Persistent Mullerian duct syndrome with bilateral abdominal testis: surgical approach and review of the literature.

Objective: We present two cases of persistent Mullerian duct syndrome (PMDS) with bilateral intra-abdominal testes and review the world's literature with special attention to diagnosis and surgical management.

Patients And Methods: Two consecutive cases of PMDS with bilateral intra-abdominal testes from our institution are presented with detailed descriptions of the presentation, physical examination, laboratory profiles, surgical findings, and treatment undertaken. Follow-up at 1 year postoperatively is included.

Results: Bilateral orchiopexy was accomplished in both the cases. In one case this was possible after division of the persistent Mullerian structures in the midline to achieve testicular mobility. In a subsequent case, splitting of the Mullerian complex did not provide adequate mobilization and microvascular autotransplantation was performed with an excellent surgical outcome.

Conclusions: Bilateral intra-abdominal testes in the setting of persistent Mullerian duct syndrome are a rare entity and controversy remains as to the ideal surgical treatment. Our two cases represent the first reported examples of open single-stage bilateral orchiopexy with division of the Mullerian complex and preservation of the vas deferens (1-year-old boy) and microvascular autotransplantation (5-year-old boy).