88 2.29 - 3.56
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 28 of 60
Table 4: Prevalence of Birth Defects by Mother's Race/Ethnicity, Texas, 1998-1999
DEFECTMOTHER’S
RACE/ETHNICITYCASES RATE†
95% CONFIDENCE INTERVAL FOR RATE
Diaphragmatic hernia White 68 2.63 2.05 - 3.34African American 17 2.39 1.39 - 3.83
Hispanic 89 3.05 2.45 - 3.75Omphalocele White 59 2.29 1.74 - 2.95
African American 22 3.10 1.94 - 4.69Hispanic 55 1.88 1.42 - 2.45
Gastroschisis * (p=0.0076)
White 98 3.80 3.08 - 4.63African American 14 1.97 1.08 - 3.31
Hispanic 127 4.35 3.59 - 5.10CHROMOSOMAL
Trisomy 21 (Down syndrome) * (p=0.0029)
White 260 10.07 8.85 - 11.30African American 49 6.90 5.10 - 9.12
Hispanic 329 11.26 10.04 - 12.48Trisomy 13 (Patau syndrome) White 27 1.05 0.69 - 1.52
African American 11 1.55 0.77 - 2.77Hispanic 35 1.20 0.83 - 1.67
Trisomy 18 (Edwards syndrome) White 61 2.36 1.81 - 3.04African American 12 1.69 0.87 - 2.95
Hispanic 51 1.75 1.30 - 2.30OTHER
Fetal alcohol syndrome or other alcohol related birth defects
White 6 0.23 0.09 - 0.51
African American 4 0.56 0.15 - 1.44Hispanic 6 0.21 0.08 - 0.45
† cases per 10,000 live births* statistically significant by Poisson regression (p<0.05)
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 29 of 60
Table 5: Prevalence of Birth Defects by Sex of Infant or Fetus, Texas, 1998-1999
DEFECT SEX CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEM
Anencephaly Male 76 2.32 1.83 - 2.90
Female 95 3.03 2.45 - 3.70Spina bifida without anencephaly Male 130 3.97 3.28 - 4.65
Female 123 3.92 3.23 - 4.61Encephalocele Male 29 0.88 0.59 - 1.27
Female 29 0.92 0.62 - 1.33Microcephaly * (p<0.001)
Male 155 4.73 3.99 - 5.47Female 228 7.27 6.33 - 8.21
Holoprosencephaly Male 36 1.10 0.77 - 1.52Female 43 1.37 0.99 - 1.85
Hydrocephaly * (p=0.0148)
Male 280 8.54 7.54 - 9.54Female 215 6.86 5.94 - 7.77
EYE AND EARAnophthalmia Male 8 0.24 0.11 - 0.48
Female 11 0.35 0.18 - 0.63Microphthalmia Male 87 2.65 2.13 - 3.27
Female 76 2.42 1.91 - 3.03Cataract Male 33 1.01 0.69 - 1.41
Female 42 1.34 0.97 - 1.81Aniridia Male 5 0.15 0.05 - 0.36
Female 1 0.03 0.00 - 0.18Anotia or microtia Male 79 2.41 1.91 - 3.00
Female 80 2.55 2.02 - 3.17CARDIAC AND CIRCULATORY
Common truncus Male 25 0.76 0.49 - 1.13Female 18 0.57 0.34 - 0.91
Transposition of the great vessels * (p=0.0001)
Male 193 5.89 5.06 - 6.72Female 119 3.79 3.11 - 4.48
Tetralogy of Fallot Male 97 2.96 2.40 - 3.61Female 89 2.84 2.28 - 3.49
Ventricular septal defect * (p<0.001)
Male 1168 35.64 33.60 - 37.69Female 1315 41.93 39.66 - 44.20
Atrial septal defect Male 1298 39.61 37.45 - 41.76Female 1205 38.42 36.25 - 40.59
Endocardial cushion defect Male 101 3.08 2.48 - 3.68Female 112 3.57 2.91 - 4.23
Pulmonary valve atresia or stenosis Male 194 5.92 5.09 - 6.75Female 206 6.57 5.67 - 7.47
Tricuspid valve atresia or stenosis Male 74 2.26 1.77 - 2.83Female 54 1.72 1.29 - 2.25
Ebstein anomaly Male 22 0.67 0.42 - 1.02Female 21 0.67 0.41 - 1.02
Aortic valve stenosis Male 89 2.72 2.18 - 3.34Female 68 2.17 1.68 - 2.75
Hypoplastic left heart syndrome Male 73 2.23 1.75 - 2.80Female 50 1.59 1.18 - 2.10
Patent ductus arteriosus * (p=0.0380)
Male 1458 44.49 42.21 - 46.77Female 1289 41.10 38.86 - 43.34
Coarctation of the aorta * (p=0.0155)
Male 166 5.07 4.29 - 5.84Female 119 3.79 3.11 - 4.48
RESPIRATORYChoanal atresia or stenosis Male 35 1.07 0.74 - 1.49
Female 37 1.18 0.83 - 1.63Agenesis, aplasia, or hypoplasia of the lung Male 160 4.88 4.13 - 5.64
Female 139 4.43 3.70 - 5.17
ORAL CLEFTS -Cleft palate alone (without cleft lip) * (p=0.0007)
Male 156 4.76 4.01 - 5.51Female 213 6.79 5.88 - 7.70
Cleft lip with or without cleft palate * (p=0.0006)
Male 384 11.72 10.55 - 12.89Female 281 8.96 7.91 - 10.01
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia Male 69 2.11 1.64 - 2.66
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 30 of 60
Table 5: Prevalence of Birth Defects by Sex of Infant or Fetus, Texas, 1998-1999
DEFECT SEX CASES RATE†95% CONFIDENCE
INTERVAL FOR RATEFemale 63 2.01 1.54 - 2.57
Pyloric stenosis * (p<0.0001)
Male 926 28.26 26.44 - 30.08Female 213 6.79 5.88 - 7.70
Stenosis or atresia of small intestine Male 90 2.75 2.21 - 3.38Female 103 3.28 2.65 - 3.92
Stenosis or atresia of large intestine, rectum, or anal canal * (p=0.0013)
Male 181 5.52 4.72 - 6.33Female 119 3.79 3.11 - 4.48
Hirschsprung disease * (p=0.0008)
Male 58 1.77 1.34 - 2.29Female 26 0.83 0.54 - 1.21
