【摘要】 目的　从流行病学角度 ,了解中国先天性无耳及小耳畸形发病率的现状。方法　全国出生缺陷监测采用以医院为基础的监测方案 ,监测对象为孕满 2 8周至生后 7d的围产儿。 5年中全国 30个省 (市、自治区 )的 44 3～ 5 88所医院参加了监测。结果　 1 988～ 1 992年全国共监测 32 46 40 8例围产儿 ,发现无耳及小耳畸形 45 3例 ,5年总发病率为 1 4 0 /万 ,发病率呈下降趋势 (P <0 0 5 )。城镇发病率高于农村 ,发病率无性别差异。全国以新疆发病率最高 ,为 2 0 8/万 ,内蒙最低 ,为 0 33/万。6 0 4 %的病例为多发畸形 ,与单发畸形 (占 39 6 % )比较差异有显著性 (P <0 0 1 )。无耳和小耳以伴发颌面部畸形最多 ,其次为肌肉骨骼系统和中枢神经系统 ,3 0 %的病例确认为综合征。结论　中国无耳和小耳畸形伴多发畸形比例明显较高 ;全国各省市发生率存在较大差异 ;对于无耳及小耳特别是多发畸形患儿 ,还需提高对先天性心脏病、染色体异常的诊断。更多还原

【Abstract】 Objective To investigate the epidemiological characteristics of cases with anotia and microtia in China Method The birth defect monitoring program was undertaken by a hospital based monitoring method in 443 588 hospitals from 30 provinces, cities and autonomous regions across China Data including intrauterine death and stillbirth from 28 weeks of gestation to a period of 7 days postpartum were collected between 1988 and 1992 Results A total of 3 246 408 births was monitored from 1988 to 1992, in which 453 cases had anotia and microtia The average incidence was 1 40 per 10 000 during the five year period The decreased tendency of incidence was noted during the period (χ 2 =5 5588, P <0 05) The incidence in the urban area was significantly higher than that in the rural area There was no sex difference in the incidence of anotia and microtia The incidence varied among 30 provinces with highest incidence in Xinjiang province (2 08 per 10 000 births) and lowest in the Inner Mongolia Automous Regien (0 33 per 10 000 births) In cases with the defects of anotia and microtia, 60 4% were accompanied with other congenital malformations The proportion of multiple malformations with microtia and anotia was significantly higher than that of isolated case (χ 2=36 927 7, P <0 01) the highest incidence of concurrent malformations was anophthalmia or microphthalmia (13 1%), followed by facial cleft (12 6%), neural tube defects (10 0%), limb reduction defects (9 6%) and polydactyly (5 4%) There were also 3 5% cardiac defects Conclusions The prevalence of anotia and microtia varied among provinces across China The high proportions of cases with anotia and microtia had multiple malformations Therefore, careful examination of other malformations in patients with anotia and microtia is necessary更多还原