Giant omphalocele

see Omphalocele


The most severe form of omphalocele (or "hepatocele") where the pouch contains at least 75 % of the liver or when the diameter of the herniary orifice is > 5 cm. It is often associated with hypoplasia of the chest (narrow and elongated thorax) and, in about 1/3 of cases, pulmonary hypertension.