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先天性肺腺瘤样疾病:产前诊断与围产期管理

Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management.

作者信息

Sapin E, Lejeune V, Barbet JP, Carricaburu E, Lewin F, Baron JM, Barbotin-Larrieu F, Helardot PG

机构信息

Department of Pediatric Surgery, Saint Vincent De Paul's Hospital, 78 - 82, avenue Denfort-Rochereon, F-75674 Paris Cedex 14, France

出版信息

Pediatr Surg Int. 1997 Mar 21;12(2/3):126-9.

PMID:9069212
Abstract

Prenatal ultrasonographic (US) detection of congenital adenomatoid malformation (CAM) was made in 18 fetuses at 17 - 36 weeks' gestation and managed in our institution during a 10-year period (1985-1994). The lesion was left-sided in 13 cases, right-sided in 4, and bilateral in 1. According to Stocker's classification, 12 cases were type I, 4 type II, and 2 type III. The prenatal course was followed with serial US examinations in 13 cases; the size of the lesion was stable in 8 and decreased in 5. Mediastinal shift was usually observed, and amniotic fluid volume was increased in 4 cases. One fetus was aborted. Six infants presented with respiratory distress syndrome and required neonatal surgery; delayed surgery was performed in 9 cases. Spontaneous regression of the lesion was observed on follow-up in 2 cases. Surgery consisted in lobectomy in 8 cases and segmentectomy in 6. The presence of fetal hydrops, type III lesions, and bilateral lung involvement are prenatal factors known to be associated with a poor prognosis. However, this series and a review of the literature suggest that caution should be observed with regard to the initial impression when counseling the parents regarding prognosis.

摘要

在1985年至1994年的10年间,本机构对18例孕17至36周经产前超声(US)检查发现先天性腺瘤样畸形(CAM)的胎儿进行了管理。病变位于左侧13例,右侧4例,双侧1例。根据斯托克分类,12例为I型,4例为II型,2例为III型。13例通过系列超声检查对产前病程进行了随访;其中8例病变大小稳定,5例减小。通常观察到纵隔移位,4例羊水过多。1例胎儿流产。6例婴儿出现呼吸窘迫综合征,需要进行新生儿手术;9例延迟手术。随访中观察到2例病变自发消退。手术方式为肺叶切除术8例,肺段切除术6例。胎儿水肿、III型病变和双侧肺受累是已知与预后不良相关的产前因素。然而,本系列研究及文献综述表明,在向父母咨询预后时,对于初始印象应谨慎对待。

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