MacGillivray T E, Jennings R W, Rudolph A M, Ring E J, Adzick N S, Harrison M R
Department of Surgery, University of California, San Francisco 94143.
J Pediatr Surg. 1994 Aug;29(8):992-6. doi: 10.1016/0022-3468(94)90265-8.
In utero surgical correction of fetal diaphragmatic hernia is a new therapeutic alternative for selected prenatally diagnosed patients. With increasing experience, the authors have found that fetuses with herniation of the liver through the diaphragmatic defect have a high incidence of perioperative death. The hypothesis was that reduction of the liver during fetal diaphragmatic hernia repair caused distortion of fetal vascular anatomy resulting in fetal demise. To study this, the authors performed angiograms through the umbilical vein in fetal and neonatal cadavers, with and without diaphragmatic hernias, and simulated in utero repair. Large fetal congenital diaphragmatic hernias can be associated with dramatic changes in the vascular anatomy of the liver. The liver does not simply rotate up into the chest through a diaphragmatic defect; instead, the liver more likely develops in the chest, with its vascular anatomy situated accordingly. Attempts to reduce a herniated liver can result in significant distortion of the fetal vasculature, leading to fetal death. Techniques to prevent vascular compromise during hepatic manipulation may improve the outcome for fetuses during in utero repair of diaphragmatic hernias.
胎儿膈疝的宫内手术矫正对于部分产前诊断的患者来说是一种新的治疗选择。随着经验的增加，作者发现肝脏通过膈肌缺损发生疝出的胎儿围手术期死亡率很高。假说是在胎儿膈疝修复过程中肝脏复位导致胎儿血管解剖结构扭曲，从而导致胎儿死亡。为了研究这一点，作者在有或没有膈疝的胎儿及新生儿尸体上通过脐静脉进行血管造影，并模拟宫内修复。大型胎儿先天性膈疝可能与肝脏血管解剖结构的显著变化有关。肝脏并非简单地通过膈肌缺损向上旋转进入胸腔；相反，肝脏更有可能在胸腔内发育，其血管解剖结构也相应地处于该位置。试图将疝出的肝脏复位可能会导致胎儿血管系统严重扭曲，从而导致胎儿死亡。在肝脏操作过程中预防血管受损的技术可能会改善胎儿膈疝宫内修复的结局。
