In male patients with undescended testes, persistent Mullerian duct syndrome (PMDS) is generally discovered by chance during orchidopexy or normal inguinal hernia treatment. It is a rare form of internal male pseudohermaphroditism caused by a failure in the synthesis or activity of Mullerian inhibitory factor, which results in the presence of Mullerian derivatives in 46XY males' uterus, fallopian tube, and upper vagina. PMDS is inherited in an autosomal recessive manner. It is vital to screen the siblings. An operating surgeon's familiarity with this illness state would improve his or her odds of appropriately identifying the case. It's crucial to distinguish PMDS from other intersex disorders. To confirm both genetic sex and the presence of functioning testicular tissue, karyotyping and measurement of testicular responsiveness to chorionic gonadotropin stimulation are required. We present a case of a 7-year-old boy with bilateral undescended testes.
Author(s) Details
Shabnam Izhar
Department of Pathology, Vivekananda Polyclinic Institute of Medical
Science(VPIMS), Lucknow, India.
Purnima Singh
Department of Pathology, Vivekananda Polyclinic Institute of Medical
Science(VPIMS), Lucknow, India.
Kusum Gupta
Department of Pathology, Vivekananda Polyclinic Institute of Medical
Science(VPIMS), Lucknow, India.
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