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Introduction DSD is an umbrella term referring to a collection of conditions in which chromosomal, gonadal or anatomical sex development is affected. Its prevalence widely varies from 1 in 5000 to 6000 in general population. They mainly present with ambiguous genitalia, amenorrhoea or infertility in later stage of life. Aims and objectives This is a rare case report of 46XY DSD with 17 beta hydroxysteroid dehydrogenase deficiency. Materials and Methods An 18 yr old individual, reared as a female since birth presented to the OPD at SCB MCH with chief complaint of primary amenorrhoea. On examination her breast showed tanner stage 2, axillary pubic hair tanner stage 4, bilaterally gonads were palpable in the inguinal region, a phallus of length 5 cm was present with external urethral meatus at the base of the phallus. She had a blind ended vagina of 1.5cm length. Karyotype-46 XY. USG showed B/L undescended testis with no evidence of uterus, ovaries or prostate. Result After thorough examination and investigation, diagnosis of 46XY DSD with 17 beta HSD3 was made. Patient was counseled about the nature of the disease and fertility aspect. After psychiatry consultation, the patient wanted to continue as a female. So she was planned for sex reversal surgery. She underwent bilateral orchidectomy with vaginoplasty. She was prescribed Tab Estradiol valerate 1mg. Conclusion DSD can be identified in childhood,puberty or adulthood. Evaluation includes physical examination, genetic, biochemical tests and imaging studies. A patient with DSD requires a multidisciplinary approach, inclusion of parents and most importantly assuring the psychological well being of the patient.