Background: Congenital diaphragmatic eventration (CDE) is defined as an abnormal displacement of the whole or a portion of an attenuated but otherwise intact diaphragm into
the thoracic cavity. Clinically it mimics the features of congenital diaphragmatic hernia (CDH) with displacement of abdominal organs into the thorax.
Methods : We report 2 cases of non-immune hydrops fetalis (NIHF) in which autopsy findings revealed an association with right-sided CDE. Both patients born at 30 weeks of gestation presented skin oedema, pleural effusions and ascitis. They died shortly after birth of
cardiorespiratory insufficiency with lung hypoplasia and low output failure.
Results : The autopsy revealed the presence of herniated visceral organs including part of the liver, small bowel and colon in the right thoracic cavity. But these organs were covered and separated from the thoracic cavity by a membraneous but intact right diaphragm. As for the
situation seen in CDH, these displaced organs led to important lung hypoplasia and obstructed venous return. The liver dysfunction resulted from the venous congestion and cardiac failure in extravascular liquid accumulation.
Conclusion(s): CDE is a rare condition resulting from impaired ingrowth of muscle fibres into the diaphragm during the first trimester of gestation. The association of CDE and NIHF has not been previously described.