Hernia diafragmática congénita / Congenital diaphragmatic hernia

The congenital diaphragmatic hernia (CDH) is a congenital defect of the formation and /or closure of the diaphragm that permits the herniation of abdominal contents into the thorax. It occurs when the diaphragmatic muscle fails to close during the prenatal development, and the contain of the abdomen migrate into the chest through this hole. When the abdominal organs are in the chest, there is limited room for the lungs to grow. This prevents the lungs for developing normally, resulting in pulmonary hypoplasia (or underdeveloped lungs). This can cause reduced blood flow to the lungs and pulmonary hypertension, as well as gastrointestinal reflux, feeding disorders and developmental delays. CDH can occur on the left side, right side or, very rarely, on both sides, and it can be life-threatening. The etiology is usually unknown. The incidence of CDH may be as high as 1 in 2000 to 1 in 5000 newborns alive. The sex relationship male/female is 1:1.8. Because of associated persistent pulmonary hypertension of the newborn and pulmonary hypoplasia, medical therapy in patients with CDH is directed toward optimizing oxygenation while avoiding definitive therapy. No time for repair of CDH is ideal, but it is suggested that the opportunity is 24-48 hours after birth to achieve pulmonary development. The key to survival lies in prompt diagnosis and treatment. Over the past two decades antenatal diagnosis rates have increased the knowledge of the pathophysiology of CDH and has become better understand with advances in clinical care including prenatal surgery, with a reported mortality of almost 35 % in live-born patients and a higher mortality when in utero deaths are conected. All these considerations are described in the article, with special reference to pre and post-natal treatment, complications management, diagnosis and prognosis

VEGF receptor expression decreases during lung development in congenital diaphragmatic hernia induced by nitrofen

Changes in vascular endothelial growth factor (VEGF) in pulmonary vessels have been described in congenital diaphragmatic hernia (CDH) and may contribute to the development of pulmonary hypoplasia and hypertension; however, how the expression of VEGF receptors changes during fetal lung development in CDH is not understood. The aim of this study was to compare morphological evolution with expression of VEGF receptors, VEGFR1 (Flt-1) and VEGFR2 (Flk-1), in pseudoglandular, canalicular, and saccular stages of lung development in normal rat fetuses and in fetuses with CDH. Pregnant rats were divided into four groups (n=20 fetuses each) of four different gestational days (GD) 18.5, 19.5, 20.5, 21.5: external control (EC), exposed to olive oil (OO), exposed to 100 mg nitrofen, by gavage, without CDH (N-), and exposed to nitrofen with CDH (CDH) on GD 9.5 (term=22 days). The morphological variables studied were: body weight (BW), total lung weight (TLW), left lung weight, TLW/BW ratio, total lung volume, and left lung volume. The histometric variables studied were: left lung parenchymal area density and left lung parenchymal volume. VEGFR1 and VEGFR2 expression were determined by Western blotting. The data were analyzed using analysis of variance with the Tukey-Kramer post hoc test. CDH frequency was 37% (80/216). All the morphological and histometric variables were reduced in the N- and CDH groups compared with the controls, and reductions were more pronounced in the CDH group (P<0.05) and more evident on GD 20.5 and GD 21.5. Similar results were observed for VEGFR1 and VEGFR2 expression. We conclude that N- and CDH fetuses showed primary pulmonary hypoplasia, with a decrease in VEGFR1 and VEGFR2 expression.