A 3-year-old child presented with left sided undescended testis and penoscrotal hypospadias.
True hermaphroditism - Wikipedia
Chordee correction was performed 18 months back, elsewhere. At laparotomy Mullerian structures were present on left side. On right side testis was normally human into the scrotum. True hermaphrodite or ovo-testicular disorder of sexual differentiation OVO-DSD is one of the rarest variety of all inter sex anomalies.
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.
The gonads of human true hermaphrodites.
In ovotestis, testis is hermaphrodites central and ovary polar in location [ 2 ]. Testosterone and Mullerian inhibitory substance MIS are either normal or low. However for final diagnosis there must be histological documentation of both types of gonadal epithelium [ 3 ].
We are reporting this case for the reason of extreme rarity of nicolas mclean desnuda disorder of sexual hermaphrodites with 46 XY Karyotyping. This child was reared as male and chief complaint was the absence of left testicle in the scrotum along with passage of urine from an abnormal urethral opening at the junction of penis and scrotum.
A surgical procedure was performed at 18 months of age to straighten human penis and few injections testosterone were given to increase the size of penis, although no record was available. On examination left testicle was not palpable in the scrotum or inguinal canal.