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Persistent Mullerian duct syndrome: A case report and review of the literature

Author(s): Odi Temitope | Abdur-Rahman Lukman | Nasir Abdulrasheed

Journal: African Journal of Paediatric Surgery
ISSN 0189-6725

Volume: 7;
Issue: 3;
Start page: 191;
Date: 2010;
Original page

Keywords: Anti-Mullerian hormone | male pseudohermaphrodite | persistent Mullerian duct syndrome

Persistent Mullerian duct syndrome is a rare form of internal male pseudohermaphroditism, in which Mullerian duct derivatives (uterus and fallopian tubes) are present in a genotypic (46XY) and phenotypic male. Over 150 cases have been reported, mainly from outside the African setting. This article presents an unexpected case encountered in an African setting. Handicaps in the management were unavailability of necessary diagnostic tools as well as lack of finance to assess those available. Although a diagnosis was eventually arrived at and the parents thoroughly counseled, the patient has not represented for definitive surgery.
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