Management of the abdominal wall defect in the prune belly syndrome by muscle transposition: an 18-year follow-up.

Prune belly syndrome (PBS), an uncommon anomaly, consists of genito-urinary abnormalities and a partial or complete absence of abdominal wall musculature. Although the patient's genito-urinary problems are addressed, the attention currently directed toward the abdominal wall deficiency has been mostly aimed at improving the cosmetic appearance of patients and does little to replace the important functions of the musculature. These functions are the support of the viscera and their compression as well as the movement of the trunk. A case report with an 18-year follow-up is presented where thigh muscles were transposed to act as substitutes for the missing musculature. Although this presentation is based on a single case, it is intended to alert patients and physicians to a method of improving the lot of patients with PBS. In this patient, the result was very satisfactory. From being unable to move his trunk as a schoolboy before the surgical procedure, postoperatively he was able to participate in all school activities. As an adult, he is fully active and is a lead guitarist in a band. His excretory functions are normal and the scoliosis, present when first seen, has not progressed. It is suggested that consideration be given to the management of the abdominal wall deficiency in PBS by the transposition of muscle(s), because no alternative effective treatment directed to restoring absent muscle function appears to exist. Because the operative procedure is well tested and successful in adult patients who lose their abdominal wall musculature, the dissemination of this data appears to be necessary.