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J Pediatr Urol. 2005 Dec;1(6):423-7. doi: 10.1016/j.jpurol.2005.03.011.

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

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  • 1Department of Pediatric Urology, Riley Children Hospital, Indiana University School of Medicine Indianapolis, IN, USA.

Abstract

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).

PMID:
18947583
DOI:
10.1016/j.jpurol.2005.03.011
[PubMed]
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