In 21OHD, fertility has been shown to be impaired in both females and males [4, 79,80,81,82,83,84,85,86], however, the fertility may be normal if the male has been diagnosed and treated early on since the neonatal period. If this is also true in 3βHSD2D is unknown. During abdominal surgery, the presence of ectopic adrenocortical tissue is a common incidental finding in otherwise healthy individuals without clinical significance [87]. In patients with CAH and during period of suboptimal treatment, high levels of ACTH and angiotensin II can stimulate adrenal-like cells causing development of testicular adrenal rest tumors (TARTs) and rarely ovarian adrenal rest tumors [75, 87]. The prevalence of TARTs varies between 34 and 94% according to different reports in males with CAH due to 21OHD [82, 85, 88, 89]. TARTs have been reported in some patients with 3βHSD2D [15, 63, 75], but it is difficult to estimate the prevalence.
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