Introduction And Significance: Hepatic-pulmonary fusion is a very rare condition that occurs in the right congenital diaphragmatic hernia and appears to affect both sexes equally. There are no specific diagnostic methods before surgery for this anomaly. Most cases are discovered during surgical repair of the right congenital diaphragmatic hernia. This anomaly is considered to have a poor prognosis because of the associated vascular abnormalities and pulmonary hypoplasia.
Case Presentation: We present the case of a neonate who suffered from respiratory distress since birth. Later, a congenital right diaphragmatic hernia was diagnosed. At surgery, it was found that hepatic-pulmonary fusion was present, accompanied by an anomaly of pulmonary venous outflow. A complete detachment of the connective tissue was performed and a patch was placed to close the defect in the diaphragm.
Clinical Discussion: The few cases of hepatic pulmonary fusion reported in the medical literature make it difficult to develop a clear plan for diagnosis and surgical management. However, this anomaly should be considered when right-sided congenital diaphragmatic hernia is present, and the case should be well investigated and the surgical decision should not be rushed.
Conclusion: Hepatic-pulmonary fusion has a poor prognosis and a high mortality rate. No pathological mechanism for this anomaly has been identified, so we could not identify a specific diagnostic mechanism or a specific surgical method to treat this anomaly.
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http://dx.doi.org/10.1016/j.ijscr.2023.108845 | DOI Listing |
Int J Surg Case Rep
January 2025
Faculty of Medicine, Damascus University, Damascus, Syria.
Introduction: Congenital pyloric web or diaphragm are rare causes of Gastric Outlet Obstruction (GOO) after infancy, representing approximately 1 % of gastrointestinal obstructions. While it typically presents in the neonatal period with nonbilious vomiting, delayed diagnosis beyond infancy is exceptionally rare.
Presentation Of The Case: An 11-year-old girl with presented with one-month history of postprandial vomiting.
Stem Cells Transl Med
January 2025
Developmental and Stem Cell Biology Program, Peter Gilgan Centre for Research and Learning, The Hospital for Sick Children, Toronto, ON, Canada M5G 0A4.
Disruption of developmental processes affecting the fetal lung leads to pulmonary hypoplasia. Pulmonary hypoplasia results from several conditions including congenital diaphragmatic hernia (CDH) and oligohydramnios. Both entities have high morbidity and mortality, and no effective therapy that fully restores normal lung development.
View Article and Find Full Text PDFPediatr Surg Int
January 2025
Division of Neonatology, The Hospital for Sick Children, Toronto, ON, Canada.
Introduction: Congenital diaphragmatic hernia (CDH) in the preterm population is increasingly common in the current era of fetal endoluminal tracheal occlusion (FETO) therapy. There remains a lack of clinical guidance for clinicians and surgeons regarding optimal management strategies for such infants. We aimed to describe our experience in managing preterm CDH in a single quaternary neonatal intensive care unit (NICU).
View Article and Find Full Text PDFBirth 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.
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