Disorder of sexual differentiation is the terms used for a child born without clear male or female phenotype. The term “hermaphrodite” is derived from Greek mythological God “Hermaphroditos” son of Hermes and Aphrodite, whose body after being merged with nymph Salmakis assumed a more perfect form with both male and female attributes [4
Proper gender assignment to a neonate born with DSD is a social emergency of the newborn period. Infants and children born with DSD pose a diagnostic and therapeutic challenge to the clinicians. Success depends upon rapid and precise diagnosis, appropriate gender assignment, proper medical therapy and meticulous surgical technique [5
The causes of true hermaphroditism remain enigmatic and the commonest presentation is an abnormal external genitalia ranging from normal male to normal female. In many of these cases such distinction may not be present and chordee, hypospadias and cryptorchidism may be noted. Similar picture is found in our case. Other presenting symptoms are hematuria, amenorrhea, lower abdominal pain and inguinal hernia [6
Documentation of location of gonads is important. In true hermaphrodites gonads are always asymmetrical with predominant testis descends and predominant ovary lies in the abdomen above the external ring as noted in index case. On the basis of location of gonads and histology these patients are classified as:
·Lateral: Testis and contralateral ovary (30%).
·Bilateral: Testicular and ovarian tissue identified on both sides, usually as ovotestis (50 %).
·Unilateral: Ovotestis on one side and testis or ovary on other side (20%).
Our patient was of lateral variety in which testis was on right side and ovary on left side. The choice of rearing hermaphrodite as male or female sex is governed by phallus size [9
]. In our patient penis was of adequate size thus plan in consultation with parents was made to rear him as a male. All female structures were thus removed. A repair of hypospadias will be performed in the next stage. Prosthesis can be placed in left hemiscrotum for psychological comfort.
True hermaphroditism is rarely associated with gonadal tumours, unlike in mixed gonadal dysgenesis, where the presence of a dysgenetic gonad predisposes to gonadal malignancy. However a few cases of malignancies like dysgerminoma and gonadoblastoma have been reported in true hermaphroditism [10
]. Hence this patient will require close follow up to diagnose any malignancy arising in his remaining testis. Since the incidence of gonadal malignancy is low, estimated at 4.6 %, [1
] prophylactic removal of his remaining testis is not justified.
Our case was unique as chromosomal analysis was 46 XY, which is very rare in a case of true hermaphrodite DSD.