Harrod Syndrome

Harrod et al. (1977) reported 2 brothers with an unusual syndrome of mental retardation, unusual facial appearance, large protruding ears, arachnodactyly, hypogenitalism, failure to thrive, and minor anomalies. The younger child also had malrotation of the small bowel and died at the age of 2 months before surgery for pyloric stenosis. Autopsy showed multiple microcysts of the renal cortex. Jurenka and Van Allen (1996) reported the case of a 46-year-old man with striking resemblance to the children described by Harrod et al. (1977). In addition to the features described by Harrod et al. (1977), he had megacolon and varicose veins, suggestive of a connective tissue disorder. An unusual facial appearance consisted of hypotelorism, long nose, highly arched palate, pointed chin, and a small mouth with malocclusion. Both brothers reported by Harrod et al. (1977) had an aberrant subclavian artery, and both had undescended testes and hypospadias. The appearance of the ears was particularly striking, as illustrated by photographs.