Biliary atresia Male 17 0.52 0.30 - 0.83Female 20 0.64 0.39 - 0.98
GENITOURINARYHypospadias or epispadias * (p<0.0001)
Male 1722 52.55 50.06 - 55.03Female 0 0.00 0.00 - 0.12
Renal agenesis or dysgenesis * (p<0.0001)
Male 214 6.53 5.66 - 7.41Female 113 3.60 2.94 - 4.27
Obstructive genitourinary defect * (p<0.0001)
Male 869 26.52 24.75 - 28.28Female 345 11.00 9.84 - 12.16
Bladder exstrophy Male 5 0.15 0.05 - 0.36Female 4 0.13 0.03 - 0.33
MUSCULOSKELETALCongenital hip dislocation * (p<0.0001)
Male 87 2.65 2.13 - 3.27Female 248 7.91 6.92 - 8.89
Reduction defects of the upper limbs Male 120 3.66 3.01 - 4.32Female 127 4.05 3.35 - 4.75
Reduction defects of the lower limbs Male 70 2.14 1.67 - 2.70Female 56 1.79 1.35 - 2.32
Craniosynostosis * (p<0.0001)
Male 146 4.46 3.73 - 5.18Female 80 2.55 2.02 - 3.17
Diaphragmatic hernia * (p=0.0292)
Male 106 3.23 2.62 - 3.85Female 73 2.33 1.82 - 2.93
Omphalocele * (p=0.0282)
Male 80 2.44 1.94 - 3.04Female 52 1.66 1.24 - 2.17
Gastroschisis Male 123 3.75 3.09 - 4.42Female 119 3.79 3.11 - 4.48
CHROMOSOMALTrisomy 21 (Down syndrome) Male 359 10.95 9.82 - 12.09
Female 298 9.50 8.42 - 10.58Trisomy 13 (Patau syndrome) Male 35 1.07 0.74 - 1.49
Female 44 1.40 1.02 - 1.88Trisomy 18 (Edwards syndrome) Male 60 1.83 1.40 - 2.36
Female 72 2.30 1.80 - 2.89OTHER
Fetal alcohol syndrome or other alcohol related birth defects
Male 9 0.27 0.13 - 0.52
Female 7 0.22 0.09 0.46
† cases per 10,000 live births * statistically significant by Poisson regression (p<0.05)
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 31 of 60
Table 6: Prevalence of Birth Defects, Texas Region 1, 1998-1999
DEFECTCASE
SRATE†
95% CONFIDENCE INTERVAL FOR
RATE
CENTRAL NERVOUS SYSTEMAnencephaly 4 1.64 0.45 - 4.21Spina bifida without anencephaly 11 4.52 2.26 - 8.09Encephalocele 3 1.23 0.25 - 3.60Microcephaly 18 7.40 4.39 - 11.7
0Holoprosencephaly 6 2.47 0.91 - 5.37Hydrocephaly 13 5.34 2.85 - 9.14
EYE AND EARAnophthalmia 0 0.00 0.00 - 1.52Microphthalmia 6 2.47 0.91 - 5.37Cataract 3 1.23 0.25 - 3.60Aniridia 1 0.41 0.01 - 2.29Anotia or microtia 6 2.47 0.91 - 5.37
CARDIAC AND CIRCULATORYCommon truncus 2 0.82 0.10 - 2.97Transposition of the great vessels 18 7.40 4.39 - 11.7
0Tetralogy of Fallot 5 2.06 0.67 - 4.80Ventricular septal defect 80 32.89 26.08 - 40.9
3Atrial septal defect 70 28.78 22.43 - 36.3
6Endocardial cushion defect 10 4.11 1.97 - 7.56Pulmonary valve atresia or stenosis 16 6.58 3.76 - 10.6
8Tricuspid valve atresia or stenosis 3 1.23 0.25 - 3.60Ebstein anomaly 1 0.41 0.01 - 2.29Aortic valve stenosis 5 2.06 0.67 - 4.80Hypoplastic left heart syndrome 9 3.70 1.69 - 7.02Patent ductus arteriosus 118 48.51 39.76 - 57.2
6Coarctation of the aorta 9 3.70 1.69 - 7.02
RESPIRATORYChoanal atresia or stenosis 2 0.82 0.10 - 2.97Agenesis, aplasia, or hypoplasia of the lung 6 2.47 0.91 - 5.37
ORAL CLEFTSCleft palate alone (without cleft lip) 18 7.40 4.39 - 11.7
0Cleft lip with or without cleft palate 38 15.62 11.06 - 21.4
4GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 8 3.29 1.42 - 6.48Pyloric stenosis 58 23.84 18.11 - 30.8
2Stenosis or atresia of small intestine 7 2.88 1.16 - 5.93Stenosis or atresia of large intestine, rectum, or anal canal
20 8.22 5.02 - 12.70
Hirschsprung disease 7 2.88 1.16 - 5.93Biliary atresia 0 0.00 0.00 - 1.52
GENITOURINARYHypospadias or epispadias 62 25.49 19.54 - 32.6
8Renal agenesis or dysgenesis 15 6.17 3.45 - 10.1
7Obstructive genitourinary defect 45 18.50 13.49 - 24.7
5Bladder exstrophy 0 0.00 0.00 - 1.52
MUSCULOSKELETALCongenital hip dislocation 10 4.11 1.97 - 7.56Reduction defects of the upper limbs 11 4.52 2.26 - 8.09Reduction defects of the lower limbs 4 1.64 0.45 - 4.21Craniosynostosis 12 4.93 2.55 - 8.62Diaphragmatic hernia 9 3.70 1.69 - 7.02Omphalocele 4 1.64 0.45 - 4.21Gastroschisis 11 4.52 2.26 - 8.09
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 32 of 60
Table 6: Prevalence of Birth Defects, Texas Region 1, 1998-1999
DEFECTCASE
SRATE†
95% CONFIDENCE INTERVAL FOR
RATE
CHROMOSOMALTrisomy 21 (Down syndrome) 29 11.92 7.98 - 17.1
2Trisomy 13 (Patau syndrome) 2 0.82 0.10 - 2.97Trisomy 18 (Edwards syndrome) 3 1.23 0.25 - 3.60
OTHERFetal alcohol syndrome or other alcohol related birth defects
1 0.41 0.01 - 2.29
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 33 of 60
Table 7: Prevalence of Birth Defects, Texas Region 2, 1998-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 10 6.72 3.22 - 12.3
