Perinatology Unit, Zeynep Kamil Gynecologic and Pediatric Training Research Hospital, Health Sciences University, Istanbul, Turkey.
Pediatric Surgery Unit, Zeynep Kamil Gynecologic and Pediatric Training Research Hospital, Health Sciences University, Istanbul, Turkey.
J Matern Fetal Neonatal Med. 2022 Mar;35(5):837-845. doi: 10.1080/14767058.2020.1731466. Epub 2020 Apr 2.
Congenital arteriovenous intrahepatic fistulas, which are hepatic hemangiomas and arteriovenous malformations (AVMs) are rare and they confused with each other. Knowledge of prenatal medical treatment of AVMs is insufficient.
First is to emphasize the distinction between hepatic hemangioma and AVMs. Second is discussion of the first case of hepatic AVM that responded well to steroid-propranolol treatment in the prenatal period.
Color Doppler ultrasonography, fetal and postnatal MR were used for diagnosis.
The first case is a giant hepatic hemangioma diagnosed and progressively growing in the prenatal period and gradually shrinking in the postnatal period. The second case was hepatic AVM with no signs of heart failure during the prenatal period and postnatal right extended hepatectomy was performed as the anastomosis was enlarged and intraportal collateral vessels were developed. The third case is the first hepatic AVM which reaches a term that was prenatally diagnosed and responded to treatment with marked reduction.
Color flow and pulse Doppler imaging have a key role in the prenatal diagnosis of arteriovenous fistulas. Intrahepatic AVM are abnormal intrahepatic vascular network formation primarily fed by the hepatic artery or its branches and drained by the hepatic venous system. This vascular region looks like a mass, but it does not contain a solid area, which allows the separation of hepatic AVMs from hepatic hemangiomas. Steroid and propranolol therapy should be considered in management.
先天性肝动静脉内瘘(arteriovenous fistulas,AVFs),包括肝血管瘤和动静脉畸形(arteriovenous malformations,AVMs),较为罕见,且两者容易混淆。目前对于 AVMs 的产前治疗的认识还不足。
首先强调肝血管瘤和 AVMs 的区别。其次讨论第一例在产前对类固醇-普萘洛尔治疗有良好反应的肝 AVM 病例。
采用彩色多普勒超声、胎儿和产后磁共振成像(MR)进行诊断。
第一例为产前诊断的巨大肝血管瘤,在产前逐渐增大,产后逐渐缩小。第二例为肝 AVM,产前无心力衰竭迹象,产后行右半肝扩大切除术,因吻合口增大并出现门静脉侧支循环。第三例为首例产前诊断并经治疗明显缩小的肝 AVM。
彩色血流和脉冲多普勒成像在动静脉瘘的产前诊断中具有关键作用。肝 AVM 是主要由肝动脉或其分支供血、肝静脉系统引流的异常肝内血管网络形成。该血管区域看似肿块,但不含实体区域,这使得肝 AVM 与肝血管瘤得以区分。在管理上应考虑类固醇和普萘洛尔治疗。