Peranteau William H, Merchant Aziz M, Hedrick Holly L, Liechty Kenneth W, Howell Lori J, Flake Alan W, Wilson R Douglas, Johnson Mark P, Bebbington Michael W, Adzick N Scott
Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Fetal Diagn Ther. 2008;24(3):190-6. doi: 10.1159/000151337. Epub 2008 Aug 28.
Peripheral bronchial atresia (PBA), a newly identified fetal lung lesion, is often asymptomatic and managed nonoperatively. However, recent studies suggest that bronchial atresia plays a role in the etiology of microcystic maldevelopment present in congenital cystic adenomatoid malformations (CCAM) which require resection to decrease the risk of infection, pneumothorax and malignant degeneration. The purpose of this study was to evaluate the prenatal radiographic and postnatal computed tomography (CT) scan/pathology findings with attention to the pathologic diagnosis of microcystic maldevelopment in infants with the presumptive diagnosis of PBA.
A retrospective review of prenatal and postnatal records of patients diagnosed with fetal lung lesions was performed. Two groups of patients were identified: (1) patients diagnosed with PBA on postnatal CT scan (n = 16), and (2) patients with the pathologically confirmed diagnosis of PBA independent of postnatal CT findings (n = 23).
Prenatal ultrasound diagnosis of these lesions included CCAMs, hybrid lesions, bronchopulmonary sequestrations and bronchial atresia. Eleven of the 16 patients in group 1 with the postnatal radiologic diagnosis of PBA underwent surgical resection, 6 of which were found to have microcystic changes consistent with CCAM. Evaluation of the 23 patients in group 2 with pathologically confirmed PBA identified 65% that had evidence of microcystic maldevelopment consistent with the small cyst type of CCAM.
Radiographically diagnosed PBA as well as pathologically confirmed PBA is frequently associated with microcystic changes consistent with the small cyst type of CCAM. Thus, operative management should be considered for PBA to decrease CCAM-associated risks.
外周性支气管闭锁（PBA）是一种新发现的胎儿肺部病变，通常无症状且无需手术治疗。然而，最近的研究表明，支气管闭锁在先天性囊性腺瘤样畸形（CCAM）中存在的微囊性发育不良的病因中起作用，而CCAM需要切除以降低感染、气胸和恶性变的风险。本研究的目的是评估产前影像学及产后计算机断层扫描（CT）和病理检查结果，重点关注疑似诊断为PBA的婴儿微囊性发育不良的病理诊断。
对诊断为胎儿肺部病变的患者的产前和产后记录进行回顾性分析。确定了两组患者：（1）产后CT扫描诊断为PBA的患者（n = 16），以及（2）病理确诊为PBA且与产后CT结果无关的患者（n = 23）。
这些病变的产前超声诊断包括CCAM、混合型病变、肺隔离症和支气管闭锁。第1组中16例产后影像学诊断为PBA的患者中有11例接受了手术切除，其中6例发现有与CCAM一致的微囊性改变。对第2组中23例病理确诊为PBA的患者进行评估，发现65%有与小囊肿型CCAM一致的微囊性发育不良的证据。
影像学诊断的PBA以及病理确诊的PBA常与小囊肿型CCAM一致的微囊性改变相关。因此，对于PBA应考虑手术治疗以降低与CCAM相关的风险。
