Two cases of gonad retention in adolescent patients with complete androgen insensitivity syndrome (CAIS)

Bilateral gonadectomy was the historical recommendation for patients diagnosed with complete androgen insensitivity syndrome (CAIS) due to the perceived risk of malignancy in the gonads. However, new shared-decision making approaches are allowing patients to explore the option to defer surgery. Here we report two patients who presented with primary amenorrhea to their primary care provider (PCP). After imaging and karyotyping, these patients were diagnosed with CAIS. They underwent exams under anesthesia and diagnostic laparoscopies in which the gonads did not present any immediate concerns or indications for removal. After discussing their options using the shared-decision making approach with the differences in sex development (DSD) team, they opted to defer gonadectomy and follow up annually with imaging to monitor the gonads.