Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele

Correlation between the presence of liver herniation and perinatal outcome in prenatally diagnosed fetal omphalocele
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DOI:
10.1515/jpm.2009.019
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发表时间:
2009-01-01
影响因子:
2.4
通讯作者:
Wake, Norio
Wake, Norio
中科院分区:
医学4区
文献类型:
--
作者:
Hidaka, Nobuhiro;Tsukimori, Kiyomi;Wake, Norio

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目的:目的:探讨肝疝的存在与胎儿脐膨出的围生期病程及预后的关系。方法:回顾性分析1990 ~ 2006年我院收治的33例胎儿脐膨出病例,根据肝疝的部位进行分组。对33例胎儿中的29例进行了染色体检查。体外肝移植组的染色体异常率(2/18)显著低于肝移植组(6/11)(P = 0.028)。在染色体正常的病例中,4例体外肝移植导致早期新生儿死亡,而肝内移植则无一例。21例染色体正常的胎儿中有5例羊水量异常。所有5例体外肝脏,其中两个导致新生儿death.Conclusions:胎儿体外肝脏染色体异常率较低,比那些在intracerebral肝组。然而,在染色体正常的情况下,与体内肝脏组相比,体外肝脏似乎可能会导致更多危及生命的异常、羊水量异常以及更高的死亡率。诊断胎儿脐膨出后,咨询前应仔细寻找肝脏位置。
Aims: To investigate the association between the presence of liver herniation and perinatal course and outcome of fetal omphalocele.Methods: Cases of fetal omphalocele managed at our hospital between 1990 and 2006 were retrospectively reviewed and grouped according to the location of the liver.Results: Thirty-three fetal omphalocele cases were diagnosed. The chromosomal status of 29 of 33 fetuses was determined. The rate of chromosomal abnormalities in cases with an extracorporeal liver was significantly lower (2/18) than in the intracorporeal group (6/11) (P = 0.028). In chromosomally normal cases, four with extracorporeal liver resulted in early neonatal death compared to none with intracorporeal liver. Five of the 21 chromosomally normal fetuses showed an abnormal volume of amniotic fluid. All five cases had extracorporeal liver and two of them resulted in neonatal death.Conclusions: Fetuses with an extracorporeal liver had a lower rate of chromosomal abnormalities than those in the intracorporeal liver group. However, in chromosomally normal cases, it appeared that extracorporeal livers might be associated with more life-threatening anomalies, amniotic fluid volume abnormalities, and a higher rate of mortality than in the group with an intracorporeal liver. Upon diagnosis of fetal omphalocele, a careful search for liver location should be conducted before counseling.