Department of Paediatric Surgery, Erasmus University Medical Centre, Sophia Children's Hospital, Rotterdam, the Netherlands.
Department of Paediatric Pulmonology, Erasmus University Medical Centre, Sophia Children's Hospital, Rotterdam, the Netherlands.
Pediatr Pulmonol. 2020 Nov;55(11):3152-3161. doi: 10.1002/ppul.25032. Epub 2020 Aug 26.
To date, no consensus has been reached on the optimal management of congenital lung abnormalities, and factors predicting postnatal outcome have not been identified. We developed an objective quantitative computed tomography (CT) scoring method, and assessed its value for clinical decision-making.
Volumetric CT-scans of all patients born with a congenital lung abnormality between January 1999 and 2018 were assessed. Lung disease was quantified using the newly-developed congenital lung abnormality quantification (CLAQ) scoring method. In 20 equidistant axial slices, cells of a square grid were scored according to the abnormality within. The scored CT parameters were used to predict development of symptoms, and SD scores for spirometry and exercise tolerance (Bruce treadmill test) at 8 years of age.
CT-scans of 124 patients with a median age of 5 months were scored. Clinical diagnoses included congenital pulmonary airway malformation (49%), bronchopulmonary sequestration (27%), congenital lobar overinflation (22%), and bronchogenic cyst (1%). Forty-four patients (35%) developed symptoms requiring surgery of whom 28 (22%) patients became symptomatic before a CT-scan was scheduled. Lesional hyperdensity was found as an important predictor of symptom development and decreased exercise tolerance. Using receiver operating characteristic analysis, an optimal cut-off value for developing symptoms was found at 18% total disease.
CT-quantification of congenital lung abnormalities using the CLAQ method is an objective and reproducible system to describe congenital lung abnormalities on chest CT. The risk for developing symptoms may increase when more than a single lung lobe is affected.
迄今为止，对于先天性肺异常的最佳治疗方法尚未达成共识，也未确定预测出生后结果的相关因素。我们开发了一种客观的定量 CT（computed tomography，计算机断层扫描）评分方法，并评估了其在临床决策中的价值。
评估了 1999 年 1 月至 2018 年期间所有患有先天性肺异常的患者的容积 CT 扫描。使用新开发的先天性肺异常量化（Congenital Lung Abnormality Quantification，CLAQ）评分方法对肺部疾病进行量化。在 20 个等距轴位切片中，根据异常情况对正方形网格中的细胞进行评分。使用评分的 CT 参数预测症状发展，并预测 8 岁时的肺功能和运动耐量（Bruce 跑步机测试）的 SD 分数。
对 124 名中位年龄为 5 个月的患者进行了 CT 扫描评分。临床诊断包括先天性肺气道畸形（49%）、支气管肺隔离症（27%）、先天性肺过度充气（22%）和支气管囊肿（1%）。44 名（35%）患者出现需要手术的症状，其中 28 名（22%）患者在安排 CT 扫描前出现症状。病变高密度被认为是症状发展和运动耐量下降的重要预测因素。使用受试者工作特征分析，发现出现症状的最佳截断值为 18%的总病变。
使用 CLAQ 方法对先天性肺异常进行 CT 定量是一种客观且可重复的方法，可用于描述胸部 CT 上的先天性肺异常。当多个肺叶受累时，出现症状的风险可能会增加。