5Spina bifida without anencephaly 7 4.70 1.89 - 9.69Encephalocele 1 0.67 0.02 - 3.74Microcephaly 14 9.40 5.14 - 15.7
8Holoprosencephaly 3 2.02 0.42 - 5.89Hydrocephaly 7 4.70 1.89 - 9.69
EYE AND EARAnophthalmia 1 0.67 0.02 - 3.74Microphthalmia 5 3.36 1.09 - 7.84Cataract 4 2.69 0.73 - 6.88Aniridia 0 0.00 0.00 - 2.48Anotia or microtia 9 6.05 2.76 - 11.4
8CARDIAC AND CIRCULATORY
Common truncus 0 0.00 0.00 - 2.48Transposition of the great vessels 9 6.05 2.76 - 11.4
8Tetralogy of Fallot 8 5.37 2.32 - 10.5
9Ventricular septal defect 55 36.94 27.83 - 48.0
9Atrial septal defect 70 47.02 36.65 - 59.4
0Endocardial cushion defect 5 3.36 1.09 - 7.84Pulmonary valve atresia or stenosis 10 6.72 3.22 - 12.3
5Tricuspid valve atresia or stenosis 2 1.34 0.16 - 4.85Ebstein anomaly 1 0.67 0.02 - 3.74Aortic valve stenosis 2 1.34 0.16 - 4.85Hypoplastic left heart syndrome 6 4.03 1.48 - 8.77Patent ductus arteriosus 45 30.23 22.05 - 40.4
4Coarctation of the aorta 6 4.03 1.48 - 8.77
RESPIRATORYChoanal atresia or stenosis 2 1.34 0.16 - 4.85Agenesis, aplasia, or hypoplasia of the lung 5 3.36 1.09 - 7.84
ORAL CLEFTSCleft palate alone (without cleft lip) 11 7.39 3.69 - 13.2
2Cleft lip with or without cleft palate 20 13.43 8.21 - 20.7
5GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 4 2.69 0.73 - 6.88Pyloric stenosis 30 20.15 13.60 - 28.7
7Stenosis or atresia of small intestine 5 3.36 1.09 - 7.84Stenosis or atresia of large intestine, rectum, or anal canal
13 8.73 4.65 - 14.93
Hirschsprung disease 0 0.00 0.00 - 2.48Biliary atresia 0 0.00 0.00 - 2.48
GENITOURINARYHypospadias or epispadias 30 20.15 13.60 - 28.7
7Renal agenesis or dysgenesis 5 3.36 1.09 - 7.84Obstructive genitourinary defect 29 19.48 13.05 - 27.9
7Bladder exstrophy 2 1.34 0.16 - 4.85
MUSCULOSKELETALCongenital hip dislocation 10 6.72 3.22 - 12.3
5Reduction defects of the upper limbs 6 4.03 1.48 - 8.77Reduction defects of the lower limbs 1 0.67 0.02 - 3.74Craniosynostosis 4 2.69 0.73 - 6.88
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 34 of 60
Table 7: Prevalence of Birth Defects, Texas Region 2, 1998-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
Diaphragmatic hernia 7 4.70 1.89 - 9.69Omphalocele 6 4.03 1.48 - 8.77Gastroschisis 8 5.37 2.32 - 10.5
9CHROMOSOMAL
Trisomy 21 (Down syndrome) 15 10.08 5.64 - 16.62
Trisomy 13 (Patau syndrome) 2 1.34 0.16 - 4.85Trisomy 18 (Edwards syndrome) 2 1.34 0.16 - 4.85
OTHERFetal alcohol syndrome or other alcohol related birth defects
1 0.67 0.02 - 3.74
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 35 of 60
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 36 of 60
Table 8: Prevalence of Birth Defects, Texas Region 3, 1998-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 46 2.55 1.87 - 3.40Spina bifida without anencephaly 78 4.32 3.42 - 5.40Encephalocele 24 1.33 0.85 - 1.98Microcephaly 131 7.26 6.02 - 8.51Holoprosencephaly 32 1.77 1.21 - 2.50Hydrocephaly 156 8.65 7.29 - 10.0
0EYE AND EAR
Anophthalmia 6 0.33 0.12 - 0.72Microphthalmia 52 2.88 2.15 - 3.78Cataract 26 1.44 0.94 - 2.11Aniridia 2 0.11 0.01 - 0.40Anotia or microtia 42 2.33 1.68 - 3.15
CARDIAC AND CIRCULATORYCommon truncus 15 0.83 0.47 - 1.37Transposition of the great vessels 101 5.60 4.51 - 6.69Tetralogy of Fallot 46 2.55 1.87 - 3.40Ventricular septal defect 687 38.08 35.23 - 40.9
3Atrial septal defect 937 51.94 48.61 - 55.2
7Endocardial cushion defect 74 4.10 3.22 - 5.15Pulmonary valve atresia or stenosis 122 6.76 5.56 - 7.96Tricuspid valve atresia or stenosis 39 2.16 1.54 - 2.96Ebstein anomaly 9 0.50 0.23 - 0.95Aortic valve stenosis 55 3.05 2.30 - 3.97Hypoplastic left heart syndrome 26 1.44 0.94 - 2.11Patent ductus arteriosus 808 44.79 41.70 - 47.8
8Coarctation of the aorta 91 5.04 4.06 - 6.19
RESPIRATORYChoanal atresia or stenosis 23 1.27 0.81 - 1.91Agenesis, aplasia, or hypoplasia of the lung 92 5.10 4.11 - 6.25
ORAL CLEFTSCleft palate alone (without cleft lip) 121 6.71 5.51 - 7.90Cleft lip with or without cleft palate 203 11.25 9.70 - 12.8
0GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 44 2.44 1.77 - 3.27Pyloric stenosis 250 13.86 12.14 - 15.5
8Stenosis or atresia of small intestine 58 3.22 2.44 - 4.16Stenosis or atresia of large intestine, rectum, or anal canal
74 4.10 3.22 - 5.15
Hirschsprung disease 27 1.50 0.99 - 2.18Biliary atresia 9 0.50 0.23 - 0.95
GENITOURINARYHypospadias or epispadias 571 31.65 29.06 - 34.2
