Division of Paediatric Respiratory Medicine and Allergology, Department of Paediatrics Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland.
Graduate School for Health Sciences, University of Bern, Bern, Switzerland.
Eur Radiol. 2024 Jun;34(6):3773-3785. doi: 10.1007/s00330-023-10395-8. Epub 2023 Nov 20.
In patients with congenital diaphragmatic hernia (CDH) the exact functional outcome of the affected lung side is still unknown, mainly due to the lack of spatially resolved diagnostic tools. Functional matrix-pencil decomposition (MP-) lung MRI fills this gap as it measures side-specific ventilation and perfusion. We aimed to assess the overall and side-specific pulmonary long-term outcomes of patients with CDH using lung function tests and MP-MRI.
Thirteen school-aged children with CDH (seven with small and six with large defect-sized CDH, defined as > 50% of the chest wall circumference being devoid of diaphragm tissue) and thirteen healthy matched controls underwent spirometry, multiple-breath washout, and MP-MRI. The main outcomes were forced expiratory volume in 1 second (FEV), lung clearance index (LCI), ventilation defect percentage (VDP), and perfusion defect percentage (QDP).
Patients with a large CDH showed significantly reduced overall lung function compared to healthy controls (mean difference [95%-CI]: FEV (z-score) -4.26 [-5.61, -2.92], FVC (z-score) -3.97 [-5.68, -2.26], LCI (TO) 1.12 [0.47, 1.76], VDP (%) 8.59 [3.58, 13.60], QDP (%) 17.22 [13.16, 21.27]) and to patients with a small CDH. Side-specific examination by MP-MRI revealed particularly reduced ipsilateral ventilation and perfusion in patients with a large CDH (mean difference to contralateral side [95%-CI]: VDP (%) 14.80 [10.50, 19.00], QDP (%) 23.50 [1.75, 45.20]).
Data indicate impaired overall lung function with particular limitation of the ipsilateral side in patients with a large CDH. MP-MRI is a promising tool to provide valuable side-specific functional information in the follow-up of patients with CDH.
In patients with congenital diaphragmatic hernia, easily applicable MP-MRI allows specific examination of the lung side affected by the hernia and provides valuable information on ventilation and perfusion with implications for clinical practice, making it a promising tool for routine follow-up.
• Functional matrix pencil decomposition (MP) MRI data from a small sample indicate reduced ipsilateral pulmonary ventilation and perfusion in children with large congenital diaphragmatic hernia (CDH). • Easily applicable pencil decomposition MRI provides valuable side-specific diagnostic information on lung ventilation and perfusion. This is a clear advantage over conventional lung function tests, helping to comprehensively follow up patients with congenital diaphragmatic hernia and monitor therapy effects.
在先天性膈疝（CDH）患者中，受累肺侧的确切功能预后仍不清楚，主要是因为缺乏空间分辨的诊断工具。功能矩阵-铅笔分解（MP-）肺部 MRI 填补了这一空白，因为它可以测量特定于肺侧的通气和灌注。我们旨在使用肺功能测试和 MP-MRI 评估 CDH 患者的整体和特定于肺侧的长期肺结局。
13 名学龄期 CDH 患儿（7 名小缺陷和 6 名大缺陷 CDH，定义为> 50%的胸壁周长没有膈肌组织）和 13 名健康匹配的对照组接受了肺活量测定、多次呼吸冲洗和 MP-MRI。主要结局是 1 秒用力呼气量（FEV）、肺清除指数（LCI）、通气缺陷百分比（VDP）和灌注缺陷百分比（QDP）。
与健康对照组相比，大 CDH 患儿的整体肺功能明显降低（平均差异[95%-CI]：FEV（z 分数）-4.26[-5.61，-2.92]，FVC（z 分数）-3.97[-5.68，-2.26]，LCI（TO）1.12[0.47，1.76]，VDP（%）8.59[3.58，13.60]，QDP（%）17.22[13.16，21.27]），且低于小 CDH 患儿。MP-MRI 的特定于肺侧的检查显示，大 CDH 患儿的患侧通气和灌注明显降低（与对侧相比的平均差异[95%-CI]：VDP（%）14.80[10.50，19.00]，QDP（%）23.50[1.75，45.20]）。
数据表明，大 CDH 患儿的整体肺功能受损，患侧尤其受限。MP-MRI 是一种很有前途的工具，可以为 CDH 患者的随访提供有价值的特定于肺侧的功能信息。
在先天性膈疝患者中，易于应用的 MP-MRI 可以对疝侧肺进行特定检查，并提供有关通气和灌注的有价值信息，对临床实践有影响，因此是常规随访的一种很有前途的工具。
•来自小样本的功能矩阵铅笔分解（MP）MRI 数据表明，患有大先天性膈疝（CDH）的儿童患侧肺的通气和灌注明显减少。•易于应用的铅笔分解 MRI 提供了有价值的特定于肺侧的肺通气和灌注的诊断信息。与传统的肺功能测试相比，这是一个明显的优势，可以全面随访先天性膈疝患者并监测治疗效果。
