Hypergonadotropic Hypogonadism (HHG) is a condition typified by defective gonadal development and/or function, as a result of elevated levels of gonadotropins. In terms of symptoms, HHG may be very similar to the condition Hypergonadotropic Hypogonadism. However, the underlying cause is the exact opposite. A simple test to measure the levels of gonadotropins in the serum is enough to distinguish between these two conditions. However, differentiating between HHG and constitutional delay in growth is more difficult. This is especially true, if no family history is available.
Treatment involves management with sex steroids; ethinyl estradiol followed by a progestational agent for girls, and testosterone enathate along with human chorionic gonadotropin for boys. Most patients can achieve fertility with the help of hormone treatment.
Bhat and Hamdi (2005) reported a 20-year old married female with normal external genitalia, under developed breasts and normal hair distribution, who presented with primary amenorrhea. She had no coital problems, and speculum and vaginal examination revealed a small nulliparous healthy cervix and a small retroverted uterus, which appeared as infantile on ultrasound with streak ovaries. Blood investigations revealed high levels of follicle stimulating hormone (FSH) of 39.05 IU and luteinizing hormone (LH) of 84.9 IU, but normal levels of prolactin and thyroid function tests and normal karyotype of 46 XX. Diagnostic laparoscopy revealed small uterus with normal tubes and bilateral streak ovaries (left smaller than right) which were confirmed by biopsy, as it revealed primary and secondary follicles and corpora albicantia with small follicles with cystic dilatation and attenuation of the cortex, suggestive of gonadal dysgenesis. The patient was subjected to progesterone challenge test which was positive and was started on hormonal therapy (estrogen and progesterone) for life, and was advised on assisted reproductive techniques for conception.