Antenatal detection of idiopathic arterial calcification with hydrops fetalis

Abstract

Idiopathic arterial calcification of infancy, or occlusive infantile arterial calcification, is a rare cause of arterial calcification. This condition is inherited as an autosomal recessive pattern, which is almost always fatal. In most of the 100 cases described in the literature, the diagnosis was made at autopsy; a few cases have been reported in which an antemortem diagnosis was made on the basis of radiographic or sonographic demonstration of arterial calcification. The clinical characteristics are extremely variable, and respiratory distress with cardiac failure is the most common finding. Hypertension that is refractory to treatment is present in most cases. Most infants die before the age of 6 months, and very few have survived for more than 1 year. Mortality in this condition is caused by myocardial ischemia due to coronary artery involvement or refractory cardiac failure. The purpose of this report is to describe the prenatal diagnosis of idiopathic arterial calcification. Fewer than 10 cases of antenatal detection of this condition have been reported in the literature. This case is 1 of 2 cases with the earliest diagnosis of this condition so far (Table 1).