Hamanaka Rurika, Yagasaki Hidehiko, Kohno Mitsutomo, Masuda Ryota, Iwazaki Masayuki
Division of General Thoracic Surgery, Department of Surgery, Tokai University School of Medicine, Japan.
Respir Med Case Rep. 2018 Feb 25;26:328-332. doi: 10.1016/j.rmcr.2018.02.002. eCollection 2019.
Congenital cystic adenomatoid malformation (CCAM) is a congenital pulmonary cystic disease that is mostly detected and diagnosed prenatally or during the neonatal period, while rarely being observed in adults. Here, we report an adult case of CCAM that was diagnosed following surgery for a recurrent pneumothorax. We further review 60 case reports on adult CCAM that have been previously published. The patient was a 29-year-old woman with a severe left pneumothorax. Her computed tomography scan showed the presence of multiple pulmonary cysts at the base of the left lower lobe. Since she had experienced a left pneumothorax twice previously, surgery was indicated. A wedge lung resection of the pulmonary cysts was performed thoracoscopically. The postoperative pathological diagnosis was type I CCAM. From the review, 7 adult CCAM patients (11.7%) out of 61, including the patient in the present case, presented with pneumothorax, while 21 patients (35%) presented with infection. Thirty-nine foci of CCAM (65%) were located in lower lobes. Moreover, malignancies were associated in 8 cases (13.3%). We propose that if multicystic lung lesions are found in pneumothorax patients, particularly in lower lobes, CCAM should be considered during the differential diagnosis, even in adults.
先天性囊性腺瘤样畸形（CCAM）是一种先天性肺囊性疾病，大多在产前或新生儿期被检测和诊断，而在成人中很少见。在此，我们报告一例因复发性气胸手术后确诊的成人CCAM病例。我们还进一步回顾了此前发表的60例成人CCAM病例报告。患者为一名29岁女性，患有严重的左侧气胸。她的计算机断层扫描显示左下叶底部存在多个肺囊肿。由于她之前曾两次发生左侧气胸，因此建议进行手术。通过胸腔镜对肺囊肿进行了楔形肺切除术。术后病理诊断为I型CCAM。通过回顾发现，61例成人CCAM患者（包括本病例患者）中有7例（11.7%）出现气胸，21例（35%）出现感染。39个CCAM病灶（65%）位于下叶。此外，8例（13.3%）伴有恶性肿瘤。我们建议，如果在气胸患者中发现多囊性肺病变，特别是在下叶，即使是成人，在鉴别诊断时也应考虑CCAM。
