From 1981 through 1986, 8 newborns with congenital diaphragmatic hernia required herniorrhaphy in the first 8 hours of life. Extracorporeal membrane oxygenation (ECMO) was employed in 7 after they met local criteria predictive of 95% mortality. These criteria were an alveolar-postductal arterial oxygen gradient greater than 600 mm Hg for 12 hours or hemodynamic instability. Four of these 7 patients had unremitting hypoxemia after herniorrhaphy (no "honeymoon" period), 3 of whom survived. One additional patient died, producing a mortality of 29%. ECMO used for 68 to 241 hours (mean, 163 hours) provided reliable oxygenation in all. Deaths resulted from disseminated intravascular coagulation and bleeding, and bleeding and pulmonary failure after ligation of a patent ductus arteriosus. Complications occurred in 6 patients and included bleeding (3), hernia recurrence (3), and air embolism (1). Follow-up ranging from 1 year to 6 years after discharge of the 5 survivors shows normal growth and development in 4. The reported mortality without ECMO following congenital diaphragmatic herniorrhaphy in the first 8 hours of life ranges between 60 and 80%. While bleeding may present problems, survival of newborns with refractory hypoxemia after diaphragmatic repair has improved with ECMO.
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http://dx.doi.org/10.1016/s0003-4975(10)62064-4 | DOI Listing |
Birth Defects Res
January 2025
National Center for Birth Defects Monitoring, West China Second University Hospital, Sichuan University, Chengdu, China.
Background: The landscape of co-occurring birth defects among infants with congenital diaphragmatic hernia (CDH) remains underexplored.
Aims: This study aims to elucidate the complex patterns of co-occurring defects in Chinese population.
Materials And Methods: We analyzed cases from the Chinese Birth Defects Monitoring Network (2007-2019) with CDH that presented along with at least one additional defect but without a syndromic diagnosis.
Cureus
December 2024
Anesthesiology, Emirates Health Services, Sharjah, ARE.
Congenital diaphragmatic hernia (CDH) presents significant challenges in neonatal management, particularly in the context of anesthesia. This case report details the successful anesthetic management of a five-day-old neonate with left-sided CDH requiring thoracoscopic repair. A five-day-old neonate, delivered via emergency cesarean section due to breech presentation, presented with severe respiratory distress and was diagnosed with left-sided CDH.
View Article and Find Full Text PDFTaiwan 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.
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