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联体双胎先天性畸形的不对称性与不一致性:6例报告

Asymmetry and discordance for congenital anomalies in conjoined twins: a report of six cases.

作者信息

Ornoy A, Navot D, Menashi M, Laufer N, Chemke J

出版信息

Teratology. 1980 Oct;22(2):145-54. doi: 10.1002/tera.1420220203.

Abstract

Six pairs of conjoined twins have been studied. The first case was a pair of 13-week-old omphalopagus fetuses. One was a holoacradius amorphus and the other had rachischisis and anencephaly. The second case was a pair of omphalopagus twins. One of the twins was macerated and corresponded to a developmental age of 13-14 weeks, while the other was developed to 28-30 weeks of gestation and exhibited urogenital and gastrointestinal defects not found in the smaller twin. In the third case, that of a thoracoomphalopagus, one had cleft lip and palate, pulmonic stenosis, and atresia of the ileocecal valve, while the other did not show these anomalies. In the fourth cae, also omphalopagus twins, one had a lumbosacral meningomyelocele and severe gastrointestinal and urogenital anomalies not found in the second twin. The fifth case was a pair of thoracoomphalopagus twins, sharing a common heart with asymmetrical anomalies. The sixth case was a diprosopus anencephalic conjoined twin. The first pairs of conjoined twins were discordant for several abnormalities in nonshared organs, in addition to having abnormalities of the conjoined organs. It seems that discordance in conjoined twins is not a rare finding. The factors that play a role in discordance of anomalies in conjoined twins are probably similar to the factors in monozygotic twins--i.e., environmental, genetic, and abnormal placental and/or fetal circulation.

摘要

对六对连体双胞胎进行了研究。第一例是一对孕13周的胸腹联体胎儿。一个是全无肢无形态胎儿,另一个有脊柱裂和无脑畸形。第二例是一对胸腹联体双胞胎。其中一个胎儿已浸软,发育年龄为13 - 14周,而另一个发育至孕28 - 30周,表现出较小胎儿未发现的泌尿生殖系统和胃肠道缺陷。第三例是一个胸腹联体儿,其中一个有唇腭裂、肺动脉狭窄和回盲瓣闭锁,而另一个未表现出这些异常。第四例也是胸腹联体双胞胎,其中一个有腰骶部脊髓脊膜膨出以及严重的胃肠道和泌尿生殖系统异常,而另一个未出现这些异常。第五例是一对胸腹联体双胞胎,共用一个心脏,存在不对称异常。第六例是一个双面无脑联体双胞胎。除了联体器官存在异常外,最初几对连体双胞胎在非共享器官上也存在几种异常不一致的情况。似乎连体双胞胎中异常不一致并非罕见现象。导致连体双胞胎异常不一致的因素可能与单卵双胞胎中的因素相似,即环境、遗传以及胎盘和/或胎儿循环异常。

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