Two cases of male patients followed for a classical form of congenital adrenal hyperplasia (CAH), presenting an azoospermia: analysis and review of the literature.

BACKGROUND: Congenital hyperplasia of the adrenal glands is a rare pathology, which can have an impact on male fertility. We report 2 cases of azoospermia in patients followed for a classical form of congenital adrenal hyperplasia. CASES PRESENTATION: 1st case: After 18 months of infertility of the couple, explorations showed a high level of ACTH on the hormonal biological analysis. A therapeutic strategy combining hydrocortisone with dexamethasone induced a normal semen analysis, and the female partner of the patient subsequently had three spontaneous pregnancies.2nd case: After two years of infertility of the couple, explorations showed adrenal testicular inclusions invading the 4/5th of the testis with a hypergonadotropic hypogonadism, the therapeutic reinforcement did not allow the improvement of semen analysis. DISCUSSION: Sertolian deficiency can be explained by: gonadotropic deficiency by excess of adrenal androgens and adrenal testicular lesions (risk of major spermatic alteration). CONCLUSION: Congenital hyperplasia of the adrenal glands is a rare pathology in the context of male infertility. A semen analysis could be performed after puberty and a semen preservation may be proposed.

CONTEXTE: L'hyperplasie congénitale des surrénales est. une pathologie rare, qui peut avoir un impact sur la fertilité masculine. Nous rapportons 2 cas de forme classique d'hyperplasie congénitale des surrénales présentant une azoospermie. PRÉSENTATION DES CAS: Premier cas: Après une infertilité de 18 mois du couple, les explorations biologiques montraient un taux élevé d'ACTH. Une modification du traitement combinant l'hydrocortisone à la dexaméthasone a permis une normalisation du spermogramme: trois grossesses spontanées ont été obtenues.Second cas: Après une infertilité de 2 ans, les explorations révélaient des inclusions testiculaires surrénaliennes envahissant les 4/5 Emes des testicules avec un bilan retrouvant un hypogonadisme-hypergonadotrope et le renforcement thérapeutique n'a pas permis l'amélioration du spermogramme. DISCUSSION: L'insuffisance Sertolienne peut être expliquée par: le déficit gonadotrope secondaire à l'excès d'androgènes surrénaliens et la présence de lésions testiculaires surrénaliennes (risque d'altération spermatique majeure). CONCLUSION: L'hyperplasie congénitale des glandes surrénales est. une pathologie rare dans le cadre de l'infertilité masculine. Un spermogramme pourrait être réalisé dès la puberté et une auto-conservation du sperme pourrait être proposée.

Magnetic resonance hysterosalpingography in diagnostic work-up of female infertility - comparison with conventional hysterosalpingography: a randomised study.

OBJECTIVE: To compare diagnostic accuracy of MR-hysterosalpingography (MR-HSG) and conventional hysterosalpingography (X-HSG) in the evaluation of female infertility. METHODS: Forty women received prospectively both X-HSG, the gold standard technique, and MR-HSG on the same day but the order in which they were conducted was randomised. A 1.5 Tesla MRI was performed with classical sequences for pelvic analysis and an additional 3D T1-weighted sequence with intra-uterine injection of gadolinium. Two radiologists independently interpreted X-HSG and MR-HSG according to randomisation, blinded to the other results. They both then performed a second interpretation of MR-HSG blinded to the first reading with a minimum time delay of 1 week. Diagnostic performance of MR-HSG for analysis of tubal and intracavity abnormalities was evaluated by calculating sensitivity (Se), specificity (Sp), positive predictive value (PPV) and negative predictive value (NPV). RESULTS: Twenty-six patients were included. Diagnostic performance of MR-HSG was: Se: 91.7% (95% CI 61.5-99.8); Sp: 92.9% (95% CI 66.1-99.8) ; PPV: 91.7% (95% CI 61.5-99.8); NPV: 92.9% (95% CI 66.1-99.8). Pain analysis showed a significant statistical difference between the two procedures: average VAS for X-HSG was 4.43 (95% CI 3.50-5.36) versus 3.46 (95% CI 2.62-4.31) for MR-HSG, p=0,01. Intra- and inter-rater agreements for detection of tubal or intracavity abnormalities were 0.92 (95% CI 0.78-1.00) and 0.76 (95% CI 0.52-1.00). CONCLUSION: MR-HSG is a well-tolerated technique demonstrating high accuracy in investigating tubal patency and intra-uterine abnormalities for diagnostic work-up of female infertility. KEY POINTS: • MR-hysterosalpingography is an innovative technique. • Hysterosalpingography can be used to investigate tubal patency and intracavity abnormalities. • Hysterosalpingography is a potential 'one-stop-shop' imaging technique for a single comprehensive examination of female infertility.