5Renal agenesis or dysgenesis 107 5.93 4.81 - 7.06Obstructive genitourinary defect 423 23.45 21.21 - 25.6
8Bladder exstrophy 2 0.11 0.01 - 0.40
MUSCULOSKELETALCongenital hip dislocation 111 6.15 5.01 - 7.30Reduction defects of the upper limbs 87 4.82 3.86 - 5.95Reduction defects of the lower limbs 39 2.16 1.54 - 2.96Craniosynostosis 83 4.60 3.66 - 5.70Diaphragmatic hernia 47 2.61 1.91 - 3.46Omphalocele 43 2.38 1.73 - 3.21Gastroschisis 63 3.49 2.68 - 4.47
CHROMOSOMALTrisomy 21 (Down syndrome) 228 12.64 11.00 - 14.2
8Trisomy 13 (Patau syndrome) 35 1.94 1.35 - 2.70Trisomy 18 (Edwards syndrome) 51 2.83 2.10 - 3.72
OTHERFetal alcohol syndrome or other alcohol related birth defects
3 0.17 0.03 - 0.49
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 37 of 60
† cases per 10,000 live births ‡ 1998 data are limited to
January 1-June 30 in Region 4
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 38 of 60
Table 9: Prevalence of Birth Defects, Texas Region 4, 1998‡-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 10 4.93 2.36 - 9.06Spina bifida without anencephaly 8 3.94 1.70 - 7.77Encephalocele 5 2.46 0.80 - 5.75Microcephaly 5 2.46 0.80 - 5.75Holoprosencephaly 0 0.00 0.00 - 1.82Hydrocephaly 13 6.40 3.41 - 10.95
EYE AND EARAnophthalmia 0 0.00 0.00 - 1.82Microphthalmia 4 1.97 0.54 - 5.05Cataract 0 0.00 0.00 - 1.82Aniridia 0 0.00 0.00 - 1.82Anotia or microtia 1 0.49 0.01 - 2.75
CARDIAC AND CIRCULATORYCommon truncus 0 0.00 0.00 - 1.82Transposition of the great vessels 7 3.45 1.39 - 7.11Tetralogy of Fallot 7 3.45 1.39 - 7.11Ventricular septal defect 38 18.72 13.25 - 25.70Atrial septal defect 72 35.47 27.76 - 44.67Endocardial cushion defect 2 0.99 0.12 - 3.56Pulmonary valve atresia or stenosis 5 2.46 0.80 - 5.75Tricuspid valve atresia or stenosis 4 1.97 0.54 - 5.05Ebstein anomaly 0 0.00 0.00 - 1.82Aortic valve stenosis 3 1.48 0.30 - 4.32Hypoplastic left heart syndrome 4 1.97 0.54 - 5.05Patent ductus arteriosus 39 19.21 13.66 - 26.27Coarctation of the aorta 4 1.97 0.54 - 5.05
RESPIRATORYChoanal atresia or stenosis 1 0.49 0.01 - 2.75Agenesis, aplasia, or hypoplasia of the lung 6 2.96 1.08 - 6.43
ORAL CLEFTSCleft palate alone (without cleft lip) 13 6.40 3.41 - 10.95Cleft lip with or without cleft palate 18 8.87 5.26 - 14.02
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia 3 1.48 0.30 - 4.32Pyloric stenosis 29 14.29 9.57 - 20.52Stenosis or atresia of small intestine 4 1.97 0.54 - 5.05Stenosis or atresia of large intestine, rectum, or anal canal
10 4.93 2.36 - 9.06
Hirschsprung disease 1 0.49 0.01 - 2.75Biliary atresia 0 0.00 0.00 - 1.82
GENITOURINARYHypospadias or epispadias 62 30.55 23.42 - 39.16Renal agenesis or dysgenesis 3 1.48 0.30 - 4.32Obstructive genitourinary defect 33 16.26 11.19 - 22.83Bladder exstrophy 0 0.00 0.00 - 1.82
MUSCULOSKELETALCongenital hip dislocation 5 2.46 0.80 - 5.75Reduction defects of the upper limbs 5 2.46 0.80 - 5.75Reduction defects of the lower limbs 3 1.48 0.30 - 4.32Craniosynostosis 4 1.97 0.54 - 5.05Diaphragmatic hernia 6 2.96 1.08 - 6.43Omphalocele 3 1.48 0.30 - 4.32Gastroschisis 7 3.45 1.39 - 7.11
CHROMOSOMALTrisomy 21 (Down syndrome) 10 4.93 2.36 - 9.06Trisomy 13 (Patau syndrome) 1 0.49 0.01 - 2.75Trisomy 18 (Edwards syndrome) 4 1.97 0.54 - 5.05
OTHERFetal alcohol syndrome or other alcohol related birth defects
0 0.00 0.00 - 1.82
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 39 of 60
Table 10: Prevalence of Birth Defects, Texas Region 5, 1998‡-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 1 0.66 0.02 - 3.66Spina bifida without anencephaly 8 5.26 2.27 - 10.3
6Encephalocele 0 0.00 0.00 - 2.42Microcephaly 11 7.23 3.61 - 12.9
3Holoprosencephaly 3 1.97 0.41 - 5.76Hydrocephaly 11 7.23 3.61 - 12.9
3EYE AND EAR
Anophthalmia 3 1.97 0.41 - 5.76Microphthalmia 1 0.66 0.02 - 3.66Cataract 2 1.31 0.16 - 4.75Aniridia 0 0.00 0.00 - 2.42Anotia or microtia 3 1.97 0.41 - 5.76
CARDIAC AND CIRCULATORYCommon truncus 0 0.00 0.00 - 2.42Transposition of the great vessels 3 1.97 0.41 - 5.76Tetralogy of Fallot 2 1.31 0.16 - 4.75Ventricular septal defect 42 27.60 19.89 - 37.3
1Atrial septal defect 15 9.86 5.52 - 16.2
6Endocardial cushion defect 3 1.97 0.41 - 5.76Pulmonary valve atresia or stenosis 4 2.63 0.72 - 6.73Tricuspid valve atresia or stenosis 2 1.31 0.16 - 4.75Ebstein anomaly 0 0.00 0.00 - 2.42Aortic valve stenosis 1 0.66 0.02 - 3.66Hypoplastic left heart syndrome 1 0.66 0.02 - 3.66Patent ductus arteriosus 46 30.23 22.13 - 40.3
