40例先天性肺发育异常病例系列及先天性囊性腺瘤样畸形病变比率的信息价值

A Series of 40 Congenital Lung Malformation Cases and the Informative Value of CPAM Lesion Ratios.

作者信息

Le Melanie, Harms Phillip, Peldschus Kersten, Junge Carl-Martin, Tomuschat Christian, Reinshagen Konrad

机构信息

Clinic for Pediatric Surgery, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.

Department of Pediatric Radiology, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.

出版信息

Pediatr Rep. 2025 Jan 9;17(1):5. doi: 10.3390/pediatric17010005.

Abstract

INTRODUCTION

In this single-center retrospective analysis, we present case data and insights gathered over the past eight years. Additionally, we computed postnatal, pre-therapy lesion-to-lung ratios of Congenital Pulmonary Airway Malformations (CPAMs) to retrospectively assess potential outcome prediction using lesion-to-lung ratios.

METHODS

Data were collected between 2015 and 2022. Information such as chosen therapy, surgical duration, postoperative hospital stay, and follow-up was obtained from electronic case records. Pre-therapy pulmonary lesion volumes [mm], lesion-to-ipsilateral-lung ratio, and lesion-to-both-lungs ratio of CPAMs were retrospectively calculated from computed tomography images using specialized software.

RESULTS

Of the 40 identified cases, 27 had CPAM, 7 had pulmonary sequestration, 4 had bronchogenic cysts, and 2 had congenital lobar emphysema. Histological examinations of resected specimens revealed no malignancy. For CPAMs, the median surgery age was 7 months (interquartile range (IQR): 0.45-11), averaging 9.54 ± 15.01 months. The CPAM surgery lasted on average 126 ± 53 min (median 124 min (IQR 108-172)). The mean length of hospital stay was 6 ± 1.41 days for thoracoscopic surgery and 17 ± 18.23 days for open surgery. No clear link was found between the lesion ratio and management choice or surgical length. Notably, larger lesions tended to result in longer postoperative stays. CPAMs with a lesion-to-ipsilateral-lung ratio of ≤0.11 were asymptomatic.

DISCUSSION AND CONCLUSIONS

If patients present no symptoms, mild symptoms, or smaller CPAM lesions, "wait and watch" and a CT scan of the thorax up to approximately six months of age remain a reasonable approach. The true risk of malignancy remains ambiguous, especially since there was no evidence of malignancy in our biopsies. On the other hand, prophylactic surgery before symptoms arose led to earlier discharge and overall low intraoperative complications compared to symptomatic counterparts. Ultimately, the adopted therapy pathway remains a parental choice. For CPAMs, an increased lesion-to-lung ratio correlated with extended hospital stay and symptomatic presentation. However, there was no cut-off value for conservative or surgical treatment.

摘要

引言

在这项单中心回顾性分析中,我们展示了过去八年收集的病例数据和见解。此外,我们计算了先天性肺气道畸形(CPAM)的产后、治疗前病变与肺的比率,以回顾性地评估使用病变与肺比率进行潜在结果预测的情况。

方法

收集了2015年至2022年的数据。从电子病例记录中获取了所选治疗方法、手术时长、术后住院时间和随访等信息。使用专门软件从计算机断层扫描图像中回顾性计算CPAM的治疗前肺病变体积[mm]、病变与同侧肺的比率以及病变与双肺的比率。

结果

在40例确诊病例中,27例为CPAM,7例为肺隔离症,4例为支气管囊肿,2例为先天性大叶性肺气肿。切除标本的组织学检查未发现恶性肿瘤。对于CPAM,中位手术年龄为7个月(四分位间距(IQR):0.45 - 11),平均为9.54 ± 15.01个月。CPAM手术平均持续126 ± 53分钟(中位值124分钟(IQR 108 - 172))。胸腔镜手术的平均住院时间为6 ± 1.41天,开放手术为17 ± 18.23天。未发现病变比率与治疗选择或手术时长之间存在明确关联。值得注意的是,较大的病变往往导致术后住院时间更长。病变与同侧肺比率≤0.11的CPAM无症状。

讨论与结论

如果患者无症状、症状轻微或CPAM病变较小,“观察等待”并在大约六个月大时进行胸部CT扫描仍是一种合理的方法。恶性肿瘤的真正风险仍不明确,特别是因为我们的活检中没有恶性肿瘤的证据。另一方面,与有症状的患者相比,在症状出现前进行预防性手术可使患者更早出院,且术中总体并发症较低。最终,所采用的治疗途径仍是家长的选择。对于CPAM而言,病变与肺比率增加与住院时间延长和症状表现相关。然而,保守治疗或手术治疗没有临界值。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4a89/11755569/d5640ba142c5/pediatrrep-17-00005-g001.jpg

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