Evidence map›Paper›PMID 41686267›Full record

ArticlePediatric surgery international2026

Long-Term pulmonary function outcomes in survivors of congenital diaphragmatic hernia.

Carolin Riemer, Philipp Utz, Florian Kipfmueller, Thomas Schaible, Eda Yildirim, Meike Weiss, Greta Thater, Christoph Mohr, Kaja Riebesell, Jana Hoffmann and 3 more

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Article in Pediatric surgery international, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

13 authors.

Carolin RiemerDepartment of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany. carolin.riemer@umm.de.
Philipp UtzDepartment of Pediatrics, University Medical Center Mannheim, Pediatric Pulmonology and Allergy Associates, University of Heidelberg, Hirschberg, Germany.
Florian KipfmuellerDepartment of Neonatology, University Medical Center Mannheim, Heidelberg University, Mannheim , Germany.
Thomas SchaibleDepartment of Neonatology, University Medical Center Mannheim, Heidelberg University, Mannheim , Germany.
Eda YildirimDepartment of Neurosurgery, Heidelberg University Hospital, University of Heidelberg, Mannheim, Germany.
Meike WeissDepartment of Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Heidelberg University, Mannheim , Germany.
Greta ThaterDepartment of Clinical Radiology and Nuclear Medicine, University Medical Center Mannheim, Heidelberg University, Mannheim , Germany.
Christoph MohrDepartment of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.
Kaja RiebesellDepartment of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.
Jana HoffmannDepartment of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.
Michael BoettcherDepartment of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.
Michaela Klinke *Department of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.
Julia Elrod *Department of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Mannheim, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundCongenital diaphragmatic hernia (CDH) is a rare developmental defect associated with pulmonary hypoplasia and long-term respiratory morbidity. Data on pulmonary function trajectories and structural predictors in CDH survivors remain limited. Objective of this study was to quantify long-term pulmonary dysfunction and its predictors in survivors of congenital diaphragmatic hernia (CDH).

methodsA prospective single-centre cohort of CDH survivors who underwent pulmonary function testing (PFT) between 2013 and 2023 was analysed. A total of 338 PFTs from 260 patients (57.7% male; birth years 2000–2017) were included; body plethysmography was available in 156 cases. Thoracic morphology was assessed by computed tomography or magnetic resonance imaging performed within six months of PFT. Spirometric and plethysmographic parameters were evaluated using z-scores derived from Global Lung Function Initiative reference equations. Associations with clinical characteristics, neonatal course, surgical technique, and imaging-derived thoracic indices were analysed using non-parametric testing, correlation analysis, and multiple linear regression.

resultsObstructive ventilatory impairment occurred in 24.6% (mild 20.6%, moderate 44.4%, severe 22.2%); restrictive ventilatory impairment in 22.4% (mild 51.4%, moderate 40.0%, severe 8.6%). Lower zFEV₁ was associated with prenatal diagnosis, right-sided hernia, large defect, liver herniation, extracorporeal membrane oxygenation (ECMO), open repair and patch closure (all p ≤ 0.003). Regression (R² = 0.32) showed + 0.51 zFEV₁ per kg birth weight; negative coefficients for large defect (-0.39), right-sided hernia (-0.57), ECMO (-0.64) and open repair (-0.97). Haller index > 2.7, correction index ≥ 0.1 or asymmetry index outside 0.95–1.05 further reduced zFEV₁ and/or zTLC (p ≤ 0.03). During 3–5-year follow-up (n = 50), lung function stabilised or improved in 82%, but progressive obstruction developed in 18%.

conclusionApproximately one quarter of CDH survivors exhibit persistent ventilatory deficits; defect severity, intensive therapy and chest-wall deformity independently predict poorer outcomes. The high incidence of long-term pulmonary impairment in CDH survivors supports the need for standardized long-term surveillance, particularly in children who already show pulmonary function impairment or other clinical signs consistent with respiratory limitation.

Indexed as

Hernias, Diaphragmatic, CongenitalLungFemaleHumansInfant, NewbornMaleProspective StudiesRespiratory Function TestsSurvivorsCDHCongenital diaphragmatic herniaObstructivePulmonary functionRestrictive

Identifiers

PMID41686267
PMCPMC12904957

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.