This case report, it's about a patient I met during prenatal consultation, she was in the 2 nd semester of her pregnancy, exactly at 25 weeks. During the physical examination, we found, she had a hypoplasia of minor labia, an incomplete vaginal diaphragm with a vaginal cup, and a hypertrophy of clitoris. We decide to make some biological and instrumental examinations. After getting the result, we concluded she has a pseudo hermaphroditism associated with malformation of 1/3 of vagina. Reason why the way of the delivery choose was a C-section. Female pseudo hermaphroditism is the most frequent (FPH) sexual ambiguity. It is characterized by the extension of abnormality of the sexual differentiation during the fetal life in the female fetus by a maternal or fetal hyper-genesis. The authors propose through a clinical case with a patient 46XX DSD pregnant. The main cause of this pathology is congenital adrenal hyperplasia, which we lead to deficits of 21-hydoxylase, 11-beta-hydroxylase, 3-beta-hydroxysteroid dehydrogenase, but 95% of the congenital adrenal hyperplasia is the deficiency of 21-hydoxylase. The treatment of congenital adrenal hyperplasia depend of the cause, hormone replacement therapy like (dexamethasone, hydrocortisone), most often the treatment for life is needed. From this we propose to make review of literature focusing on it.
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