Infantile Arteriosclerosis

Abstract

Calcification and sclerosis of the arteries in infants and young children may be encountered in a variety of clinical conditions. Foremost among these are disorders in which disturbances of the calcium and phosphorus metabolism play an important role. Thus infantile arteriosclerosis has been recognized in renal disease (1), hyperparathyroidism, and vitamin D intoxication (2). Similar arterial changes have also been noted in progeria, in which the arteriosclerotic process is associated with extensive growth disturbances and profound metabolic changes (3). Aside from these conditions, infantile arteriosclerosis has been observed in an increasing number of cases in which no obvious etiologic factors are apparent but which show considerable resemblance in their clinical manifestations and striking similarity in their pathologic findings. The principal morphologic changes of the arteries in these patients are a marked disorganization of the internal elastic membrane accompanied by deposition of lime salts in its proximity. Concurrently a marked fibroblastic proliferation of the intima takes place, which may attain such proportions that the involved vascular lumen becomes markedly narrowed or even obliterated. The large majority of these cases have been discovered on necropsy and described in the pathologic or pediatric literature, where it has been repeatedly suggested that the arterial calcifications should be recognizable on radiologic examination. Unfortunately, radiologists are frequently unacquainted with this clinical entity, though the x-ray examination may well prove to be the most important means of establishing a diagnosis during life. The following two case reports may illustrate the salient features of this condition. Case I: R. H., a colored male, was admitted at the age of ten months on account of progressive loss of weight and enlargement of the abdomen. The family history was not contributory. Delivery had been normal but neonatal weight gain had been poor. The essential findings on physical examination were emaciation, abdominal distention, and a small umbilical hernia. The radiologie examination of the chest was not remarkable. Roentgenograms of the long bones revealed a delicate but widespread calcification of the arteries which was most pronounced throughout the soft tissues of the forearm and lower extremities, where the blood vessels showed considerable tortuosity (Figs. 1 and 2). Vascular calcification could not be recognized throughout the abdomen, thorax, or skull The laboratory findings, including serology, hemogram, blood chemistry, electrocardiogram, and duodenal trypsin activity were within normal range. The blood pressure, determined by the flush method, was recorded as follows: Right arm, 170; left arm, 150; right leg, 180; left leg, 200. Repeated studies gave similar results. After discharge and improvement, the child was readmitted at the age of sixteen months, with edema of the lower extremities.