Complete androgen insensitivity (CAIS) occurs in approximately 1 in 13,000 to 1 in 62,000 girls and results in a female phenotype but the presence of XY gonads. Fortunately there is very little controversy in regards to gender rearing these patients as females. Rather, the controversy centers primarily around the timing of the gonadectomy. All clinicians agree that XY gonads should be removed in patients with CAIS to avoid the potential risk of germ cell malignancies but when this should occur continues to be a source of debate. In this clinical opinion, I have asked Dr. Lisa Allen, a pediatric gynecologist, and Dr. Darcie Kiddoo, a pediatric urologist, to provide their opinion in favor of either an early or a delayed gonadectomy.
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