Original Communications
The incidence and epidemiology of congenital upper limb anomalies: A total population study*,**

https://doi.org/10.1053/jhsu.2001.26121Get rights and content

Abstract

An 11-year total population study of Western Australia examined the prevalence and epidemiology of congenital upper limb anomalies. All anomalies were classified according to the International Federation of Surgical Societies of the Hand classification. We found the prevalence of babies born with upper limb anomalies to be 1 in 506. Forty-six percent of those affected had another nonhand congenital anomaly. Fifty-one percent had bilateral hand anomalies, and 17% had multiple different hand anomalies. The most common anomalies were failures of differentiation (35%), duplications (33%), and failures of formation (15%). Congenital upper limb anomalies were more common in boys; preterm, postterm, and multiple births; and older mothers. No significant differences in prevalence or frequency of anomalies were found between whites and nonwhites, left and right sides, and in babies that survived and those who died shortly after birth. (J Hand Surg 2001;26A:628-634. Copyright © 2001 by the American Society for Surgery of the Hand.)

Section snippets

Methods/Materials

Data on babies born with CULA were obtained from the Western Australian Birth Defects Registry, a population-based register of major and minor birth defects diagnosed up to the age of 6 years in children born in Western Australia.10

Data were collated from notifications submitted by midwives, pediatricians, obstetricians, geneticists, surgeons, and other clinicians who may have had contact with any children during this period. These data were cross-referenced with the other sources as they were

Prevalence data

During the 11-year period from 1980 to 1990, 509 live births with CULA were reported. There were 257,430 live births during this period. Thus, the prevalence of babies born with 1 or more CULA was 1 in 506, or 1.97 per 1,000 live births. Forty-six percent of the babies with a CULA had some other anomaly of which 101 (20%) were syndromic, 48 (9%) had multiple anomalies that were not classifiable into a syndrome, 45 (9%) had a foot anomaly, and 40 (8%) a single other anomaly.

The 509 babies had

Discussion

The prevalence of 1 in 506 live births born with a CULA compares well with the extrapolated estimate proposed by Conway and Bowe8 of 1 in 626. This comparison is especially close when we consider our prevalence of potential clinic attenders (1 in 610) which resembles the Conway and Bowe study population.

In our study, more than half the babies had bilateral anomalies and more than 16% had multiple anomalies; thus, if one studies only the anomalies, an inflated prevalence figure is obtained. We

Acknowledgements

The authors are very grateful to Louise Grace for her assistance in data retrieval, interpretation, and statistics.

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    In his 'the care of congenital hand anomalie's’, Flatt describes the relative frequency of anomalies for 2758 children over 34 years.6 To our knowledge, there are only a handful of epidemiological studies on congenital hand deformities, one from Western Australia,7 followed by Stockholm, Sweden,89 Middle west, United States,10 and Finland.11 In India, the documented literature on the epidemiology of congenital hand deformity is by Mittal et al.12 on the rural population, Agarwal et al.,13 who surveyed a tertiary care hospital, and by Yesilada AK et al.14

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*

No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.

**

Reprint requests: Henk Giele, MD, Department of Plastic Surgery, Radcliffe Infirmary, Woodstock Road, Oxford. OX2 6HE, England.

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