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Persistent mullerian duct syndrome with transverse testicular ectopia
Mehmet Emin Boleken1, Mete Kaya, Sefik Güran
1Department of Pediatric Surgery, Harran University Medical Faculty, Sanliurfa, Turkey. mboleken@yahoo.com
International Urology and Nephrology
|March 3, 2007
Summary
A 15-month-old boy with persistent Mullerian duct syndrome (PMDS) presented with internal female genitalia. Surgical management involved removing Müllerian remnants and performing bilateral orchiopexy, highlighting a rare case of this disorder.
Area of Science:
- Pediatric Surgery
- Endocrinology
- Genetics
Background:
- Persistent Mullerian Duct Syndrome (PMDS) is a rare disorder of sex development where individuals with male karyotype (46XY) possess Müllerian duct remnants (uterus, fallopian tubes).
- It typically presents with disorders of testicular descent or intersex ambiguity.
Observation:
- A 15-month-old boy with a 46XY karyotype was incidentally found to have internal female genitalia during surgery for bilateral inguinal hernia.
- Biopsies confirmed normal testicular tissue, leading to the diagnosis of PMDS.
Findings:
- Surgical intervention included the excision of Müllerian duct remnants (uterine fundus and fallopian tubes).
- Bilateral orchiopexy was performed to address testicular position.
Implications:
- This case highlights a rare clinical presentation of PMDS requiring surgical management.
- Understanding the genetic basis and surgical strategies for PMDS is crucial for affected individuals.
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