CONGENITAL DIAPHRAGMATIC-HERNIA IN THE BRACHMANN-DELANGE SYNDROME

We present 12 children with typical Brachmann-de Lange syndrome and co ngenital diaphragmatic hernia. Affected children were more likely to b e of low birth weight and to have major upper limb malformations. Hern ia repair was attempted in 4 of these children, and only one survived past 12 months. Newborn infants with congenital diaphragmatic hernia s hould be examined carefully for evidence of the Brachmann-de Lange syn drome because diagnosis of this condition may influence their clinical management and prognosis. (C) 1993 Wiley-Liss, Inc.