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Preservation of müllerian structures with laparoscopic management of intra-abdominal testes in persistent müllerian duct syndrome

  • Diana K. Bowen
    Affiliations
    Division of Pediatric Urology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA

    Department of Urology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA
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  • Richard S. Matulewicz
    Affiliations
    Division of Pediatric Urology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA

    Department of Urology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA
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  • Edward M. Gong
    Correspondence
    Correspondence to: E.M. Gong, 225 East Chicago Ave, Box 24, Chicago, IL, 60611, USA, Tel.: +1 312 227 6340; fax: +1 312 227 9412
    Affiliations
    Division of Pediatric Urology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA

    Department of Urology, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA
    Search for articles by this author
Published:November 05, 2015DOI:https://doi.org/10.1016/j.jpurol.2015.08.024

      Summary

      Background

      Laparoscopic management of remnant uterine structures for patients with persistent müllerian duct syndrome (PMDS) and bilateral intra-abdominal testes include supracervical hysterectomy or splitting of the uterine structure to facilitate orchiopexy. A laparoscopic uterine-sparing approach, however, has not been described in the literature.

      Methods

      We present a case of a 10-year-old male with PMDS who underwent laparoscopic two-step Fowler–Stephens orchiopexy (FSO) with uterine preservation. Diagnostic laparoscopy revealed bilateral intra-abdominal testes, a robust right vas deferens but diminutive left vas deferens, and a rudimentary uterine structure posterior to the bladder. At the time of the second-stage FSO, the decision was made to preserve the uterine structure to keep all future fertility options viable. A more extensive dissection was undertaken on the left side to gain adequate length for both testes to reach the scrotum and give the best chance for survival to the right testis with its accompanying robust vas deferens.

      Conclusion

      Our case highlights a laparoscopic approach to a challenging problem in pediatric urology. If uterine preservation is preferred, a laparoscopic two-step FSO with uterine preservation is technically feasible and should be a consideration for patients with PMDS and intra-abdominal testes.

      Keywords

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