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先天性主动脉弓异常与血管环(作者译)

[Congenital aortic arch anomalies and vascular rings (author's transl)].

作者信息

Vincent J, Lacquet L K, Kuijpers P J, Brom A G

出版信息

Acta Chir Belg. 1975 May;74(3):305-16.

PMID:1224893
Abstract

The anomalies of the aortic arch are consequences of malformations in the first twelve weeks of the intrauterine life. Embryologically it is possible to explain and classify all of them. Most remain asymptomatic and do not need treatment. About one third of all anomalies are causing tracheo-esophageal compression with consequent complaints. In most cases the symptomatology starts in the first days of life. Some of them can be temporarily treated conservatively. The prognosis of patients, who need surgery is good, even in small infants. Surgery consists mainly in section of the atretic or hypoplastic part of the double aortic arch, section of the aberrant subclavian artery, section of the Botal ligament or duct and liberation of the trachea and esophagus. A normal configuration of trachea and esophagus is reached after several months or even more than a year. There are no reports on reoperations for secondary strictures. In the University Hospital of Nijmegen and Leiden we operated on 17 patients. There were 8 patients of group I A --- Edwards classification -- double aortic arch with both aortic arches open and left Botal duct or ligament; 4 patients of group I B, double aortic arch with one atretic aortic arch with a left Botal ligament; 3 patients of group II B, with a left aortic arch and aberrant right subclavian artery and a left Botal ligament; 2 patients of group III B with a right aortic arch and an aberrant left subclavian artery and a left Botal duct or ligament. Of our 17 patients one died during the operation because of a haemorrhage and overtransfusion; an other one died three weeks postoperatively from an endotracheal bleeding. All the other patients are without complaints and remained so.

摘要

主动脉弓异常是子宫内生命最初十二周内畸形的后果。从胚胎学角度可以对所有这些异常进行解释和分类。大多数异常无症状,无需治疗。所有异常中约三分之一会导致气管食管受压,从而引发相应症状。在大多数情况下,症状在出生后的头几天就会出现。其中一些可以通过保守治疗暂时缓解。即使是小婴儿,需要手术治疗的患者预后也良好。手术主要包括切断双主动脉弓的闭锁或发育不全部分、切断迷走锁骨下动脉、切断动脉导管韧带或动脉导管以及松解气管和食管。数月甚至一年多后,气管和食管可恢复正常形态。目前尚无关于二次狭窄再次手术的报道。在奈梅亨和莱顿大学医院,我们为17例患者进行了手术。其中,根据爱德华兹分类法,有8例属于IA组——双主动脉弓,两个主动脉弓均开放,伴有左侧动脉导管韧带或动脉导管;4例属于IB组,双主动脉弓,其中一个主动脉弓闭锁,伴有左侧动脉导管韧带;3例属于IIB组,有左主动脉弓、迷走右锁骨下动脉和左侧动脉导管韧带;2例属于IIIB组,有右主动脉弓、迷走左锁骨下动脉和左侧动脉导管或动脉导管韧带。我们的17例患者中,1例因出血和输血过多在手术中死亡;另1例在术后三周因气管内出血死亡。其他所有患者均无不适症状,且一直保持良好状态。

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