Birth prevalence for congenital limb defects in the northern Netherlands: a 30-year population-based study

Table 1 Total birth prevalence per type of congenital limb defects for the period 1981–2010 in the northern Netherlands

CLD	Isolated CLD, n	Multiple congenital anomalies, n	CLD is part of a recognised condition, n	Total, n	Prevalence per 10,000
Polydactyly	291	45	83	419	8.4
Upper limb	228	38	63^a	329	6.6
Preaxial	73	13	20	106	2.1
Postaxial	141	21	39	201	4.0
NOS	14	4	6	24	0.5
Lower limb	75	9	32	116	2.3
Preaxial	12	1	8	21	0.4
Postaxial	59	7	19	85	1.7
NOS	4	1	5	10	0.2
NOS	4	0	2	6	0.1
Upper and lower limb	16	2	14	32	0.6
Reduction defects	180	42	120	342	6.9
Upper limb	128	28	94	250	5.0
Transverse	89	17	39	145	2.9
Longitudinal	21^b	9	51	81	1.6
Preaxial	12	7	33	52	1.0
Postaxial	10	2	18	30	0.6
Intercalary	3	1	5	9	0.2
Central	16	1	4	21	0.4
Multiple	1	0	5	6	0.1
Lower limb	66	25	58	149	3.0
Transverse	29	15	31	75	1.5
Longitudinal	23^c	5	24	52	1.0
Preaxial	4	3	9	16	0.3
Postaxial	21	2	16	39	0.8
Intercalary	7	5	10	22	0.4
Central	13	1	2	16	0.3
Multiple	7	2	8^d	17	0.3
NOS	1	1	0	2	0.04
NOS	0	0	1	1	0.02
Upper and lower limb	14	11	33	58	1.2
Syndactyly	126	29	77	232	4.7
Upper limb	77	17	46	140	2.8
Lower limb	59	12	46	117	2.4
NOS	0	1	0	1	0.02
Upper and lower limb	10	1	15	26	0.5
Other CLD^e	41	34	88	163	3.3
Upper limb	18	14	66	98	2.0
Lower limb	25	22	53	100	2.0
NOS	0	1	0	1	0.02
Upper and lower limb	2	3	31	36	0.7
Multiple CLD^f	38^g	14^g	49^g	101	2.0
Total no. of cases	598	135	315	1048	21.1
Total no. of live births	587	77	159	823	16.5

Total number of births for the period 1981–2010: n = 497,751.
Abbreviations: CLD–congenital limb defects, NOS–not otherwise specified, n–number of children with CLD.
^aTwo children had preaxial and postaxial polydactyly of upper limbs.
^bOne child had preaxial and postaxial longitudinal reduction defects of upper limbs.
^cTwo children had preaxial and postaxial longitudinal reduction defects of lower limbs.
^dOne child had transversal, longitudinal and intercalary reduction defects of lower limbs.
^eThe category consisted of CLD like arthrogryposis (n = 40), hemihypertrophy (n = 25), contractures of elbows/knees/fingers (n = 19), undergrowth of limbs (n = 9), radio-ulnar synostosis (n = 5), macrodactyly (n = 4).
^fCategory containing cases with several CLD included in the study.
^gChildren in the group with isolated (n = 2), multiple congenital anomalies (n = 1), and CLD as part of a recognised condition (n = 4) had three types of CLD, whereas the rest of the children with multiple limb defects had two types of CLD.

ISSN: 1471-2474