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High Prevalence of Reduced Fecundity in Men with Congenital Adrenal Hyperplasia

Endocrinology and Metabolism (N.R., L.F., M.B., F.B., M.Re.), Psychosomatic Ambulance and Outpatient Clinic (F.P.G.), Medizinische Klinik-Innenstadt, Department of Radiology (M.S.), Outpatient Clinic of Andrology, Department of Dermatology (H.W.), and Department of Endocrinology, von Haunersches Kinderspital (H.-P.S.), University Hospital München, D-80336 München, Germany; Institute of Medical Information Technology, Biometry and Epidemiology (M.Ro.), Ludwig-Maximilians-Universität, München D-81377, Germany; and Clinical Endocrinology (M.Q.), Charité Campus Mitte, Charité University Medicine Berlin, Berlin 13353, Germany

Address all correspondence and requests for reprints to: Nicole Reisch, M.D., Medizinische Klinik-Innenstadt, Klinikum der Universität München, Ziemssenstr. 1, D-80336 München, Germany. E-mail: nicole.reisch{at}med.uni-muenchen.de.

Context: Testicular adrenal rest tumors (TARTs) are regarded to contribute to the high prevalence of subfertility in males with congenital adrenal hyperplasia (CAH).

Objectives: Our objectives were to evaluate reduced fecundity and its possible causes in well-controlled adult males with CAH, and to investigate diagnostic tools for improved treatment monitoring with respect to fertility outcomes.

Design: In a cross-sectional study at the Department of Endocrinology at the University Hospital München, Germany, 22 adult male CAH patients (15 salt wasting and seven simple virilizing, age 19–48 yr) were clinically assessed according to their hormonal control. We performed testicular ultrasound (22 of 22), magnetic resonance imaging (18 of 22), and a semen analysis (19 of 22) in the participants.

Results: All patients had a pathological semen analysis. TART prevalence was 10 of 22 (eight salt wasting, two simple virilizing). Poor therapy control was present in five patients, and all five had TARTs. Of the other 17 well-controlled patients with normal or suppressed adrenal androgens and 17-hydroxyprogesterone levels, five presented with TARTs. There was a significant correlation between sperm concentration and functional testicular volume (r = 0.70; P = 0.002), TART volume (r = –0.70; P = 0.036), as well as inhibin B levels (r = 0.75; P < 0.0001), respectively. In several men, hormonal control parameters suggested hypogonadism, with glucocorticoid overtreatment as a relevant factor for poor semen quality.

Conclusions: Poor semen parameters are common in male CAH patients. TARTs, most likely reflecting undertreatment, as well as inhibin B are important indicators of fecundity. On the other hand, long-term glucocorticoid overtreatment also seems to contribute to low semen quality.