Complete androgen insensitivity syndrome

Abstract

Presentation of the case. Phenotypically female patient, 18 years old, 46 XY karyotype, who was evaluated in a tertiary care center for pediatric medicine due to primary amenorrhea. Physical evaluation found adequate breast development, female external genitalia with slight hypoatrophy of the labia majora and minora, vaginal canal of 11 cm, ultrasound reports a linear rudimentary uterus and images suggestive of ovaries, magnetic resonance describes the presence of a bilobed tubular structure composed of two nodular images, which are located adjacent to the bladder wall in their aspect posterior, lateral and
superior right 27,4 × 15,4× 11,0 mm, left inguinal canal two images, one solid nodular, ovoid, isointense 37 × 21 × 13,8 mm (nodular), cystic 22,7 × 14,0 mm, congenital absence of uterus. Treatment. In the laparoscopic examination, the presence of gonads in the inguinal and para-bladder canals was detected, and both gonads were resected. Outcome. She was managed with analgesia, antibiotics and was discharged three days after surgery. The biopsy reported cryptorchidism of the right and left testis.