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Disorders of Menstruation

Primary amenorrhea means that the patient has never menstruated. Definitions are no menarche by 14. If no secondary sexual characteristics, then this is delayed puberty. No menarche by 16, and no menarche by five years after breast development. Whatís the normal time between breast development and menarche? Right, two to two-and-one-half years. Secondary amenorrhea means you had it and you lost it. What are the etiologies? Iím going to do a structured analysis of it starting from the GU tract and moving up. Uterus outflow triad; you can have absence of the uterus, an imperforate hymen, a septum or androgen insensitivity. In terms of ovary; you can have Turnerís.

Now this is a little daunting. I will break it down, but it is in your chart and I had them make it as large as they could with the fonts. Iíll go over it piece by piece. Always do a physical examination, in spite of all the lab tests and everything else you can do, examine the patient first. If there is normal breast and normal uterus and there is cyclic pain, then think about an obstruction. Like an vaginal outlet obstruction, like imperforate hymen or transverse vaginal septum.

Now to orient you, this is a transverse vaginal septum. This is blood in the vagina, this is a thickened endometrial cavity and this is the bladder. So this is a transverse vaginal septum. If you looked at the external genitalia it would be normal. You put a Q-tip in and you hit a little block, and thatís the septum. Once again, physical exam. No breasts, no uterus, you would do a karyotype and you could have either gonadal enzyme deficiency - which Iíve never seen, the endocrinologists I work with have never seen.

You do an FSH. If the FSH is high - weíll cover that in a second - if itís low, it means that the brain is not working right. Remember there is either center or peripheral function of estrogen. Estrogen is produced by the ovaries, so if the FSH is high the ovaries arenít working. If itís low, the brain is not telling the ovaries what to do. So if itís low or normal, hypogonadotropic hypogonadism. And you need to do an MRI or a CT-scan. If the FSH is high, what does that mean? Whatís not working? The ovaries arenít working. So you do a blood pressure. If itís normal you do a karyotype. If itís normal you have total gonadal dysgenesis. If itís abnormal, itís Turnerís, which is one of the most common endocrine abnormalities in women. If the blood pressure is high then you could have a 17-hydroxylase deficiency. Progesterone is high.

Whatís the diagnosis? Thatís short. Short amenorrhea. Turnerís. Not a real wide neck, maybe a little bit of a shield chest. But hint; short patient with amenorrhea, think Turnerís first.

The last one; you do a physical exam. Normal breast development but vaginal pouch and no uterus. You do a testosterone analysis and a karyotype. If you have a normal male level of testosterone, then you have androgen insensitivity or testicular feminization. If you have a female level of testosterone and the chromosomes are 46 XX, then itís Rokitansky-Mayer congenital absence of the uterus and mullerian agenesis.

Here are two patients, twins, with testicular feminization. Hint: they have no pubic hair, no axillary hair, and are not troubled by acne. They are insensitive to all the androgens. The reason they have breast development is they have peripheral conversion of the androgens to estrogens. Here is a patient with mullerian agenesis. You look and it looks very similar to what the imperforate hymen looked like. If you looked at a schematic, thereís no uterus, there is partial tubes, and there is no real vagina. Itís non-cannulized.

Okay, so secondary amenorrhea: once again, starting from the bottom working up, Ashermanís syndrome. Whatís that? Thatís when youíve had some kind of surgery or something causing uterine synechiae. Pregnancy, secondary amenorrhea is pregnancy. I donít care if they say they are not pregnant or they are not having sex, do a pregnancy test. Ovarian causes; ovarian cysts, premature ovarian failure, Turnerís. You can have a Turnerís syndrome thatís late onset. Pituitary; Sheehanís. This is where you have ablation of the pituitary. Pituitary adenomas, microadenomas, idiopathic. In terms of adrenals.

Daunting diagram. Iíll break it down. First thing, rule out pregnancy. Do a T4, TSH, prolactin, progesterone challenge. You can challenge with 30 mg a day for three days, ten a day for ten days, five a day for five days. Whatever textbook you read will have their own regimen. If you withdraw, that means sheíll bleed and thatís even a scant amount of bleeding. Even a little bit of brown discharge. And you have a normal prolactin, normal T4, TSH then itís anovulation from a variety of causes, including PCO. If prolactin is elevated then you should do an cone-down MRI of the cella. If there is no withdrawal bleeding; youíve ruled out pregnancy, you do the TSH, prolactin, you have no withdrawal bleeding, if theyíve ever had surgery.

If there is androgen excess, meaning hirsutism, virilization such as clitoromegaly, you want to do a DHEAS - dehydroepiandrosterone sulfate - a testosterone and first morning 17-hydroxyprogesterone. Differential diagnosis would be polycystic ovarian syndrome, ovarian or renal tumor, congenital adrenal hyperplasia. If they have absent uterus, normal breasts, then Ö we did that before.

This is hirsutism. This is not a guy. Thatís clitoromegaly and this is a patient with 17-hydroxylase deficiency.