Limb-body wall complex
Overview
Limbbody wall complex (LBWC) is a rare,complicated, polymalformative fetal malformation syndrome with the essential features of : a) exencephaly/ encephalocele with facial clefts, b) thoraco-and/or abdominoschisis and c) limb defect . Generally, the diagnosis is based on two of three of the above features. Two phenotypes have been described, the “placento- cranial” and “placento-abdominal” adhesion phenotypes.
Symptoms
* Limb defects * Exencephaly * Encephalocele * Facial clefts * Thoraco-abdominoschisis
Treatment
* Since underlying cause of an asymmetric limb is very broad, treatment varies depending on the etiology * In certain cases, especially in those in which specific treatment would make a difference (DDH, SCFE, infection, inflammation malignancy), diagnosis must be made as soon as possible to prevent permanent damage * Consider surgical (shortening or lengthening procedures, prostheses) and nonsurgical (orthotics, pressure stockings) * Angular deformity should be looked at carefully and corrected before or at the same time of length equalization to avoid joint dislocation