Endocrine Abstracts

Introduction: Androgen insensitivities are rare genetic diseases, characterized by a more or less complete defect of tissue sensitivity to testosterone. It ranges from a more or less complete lack of masculinization to isolated infertility in a 46, XY individual. Many surgical methods of vaginoplasty have been opted with the aim of reconstructing the anatomy to allow future sexual activity as well as an improvement in the quality of life. We report the case of two patients with complete androgen resistance syndrome discovered during the exploration of primary amenorrhea

Observation 1: 33-year-old patient, operated in 2016 for bilateral intra-abdominal gonadectomy, with an anatomopathological examination of an ectopic testicle on the right without signs of malignancy, and an embryonic carcinoma on the left, the patient underwent chemotherapy sessions, she presents a harmonious and feminine Morphological development, with absence of vaginal orifice, a carotype was done in favor of a 46 XY, pelvic MRI with absence of internal genitalia, and genitography with absence of vaginal orifice, the patient was put on estrogen and progestin, with good clinical evolution

Observation 2: 20-year-old patient, who underwent bilateral gonadectomy in 2017 with pathological examination of hypoplastic testicular pulp with hamartomatous nodules and hypoplastic foci of Leydig cells compatible with androgen insensitivity syndrome without malignancy, with a gynecological examination presence of labia majora and labia minora not well formed with an incipient vagina. a carotype was done in favor of a 46 XY, pelvic MRI with absence of internal genitalia, and genitography with absence of vaginal orifice, the patient was put on estrogen and progestin, with good clinical evolution The first patient underwent a rectosigmodian vaginoplasty and the second a vaginal enlargement surgery. The result was excellent with the obtaining of a 6 cm deep neovagina. After six months of follow-up, the two patients keep neovaginal cavities with regular digital dilation

Conclusion: The choice of the most appropriate surgical technique is conditioned by the results of the clinical examination, ultrasound, genitography and endoscopic exploration.