Unlabelled: To evaluate the effectiveness of a novel protocol, adopted in our institution, as a quality improvement project for congenital diaphragmatic hernia (CDH). A maximal lung protection (MLP) protocol was implemented in 2019. This strategy included immediate use of high-frequency oscillatory ventilation (HFOV) after birth, during the stay at the Neonatal Intensive Care Unit (NICU), and during surgical repair. HFOV strategy included low distending pressures and higher frequencies (15 Hz) with subsequent lower tidal volumes. Surgical repair was performed early, within 24 h of birth, if possible. A retrospective study of all inborn neonates prenatally diagnosed with CDH and without major associated anomalies was performed at the NICU of Schneider Children's Medical Center of Israel between 2009 and 2022. Survival rates and pulmonary outcomes of neonates managed with MLP were compared to the historical standard care cohort. Thirty-three neonates were managed with the MLP protocol vs. 39 neonates that were not. Major adverse outcomes decreased including death rate from 46 to 18% (p = 0.012), extracorporeal membrane oxygenation from 39 to 0% (p < 0.001), and pneumothorax from 18 to 0% (p = 0.013).
Conclusion: MLP with early surgery significantly improved survival and additional adverse outcomes of neonates with CDH. Prospective randomized studies are necessary to confirm the findings of the current study.
What Is Known: • Ventilator-induced lung injury was reported as the main cause of mortality in neonates with congenital diaphragmatic hernia (CDH). • Conventional ventilation is recommended by the European CDH consortium as the first-line ventilation modality; timing of surgery is controversial.
What Is New: • A maximal lung protection strategy based on 15-Hz high-frequency oscillatory ventilation with low distending pressures as initial modality and early surgery significantly reduced mortality and other outcomes.
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http://dx.doi.org/10.1007/s00431-023-05328-y | DOI Listing |
Taiwan J Obstet Gynecol
January 2025
Ultrasound, Chongqing Health Center for Women and Children, Chongqing, China; Ultrasound, Women and Children's Hospital of Chongqing Medical University, Chongqing, China. Electronic address:
Objective: To explore outcomes of fetuses with severe congenital diaphragmatic hernia (CDH) after fetal endoluminal tracheal occlusion (FETO) treatment.
Case Report: Fetuses diagnosed with severe CDH and taken FETO for intrauterine treatment from January 2020 to December 2023 were recruited. There was no significant difference in general conditions, as well as O/E LHR and measurements related to CDH (p > 0.
Pediatr Cardiol
January 2025
Department of Pediatrics, Cincinnati Children's Hospital Medical Center, The Heart Institute, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Cardiac-Urogenital Syndrome (CUGS) is a recently identified genetic disease characterized by urogenital, diaphragmatic, ophthalmic, and cardiac abnormalities caused by heterozygous pathogenic variants in the Myelin Regulatory Factor (MYRF) gene. The complete spectrum of disease characteristics and prevalence is not yet defined. This report documents the first known cases of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) in MYRF-associated Cardiac-Urogenital Syndrome (MYRF-CUGS).
View Article and Find Full Text PDFCongenit Anom (Kyoto)
January 2025
Department of Neonatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
J Pediatr Surg
December 2024
Department of Pediatric Surgery, University Medical Center Mannheim, University of Heidelberg, Theodor-Kutzer-Ufer 1-3, Mannheim 68167, Germany. Electronic address:
Introduction: Open repair of Congenital diaphragmatic hernia (CDH) in neonates often involves reconstruction of the abdominal wall using a patch. Data on predictors for the need of a patch and associated postoperative risks, such as infection or patch detachment, are limited. Specifically, the question regarding the ideal timepoint of patch removal remains unanswered.
View Article and Find Full Text PDFJ Cardiothorac Surg
January 2025
Internal Medicine, University of Arkansas for Medical Sciences - Northwest, Fayetteville, USA.
Introduction: The rarest form of renal ectopia, the thoracic kidney, has been documented in only about 200 cases worldwide. There are four recognized causes of congenital thoracic renal ectopia: renal ectopia with an intact diaphragm, diaphragmatic eventration, diaphragmatic hernia, and traumatic diaphragmatic rupture. This condition often presents as an incidental finding in asymptomatic patients.
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