Martínez-Frías María Luisa, Bermejo Eva, Mendioroz Jacobo, Rodríguez-Pinilla Elvira, Blanco Manuel, Egüés Javier, Félix Valentín, García Angel, Huertas Héctor, Nieto Carmen, López José Antonio, López Santiago, Paisán Luis, Rosa Alejandro, Vázquez María Socorro
ECEMC, Centro de Investigación sobre Anomalías Congénitas, del Instituto de Salud Carlos III, Madrid, Spain.
J Pediatr Surg. 2009 Apr;44(4):811-20. doi: 10.1016/j.jpedsurg.2008.07.002.
The aim of the study was to analyze the frequency and certain epidemiological characteristics of a consecutive series of conjoined twins born in Spain.
We used data from the Spanish Collaborative Study of Congenital Malformations for the period April 1976 to 2006. Because the Spanish law permitting voluntary termination of pregnancies (TOP) when the fetus presented malformations was effective by the end of 1985, we analyzed the data in 4 periods, 2 before 1986 and 2 after. During the first period (1976-1979) only live births were recorded, whereas both still and live births were included in the other three (1980-1985, 1986-1995, and 1996-2006). In the present study, the cases were classified as symmetrical (16 pairs) and asymmetrical (1 pair) conjoined twins. Each pair of conjoined twins was considered as only one case for calculations, regardless of the type of union.
Among a total of 2,281,604 consecutive births between 1980 and 2006, there were a total of 15 cases of symmetrical conjoined twins giving a frequency of 0.70 per 100,000 (1/152,107), whereas there was only 1 stillborn asymmetrical conjoined twin pair (0.04/100,000). Among the 13,418 consecutive stillborns surveyed, 6 cases of conjoined twins were identified (either symmetrical or asymmetrical) giving a frequency of 44.72 per 100,000, and 11 pairs were identified among the 2,425,583 total live births surveyed during the first period 1976 to 1979, a frequency of 0.45 per 100,000. Thus, the frequency among stillborn infants is 99.34 times higher than that observed among live births. However, the frequency for the total births (3 last periods) showed a decreasing trend from 1.47 per 100,000 birth in the first period (1980-1985) when TOP was illegal, to a value of 0.09 per 100,000 in the last period, more than 16-fold lower, probably because of the TOP of affected fetuses. Therefore, we consider that the frequencies observed in the period 1980 to 1985 are the basal values in our population. The most frequent type observed was thoracopagus, with an overall prevalence at birth of 0.44 per 100,000 (1/228,160) from 1980 to 2006, representing 58.82% of the total population of symmetric conjoined twin pairs. Diprosopus pairs were the next most common group (11.76%). Most of the cases were females (4 males/11 females), and although this appeared to be mainly because of the thoracopagus pairs (males-females, 2:8), in such a small number of cases, it is not possible to determine the ratios for the other groups. Gestational age was significantly shorter than in control twins for each type studied.
We conclude that it is incorrect to consider that all types of conjoined twins have the same epidemiological characteristics, such as the frequency at birth. The differences observed may be related with the distinct embryo-fetal mortality of each type of conjoined twins in different populations, and the sex ratio, among others.
本研究旨在分析西班牙出生的一系列联体双胎的发生频率及某些流行病学特征。
我们使用了1976年4月至2006年期间西班牙先天性畸形协作研究的数据。由于西班牙允许在胎儿出现畸形时自愿终止妊娠(TOP)的法律于1985年底生效,我们将数据分为4个时期进行分析,其中2个时期在1986年之前,2个时期在1986年之后。在第一个时期(1976 - 1979年)仅记录活产,而在其他三个时期(1980 - 1985年、1986 - 1995年和1996 - 2006年)则同时包括死产和活产。在本研究中,联体双胎病例分为对称型(16对)和不对称型(1对)。计算时,每对联体双胎仅视为1例,无论联体类型如何。
在1980年至2006年期间的2,281,604例连续出生中,共有15例对称型联体双胎,发生率为每100,000例中有0.70例(1/152,107),而仅有1例不对称型联体双胎死产(0.04/100,000)。在13,418例连续死产中,识别出6例联体双胎(对称型或不对称型),发生率为每100,000例中有44.72例,在1976年至1979年第一个时期的2,425,583例总活产中识别出11对,发生率为每100,000例中有0.45例。因此,死产儿中的发生率比活产儿中观察到的发生率高99.34倍。然而,总出生数(最后3个时期)的发生率呈下降趋势,从TOP为非法的第一个时期(1980 - 1985年)的每100,000例出生中有1.47例,降至最后一个时期的每100,000例中有0.09例,降低了16倍多,这可能是由于对受影响胎儿进行了TOP。因此,我们认为1980年至1985年期间观察到的发生率是我们人群中的基础值。观察到的最常见类型是胸部联体双胎,1980年至2006年期间出生时的总体患病率为每100,000例中有0.44例(1/228,160),占对称型联体双胎总数的58.82%。双面联体双胎是第二常见的类型(11.76%)。大多数病例为女性(4例男性/11例女性),尽管这似乎主要是由于胸部联体双胎(男性 - 女性,2:8),但在如此少量的病例中,无法确定其他类型的比例。研究的每种类型联体双胎的孕周均明显短于对照双胎。
我们得出结论,认为所有类型的联体双胎具有相同的流行病学特征(如出生时的发生率)是不正确的。观察到的差异可能与不同人群中每种类型联体双胎独特的胚胎 - 胎儿死亡率以及性别比例等有关。