Limb-Body Wall Complex: an Update

Abstract

Limb-body wall complex is a complicated fetal malformation with the essential features of : 1) exencephalylencephalocele with facial clefts, 2) thoraco- and/or abdominoschisis, and 3) limb defect. The diagnosis is based on two out of three of the above features. Prevalent rate varies between 1:4000 to 1:39000 births. Etiology is generally unknown. Chromosome does not play role. Specific teratogen is not proved but cocaine is thought to be possible. Up to date, pathogenesis may be explained by two mechanisms; 1) an early vascular disruption and 2) an intrinsic embryonal maldevelopment. Diagnosis can be made prenatally by ultrasonography. Three dimentional ultrasonography may aid diagnosis. Prognosis is uniformly poor. Termination of pregnancy is the proper management.