Francis Babu, Hallam Lavinia, Kecskes Zsuzsoka, Ellwood David, Croaker David, Kent Alison
Department of Neonatology, Fetal Medicine Unit, The Canberra Hospital, Woden, and Australian National University Medical School, Canberra, ACT, Australia.
Pediatr Dev Pathol. 2007 Jan-Feb;10(1):50-4. doi: 10.2350/06-03-0066.1.
Placental mesenchymal dysplasia is an uncommon disorder in which the placenta is enlarged with abnormal, large, and often cystic villi with dilated and/or thick-walled vessels. These placental changes can mimic a partial hydatidiform mole but in contrast to a partial mole can coexist with a fully viable fetus. Fetal anatomical and vascular anomalies frequently coexist with placental mesenchymal dysplasia. In this case, placental mesenchymal dysplasia was associated with preterm labor at 33 weeks' gestation, fetal compromise, and a large abdominal mass with a large hepatic cyst that was de-roofed at exploratory laparotomy. The neonate remained critically ill with hypoxic ischaemic encephalopathy and coagulopathy and died despite intensive care. Biopsy and autopsy findings showed a large cystic mesenchymal hamartoma affecting the left lobe of the liver. This appears to be the 3rd histologically confirmed association of placental mesenchymal dysplasia with mesenchymal hamartoma of the liver in the English language literature.
胎盘间质性发育异常是一种罕见的疾病，其胎盘增大，伴有异常、巨大且常为囊性的绒毛以及扩张和/或壁厚的血管。这些胎盘变化可类似部分性葡萄胎，但与部分性葡萄胎不同的是，它可与完全存活的胎儿共存。胎儿解剖和血管异常常与胎盘间质性发育异常并存。在该病例中，胎盘间质性发育异常与妊娠33周时的早产、胎儿窘迫以及一个巨大的腹部肿块相关，该肿块内有一个大的肝囊肿，在剖腹探查时进行了囊肿去顶术。新生儿因缺氧缺血性脑病和凝血病病情危重，尽管进行了重症监护仍死亡。活检和尸检结果显示为一个累及肝左叶的巨大囊性间质性错构瘤。这似乎是英文文献中第3例经组织学证实的胎盘间质性发育异常与肝间质性错构瘤的关联。
