Chiari-Frommel Syndrome is a rare endocrine disorder that affects women who have recently given birth (postpartum) and is characterized by the over-production of breast milk (galactorrhea), lack of ovulation (anovulation), and the absence of regular menstrual periods (amenorrhea). In Chiari-Frommel Syndrome, these symptoms persist long (for more than six months) after childbirth. The absence of normal hormonal cycles may result in reduced size of the uterus (atrophy). Some cases of Chiari-Frommel Syndrome resolve completely without treatment (spontaneously); hormone levels and reproductive function return to normal.
Chiari-Frommel Syndrome is a rare disorder characterized by the abnormal production of breast milk (galactorrhea), and the absence of regular menstrual periods (amenorrhea) and ovulation (anovulatory) for more than 6 months after childbirth. These symptoms occur even though the mother is not nursing the baby. The pregnancy which precedes the onset of Chiari-Frommel Syndrome is usually normal, and childbirth and initial lactation are uneventful. However, normal menstrual periods and ovulation do not resume, and persistent discharge from the nipples occurs, which can sometimes last for years. Other symptoms may include emotional distress, anxiety, headaches, backaches, abdominal pain, impaired vision, and occasionally obesity. Women who have Chiari-Frommel Syndrome for a long time may also have a loss of muscle tone in the uterus and diminished uterine size (atrophy).
The exact cause of Chiari-Frommel Syndrome is not fully understood but may be related to an abnormality of the hypothalamus and/or pituitary glands. Some research suggests that microscopic tumors of the pituitary gland (microadenomas), stimulated by the hormones associated with pregnancy (e.g., prolactin, a stimulator of lactation) are responsible. When such microtumors grow, they may be detected by imaging techniques (CT scan or MRI). Approximately 50 percent of affected women eventually resume normal menstruation over a period of months or years.
The cause of the abnormal hormonal relationship between the pituitary and hypothalamus gland associated with Chiari-Frommel Syndrome is not known. Some studies suggest that microscopic lesions of the hypothalamus may also cause Chiari-Frommel Syndrome. An association with the use of oral contraceptives has also been suggested.
Chiari-Frommel Syndrome is a rare disorder that affects females who have recently given birth (postpartum).
Some women with Chiari-Frommel Syndrome may have abnormally high levels of prolactin in the blood. Other women have normal prolactin levels. Additional laboratory findings may include abnormally low levels of estrogen and other hormones (gonadotropins) in the urine.
The drug bromocriptine may be prescribed to help reduce prolactin levels. When these levels are reduced, normal ovulation cycles may be restored along with regular menstrual periods.
If the symptoms persist for a long period of time, affected individuals should be monitored (CT scan or MRI) for the presence of a pituitary tumor. If a tumor is discovered, it may be difficult to treat if it is very small. Larger tumors may be surgically removed.
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