Percutaneous Aortic Bioprosthesis: Necropsy Images
2016
Thus, the incidence of the lesion we describe (left anterior congenital diaphragmatic hernia or MorgnaniLarrey hernia) is exceptionally low. Although cases have been reported in fetuses and newborn infants, it is generally produced as a consequence of acquired mechanisms that increase the intraabdominal pressure, resulting in herniation of the abdominal organs into the thoracic cavity through a congenital anatomical defect in the left sternocostal triangle of the diaphragm. In adults, it usually goes undetected and the diagnosis is incidental, whereas in children, it frequently produces respiratory symptoms (dyspnea, tachypnea, and recurrent pneumonia).1,2 Cases have been reported of newborn infants with associated severe pericardial effusion, often treated by means of emergency pericardiocentesis followed by corrective surgery. However, the slow development of the effusion due to the direct irritation of the pericardial sac can result in the accumulation of a significant amount of fluid with no hemodynamic impact. Thus, it would appear to be reasonable to adopt an initial conservative approach and perform definitive surgical treatment electively.3 In our case, we decided to initiate medical treatment in order to reduce the effusion, which proved to be effective, and subsequent elective surgery. Initially, we suspected a thyroid hormone deficiency (a disorder associated with trisomy 21) as a possible cause, despite the fact that a complete etiological study was carried out and all the results were negative. Since an association between Morgagni hernia and trisomy 21, although not very common, has been demonstrated, the former should be considered in the presence of recurrent respiratory and/or gastrointestinal conditions or those of unknown cause in patients with chromosomal abnormality.4,5 In a case of Morgagni-Larrey hernia as an unusual cause of pericardial effusion in a child with Down’s syndrome, the slow and progressive development of the effusion makes it possible, in many cases, to adopt an initial conservative approach, to be followed subsequently by definitive surgery performed electively.
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