Long-Term Follow-Up of Total Abdominal Wall Reconstruction for Prune Belly Syndrome

BACKGROUND: Prune belly syndrome is a rare, congenital condition that consists of a major deficiency or hypoplasia of the abdominal wall musculature, bilateral cryptorchidism, and genitourinary tract malformations. Reconstruction of the abdominal wall in these patients has presented a challenge to plastic surgeons throughout the years. METHODS: The authors previously described a technique for total abdominal wall reconstruction that permitted simultaneous urinary tract reconstruction and bilateral orchiopexy. This innovative procedure used medial advancement of the fascia in a "double-breasted" fashion with preservation of the umbilicus. The authors reviewed their experience with this particular technique in one of the largest series of patients in the literature and the series with the longest follow-up. RESULTS: Twenty patients underwent total abdominal wall reconstruction with simultaneous urinary tract reconstruction and orchiopexy with a mean follow-up of 20.4 years. There were no major complications noted during this period, and all patients were extremely satisfied with their postoperative result. CONCLUSION: Total abdominal wall reconstruction using the double-breasted technique in patients with prune belly syndrome is a safe and durable procedure that achieves excellent cosmetic results. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.