Wagner D S, Klein R L, Robinson H B, Novak R W
Department of Surgery, Akron City Hospital, OH.
J Pediatr Surg. 1990 May;25(5):538-42. doi: 10.1016/0022-3468(90)90568-t.
A syndrome in monozygotic twins that consists of a macerated twin fetus (fetus papyraceous) and a live-born twin with various anatomical defects has been described. The etiology is thought to be placental transfer of emboli or thromboplastic material through vascular shunts. Thromboplastic material precipitates disseminated intravascular coagulation (DIC) in the fetus, with a resultant hypercoagulable state due to relative fetal antithrombin III deficiency. Two cases of this syndrome will be discussed. The case of a live-born twin with intestinal atresia, who developed in utero with a fetus papyraceous, is reported. Emboli were demonstrated in vascular shunts of the diamniotic-monochorionic placenta. The hypothesis of intestinal atresia as a result of a vascular accident is reviewed. Another case involving a live-born twin with congenital skin defects, who developed in utero with a fetus papyraceous, is also reported. The skin defects were a congenital disruption from fetal DIC with resultant hypercoagulable state. Several other manifestations of the placental emboli syndrome will be discussed and the vascular etiology of the disruptions explained.
已描述了一种单卵双胞胎综合征，其包括一个浸软的双胎胎儿（纸样胎儿）和一个患有各种解剖学缺陷的活产双胞胎。病因被认为是栓子或促凝物质通过血管分流进行胎盘转移。促凝物质在胎儿体内引发弥散性血管内凝血（DIC），由于胎儿相对抗凝血酶III缺乏，导致高凝状态。将讨论该综合征的两个病例。报告了一例活产双胞胎，其患有肠道闭锁，在子宫内与一个纸样胎儿共同发育。在双羊膜-单绒毛膜胎盘的血管分流中发现了栓子。对因血管意外导致肠道闭锁的假说进行了综述。还报告了另一例病例，涉及一个患有先天性皮肤缺陷的活产双胞胎，其在子宫内与一个纸样胎儿共同发育。皮肤缺陷是胎儿DIC导致的先天性破坏，进而产生高凝状态。将讨论胎盘栓子综合征的其他几种表现，并解释破坏的血管病因。
