Congenital Hemihypertrophy with Abnormalities of The Aortic Arch, and of the Skeletal, Cutaneous and Ocular Systems

The authors record a case of congenital hemihypertrophy in a woman of 24 years. Diagnosis is usually made earlier in infancy or childhood. The principal features of this curious malformation are described. Associated malformations are common. In the present case, a particular malformation is stressed, namely an abnormality of the aortic arch with a right-sided aorta and low implantation of the left subclavian artery which arose from an aortic diverticulum. Another interesting point is the presence of anomalies of the extremities, brachydactyly, and syndactyly. Cutaneous and ocular anomalies are more common and the condition appears to be related to phacomatosis. The different theories of the pathogenesis of the condition are discussed.