Amen Or Rhea

Amenorrhea # Definition  Absence of menstruation. # Types:

1- Primary amenorrhea (2.5%): - No menstruation by the age of 14 years in the absence of secondary sexual characteristics, which includes: thelarchae (enlarged breasts), puberchae (growth of pubic and axillary’s hair). - No menstruation by the age of 16 years with the presence pf secondary sexual characteristics. 2- Secondary amenorrhea (3 – 100%): - The stopping of the menstruation process for 6 months in a woman with regular periods or 1 year in a women with irregular period.

# Causes:

1- Physiologic causes: - Pre-pubertal. - Pregnancy. - Lactation. - Postmenopausal. - Following hysterectomy. 2- Anatomical and pathological causes: - Disorders of outflow tract (hymen, vagina and uterus). - Disorders of ovary. - Disorders of anterior pituitary gland. - Disorders of hypothalamus can cause primary or secondary.

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Disorders of out flow tract A. Cryptomenorrhea:

Congenital obstruction caused by: 1- Imperforate hymen (most common). 2- Vaginal atresia or septum.

# Usually presented with: 1234-

Primary amenorrhea. Teenage girl with regular cycle period but no blood flow. Intermittent abdominal pain every month. Difficult urination because of the compression on the neck of the bladder (most common presentation). 5- Abdominal; mass. 6- Bluish bulging of the hymen because of the accumulation of the blood first in the vagina (hematocolpus), then the uterus (hematometra) and finally in the tubes (hematosalpins).

# Treatment  cruciade incision not to close again. ______________________________________

B. Mayer – Rokitausky – Kuster – Hauser syndrome: A very rare condition, where the vagina could be completely or partially absent. Usually presented with: 1- Primary amenorrhea. 2- Gross development and ovarian functions are normal. 3- The uterus is rudimentary. 4- 30% of the patients have renal anomalies. 5- Karyotype 44XX (female). 6- No menstruation and she will no have children.

# Treatment  Create a vagina for intercourse and explain to her that she’ll not have children. ______________________________________

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C. Testicular feminization (androgen insensitivity): Congenital abnormality characterized by: 1- A 44XY genotype (actually male). 2- Female phenotype because of the androgen insensitivity. 3- There are non functional testis (Y chromosome). 4- Inherited as X-linked recessive disorder resulting in the absence of cytosol androgen receptors. 5- Features of the patient: - Normal growth and development. - Breast is well developed. - 50% of the patients have palpable inguinal hernia containing testis. - Absence of axillary and pubic hair. - Labia minora are under developed. - Testis is in the abdomen or inguinal canal (no spermatogenesis). - Testosterone level is normal but no response to it because of the absence of the androgen receptors. - High chance of testicular malignancy (30%). So, you have to remove the testis after puberty because it’s needed prepuberty for development and growth. - Primary amenorrhea. - No uterus, tubes or ovaries. And the vagina ends blindly.

# Treatment  1234-

Never tell her that she’s not a female. Remove the testis after puberty. Tell her she will not have children. Hormone replacement therapy (estrogen: estradiol). Continuous administration cause there is no uterus. If there is uterus you give cyclic estrogen to prevent endometrial cancer. ______________________________________

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D. Asherman’s syndrome:

Destruction of endometrium by over curettage leading to removal of endometrium, which is replaced by fibrosis (synechiae). Other causes include: 1- PPH, which end with D & C. 2- Repeated infections (endometritis). 3- TB of uterus. 4- Schistosoma of uterus (very rare now).

# Presentations:

1- Secondary amenorrhea. 2- History of repeated D & C followed by amenorrhea. 3- Hystrosalpingogram.

# Treatment  Hysteroscopy and cut the adhesions and put TUCD for 1 week to prevent re-adhesions and allow regeneration of endometrium.

