Table 2.
Population-based unduplicated rates per 10 000 live births of individuals with selected congenital malformations in Rivne, Ukraine1
| Categories | Polissia | Non-Polissia | Polissia | Non-Polissia | Polissia vs. non-Polissia | ||||
|---|---|---|---|---|---|---|---|---|---|
| 2000–2004 | 2005–2009 | 2000–2004 | 2005–2009 | 2000–2009 | 2000–2009 | OR | P-value | CL | |
| Neural tube defects (NTD) | 29.1 | 23.5 | 18.4 | 14.8 | 26.1 | 16.4 | 1.59 | *** | 1.26, 2.02 |
| Cephalad2 | 9.5 | 10.1 | 7.8 | 4.8 | 9.8 | 6.2 | 1.59 | ** | 1.08, 2.37 |
| Anencephaly | 6.2 | 4.1 | 5.1 | 3.0 | 5.1 | 4.0 | – | n/s | – |
| Isolated | 5.6 | 3.9 | 4.8 | 3.0 | 4.7 | 3.8 | – | n/s | – |
| Non-NTD malformations3 | n/c | n/c | n/c | – | n/c | n/c | – | n/c | – |
| Cranio-inien-rachis-schisis4 | 3.3 | 5.9 | 2.7 | 1.8 | 4.7 | 2.2 | 2.15 | ** | 1.15, 4.16 |
| Isolated | 2.1 | 4.1 | 1.8 | 1.5 | 3.2 | 1.6 | 1.94 | * | 0.92, 4.27 |
| Other malformations | n/c | 1.8 | n/c | n/c | 1.5 | n/c | – | n/s | – |
| Spina bifida cervico-thoracic | 3.3 | 1.6 | – | n/c | 2.3 | n/c | 4.29 | ** | 1.40, 17.5 |
| Isolated | 2.4 | 1.6 | – | n/c | 1.9 | n/c | 3.53 | * | 1.11, 14.7 |
| Other malformations | n/c | – | – | – | n/c | – | – | n/c | – |
| Spina bifida lumbo-sacral | 10.1 | 10.3 | 7.2 | 7.0 | 10.2 | 7.1 | 1.44 | * | 0.99, 2.09 |
| Isolated | 8.9 | 9.3 | 6.0 | 6.5 | 9.1 | 6.3 | 1.45 | * | 0.98, 2.16 |
| Syndromes | n/c | – | – | – | n/c | – | – | n/c | – |
| Other malformations | n/c | n/c | n/c | n/c | 0.97 | 0.8 | – | n/s | – |
| Spina bifida site unknown | 3.3 | – | n/c | – | 1.5 | n/c | – | n/s | – |
| Isolated | 3.3 | – | n/c | – | 1.5 | n/c | – | n/s | – |
| Spina bifida combined | 16.6 | 11.9 | 8.4 | 8.0 | 14.1 | 8.2 | 1.71 | *** | 1.24, 2.40 |
| Encephalocele | 3.0 | 1.6 | 2.1 | 2.0 | 2.2 | 2.1 | – | n/s | – |
| Isolated | 2.1 | 1.3 | 1.5 | 1.5 | 1.7 | 1.5 | – | n/s | – |
| Syndromes | n/c | n/c | – | – | n/c | n/c | – | n/c | – |
| Other malformations | n/c | – | n/c | n/c | n/c | n/c | – | n/c | – |
| Microcephaly5 | 5.3 | 6.7 | 3.3 | 3.3 | 6.1 | 3.3 | 1.85 | ** | 1.10, 3.18 |
| Isolated | 2.7 | 1.3 | n/c | 1.3 | 1.9 | 1.1 | – | n/s | – |
| Syndromes | n/c | 2.8 | 2.1 | 1.8 | 2.1 | 1.9 | – | n/s | – |
| Other malformations | 1.5 | 2.6 | n/c | n/c | 2.1 | n/c | 7.57 | *** | 1.76, 68.2 |
| Microphthalmos6 | 1.8 | 3.1 | n/c | n/c | 2.5 | 0.8 | 3.03 | * | 1.15, 9.32 |
| Isolated | n/c | 1.6 | – | n/c | 1.1 | n/c | – | n/s | – |
| Syndromes | – | n/c | n/c | – | n/c | n/c | – | n/c | – |
| Other malformations | n/c | n/c | n/c | – | 1.0 | n/c | 7.07 | * | 0.91, 318 |
| Omphalocele7 | 1.8 | 1.8 | 2.4 | 4.3 | 1.8 | 3.4 | 0.52 | (*) | 0.25, 1.07 |
| Isolated | n/c | n/c | 1.8 | 2.8 | 0.7 | 2.3 | 0.30 | (**) | 0.09, 0.84 |
| Syndromes | n/c | n/c | n/c | – | n/c | n/c | – | n/c | – |
| Other malformations | n/c | n/c | n/c | 1.5 | 0.8 | 1.0 | – | n/s | – |
| Gastroschisis | n/c | 3.4 | 3.3 | 3.0 | 2.3 | 3.1 | – | n/s | – |
| Isolated | n/c | 3.4 | 3.0 | 3.0 | 2.2 | 3.0 | – | n/s | – |
| Other malformations | n/c | – | n/c | – | n/c | n/c | – | n/c | – |
| Urinary bladder exstrophy8 | n/c | n/c | n/c | n/c | 1.1 | 0.7 | – | n/s | – |
