Congenital diaphragmatic hernia in the Brachmann-de Lange syndrome.
We present 12 children with typical Brachmann-de Lange syndrome and congenital diaphragmatic hernia. Affected children were more likely to be of low birth weight and to have major upper limb malformations. Hernia repair was attempted in 4 of these children, and only one survived past 12 months. Newborn infants with congenital diaphragmatic hernia should be examined carefully for evidence of the Brachmann-de Lange syndrome because diagnosis of this condition may influence their clinical management and prognosis.
Cunniff
C
C
Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock 72205.
Curry
C J
CJ
Carey
J C
JC
Graham
J M
JM
Williams
C A
CA
Stengel-Rutkowski
S
S
Lüttgen
S
S
Meinecke
P
P
D003635:De Lange Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D005260:Female; D006548:Hernia, Diaphragmatic / Q000235:genetics; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017880:Limb Deformities, Congenital; D008297:Male; D010641:Phenotype; D011379:Prognosis