2Coarctation of the aorta 5 3.29 1.07 - 7.67
RESPIRATORYChoanal atresia or stenosis 2 1.31 0.16 - 4.75Agenesis, aplasia, or hypoplasia of the lung 9 5.91 2.70 - 11.2
3ORAL CLEFTS
Cleft palate alone (without cleft lip) 6 3.94 1.45 - 8.58Cleft lip with or without cleft palate 16 10.51 6.01 - 17.0
7GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 2 1.31 0.16 - 4.75Pyloric stenosis 34 22.34 15.47 - 31.2
2Stenosis or atresia of small intestine 7 4.60 1.85 - 9.48Stenosis or atresia of large intestine, rectum, or anal canal
11 7.23 3.61 - 12.93
Hirschsprung disease 3 1.97 0.41 - 5.76Biliary atresia 1 0.66 0.02 - 3.66
GENITOURINARYHypospadias or epispadias 31 20.37 13.84 - 28.9
2Renal agenesis or dysgenesis 10 6.57 3.15 - 12.0
9Obstructive genitourinary defect 28 18.40 12.23 - 26.5
9Bladder exstrophy 1 0.66 0.02 - 3.66
MUSCULOSKELETALCongenital hip dislocation 5 3.29 1.07 - 7.67Reduction defects of the upper limbs 3 1.97 0.41 - 5.76Reduction defects of the lower limbs 5 3.29 1.07 - 7.67Craniosynostosis 3 1.97 0.41 - 5.76Diaphragmatic hernia 3 1.97 0.41 - 5.76Omphalocele 2 1.31 0.16 - 4.75Gastroschisis 9 5.91 2.70 - 11.2
3CHROMOSOMAL
Trisomy 21 (Down syndrome) 9 5.91 2.70 - 11.23
Trisomy 13 (Patau syndrome) 3 1.97 0.41 - 5.76Trisomy 18 (Edwards syndrome) 2 1.31 0.16 - 4.75
OTHERFetal alcohol syndrome or other alcohol related birth defects
0 0.00 0.00 - 2.42
‡ 1998 data are limited to July 1-December 31 in Region 5
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 40 of 60
Table 11: Prevalence of Birth Defects, Texas Region 6, 1998‡-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 30 2.41 1.63 - 3.44Spina bifida without anencephaly 32 2.57 1.76 - 3.63Encephalocele 6 0.48 0.18 - 1.05Microcephaly 79 6.35 5.03 - 7.92Holoprosencephaly 5 0.40 0.13 - 0.94Hydrocephaly 76 6.11 4.82 - 7.65
EYE AND EARAnophthalmia 2 0.16 0.02 - 0.58Microphthalmia 15 1.21 0.68 - 1.99Cataract 13 1.05 0.56 - 1.79Aniridia 1 0.08 0.00 - 0.45Anotia or microtia 20 1.61 0.98 - 2.48
CARDIAC AND CIRCULATORYCommon truncus 10 0.80 0.39 - 1.48Transposition of the great vessels 62 4.99 3.82 - 6.39Tetralogy of Fallot 40 3.22 2.30 - 4.38Ventricular septal defect 399 32.09 28.94 - 35.2
4Atrial septal defect 195 15.68 13.48 - 17.8
9Endocardial cushion defect 38 3.06 2.16 - 4.20Pulmonary valve atresia or stenosis 69 5.55 4.32 - 7.02Tricuspid valve atresia or stenosis 26 2.09 1.37 - 3.06Ebstein anomaly 6 0.48 0.18 - 1.05Aortic valve stenosis 28 2.25 1.50 - 3.26Hypoplastic left heart syndrome 20 1.61 0.98 - 2.48Patent ductus arteriosus 333 26.79 23.91 - 29.6
6Coarctation of the aorta 40 3.22 2.30 - 4.38
RESPIRATORYChoanal atresia or stenosis 10 0.80 0.39 - 1.48Agenesis, aplasia, or hypoplasia of the lung 63 5.07 3.89 - 6.48
ORAL CLEFTSCleft palate alone (without cleft lip) 67 5.39 4.18 - 6.84Cleft lip with or without cleft palate 97 7.80 6.33 - 9.52
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia 19 1.53 0.92 - 2.39Pyloric stenosis 204 16.41 14.16 - 18.6
6Stenosis or atresia of small intestine 27 2.17 1.43 - 3.16Stenosis or atresia of large intestine, rectum, or anal canal
50 4.02 2.99 - 5.30
Hirschsprung disease 16 1.29 0.74 - 2.09Biliary atresia 8 0.64 0.28 - 1.27
GENITOURINARYHypospadias or epispadias 355 28.55 25.58 - 31.5
3Renal agenesis or dysgenesis 60 4.83 3.68 - 6.21Obstructive genitourinary defect 242 19.47 17.01 - 21.9
2Bladder exstrophy 1 0.08 0.00 - 0.45
MUSCULOSKELETALCongenital hip dislocation 73 5.87 4.60 - 7.38Reduction defects of the upper limbs 25 2.01 1.30 - 2.97Reduction defects of the lower limbs 21 1.69 1.05 - 2.58Craniosynostosis 33 2.65 1.83 - 3.73Diaphragmatic hernia 40 3.22 2.30 - 4.38Omphalocele 27 2.17 1.43 - 3.16Gastroschisis 49 3.94 2.92 - 5.21
CHROMOSOMALTrisomy 21 (Down syndrome) 104 8.37 6.76 - 9.97Trisomy 13 (Patau syndrome) 14 1.13 0.62 - 1.89Trisomy 18 (Edwards syndrome) 24 1.93 1.24 - 2.87
OTHERFetal alcohol syndrome or other alcohol related birth 3 0.24 0.05 - 0.71
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 41 of 60
Table 11: Prevalence of Birth Defects, Texas Region 6, 1998‡-1999
DEFECT CASES RATE†95% CONFIDENCE
INTERVAL FOR RATE
defects
† cases per 10,000 live births ‡ 1998 data are limited to July 1-December 31 in Region 6
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 42 of 60
