Department of Paediatric Surgery and Urology, Robert Debré Hospital, 75019 Paris, France.
J Pediatr Surg. 2012 Apr;47(4):642-5. doi: 10.1016/j.jpedsurg.2011.08.002.
The extent of surgical resection in the management of congenital cystic adenomatoid malformation (CCAM) located in 1 pulmonary lobe remains controversial. Although lobectomy seems to be the best treatment to avoid recurrent pulmonary infection and neoplasia, neither retrospective nor prospective studies have been done to our knowledge. Besides, many reports claim that there are advantages of parenchyma-saving resections. The aim of our study was to determine whether it was necessary to perform an extended resection in these cases.
We retrospectively reviewed all patients who had undergone a thoracoscopic lobectomy for CCAM located to 1 pulmonary lobe between January 2007 and January 2009. A thoracic radiologist performed a single blind review of all preoperative computed tomographic (CT) scans, mentioning the presence or absence of distant lesions from the main cysts of CCAM within the pulmonary lobe. The pathologist who analyzed the pulmonary lobectomy specimen was aware of the diagnosis but not the CT report.
Twenty-five patients underwent thoracoscopic lobectomy for congenital lung malformations, including 12 with CCAM. All had a positive prenatal sonogram. The median age at surgery was 12 months (range, 2-24 months). The preoperative CT showed only 2 cases with distant lesions within the affected pulmonary lobe, whereas the histologic study of the surgical specimen identified 6 cases. The sensitivity of the CT scan was low, 33%, whereas its specificity was high, 100%. Furthermore, the preoperative CT negative predictive value was 60%. The median time of follow-up was 7.5 months.
Adequate treatment of CCAM in children requires a lobectomy because of poor sensitivity and very poor negative predictive value (60%) of the preoperative CT to determine distal adjacent lesions. A prospective study on a larger number of patients is required to confirm these observations.
在 1 个肺叶中先天性囊性腺瘤样畸形（CCAM）的手术切除范围仍存在争议。虽然肺叶切除术似乎是避免复发性肺部感染和肿瘤的最佳治疗方法，但据我们所知，既没有回顾性研究，也没有前瞻性研究。此外，许多报道声称保肺切除术有优势。我们的研究目的是确定在这些情况下是否有必要进行广泛切除。
我们回顾性分析了 2007 年 1 月至 2009 年 1 月期间因 CCAM 位于 1 个肺叶而接受胸腔镜肺叶切除术的所有患者。一名胸部放射科医生对所有术前 CT 扫描进行了单次盲法复查，提到肺叶内 CCAM 主要囊肿是否存在远处病变。分析肺叶切除术标本的病理学家知道诊断结果，但不知道 CT 报告。
25 例患者因先天性肺畸形行胸腔镜肺叶切除术，其中 12 例为 CCAM。所有患者均有阳性产前超声检查结果。手术时的中位年龄为 12 个月（范围 2-24 个月）。术前 CT 仅显示 2 例肺叶内有远处病变，而手术标本的组织学研究发现 6 例。CT 扫描的敏感性较低，为 33%，特异性较高，为 100%。此外，术前 CT 的阴性预测值为 60%。中位随访时间为 7.5 个月。
儿童 CCAM 的充分治疗需要进行肺叶切除术，因为术前 CT 确定远端相邻病变的敏感性差，阴性预测值非常差（60%）。需要对更多患者进行前瞻性研究以证实这些观察结果。
