The co-occurrence of congenital diaphragmatic hernia, esophageal atresia/tracheoesophageal fistula, and lung hypoplasia

Objectives. To assess the effectiveness of the use of prenatal ultrasound indexes in congenital diaphragmatic hernia of the fetus to determine the postnatal prognosis. Materials. The analysis of 95 observations of left-sided congenital diaphragmatic hernia of the fetus was carried out. In the prenatal period, the composition of organs displaced into the pleural cavity was determined, the heart compression index (HCI), O/E LHR according to Jani and DeKoninck, and QLI were calculated. Results. Survival rate of newborns was 57.9%, mortality rate was 42.1%. The newborns were divided into two groups depending on the outcome of the disease. Group I — surviving newborns (n = 55), group II — deceased patients (n = 40). In the analyzed groups, there were no statistical differences in the timing of delivery, birth weight of newborns, the severity of asphyxia after birth and the type of hernia. In group I, the intestinal loops and stomach were significantly more often identified in the pleural cavity in isolation, less often the liver. HCI corresponded to 1.3, Jani O/E LHR 45.7%, DeKoninck O/E LHR 38.7%, QLI 0.7. In group II, concomitant malformations, polyhydramnios and displacement of the liver into the pleural cavity were significantly more frequent. HCI was 1.5, Jani O/E LHR 38.6%, DeKoninck O/E LHR 32.0%, QLI 0.6. Conclusions. In predicting the outcome of the disease for a newborn, the most effective is a comprehensive assessment of the location of the liver, the heart compression index and the index of lung hypoplasia (O/E LHR according to Jani). The diagnostic accuracy of the method is 80%, the sensitivity is 74.4%, and the specificity is 83.3%.

Download Full-text

Prenatal Diagnosis and Management of a Rare Central Tendon Defect Type of Congenital Diaphragmatic Hernia with a Massive Pericardial Effusion

Case Reports in Obstetrics and Gynecology ◽

10.1155/2020/6798253 ◽

2020 ◽

Vol 2020 ◽

pp. 1-5

Author(s):

Inas Babic ◽

Haifa Al-Jobair ◽

Osama Al Towaijri ◽

Huda Al-Shammary ◽

Merna Atiyah ◽

...

Keyword(s):

Pericardial Effusion ◽

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Neonatal Morbidity ◽

Lung Hypoplasia ◽

Defect Type ◽

Tendon Defect ◽

Prenatal Diagnostic ◽

Large Pericardial Effusion ◽

Prenatal Intervention

The central tendon defect type of congenital diaphragmatic hernia (CDH) is extremely rare and usually associated with a significant pericardial effusion. Prenatal diagnostic ultrasound features of this quite rare entity remain often overlooked or misdiagnosed. There is a dearth of literature about the role of prenatal intervention, often through an elective pericardiocentesis, for the prevention of lung hypoplasia and to decrease the overall neonatal morbidity and mortality. To the best of our knowledge, till date, there is only one case that was subjected to a prenatal intervention. Here, we present a second case of a central tendon defect type of CDH with a large pericardial effusion that was subjected to a prenatal transthoracic pericardiocentesis. Although smooth intubation and ventilation were performed immediately after birth, the infant suffered for several months from respiratory instability. Laparoscopic central tendon hernia repair was performed, and neonate was discharged home at seven months of age. Although prenatal pericardiocentesis may facilitate smoother postnatal intubation and ventilation, its broader effect on respiratory function is uncertain and still remains elusive.

Download Full-text

Cellular Origin(s) of Congenital Diaphragmatic Hernia

Frontiers in Pediatrics ◽

10.3389/fped.2021.804496 ◽

2021 ◽

Vol 9 ◽

Author(s):

Gabriëla G. Edel ◽

Gerben Schaaf ◽

Rene M. H. Wijnen ◽

Dick Tibboel ◽

Gabrielle Kardon ◽

...

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Diaphragmatic Hernia ◽

Birth Defect ◽

Congenital Disorder ◽

Diaphragmatic Defect ◽

Lung Hypoplasia ◽

Cellular Origin ◽

Recent Developments ◽

Mechanical Factors

Congenital diaphragmatic hernia (CDH) is a structural birth defect characterized by a diaphragmatic defect, lung hypoplasia and structural vascular defects. In spite of recent developments, the pathogenesis of CDH is still poorly understood. CDH is a complex congenital disorder with multifactorial etiology consisting of genetic, cellular and mechanical factors. This review explores the cellular origin of CDH pathogenesis in the diaphragm and lungs and describes recent developments in basic and translational CDH research.

Download Full-text

Tetralogy of Fallot with congenital diaphragmatic hernia and left lung hypoplasia: assessment of the adequacy of the peripheral pulmonary vascular growth after hernia repair

Cardiology in the Young ◽

10.1017/s104795111100134x ◽

2011 ◽

Vol 22 (2) ◽

pp. 235-238 ◽

Cited By ~ 3

Author(s):

Hong Ju Shin ◽

Won Kyoung Jhang ◽

Tae Jin Yun

Keyword(s):

Congenital Diaphragmatic Hernia ◽

Tetralogy Of Fallot ◽

Diaphragmatic Hernia ◽

Left Lung ◽

Lung Hypoplasia ◽

Vascular Growth ◽

Threatening Condition ◽

Central Pulmonary Artery ◽

Life Threatening ◽

Vascular Beds

AbstractCongenital diaphragmatic hernia is a life-threatening condition frequently associated with various congenital cardiac diseases. In congenital diaphragmatic hernia associated with tetralogy of Fallot, central pulmonary artery size of the affected side may not reflect the capacitance of peripheral pulmonary vascular beds. We report a case of congenital diaphragmatic hernia associated with tetralogy of Fallot, which was repaired after assessing the adequacy of the pulmonary vascular beds by intra-operative pulmonary blood flow study.

Download Full-text