Short ribs with pulmonary hypoplasia are
associated with a variety of other malformations. Short tubular bones,
perinatal hydrops, and macrocephaly are present in almost all children.
Midline cleft with or without cleft palate, congenital heart disease, central nervous system
malformations, and gastrointestinal and urogenital abnormalities are other
frequently encountered features. The syndrome is generally lethal; most
children die in utero or during early infancy as a result of respiratory
insufficiency secondary to pulmonary hypoplasia.