OEIS complex - A population study

Using a novel method for the analysis of infants with multiple malformation s, we investigated the cluster of associated malformations called the OEIS (omphalocele, bladder exstrophyl imperforate anus, spine defect) complex am ong 5,260 infants with multiple malformations identified in four large regi sters of congenital malformations, corresponding to 5.84 million births, Th e existence of the OEIS complex was clearly demonstrated and malformations entering it could be defined, Other than the four classical malformations, omphalocele, bladder exstrophy, imperforate anus, and spine malformation, a strong association with spina bifida and intersex was stressed, Spine malf ormations occurred not only in the lumbosacral level but also more craniall y, and an association also with upper spina bifida could be demonstrated. N o specific association with any other malformation, including cardiac defec ts, was apparent. The OEIS complex is an unusually clearly defined entity a mong the various nonrandom associations which have been described. Am. J, M ed. Genet, 92:62-68, 2000, (C) 2000 Wiley-Liss, Inc.