Mahajan Niraj N, Kaushal Nandita, Subramanyam Alka A, Bhagwat Nikhil M
Department of Obstetrics and Gynecology, Topiwala National Medical College & BYL Nair Charitable Hospital, Mumbai, India.
Department of Psychiatry, Topiwala National Medical College & BYL Nair Charitable Hospital, Mumbai, India.
J Obstet Gynaecol Res. 2022 Feb;48(2):497-501. doi: 10.1111/jog.15124. Epub 2021 Dec 14.
Androgen insensitivity syndrome (AIS) causes feminization of the external genitalia, in 46XY individuals. We report a notable case of partial AIS (PAIS), which was treated with ventral clitoroplasty and vaginal dilatation. The patient is a 17-year-old phenotypically female, presented with primary amenorrhea, infantile vagina, clitoromegaly, and presence of testes. Feminizing genitoplasty was done in form of ventral clitoroplasty with gonadectomy and was put on hormone replacement therapy and advised regular use of vaginal dilators to improve vaginal length. In ventral approach, the erectile tissues are excised without disturbing the neurovascular structure. Vibratory threshold perception of clitoris assessed by biothesiometer was normal 4 years after the surgery. Vaginal corrective surgery is not required when presentation is at later stage and has some vaginal depth to work out with vaginal dilators. Regular psychiatric consultations and support are needed in patients with PAIS to develop their confidence in gender identity and sexual orientation.
雄激素不敏感综合征（AIS）可导致46XY个体的外生殖器女性化。我们报告了一例显著的部分性雄激素不敏感综合征（PAIS）病例，该病例接受了腹侧阴蒂成形术和阴道扩张术治疗。患者为一名17岁表型女性，表现为原发性闭经、幼稚型阴道、阴蒂肥大及睾丸存在。以腹侧阴蒂成形术加性腺切除术的形式进行了女性化生殖器整形手术，并给予激素替代治疗，建议定期使用阴道扩张器以增加阴道长度。在腹侧入路中，切除勃起组织时不干扰神经血管结构。术后4年通过生物感觉阈值测量仪评估的阴蒂振动阈值感知正常。如果在较晚阶段就诊且有一定阴道深度可通过阴道扩张器改善，则无需进行阴道矫正手术。PAIS患者需要定期进行精神科咨询和获得支持，以增强其对性别认同和性取向的信心。
