FEMALE GONADAL DYSGENESIS; CAUSE OF PRIMARY AMENORRHEA: ABOUT A CASE AND REVIEW OF THE LITERATURE
Imane Laghrich*, Badiaa Ezziouani, Soukaina Laaraj, Moulay Abdellah Baba Habib, Jaouad Kouach
ABSTRACT
Female gonadal dysgenesis is characterized by the absence or insufficient development of the ovaries. This is manifested by symptoms of hypogonadism, discovered during impuberism, which can vary according to the degree of development of the gonads. We report the case of a 15-year-old girl, with no particular history, who consulted for primary amenorrhea. Clinical examination revealed impuberism with an unambiguous female phenotype. The hormonal assessment showed a profile of hypergonadotropic hypogonadism. The genetic study revealed a normal karyotype at 46 XX. pelvic MRI showed utero-vaginal hypoplasia. However, the ovaries were not seen. Laparoscopy found a hypoplastic uterus with almost absent ovaries, reduced to fibrous bands and whose biopsy found fibrous tissue devoid of ovarian parenchyma, thus confirming the diagnosis of ovarian dysgenesis.
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