Prune Belly syndrome: Epidemiologic, clinic and therapeutic aspects

Prune Belly syndrome (PBS) is a rare complex malformation with male predominance. His pathogeny is not yet completely elucidated. The goal of this work is to analyze the epidemiological, anatomoclinical and treatment aspects of a retrospective trial in Aristide-Le-Dantec Hospital. Patients and methods: We carried out a retrospective study about 22 cases collected in the departments of urology-andrology and pediatric surgery in Aristide-Le-Dantec Hospital between April 1995 and November 2004. Results: The mean age of the patients was 15 months with extremes of one day and 10 years. The somatic examination revealed 20 cases of complete abdominal muscle aplasia, one right partial form and the last case had a left partial form. Nineteen patients were managed with conservative treatment and three patients benefited a surgical act for urinary abnormalities. The Montfort intervention was performed in two patients respectively aged eight and 10 years. The orchidopexy, stage 1, by Fowler-Stephens technique was performed in 13 cases. Five cases of death and nine cases of testicular atrophy after orchidopexy occurred. The followings were satisfactory in the three operated patients for urinary abnormalities. Conclusion: The renal failure is the main cause of death. The management of the urinary tract abnormalities must be performed individually. The testis descending should be performed in newborn period to enhance the fertility chances. The abdominoplasty also should be done early for aesthetic reason and to improve pulmonary, defecation, and voiding functions. © 2008 Elsevier Masson SAS. All rights reserved.