Prune belly syndrome and abdominal wall reconstruction

The prune belly syndrome was first reported by Frolich in 1839 (Frolich F, Der Mangel der Muskeln insebesondere der Setinbauchmuskeln Dissertation, 1839) and is characterized by a triad of deficient abdominal musculature, intraabdominal testes, and dilatation of the urinary collecting system. These patients who often require urological procedures and subsequent reconstruction of the abdominal wall can prove to be an interesting plastic surgery challenge. The standard techniques for abdominal wall reconstruction can be used, but these must be modified to meet the needs of each individual patient. A 3-year-old boy with prune belly syndrome is presented who was referred to the plastic surgeons for abdominal wall reconstruction. He had already undergone multiple urological procedures and had a Mitrofanoff microstoma at the umbilicus. There have been no techniques described previously to deal with the umbilical stoma. The patient underwent a two-stage reconstruction. This included plication of the fibrous abdominal wall and deepithelialization of excess skin to provide a double layer of dermis. The patient is now 17 years old and has achieved a good result. In the techniques previously described, "redundant" excess skin was excised and discarded, together with some form of plication. We feel that excess skin in prune belly patients should not necessarily be thought of as redundant and may be used as a double layer of dermis to protect and enhance the underlying abdominal wall repair. © Springer-Verlag 2006.