The prognosis of high risk congenital diaphragmatic hernia and eventration diagnosed in the early neonatal period (before 24 h) is studied based on a series of 64 cases. Eventration has a poor prognosis with 5 deaths out of 7 cases. The replacement of the hemidiaphragm by an abdominal muscular flap seems to be the best surgical procedure (2 recoveries on 4 cases). A high mortality rate remains in the diaphragmatic hernias which are undiagnosed before birth. Out of 54 operated cases with systematic homolateral drainage, there were 35 survivors (65%). Post-operative alveolar-arterial PO2 difference less than 53 kPa appears to be a reliable criterion of good prognosis with a survival rate of 91% in this series. Thus, despite major progresses in post-operative resuscitation, the recovery rate in high risk neonatal congenital diaphragmatic hernia and eventration is only 75%. This seems to be partly related to the existence of lethal forms due to bilateral pulmonary hypoplasia and structural anomalies of the pulmonary arteries.
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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 PDFJ 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 PDFCongenit Anom (Kyoto)
January 2025
Department of Neonatology, Hyogo Prefectural Kobe Children's Hospital, Kobe, Hyogo, Japan.
J 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.
View Article and Find Full Text PDFEur J Ophthalmol
January 2025
Dept of Vitreoretinal Diseases, Sankara Nethralaya, Chennai, India.
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