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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.

Signs & Symptoms

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).

Causes

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.

Affected populations

Chiari-Frommel Syndrome is a rare disorder that affects females who have recently given

birth (postpartum).

Disorders with Similar Symptoms

Symptoms of the following disorders can be similar to those of Chiari-Frommel Syndrome.

Comparisons may be useful for a differential diagnosis:

Forbes-Albright Syndrome is one of a group of rare endocrine disorders characterized by

abnormally high levels of the hormone prolactin due to a tumor of the pituitary gland.

Symptoms include the production and secretion of milk from the breasts (lactation) without

associated childbirth or nursing (galactorrhea), and the absence of a regular menstrual period

(amenorrhea). Women with Forbes-Albright Syndrome generally have breasts and nipples of
normal size and appearance, but the pattern of body hair and sexual drive may be reduced.

(For more information on this disorder, choose “Forbes-Albright” as your search term in the

Rare Disease Database.)

Ahumada-del Castillo Syndrome is a rare endocrine disorder characterized by the abnormal

function of the hypothalamus and pituitary glands affecting the secretion of hormones. This

disorder affects only women and is not related to pregnancy. The two major symptoms of this

disorder include the production and expression of milk from the breasts not associated with

childbirth or nursing, and the lack of regular menstruation. There is normal development of

secondary sexual characteristics. (For more information on this disorder, choose “Ahumada-

del Castillo” as your search term in the Rare Disease Database.)

Standard Therapies

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.