Wang J, Luo J Y, Yang J, Yuan P B, Wang X J, Meng X L, Zhao Y Y, Wei Y
Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, China (Wang Jing is working on the Department of Obstetrics and Gynecology, Peking University International Hospital, Beijing 102206, China).
Department of Obstetrics, Fujian Provincal Maternity and Child Health Hospital, Fuzhou 350001, China.
Zhonghua Fu Chan Ke Za Zhi. 2019 Feb 25;54(2):87-92. doi: 10.3760/cma.j.issn.0529-567x.2019.02.003.
To analyze the clinical characteristics of structural malformations in one of monochorionic diamnionic twins (MCDA). The clinical data of 77 MCDA patients with structural malformations diagnosed by ultrasound were retrospectively reviewed from January 2012 to May 2017. The distribution of structural malformations, prenatal chromosomal karyotyping and pregnancy outcomes were analyzed. (1)Among the 77 MCDA patients with structural malformations, the single malformation accounted for 79%(61/77), the multiple malformations accounted for 21%(16/77). And there were a total of 94 types of malformations, the top three malformations were neurological malformations (32%, 30/94), cardiovascular malformations (29%, 27/94) and twin reversed arterial perfusion sequence (TRAPS;10%,9/94).(2)Among the 77 patients with structural malformation, 64 cases (83%, 64/77) were examined for fetal chromosomes, of whom 14 cases (22%,14/64) were examined for fetal chromosomes of both twins, with 1 case (1/14) of discordant fetal chromosome. (3)Among the 77 patients, 4 cases (5%, 4/77) with severe fetal malformations terminated pregnancy. Totally 29 cases (38%,29/77) with severe malformations were treated with selective fetal reduction, among whom 7 cases (24%, 7/29) experienced unexplained fetal death within 24 hours after the operation; 2 cases (7%, 2/29) happened inevitable abortion, and 2 cases (7%, 2/29) underwent unexplained fetal death during the late pregnancy. Of the remaining 44 patients (57%,44/77) with expectant treatment, 13 cases (30%,13/44) occurred twin transfusion syndrome (Ⅱ-Ⅳ), and were treated with fetoscopic laser occlusion. Eight patients had 2 survival twins, 4 patients delivered 1 survival twin, and 1 patient had dead twins. The most common malformations in MCDA twins are the nervous malformations, cardiovascular malformations and TRAPS. The chromosome karyotype of MCDA twins with structural malformations are sometimes discordant, and separate samling of the twins is suggested for prenatal diagnosis. Selective fetal reduction could be given to severe structural malformation in MCDA patients safely and effectively. For non-severe structural malformation in MCDA patients with twin transfusion syndrome, fetoscopic laser occlusion is safe and effective.
分析单绒毛膜双羊膜囊双胎（MCDA）之一结构畸形的临床特征。回顾性分析2012年1月至2017年5月间77例经超声诊断为结构畸形的MCDA患者的临床资料。分析结构畸形的分布、产前染色体核型及妊娠结局。（1）在77例有结构畸形的MCDA患者中，单发畸形占79%（61/77），多发畸形占21%（16/77）。共有94种畸形类型，前三位的畸形为神经系统畸形（32%，30/94）、心血管畸形（29%，27/94）和双胎反向动脉灌注序列（TRAPS；10%，9/94）。（2）在77例有结构畸形的患者中，64例（83%，64/77）进行了胎儿染色体检查，其中14例（22%，14/64）对双胎均进行了胎儿染色体检查，有1例（1/14）胎儿染色体不一致。（3）在77例患者中，4例（5%，4/77）因胎儿严重畸形终止妊娠。共有29例（38%，29/77）严重畸形患者接受了选择性减胎术，其中7例（24%，7/29）在术后24小时内发生不明原因胎儿死亡；2例（7%，2/29）发生难免流产，2例（7%，2/29）在妊娠晚期发生不明原因胎儿死亡。其余44例（57%，44/77）采取期待治疗，其中13例（30%，13/44）发生双胎输血综合征（Ⅱ - Ⅳ期），并接受了胎儿镜激光封堵治疗。8例患者双胎均存活，4例患者分娩出1例存活双胎，1例患者双胎均死亡。MCDA双胎最常见的畸形是神经系统畸形、心血管畸形和TRAPS。有结构畸形的MCDA双胎染色体核型有时不一致，建议双胎分别取样进行产前诊断。对于MCDA患者严重结构畸形可安全有效地进行选择性减胎术。对于合并双胎输血综合征的MCDA患者非严重结构畸形，胎儿镜激光封堵治疗安全有效。
