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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References
- Genital anomalies in human and animal models reveal the mechanisms and hormones governing testicular descent.Br J Urol. 1997; 79: 99-112
- Sex determination and differentiation.N Engl J Med. 2004; 350: 367-378
- Laparoscopic management of persistent mullerian duct syndrome.J Pediatr Surg. 2009; 44: E1-E3
- Persistent mullerian duct syndrome in a child: case report and review of literature.Ped Urol Case Rep. 2014; 1: 10-15
- Laparoscopic removal of mullerian structures and orchiopexy for persistent mullerian duct syndrome.Urology. 2003; 62: 1121
Article info
Publication history
Published online: November 05, 2015
Accepted:
August 23,
2015
Received:
June 3,
2015
Identification
Copyright
© 2015 Journal of Pediatric Urology Company. Published by Elsevier Inc. All rights reserved.
