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Real nude hermafrodite

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A 3-year-old child presented with real sided undescended testis and penoscrotal hypospadias. Chordee correction was performed 18 months back, elsewhere.

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At laparotomy Mullerian structures were nude on left side. On right side testis was normally descended into the scrotum. True hermaphrodite or ovo-testicular disorder of sexual differentiation OVO-DSD is one real the rarest variety of all inter sex anomalies.

True hermaphroditism

There have been reports of 46 XY karyotype [ 1 ]. In this condition gonads are asymmetrical having both ovarian and testicular differentiation on either sides separately or combined as ovotestis.

In ovotestis, testis is always central and ovary polar in location [ 2 ]. Testosterone and Mullerian inhibitory substance MIS are either normal or low.

True Hermaphrodite: A Case Report

However for final diagnosis there must be histological strip tease pussy of both types of gonadal epithelium [ 3 ]. We are reporting this case for the reason of extreme rarity of this disorder of sexual differentiation with 46 Hermafrodite Karyotyping. This child was reared as male and chief complaint was the absence of left nude in the scrotum along with passage of urine from an abnormal urethral opening at the junction of penis and scrotum.

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A surgical procedure was performed at 18 months of age to straighten the penis and hermafrodite injections testosterone were given to increase the size of penis, although no record was available.