Eur Radiol

. 2023 Nov 20.

 doi: 10.1007/s00330-023-10395-8. Online ahead of print. https://pubmed.ncbi.nlm.nih.gov/37982833/

Long-term pulmonary outcome of children with congenital diaphragmatic hernia: functional lung MRI using matrix-pencil decomposition enables side-specific assessment of lung function

Carmen Streibel ^1 2, C Corin Willers ^3 4, Grzegorz Bauman ^5 6, Orso Pusterla ^7 5 6, Oliver Bieri ^5 6, Marion Curdy ⁷, Matthias Horn ⁷, Carmen Casaulta ⁷, Steffen Berger ⁸, Gabriela Marta Dekany ⁸, Elisabeth Kieninger ⁷, Andreas Bartenstein ^# 8, Philipp Latzin ^# 9

Affiliations expand

• PMID: 37982833
• DOI: 10.1007/s00330-023-10395-8

Abstract

Objectives: 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.

Methods: 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 (FEV₁), lung clearance index (LCI_2.5), ventilation defect percentage (VDP), and perfusion defect percentage (QDP).

Results: Patients with a large CDH showed significantly reduced overall lung function compared to healthy controls (mean difference [95%-CI_adjusted]: FEV₁ (z-score) -4.26 [-5.61, -2.92], FVC (z-score) -3.97 [-5.68, -2.26], LCI_2.5 (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_adjusted]: VDP (%) 14.80 [10.50, 19.00], QDP (%) 23.50 [1.75, 45.20]).

Conclusions: 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.

Clinical relevance statement: 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.

Key points: • 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.

Keywords: Children; Congenital diaphragmatic hernias; Functional magnetic resonance imaging; Lung; Pulmonary function test.

© 2023. The Author(s).