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Disorders of the ovary E. Turner’s syndrome: A chromosomal defect where we get 45XO (gonadal desgenesis). It leads to primary amenorrhea in 99% 0f cases. Some times we get secondary amenorrhea in the mosaic pattern of the disease (XX/XO) where there is a functioning uterus and can have children. Usually these patients: 1- Short stature. 2- Webbed neck and wide chest. 3- Increase chance of cortication of the aorta. 4- Streak ovary and fibrotic. ______________________________________

B. Gonadal agenesis  A very rare condition where there is no ovaries at all. ______________________________________

C. Resistant ovary syndrome

A very rare condition, where we get secondary amenorrhea or primary in middle age women (25-30). In this disease we have normal ovarian development but the follicles lack the receptors for gonadotrophes (FSH, LH). There is increase in the FSH level caused by the absence of estrogen, which is responsible for the –ve feed back. The chance of pregnancy is very rare.

# Treatment  Cyclic estrogen and + progesterone because there is a uterus.

______________________________________

D. Premature ovarian failure: A condition where there is a secondary amenorrhea caused by early menopause at the age of 35 years. There are no more follicles in the ovary. FSH levels are high. Can be due to: 1- Natural. 2- Autoimmune disease. 3- Viral (mumps). 4- Cytotoxic drugs (cyclophasphamide). 5- Surgery for the ovary that caused impairment of blood supply.

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# Diagnosis: To differentiate between resistant ovary syndrome and premature ovarian failure we depend on: 1- Age of the patient. 2- Biopsy of the ovary by laparotomy to check the follicles (if present then resistant ovary if not then it would be premature failure),

E. Polycystic ovarian disease:          

A disease where there is anovulation and infertility. Usually seen in obese patients. There is hursitism (abnormal hair growth in male pattern). Gives secondary amenorrhea or Oligomenorrhea (menstruation 5X per year with small amount of blood). This condition is caused by steady state release of estrogen. The graphian follicle grows but does not ovulate. Follicle has very thick capsule. There is no ovulation but there’s a steady release of estrogen. And because she’s obese there is a peripheral conversion of androgen to estrogen. Because of the steady release of estrogen they can develop endometrial cancer.

# Treatment:

1- If she wants to get pregnant you must give her induction of ovulation. 2- If she does not want to get pregnant you give her progesterone to stop this steady release of estrogen. ______________________________________

F. Other ovarian causes: 1- Oopheritis. 2- Benign mass. 3- Malignancy: - Granulose cell tumor. - Androgen secretary tumor. - Arrinoblastoma and Ladyg cell tumor.

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Disorders of pituitary gland # Hyperprolactinemia

Normal levels of prolactin are 150 – 400 mg/l.

# Caused by:

1- Physiologic: - Stress. - Pelvic examination. - Intercourse. - Venipuncture (to take blood sample). - Surgery (especially in the chest). - Sleep. - Pregnancy and lactation (level of prolactin increase 10x the normal). 2- Drugs: - Phenothyazine group. - Methyldopa. - Antihistamine. - Estrogen. - Morphine and narcotics. - Contraceptive pills. - Reserpine. 3- Pathologic: - Adenoma: micro (<1cm) or macro (>1cm). - Primary hypothyroidism because it tends to increase TSH which also increase prolactin. 4- Idiopathic  More than 50% of the cases.

# Presents with: 1- Galactorrhea (milk production). 2- Secondary amenorrhea. 3- Or non-ovulating cycle- infertility.

# Treatment: 1- Dopamine agonist (Bromocriptine) 2- Try to diagnose pituitary adenoma with x-ray of the skull, CT scan and MRI.

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# Other causes (usually theses cause secondary amenorrhea): 12345-

Stress. Metabolic disorders. Missed abortion. Psychological. Anorexia nervosa and athletics where body fat decreases by 20% and increase muscle weight leading to an increase in androgens. So, we might get amenorrhea. 6- Depression. 7- Congenital adrenal hypoplasia, where there is less sex hormones because of 21-hydroxylase deficiencies. 8- Lauren – Levis syndrome: the Gonadotropin and growth hormones are affected. She has boyish features. 9- Frohelish syndrome. Patients have low intelligence. 10-Kallamar syndrome.

Post pill amenorrhea  After stopping contraceptive pills some ladies get amenorrhea that lasts up to 1 year because the ovaries needs time to recover with the fact that contraception suppress FSH and LH leading to no ovulation.  Can be considered as physiological amenorrhea.

Anovulation  No ovulation at all.  Oligo-ovulation (infrequent) where in one cycle there is ovulation and in 3 others there are not and so on.  The commonest cause is polycystic ovarian disease.

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