A rare case of severe congenital diaphragmatic hernia

Abstract

Background. Diaphragmatic hernia is a complex congenital anomaly, often associated with other structural defects with lethal potential. Congenital diaphragmatic hernia (CHD) often causes a chain of multiple complications since intrauterine development, the most frequent of which is pulmonary hypoplasia complicated, in turn, by the installation of persistent pulmonary hypertension in the newborn after birth. Although the medical and surgical management of CDH has improved considerably, morbidity and mortality rates remain high. Case report. We presented the case of a newborn diagnosed with a massive Bochdalek-type left diaphragmatic hernia, with the ascension into the thoracic cavity of the intestine, spleen, stomach, left lobe of the liver, the antenatal diagnosis was established one month before birth, with fatal evolution within 48 hours of at birth despite the intensive support applied. Conclusions. The presented case is a very rare case of severe CHD with a fatal outcome mainly due to the wide diaphragmatic defect that allowed the thoracic ascension of the spleen, stomach, intestinal loops, and the right liver lobe associated with hypo/aplasia of the left lung, severe displacement and compression of the heart and severe pulmonary hypertension.