management
Last reviewed 07/2021
These individuals have usually been treated as female since birth and therefore, it would be potentially damaging to disclose the diagnosis to the (usually teenage) patient.
The testes should be removed once puberty has occurred since they constitute a malignancy risk. Removal prior to puberty may be followed by severe climacteric symptoms. Oestrogens should be given to complete secondary sexual development.
The patient should be informed that she has no uterus, and cannot conceive a child. IQ and longevity are unaffected.