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先天性肺囊性腺瘤样畸形:一例报告并胚胎学回顾

Congenital cystic adenomatoid malformation of the lung: a case report with embryological review.

作者信息

Gosain Ratna, Motwani Rohini

机构信息

Department of Pathology, ESIC Medical College & Super Specialty Hospital, Sanathnagar, Hyderabad (Telangana), India.

Department of Anatomy, All India Institute of Medical Sciences, Bibinagar, Hyderabad (Telangana), India.

出版信息

Anat Cell Biol. 2022 Jun 30;55(2):264-268. doi: 10.5115/acb.21.260. Epub 2022 May 26.

Abstract

Congenital cystic adenomatoid malformation (CCAM) is a rare developmental dysplastic lesion that affects the fetal bronchial tree. Etiopathogenesis is still poorly understood. Most accepted view is that of abnormal branching of bronchioles during the period of morphogenesis. We observed a rare congenital anomaly of the lungs during fetal autopsy. Routine antenatal ultrasonography revealed multiple echolucent cysts in the right lung of the fetus. Thorough external and internal examination was followed by sectioning of each organ for histopathological examination. Histopathology of the right lung showed distortion of the parenchyma with dilated bronchioles. Multiple cysts lined by columnar epithelium along with loose intervening connective tissue were observed along with many congested and dilated blood vessels. Knowledge of congenital anomalies of the respiratory system would help clinicians to plan the management at a very early stage. Accurate fetal autopsy along with clinical data is important in evaluating fetal deaths and can help in reducing unexplained stillbirths.

摘要

先天性囊性腺瘤样畸形(CCAM)是一种罕见的发育异常性病变,累及胎儿支气管树。其病因发病机制仍知之甚少。最被广泛接受的观点是,在形态发生期细支气管发生异常分支。我们在胎儿尸检过程中观察到一种罕见的肺部先天性异常。常规产前超声检查显示胎儿右肺有多个无回声囊肿。在进行全面的外部和内部检查后,将每个器官切片进行组织病理学检查。右肺组织病理学显示实质扭曲,细支气管扩张。观察到多个由柱状上皮细胞衬里的囊肿,其间有疏松的结缔组织,还有许多充血和扩张的血管。了解呼吸系统的先天性异常有助于临床医生在早期阶段制定治疗方案。准确的胎儿尸检以及临床数据对于评估胎儿死亡很重要,有助于减少不明原因的死产。

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