Q:

When should gonadectomy be done in CAIS?

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A girl is brought at the age of 17 years with complaints of primary amenorrhea. She is born at full term with no perinatal complications and her motor and sensory milestones are normal. She is 165 cm in height and her predicted height is 155 cm. On examination she has B3 staging of breast with absence of axillary and pubic hairs. Her serum E2 level is low and her FSH and LH are high. Her serum testosterone is 1000 ng/mL and her USG pelvis does not show any uterus or ovaries. She has a blind vaginal pouch on vaginal examination by the gynecologist.

When should gonadectomy be done in CAIS?

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Girls with CAIS have a normal pubertal growth spurt and feminize at the time of expected puberty (secondary to aromatization of androgen to estrogen), and testicular tumors do not usually develop until after this time. Therefore, gonadectomy in women with CAIS is typically delayed until sexual maturation is complete. This approach also respects patient autonomy. However, in some situations, the convenience of combining gonadectomy with hernia repair or the need to relieve symptoms of discomfort from labial or inguinal testes justifies removal of the testes before puberty.

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