| Isolated | n/c | n/c | n/c | n/c | 1.1 | n/c | – | n/s | – |
| Other malformations | – | – | n/c | – | – | n/c | – | n/c | – |
| Conjoined twins9 | n/c | – | n/c | n/c | n/c | 0.7 | – | n/s | – |
| Isolated | n/c | – | n/c | n/c | n/c | n/c | – | n/c | – |
| Other malformations | – | – | n/c | n/c | – | n/c | – | n/c | – |
| Teratomas | 1.5 | n/c | n/c | n/c | 0.8 | n/c | – | n/s | – |
| Isolated | 1.5 | n/c | n/c | n/c | 0.8 | n/c | – | n/s | – |
| Sacro-coccygeal | 1.5 | n/c | n/c | n/c | 0.8 | n/c | – | n/s | – |
| Isolated | 1.5 | n/c | n/c | n/c | 0.8 | n/c | – | n/s | – |
| All | 42.4 | 39.8 | 29.8 | 28.4 | 41.0 | 29.0 | 1.42 | *** | 1.18, 1.70 |
| Isolated | 32.6 | 28.2 | 22.3 | 23.3 | 30.3 | 22.9 | 1.33 | ** | 1.08, 1.63 |
| Syndromes | 2.4 | 4.1 | 2.7 | 1.8 | 3.3 | 2.2 | – | n/s | – |
| Other malformations | 7.4 | 7.5 | 4.8 | 3.3 | 7.5 | 4.0 | 1.88 | ** | 1.18, 3.06 |
Actual counts of unduplicated individuals are found in Table S1b. All births in Rivne are 145 437, in Polissia, 72 379, and in non-Polissia, 73 058 (observations in non-Polissia are in italics). Individuals with congenital malformations (CM) are represented only in one category and include those liveborn or not and singletons or not; individuals with holoprosencephaly are excluded from the Tables 2, and S1a,b,c series; population rates of CM detected up to one year of age are calculated per 10 000 live births. One-tailed P-values are calculated with 95% confidence limits for categories with at least five individuals represented or alternatively are shown as not computed (n/c). P-values are denoted by
***, ≤0.001; **, ≤0.01; *, ≤0.05, or n/s for non-significant. P-values in parentheses indicate a negative association. Odds ratio (OR) and confidence limits (CL) are also shown.
Cephalad includes iniencephaly, anencephaly (or acrania) associated or not with rachis-schisis or spina bifida. Encephaloceles are computed separately.
Microphthalmia (c-1); cleft lip (c-2); omphalocele (c-3); esophageal atresia (d-1).
Includes iniencephaly and anencephaly-rachis-schisis.
Excluded are two singletons and one twin with holoprosencephaly (hol-6, 20; tw-1); also excluded is an individual incompletely described (z-2) diagnosed at 20 weeks of gestation with a holoprosencephaly spectrum and a single orbit, who is represented among conjoined twins.Included in this category as isolated microcephaly are two individuals who had microcephaly and microphthalmia not associated with extra-ocular anomalies (ri-8, 12).
Excluded is one individual represented among anencephalics (c-1); four individuals with microcephaly (ri-8, 12; t-8; s-2); and one individual (z-2), who is represented among conjoined twins and further described in footnote (5).
Excluded is one individual represented among anencephalics (c-3); seven individuals with cranio-rachis-schises (f-2, 4–8; g-4); six individuals with spina bifida (h-1, 3; l-5, m-2, 5, 6); two individuals with microcephaly (t-9, u-2); and two individuals with microphthalmia (v-1; w-1).
Excluded is one individual with spina bifida (h-3).
Excluded is one member of a set of conjoined twins (h-1) with spina bifida and represented among other individuals with spina bifida.