Hernia diafragmática congénita / Congenital diaphragmatic hernia

ABSTRACT

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 11.8. 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