Prenatal diagnosis of idiopathic infantile arterial calcification without fetal hydrops

Idiopathic infantile arterial calcification (IIAC) is a rare autosomal recessive disease that is characterized by extensive calcification of the internal elastic lamina and intimal proliferation of large- and medium-sized arteries, including the aortic, coronary, pulmonary, and iliac arteries. Most reported cases of IIAC were diagnosed in the neonatal periods. Prenatal diagnosis of this condition is extremely rare and is usually made in the third trimester when fetuses had nonimmune hydrops together with aortic and pulmonary calcification. Early prenatal diagnosis can hardly be made without fetal hydrops in the second trimester. We report a case of IIAC referred to our center because of hyperechogenic tricuspid valve. The prenatal diagnosis was made by echocardiographic detection of diffuse hyperechogenicity of the cardiac valves, annuli, aorta, pulmonary artery, renal artery and common iliac artery without fetal hydrops. To the best of our knowledge, this was the first case of IIAC accurately diagnosed prenatally in the absence of fetal hydrops.