Hardee Steven, Tuzovic Lea, Silva Cicero T, Cowles Robert A, Copel Joshua, Morotti Raffaella A
1 Department of Pathology, Yale School of Medicine, New Haven, Connecticut, USA.
2 Department of Obstetrics, Gynecology and Reproductive Sciences, Yale School of Medicine, New Haven, Connecticut, USA.
Pediatr Dev Pathol. 2017 Sep-Oct;20(5):403-410. doi: 10.1177/1093526617698604. Epub 2017 Mar 15.
Congenital cystic lung lesions are a group of rare pathologies that are usually diagnosed in the prenatal period. The majority of these lesions are diagnosed at pathology examination as congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestration (BPS). These lesions are typically managed by surgical intervention within the first year of life and have an excellent prognosis. We examined the evolution of imaging appearances from prenatal diagnosis to postnatal work-up of these lesions and correlate imaging and pathological findings. An 8-year retrospective review of the perinatal and pathology database of a single tertiary care center identified 42 cases of congenital cystic lung lesions of which 36 had known prenatal ultrasound and prenatal course available. Final pathologic diagnoses were 15 CPAM (41%), 7 BPS (19%), and 9 hybrid BPS and CPAM lesions (25%). Five cases with bronchial atresia were also identified (either in isolation or associated with CPAM or BPS). The overall characteristics of these lesions by prenatal ultrasound, postnatal imaging, and ultimate histopathologic diagnosis are described.
先天性肺囊性病变是一组罕见的病理情况，通常在孕期被诊断出来。这些病变在病理检查中大多被诊断为先天性肺气道畸形（CPAM）和肺隔离症（BPS）。这些病变通常在出生后第一年内通过手术干预进行治疗，预后良好。我们研究了这些病变从产前诊断到产后检查的影像学表现演变，并将影像学和病理结果进行关联。对一家三级医疗中心围产期和病理数据库进行的一项为期8年的回顾性研究，确定了42例先天性肺囊性病变病例，其中36例有已知的产前超声检查和产前病程记录。最终病理诊断为15例CPAM（41%），7例BPS（19%），9例BPS与CPAM混合性病变（25%）。还发现了5例支气管闭锁病例（单独存在或与CPAM或BPS相关）。描述了这些病变通过产前超声、产后影像学检查以及最终组织病理学诊断的总体特征。
