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Cover page of the Journal of Health Sciences
CASE REPORT
Year : 2014  |  Volume : 7  |  Issue : 1  |  Page : 58-60

Persistant Mullerian duct syndrome: A case report of a rare disorder of sex development


1 Department of Paediatric Surgery, KLE University's J.N. Medical College, Belgaum, Karnataka, India
2 Department of Paediatrics, KLE University's J.N. Medical College, Belgaum, Karnataka, India

Correspondence Address:
Santosh B Kurbet
Department of Paediatric Surgery, KLE University's J.N. Medical College, Belgaum, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2349-5006.135080

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Disorders of sex development (DSD) are congenital abnormalities of sexual differentiation resulting from disparity in the chromosomal, gonadal and phenotypic sex. Most of these children present with ambiguous genitalia, but can also present with hernias or undescended testis as in persistent Mullerian duct syndrome (PMDS). The social stigma attached with this disorder makes it imperative for expeditious evaluation and appropriate assignment of sex in our scenario. We report here successful management of such a case and review the literature. In conclusion, a rare case of DSD like PMDS requires a high index of suspicion and can be managed successfully.


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