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).
- Ambiguous genitalia
- Hernii uteri inguinalis
- Persistent Mullerian duct syndrome
- Undescended testis
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health