Reversible infertility in a man with 21-hydroxylase deficiency congenital adrenal hyperplasia

OBJECTIVE: To report a case of an azoospermic man diagnosed with 21-hydroxylase deficiency congenital adrenal hyperplasia who successfully conceived with intrauterine insemination (IUI) after hormonal and clomiphene citrate (CC) treatment. DESIGN: Case report. SETTING: Outpatient practice and academic hospital. PATIENT(S): A 32-year-old azoospermic man who presented for evaluation of male factor infertility. INTERVENTION(S): Semen analysis, ultrasonography, endocrinologic assays, hormonal treatment, CC, and IUI. MAIN OUTCOME MEASURE(S): Semen analysis demonstrating increased sperm count and motility, ultrasonography demonstrating persistent nodules in bilateral testes, endocrinologic assays demonstrating elevated FSH and LH after CC treatment. RESULT(S): Successful intrauterine pregnancy after IUI using the patient's sperm. CONCLUSION(S): Clomiphene citrate treatment in addition to hormonal manipulation in azoospermic patients with congenital adrenal hyperplasia can be successful in improving sperm count and motility to allow for successful conception using IUI. The presumed adrenal rests in the testes may not involute after adrenal suppression.