Aortic dissection, patent ductus arteriosus, iris hypoplasia and brachytelephalangy in a male adolescent

We describe a 11-year-old male with dissection of the descending aorta, bil ateral iris hypoplasia, striae distensae and brachytelephalangy, the latter being most marked in the thumbs. Inguinal herniae and a patent ductus arte riosus were surgically repaired in infancy. The pattern of abnormalities ma y constitute a previously undescribed syndrome. The proband died suddenly a t the age of 17 years. Clin Dysmorphol 8: 269-276 (C) 1999 Lippincott Willi ams & Wilkins.