Table 12: Prevalence of Birth Defects, Texas Region 7, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 9 1.27 0.58 - 2.41Spina bifida without anencephaly 22 3.11 1.95 - 4.70Encephalocele 8 1.13 0.49 - 2.23Microcephaly 24 3.39 2.17 - 5.04Holoprosencephaly 7 0.99 0.40 - 2.04Hydrocephaly 55 7.76 5.85 - 10.1
1EYE AND EAR
Anophthalmia 2 0.28 0.03 - 1.02Microphthalmia 23 3.25 2.06 - 4.87Cataract 6 0.85 0.31 - 1.84Aniridia 2 0.28 0.03 - 1.02Anotia or microtia 18 2.54 1.51 - 4.02
CARDIAC AND CIRCULATORYCommon truncus 2 0.28 0.03 - 1.02Transposition of the great vessels 24 3.39 2.17 - 5.04Tetralogy of Fallot 14 1.98 1.08 - 3.32Ventricular septal defect 201 28.37 24.45 - 32.2
9Atrial septal defect 106 14.96 12.11 - 17.8
1Endocardial cushion defect 8 1.13 0.49 - 2.23Pulmonary valve atresia or stenosis 41 5.79 4.15 - 7.85Tricuspid valve atresia or stenosis 7 0.99 0.40 - 2.04Ebstein anomaly 2 0.28 0.03 - 1.02Aortic valve stenosis 10 1.41 0.68 - 2.60Hypoplastic left heart syndrome 13 1.83 0.98 - 3.14Patent ductus arteriosus 203 28.65 24.71 - 32.6
0Coarctation of the aorta 23 3.25 2.06 - 4.87
RESPIRATORYChoanal atresia or stenosis 10 1.41 0.68 - 2.60Agenesis, aplasia, or hypoplasia of the lung 27 3.81 2.51 - 5.55
ORAL CLEFTSCleft palate alone (without cleft lip) 39 5.50 3.91 - 7.53Cleft lip with or without cleft palate 61 8.61 6.59 - 11.0
6GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 13 1.83 0.98 - 3.14Pyloric stenosis 96 13.55 10.98 - 16.5
5Stenosis or atresia of small intestine 24 3.39 2.17 - 5.04Stenosis or atresia of large intestine, rectum, or anal canal 23 3.25 2.06 - 4.87Hirschsprung disease 13 1.83 0.98 - 3.14Biliary atresia 7 0.99 0.40 - 2.04
GENITOURINARYHypospadias or epispadias 186 26.25 22.48 - 30.0
3Renal agenesis or dysgenesis 26 3.67 2.40 - 5.38Obstructive genitourinary defect 108 15.24 12.37 - 18.1
2Bladder exstrophy 2 0.28 0.03 - 1.02
MUSCULOSKELETALCongenital hip dislocation 36 5.08 3.56 - 7.03Reduction defects of the upper limbs 17 2.40 1.40 - 3.84Reduction defects of the lower limbs 12 1.69 0.88 - 2.96Craniosynostosis 20 2.82 1.72 - 4.36Diaphragmatic hernia 14 1.98 1.08 - 3.32Omphalocele 17 2.40 1.40 - 3.84Gastroschisis 24 3.39 2.17 - 5.04
CHROMOSOMALTrisomy 21 (Down syndrome) 46 6.49 4.75 - 8.66Trisomy 13 (Patau syndrome) 5 0.71 0.23 - 1.65Trisomy 18 (Edwards syndrome) 12 1.69 0.88 - 2.96
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 43 of 60
Table 12: Prevalence of Birth Defects, Texas Region 7, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
OTHERFetal alcohol syndrome or other alcohol related birth defects 2 0.28 0.03 - 1.02
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 44 of 60
Table 13: Prevalence of Birth Defects, Texas Region 8, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 11 1.59 0.79 - 2.85Spina bifida without anencephaly 21 3.04 1.88 - 4.64Encephalocele 3 0.43 0.09 - 1.27Microcephaly 29 4.19 2.81 - 6.02Holoprosencephaly 7 1.01 0.41 - 2.09Hydrocephaly 43 6.22 4.50 - 8.38
EYE AND EARAnophthalmia 2 0.29 0.04 - 1.04Microphthalmia 23 3.33 2.11 - 4.99Cataract 5 0.72 0.23 - 1.69Aniridia 0 0.00 0.00 - 0.53Anotia or microtia 20 2.89 1.77 - 4.47
CARDIAC AND CIRCULATORYCommon truncus 3 0.43 0.09 - 1.27Transposition of the great vessels 38 5.50 3.89 - 7.54Tetralogy of Fallot 24 3.47 2.22 - 5.16Ventricular septal defect 277 40.06 35.34 - 44.7
8Atrial septal defect 175 25.31 21.56 - 29.0
6Endocardial cushion defect 30 4.34 2.93 - 6.19Pulmonary valve atresia or stenosis 53 7.66 5.74 - 10.0
3Tricuspid valve atresia or stenosis 18 2.60 1.54 - 4.11Ebstein anomaly 11 1.59 0.79 - 2.85Aortic valve stenosis 22 3.18 1.99 - 4.82Hypoplastic left heart syndrome 12 1.74 0.90 - 3.03Patent ductus arteriosus 315 45.55 40.52 - 50.5
8Coarctation of the aorta 36 5.21 3.65 - 7.21
RESPIRATORYChoanal atresia or stenosis 5 0.72 0.23 - 1.69Agenesis, aplasia, or hypoplasia of the lung 34 4.92 3.41 - 6.87
ORAL CLEFTSCleft palate alone (without cleft lip) 33 4.77 3.29 - 6.70Cleft lip with or without cleft palate 73 10.56 8.27 - 13.2
7GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 22 3.18 1.99 - 4.82Pyloric stenosis 142 20.54 17.16 - 23.9
1Stenosis or atresia of small intestine 19 2.75 1.65 - 4.29Stenosis or atresia of large intestine, rectum, or anal canal
35 5.06 3.53 - 7.04
Hirschsprung disease 8 1.16 0.50 - 2.28Biliary atresia 5 0.72 0.23 - 1.69
GENITOURINARYHypospadias or epispadias 170 24.58 20.89 - 28.2
8Renal agenesis or dysgenesis 34 4.92 3.41 - 6.87Obstructive genitourinary defect 110 15.91 12.93 - 18.8
