Congenital Hernia of the Diaphragmatic Dome with Neonatal Revelation, Experience from the Neonatology and Neonatal Intensive Care Unit at the University Hospital Center of Oujda  

作　　者：Addou Bebana Anass Ayyad Mohammed Ec-Chebab Sahar Messaoudi Rim Amrani Addou Bebana;Anass Ayyad;Mohammed Ec-Chebab;Sahar Messaoudi;Rim Amrani(Pediatrics/Residency, Mohammed VI University Hospital, Oujda, Morocco;Department of Neonatology and Neonatal Intensive Care Unit, Mohammed VI University Hospital, Medical School, Mohammed First University, Oujda, Morocco;The Maternal-Child & Mental Health Research Laboratory, Oujda, Morocco)

机构地区：[1]Pediatrics/Residency, Mohammed VI University Hospital, Oujda, Morocco [2]Department of Neonatology and Neonatal Intensive Care Unit, Mohammed VI University Hospital, Medical School, Mohammed First University, Oujda, Morocco [3]The Maternal-Child & Mental Health Research Laboratory, Oujda, Morocco

出　　处：《Open Journal of Pediatrics》2024年第5期790-799,共10页儿科学期刊（英文）

摘　　要：Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.Congenital hernia of the diaphragmatic dome (CHDD) is an embryonic malformation in which all or part of the diaphragmatic dome fails to develop properly. In the majority of cases (80% to 90%), this malformation affects the left posterolateral part of the diaphragm, while in 10% to 15% of cases it affects the right. Bilateral cases are extremely rare, accounting for less than 1% of cases. This malformation is estimated to occur at a frequency of around 1 in 3500 births, with a male predominance. The diaphragmatic defect causes the abdominal organs to rise into the thoracic cavity during critical phases of lung development. These anomalies result in bilateral pulmonary hypoplasia, a reduced number of pulmonary vessels, and pulmonary arterial hypertension (PAH). The combination of these anatomical and functional anomalies, in varying degrees, explains the wide variability of symptoms at birth. Diagnosis is usually made prenatally by ultrasound, which enables severe forms of the disease to be detected and appropriate management initiated. The prognosis remains generally grave, with a neonatal mortality rate of between 30% and 60% depending on the study, and around half of all children will have long-term sequelae.

关 键 词：Congenital Diaphragmatic Hernia Prenatal Diagnosis Pulmonary Hypoplasia Pulmonary Hypertension Medical-Surgical Treatment 

分 类 号：R65[医药卫生—外科学]