Limb-body wall complex: a compound anomaly pattern in body-wall defects

Our presentation of four cases demonstrates the essential features of limb- body wall complex (LBWC), representing a compound anomaly pattern in body-w all defects. The diagnosis of this entity is based on two of the three foll owing characteristics: (1) exencephaly/encephalocele and facial clefts; (2) thoraco- and/or abdominoschisis; and (3) limb defects. A definite associat ion with internal anomalies and severe kyphoscoliosis makes a more distinct concept of the pathogenesis reasonable. Limb-body wall malformations resul t from a malfunction of the ectodermal placodes involving the early embryon ic folding process. The poor prognosis of LBWC calls for early antenatal di agnosis.