Hernia uterine inguinale is one of the rare causes of male pseudohermaphroditism. These individuals are phenotypically males. They commonly present with inguinal hernia or undescended testis or both. Our patient presented with obstructed hernia.

A 28 year old male presented with a left sided obstructed inguinal hernia of one day duration.

Further interrogation revealed left inguinal swelling of 10 years duration and absence of both testicles since birth. He had 8 years of happy married life with no issues. No family history of similar complaints.

Physical examination revealed male muscular built with well developed secondary sex characters. The penis, urethra and scrotum were fully formed but, both testicles were undescended.

The routine investigation was within normal limits. Exploration of the inguinal canal region reveals an indirect complete sac with bowel contents. The sac was opened, the contents were reduced and then the presence of well formed uterus and cervix attached to posterior part of prostate by thick fibrous band was seen. Both ovaries, mesorchium were attached to uterus (Fig. 1). Total hysterectomy was done along with herniorraphy.

Pathology report Gross specimen of uterus with bilateral broad ligament with tubes and ovaries measuring 7.5 X 4.5 X 2.5cm. Cut section shows atrophic endometrial lining and seminiferous tubules like structure

a) Left and right ovaries: Section shows atrophic and hyalinised seminiferous tubules. No evidence of spermatogenesis.

b) Tubular tissue : Section shows epididymal tissue and also features of fallopian tube.

Chromosomal analysis and routine karyotyping revealed normal 46XY pattern. Post operative, patient’s recovery was uneventful.

Male pseudohermaphroditism is a condition in which gonads are testis but the internal genitalia are not completely virilised, male intersex may present:

(i) With masculine external genitalia but fully developed uterus (Present case)

(ii) With purely feminine external genitalia.

(iii) With external genitalia of equivocal sexuality.

A brief review of embryology would make the understanding of this case easy. Gonads are not developed till seven weeks. It begins to differentiate as a testis under the influence of testis determining region Y.1 Foetal sertoli cells appears first and secrets anti-mullerian hormone (AMH) by seven weeks. 2,3 It causes regression of mullerian ducts in male. The gene has been localized to chromosome 19. Leydig cells appears at 8-9 weeks and begins to synthesise testosterone.3 The latter binds to androgen receptors and acts on wolffian ducts. The latter differentiates into epididymis, vas deferens, seminal vesicles, and ejaculatory ducts in male. External genitalia are not differentiated till eight weeks of foetal life.3 Testosterone is converted by enzyme 5a reductase in target cells into Dihydrotestosterone (DHT). Testosterone and DHT combines with androgen receptors. The gene for androgen receptor is located on the long arm of X chromosome. The activated androgen receptor complex binds to DNA within the nucleus, inducing protein synthesis and resulting in androgenic effects.4 DHT causes differentiation of urogenital sinus (UGS) and external genitalia into male type. Lack of AMH, testosterone and DHT causes feminine development.

The possibility of an intersex abnormality was not conceived in the present case until the herniotomy revealed the presence of the uterus. In male intersex the penis, urethra and scrotum are fully developed but one or both testicles are undescended and usually there is an inguinal hernia. The testicles are histologically normal for undescended organs but menstruation does not occur as there is no endometrial development in the uterus. The general bodily development is masculine in type as in our case.