FURTHER DELINEATION OF THE OPITZ-G BBB-SYNDROME - REPORT OF AN INFANTWITH COMPLEX CONGENITAL HEART-DISEASE AND BLADDER EXSTROPHY, AND REVIEW OF THE LITERATURE/

The combination of complex congenital heart disease (double outlet rig ht ventricle with pulmonary atresia, malalignment ventriculoseptal def ect, right-sided aortic arch with left ductus arteriosus) and bladder exstrophy occurred in an infant with Opitz syndrome. Neither of these defects has previously been reported in association with Opitz syndrom e. These malformations, which are midline defects, further characteriz e this syndrome as an impairment in midline development. The spectrum of congenital heart disease and genitourinary anomalies seen in Opitz syndrome is reviewed. (C) 1998 Wiley-Liss, Inc.