8Bladder exstrophy 0 0.00 0.00 - 0.53
MUSCULOSKELETALCongenital hip dislocation 17 2.46 1.43 - 3.94Reduction defects of the upper limbs 40 5.78 4.13 - 7.88Reduction defects of the lower limbs 19 2.75 1.65 - 4.29Craniosynostosis 18 2.60 1.54 - 4.11Diaphragmatic hernia 19 2.75 1.65 - 4.29Omphalocele 15 2.17 1.21 - 3.58Gastroschisis 31 4.48 3.05 - 6.36
CHROMOSOMALTrisomy 21 (Down syndrome) 83 12.00 9.56 - 14.8
8
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 45 of 60
Table 13: Prevalence of Birth Defects, Texas Region 8, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
Trisomy 13 (Patau syndrome) 3 0.43 0.09 - 1.27Trisomy 18 (Edwards syndrome) 10 1.45 0.69 - 2.66
OTHERFetal alcohol syndrome or other alcohol related birth defects
1 0.14 0.00 - 0.81
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 46 of 60
Table 14: Prevalence of Birth Defects, Texas Region 9, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 11 6.67 3.33 - 11.9
3Spina bifida without anencephaly 6 3.64 1.33 - 7.91Encephalocele 2 1.21 0.15 - 4.38Microcephaly 14 8.48 4.64 - 14.2
4Holoprosencephaly 5 3.03 0.98 - 7.07Hydrocephaly 17 10.30 6.00 - 16.5
0EYE AND EAR
Anophthalmia 0 0.00 0.00 - 2.24Microphthalmia 11 6.67 3.33 - 11.9
3Cataract 0 0.00 0.00 - 2.24Aniridia 0 0.00 0.00 - 2.24Anotia or microtia 8 4.85 2.09 - 9.55
CARDIAC AND CIRCULATORYCommon truncus 1 0.61 0.02 - 3.38Transposition of the great vessels 4 2.42 0.66 - 6.21Tetralogy of Fallot 5 3.03 0.98 - 7.07Ventricular septal defect 69 41.82 32.54 - 52.9
2Atrial septal defect 63 38.18 29.34 - 48.8
5Endocardial cushion defect 7 4.24 1.71 - 8.74Pulmonary valve atresia or stenosis 8 4.85 2.09 - 9.55Tricuspid valve atresia or stenosis 2 1.21 0.15 - 4.38Ebstein anomaly 0 0.00 0.00 - 2.24Aortic valve stenosis 3 1.82 0.37 - 5.31Hypoplastic left heart syndrome 3 1.82 0.37 - 5.31Patent ductus arteriosus 82 49.69 39.52 - 61.6
8Coarctation of the aorta 6 3.64 1.33 - 7.91
RESPIRATORYChoanal atresia or stenosis 1 0.61 0.02 - 3.38Agenesis, aplasia, or hypoplasia of the lung 10 6.06 2.91 - 11.1
5ORAL CLEFTS
Cleft palate alone (without cleft lip) 9 5.45 2.49 - 10.35
Cleft lip with or without cleft palate 23 13.94 8.84 - 20.91
GASTROINTESTINALTracheoesophageal fistula / esophageal atresia 3 1.82 0.37 - 5.31Pyloric stenosis 43 26.06 18.86 - 35.1
0Stenosis or atresia of small intestine 4 2.42 0.66 - 6.21Stenosis or atresia of large intestine, rectum, or anal canal
9 5.45 2.49 - 10.35
Hirschsprung disease 2 1.21 0.15 - 4.38Biliary atresia 1 0.61 0.02 - 3.38
GENITOURINARYHypospadias or epispadias 33 20.00 13.77 - 28.0
9Renal agenesis or dysgenesis 15 9.09 5.09 - 14.9
9Obstructive genitourinary defect 30 18.18 12.27 - 25.9
5Bladder exstrophy 1 0.61 0.02 - 3.38
MUSCULOSKELETALCongenital hip dislocation 8 4.85 2.09 - 9.55Reduction defects of the upper limbs 9 5.45 2.49 - 10.3
5Reduction defects of the lower limbs 5 3.03 0.98 - 7.07
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 47 of 60
Table 14: Prevalence of Birth Defects, Texas Region 9, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
Craniosynostosis 3 1.82 0.37 - 5.31Diaphragmatic hernia 1 0.61 0.02 - 3.38Omphalocele 5 3.03 0.98 - 7.07Gastroschisis 9 5.45 2.49 - 10.3
5CHROMOSOMAL
Trisomy 21 (Down syndrome) 20 12.12 7.40 - 18.72
Trisomy 13 (Patau syndrome) 4 2.42 0.66 - 6.21Trisomy 18 (Edwards syndrome) 3 1.82 0.37 - 5.31
OTHERFetal alcohol syndrome or other alcohol related birth defects
0 0.00 0.00 - 2.24
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 48 of 60
Table 15: Prevalence of Birth Defects, Texas Region 10, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 16 5.49 3.14 - 8.92Spina bifida without anencephaly 15 5.15 2.88 - 8.49Encephalocele 3 1.03 0.21 - 3.01Microcephaly 19 6.52 3.93 - 10.1
8Holoprosencephaly 5 1.72 0.56 - 4.01Hydrocephaly 35 12.01 8.37 - 16.7
1EYE AND EAR
Anophthalmia 0 0.00 0.00 - 1.27Microphthalmia 6 2.06 0.76 - 4.48Cataract 6 2.06 0.76 - 4.48Aniridia 0 0.00 0.00 - 1.27Anotia or microtia 17 5.84 3.40 - 9.34
CARDIAC AND CIRCULATORYCommon truncus 2 0.69 0.08 - 2.48Transposition of the great vessels 11 3.78 1.88 - 6.76Tetralogy of Fallot 7 2.40 0.97 - 4.95Ventricular septal defect 101 34.67 27.91 - 41.4
3Atrial septal defect 28 9.61 6.39 - 13.8
