Han Zhonglong, Yu Tong, Duan Xiaomin, Liu Dingyi, Luo Huanyu, Peng Yun
Department of Radiology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, No. 56 Nanlishi Road, Xicheng District, Beijing, 100045, China.
Department of Thoracic Surgery, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Pediatr Radiol. 2025 Jul;55(8):1652-1668. doi: 10.1007/s00247-025-06290-7. Epub 2025 Jun 23.
Identifying the blood supply from a systemic artery is crucial for diagnosing extralobar pulmonary sequestration. Contrast-enhanced computed tomography (CT) can accurately delineate the origin and course of anomalous systemic arteries and venous drainage. However, it remains challenging to make an accurate diagnosis in cases with atypical CT manifestations.
To improve our understanding of the diversity of extralobar pulmonary sequestration and facilitate a more accurate diagnosis, especially for cases with atypical CT manifestations.
A retrospective review was conducted on 58 patients who had confirmed extralobar pulmonary sequestration by surgery between November 2019 and December 2023. Patient demographics, clinical manifestations, and CT findings were reviewed.
Among the 58 patients, 38 (65.5%) were male, and the left-to-right ratio was 47 to 11. It typically appeared triangular or polygonal, with the sharp angular sign observed in 55 patients (94.8%). The low-density branch sign was identified in 45 patients (77.6%) via contrast-enhanced CT. Lesions were located as follows: above the diaphragm in 45 cases (77.6%), within the diaphragm in seven cases (12.1%), and below the diaphragm in two cases (3.4%), presenting as solid masses; two instances (3.4%) involved anterior mediastinum lesions that presented as cystic-solid masses. In two cases (3.4%), the sequestered lung communicated with the lower esophagus, presenting as multiple air-filled cystic lesions. Abnormal arterial blood supply was detected in 54 patients (93.1%), whereas venous drainage was identified in 52 patients (89.7%).
The presence of the sharp angular sign in a solid mass with clear boundaries suggests a diagnosis of extralobar pulmonary sequestration on routine CT, regardless of its location above, within, or below the diaphragm. CT angiography is a reliable imaging modality for documenting feeding arteries and draining veins, which confirms the diagnosis of extralobar pulmonary sequestration. The low-density branch sign on contrast-enhanced CT indicates that the lesion comprises lung tissue and helps establish a diagnosis of pulmonary sequestration.
识别来自体循环动脉的血供对于诊断肺叶外型肺隔离症至关重要。对比增强计算机断层扫描（CT）能够准确描绘异常体循环动脉的起源和走行以及静脉引流情况。然而，对于CT表现不典型的病例，准确诊断仍具有挑战性。
提高我们对肺叶外型肺隔离症多样性的认识，并促进更准确的诊断，尤其是对于CT表现不典型的病例。
对2019年11月至2023年12月期间58例经手术确诊为肺叶外型肺隔离症的患者进行回顾性研究。回顾患者的人口统计学资料、临床表现和CT检查结果。
58例患者中，男性38例（65.5%），左右比例为47比11。其典型表现为三角形或多边形，55例患者（94.8%）可见锐角征。45例患者（77.6%）通过对比增强CT发现低密度分支征。病变位置如下：膈上45例（77.6%），膈内7例（12.1%），膈下2例（3.4%），表现为实性肿块；2例（3.4%）累及前纵隔病变，表现为囊实性肿块。2例（3.4%）中，隔离肺与下食管相通，表现为多个含气囊性病变。54例患者（93.1%）检测到异常动脉血供，52例患者（89.7%）发现静脉引流。
边界清晰的实性肿块中出现锐角征提示在常规CT上可诊断为肺叶外型肺隔离症，无论其位于膈上、膈内还是膈下。CT血管造影是记录供血动脉和引流静脉的可靠成像方式，可确诊肺叶外型肺隔离症。对比增强CT上的低密度分支征表明病变由肺组织构成，有助于确立肺隔离症的诊断。
