Takeuchi Makoto, Nakayama Masahiro, Tamura Arihiro, Kitajima Hiroyuki
Department of Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health.
Pediatr Dev Pathol. 2009 May-Jun;12(3):239-43. doi: 10.2350/07-10-0363.1.
We describe the clinical course and autopsy findings of a male infant with hydrops fetalis due to agenesis of the ductus venosus. Fetal echocardiography at 27 weeks in gestation demonstrated hydrops fetalis due to unknown causes. The baby was born at 28 weeks in gestation by emergency caesarean section because of preeclampsia and progressive hydrop fetalis but died immediately at birth. The umbilical vein catheter ran an unusual course: left renal vein and inferior vena cava were opacified after postmortem injection of radiopaque dye into the umbilical vein. The autopsy demonstrated agenesis of the ductus venosus without extrahepatic umbilical venous drainage. The type without extrahepatic venous drainage is rare but shows a favorable outcome in general. However, our findings illustrate that in addition to sinusoidal dilatation, some cases may induce significant medial hypertrophy of portal veins, leading to hydrops fetalis and neonatal demise.
我们描述了一名因静脉导管缺如导致胎儿水肿的男婴的临床病程及尸检结果。妊娠27周时的胎儿超声心动图显示因不明原因导致的胎儿水肿。由于先兆子痫和进行性胎儿水肿，该婴儿在妊娠28周时通过紧急剖宫产出生，但出生后立即死亡。脐静脉导管走行异常：死后向脐静脉注射不透射线染料后，左肾静脉和下腔静脉显影。尸检显示静脉导管缺如，无肝外脐静脉引流。无肝外静脉引流的类型罕见，但总体预后良好。然而，我们的研究结果表明，除了窦状扩张外，一些病例可能会导致门静脉显著的中层肥厚，从而导致胎儿水肿和新生儿死亡。