9Endocardial cushion defect 11 3.78 1.88 - 6.76Pulmonary valve atresia or stenosis 11 3.78 1.88 - 6.76Tricuspid valve atresia or stenosis 6 2.06 0.76 - 4.48Ebstein anomaly 1 0.34 0.01 - 1.91Aortic valve stenosis 7 2.40 0.97 - 4.95Hypoplastic left heart syndrome 10 3.43 1.65 - 6.31Patent ductus arteriosus 82 28.15 22.39 - 34.9
4Coarctation of the aorta 17 5.84 3.40 - 9.34
RESPIRATORYChoanal atresia or stenosis 3 1.03 0.21 - 3.01Agenesis, aplasia, or hypoplasia of the lung 13 4.46 2.38 - 7.63
ORAL CLEFTSCleft palate alone (without cleft lip) 13 4.46 2.38 - 7.63Cleft lip with or without cleft palate 34 11.67 8.08 - 16.3
1GASTROINTESTINAL
Tracheoesophageal fistula / esophageal atresia 4 1.37 0.37 - 3.52Pyloric stenosis 69 23.68 18.43 - 29.9
7Stenosis or atresia of small intestine 9 3.09 1.41 - 5.86Stenosis or atresia of large intestine, rectum, or anal canal
17 5.84 3.40 - 9.34
Hirschsprung disease 4 1.37 0.37 - 3.52Biliary atresia 2 0.69 0.08 - 2.48
GENITOURINARYHypospadias or epispadias 62 21.28 16.32 - 27.2
8Renal agenesis or dysgenesis 12 4.12 2.13 - 7.19Obstructive genitourinary defect 47 16.13 11.85 - 21.4
5Bladder exstrophy 0 0.00 0.00 - 1.27
MUSCULOSKELETALCongenital hip dislocation 11 3.78 1.88 - 6.76Reduction defects of the upper limbs 10 3.43 1.65 - 6.31Reduction defects of the lower limbs 3 1.03 0.21 - 3.01Craniosynostosis 15 5.15 2.88 - 8.49Diaphragmatic hernia 6 2.06 0.76 - 4.48Omphalocele 6 2.06 0.76 - 4.48Gastroschisis 12 4.12 2.13 - 7.19
CHROMOSOMALTrisomy 21 (Down syndrome) 34 11.67 8.08 - 16.3
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 49 of 60
Table 15: Prevalence of Birth Defects, Texas Region 10, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
1Trisomy 13 (Patau syndrome) 3 1.03 0.21 - 3.01Trisomy 18 (Edwards syndrome) 4 1.37 0.37 - 3.52
OTHERFetal alcohol syndrome or other alcohol related birth defects
1 0.34 0.01 - 1.91
† cases per 10,000 live births
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 50 of 60
Table 16: Prevalence of Birth Defects, Texas Region 11, 1998-1999
DEFECT CASES RATE†
95% CONFIDENCE
INTERVAL FOR RATE
CENTRAL NERVOUS SYSTEMAnencephaly 43 5.64 4.08 - 7.60Spina bifida without anencephaly 51 6.69 4.98 - 8.79Encephalocele 7 0.92 0.37 - 1.89Microcephaly 39 5.11 3.64 - 6.99Holoprosencephaly 6 0.79 0.29 - 1.71Hydrocephaly 72 9.44 7.39 - 11.89
EYE AND EARAnophthalmia 3 0.39 0.08 - 1.15Microphthalmia 17 2.23 1.30 - 3.57Cataract 10 1.31 0.63 - 2.41Aniridia 0 0.00 0.00 - 0.48Anotia or microtia 15 1.97 1.10 - 3.24
CARDIAC AND CIRCULATORYCommon truncus 8 1.05 0.45 - 2.07Transposition of the great vessels 35 4.59 3.20 - 6.38Tetralogy of Fallot 28 3.67 2.44 - 5.31Ventricular septal defect 534 70.03 64.09 - 75.96Atrial septal defect 772 101.24 94.09 - 108.3
8Endocardial cushion defect 25 3.28 2.12 - 4.84Pulmonary valve atresia or stenosis 61 8.00 6.12 - 10.28Tricuspid valve atresia or stenosis 19 2.49 1.50 - 3.89Ebstein anomaly 12 1.57 0.81 - 2.75Aortic valve stenosis 21 2.75 1.70 - 4.21Hypoplastic left heart syndrome 20 2.62 1.60 - 4.05Patent ductus arteriosus 677 88.78 82.09 - 95.47Coarctation of the aorta 48 6.29 4.64 - 8.35
RESPIRATORYChoanal atresia or stenosis 13 1.70 0.91 - 2.92Agenesis, aplasia, or hypoplasia of the lung 36 4.72 3.31 - 6.54
ORAL CLEFTSCleft palate alone (without cleft lip) 39 5.11 3.64 - 6.99Cleft lip with or without cleft palate 84 11.02 8.79 - 13.64Tracheoesophageal fistula / esophageal atresia 10 1.31 0.63 - 2.41
GASTROINTESTINALPyloric stenosis 184 24.13 20.64 - 27.62Stenosis or atresia of small intestine 29 3.80 2.55 - 5.46Stenosis or atresia of large intestine, rectum, or anal canal
41 5.38 3.86 - 7.29
Hirschsprung disease 3 0.39 0.08 - 1.15Biliary atresia 4 0.52 0.14 - 1.34
GENITOURINARYHypospadias or epispadias 164 21.51 18.21 - 24.80Renal agenesis or dysgenesis 45 5.90 4.30 - 7.90Obstructive genitourinary defect 122 16.00 13.16 - 18.84Bladder exstrophy 0 0.00 0.00 - 0.48
MUSCULOSKELETALCongenital hip dislocation 49 6.43 4.75 - 8.49Reduction defects of the upper limbs 34 4.46 3.09 - 6.23Reduction defects of the lower limbs 15 1.97 1.10 - 3.24Craniosynostosis 31 4.07 2.76 - 5.77Diaphragmatic hernia 27 3.54 2.33 - 5.15Omphalocele 13 1.70 0.91 - 2.92Gastroschisis 22 2.88 1.81 - 4.37
CHROMOSOMALTrisomy 21 (Down syndrome) 86 11.28 9.02 - 13.93Trisomy 13 (Patau syndrome) 7 0.92 0.37 - 1.89Trisomy 18 (Edwards syndrome) 21 2.75 1.70 - 4.21
OTHERFetal alcohol syndrome or other alcohol related birth defects
4 0.52 0.14 - 1.34
Texas Birth Defects Registry Report of Defects Among 1998-1999 Deliveries Page 51 of 60
