Descriptive epidemiology of anophthalmia and microphthalmia, Hawaii, 1986-2001. 2006

Mathias B Forrester, and Ruth D Merz
Hawaii Birth Defects Program, Honolulu, Hawaii 96817-5157, USA.

BACKGROUND Population-based epidemiologic data on anophthalmia and microphthalmia in the United States are limited and have come mainly from only a few states. The intent of this study was to report on the epidemiology of these eye defects. METHODS Cases were derived from a population-based birth defects registry in Hawaii and comprised all infants and fetuses with anophthalmia and microphthalmia who were delivered during 1986-2001. Anophthalmia and microphthalmia rates per 10,000 births were determined for selected factors, and comparisons were made by calculating the rate ratios and 95% confidence intervals (CIs). RESULTS Ninety-six cases of anophthalmia and microphthalmia were identified, with a rate of 3.21 per 10,000 live births. The eye defects were isolated in 5 cases (5.2%), and 24 cases (25.0%) had confirmed chromosomal abnormalities. The risk of anophthalmia and microphthalmia varied over time and was significantly higher for live-born infants with low birth weights and gestational ages. The anophthalmia and microphthalmia rates also varied by maternal race/ethnicity, sex, and plurality, although these differences were not statistically significant. CONCLUSIONS Anophthalmia and microphthalmia frequently occurred with other birth defects, and the rate was consistent with that found in the literature. The risk of defects differed significantly with time period, birth weight, and gestational age. The impact of many factors on anophthalmia and microphthalmia in Hawaii was frequently consistent with that reported elsewhere.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D008850 Microphthalmos Congenital or developmental anomaly in which the eyeballs are abnormally small. Microphthalmia
D012042 Registries The systems and processes involved in the establishment, support, management, and operation of registers, e.g., disease registers. Parish Registers,Population Register,Parish Register,Population Registers,Register, Parish,Register, Population,Registers, Parish,Registers, Population,Registry
D005260 Female Females
D006254 Hawaii A group of islands in Polynesia, in the north central Pacific Ocean, comprising eight major and 114 minor islands, largely volcanic and coral. Its capital is Honolulu. It was first reached by Polynesians about 500 A.D. It was discovered and named the Sandwich Islands in 1778 by Captain Cook. The islands were united under the rule of King Kamehameha 1795-1819 and requested annexation to the United States in 1893 when a provisional government was set up. Hawaii was established as a territory in 1900 and admitted as a state in 1959. The name is from the Polynesian Owhyhii, place of the gods, with reference to the two volcanoes Mauna Kea and Mauna Loa, regarded as the abode of the gods. (From Webster's New Geographical Dictionary, 1988, p493 & Room, Brewer's Dictionary of Names, 1992, p2330) Midway Island,Midway Islands
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D000853 Anophthalmos Congenital absence of the eye or eyes. Anophthalmia,Anophthalmias

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