Chiari Frommel Syndrome - Causes, Symptoms, and Treatment

What Is Chiari Frommel Syndrome?

Chiari Frommel syndrome is an uncommon condition prevalent in women during the postpartum phase (time after childbirth). It is an endocrine disorder, and the following three symptoms characterize it:

• Galactorrhea (excess production of milk).

• Anovulation (absence of ovulation).

• Amenorrhea (irregular menstrual cycles).

These symptoms may persist for more than six months after childbirth in the case of Chiari Frommel syndrome. Patients should be checked for Chiari-Frommel syndrome if they experience prolonged postpartum lactation that lasts months or years, especially if there are irregular menstrual cycles present. The uterus may become smaller if there are no regular hormonal cycles (atrophy of the uterus). Frequently, the patient has a history of irregular menstrual cycles even before the pregnancy. In this syndrome, galactorrhea will still happen whether the mother breastfeeds or not. In addition to persistent lactation with potential breast engorgement, the clinical signs of Chiari-Frommel syndrome also include obesity, uterine and ovarian dysfunction, and hypothyroidism (underactive thyroid gland). Hormone levels and reproductive function can return to normal in some cases of Chiari-Frommel Syndrome, which resolves without medical intervention. In 40 % of patients, the condition spontaneously resolves within five years.

Who Is Affected by Chiari Frommel Syndrome?

Women who have recently given birth are susceptible to developing Chiari-Frommel Syndrome.

What Are the Symptoms of Chiari Frommel Syndrome?

Symptoms of Chiari Frommel syndrome include:

• Excessive production of breast milk (galactorrhea).

• Absence or irregular menstrual cycles (amenorrhea or oligomenorrhea).

• Lack of ovulation (anovulation).

• Symptoms have existed for more than six months.

• The symptoms persist in mothers who are not nursing their babies.

• The pregnancy that precedes the development of Chiari-Frommel Syndrome is typically normal, and labor, delivery, and the first few months of breastfeeding progress normally.

• However, regular ovulation and menstrual cycles are not resumed, and continuous discharge from the nipples can occasionally continue for years.

• Emotional instability.

• Anxiety.

• Headaches, backaches, and body pain.

• Pain in the abdomen.

• Vision problem.

• Obesity.

• Atrophy of the uterus may lead to reduced size and loss of muscle tone.

• Decreased libido (reduced sex drive).

• Osteoporosis (a condition characterized by weak and brittle bones).

What Causes Chiari Frommel Syndrome?

The causes of Chiari-Frommel syndrome are:

• The definitive cause of Chiari-Frommel syndrome is not known.

• However, experts suggest that Chiari-Frommel syndrome is associated with abnormalities in the hypothalamus or the pituitary gland.

• According to several studies, microadenomas (microscopic tumors of the pituitary gland) that are triggered by the pregnancy hormones (like prolactin) are responsible for the development of Chiari-Frommel syndrome.

• It is unknown what causes the unusual hormonal interaction between the pituitary and hypothalamus glands that is linked to Chiari-Frommel Syndrome.

• Chiari-Frommel Syndrome may also be caused by microscopic abnormalities of the hypothalamus, according to some research.

• The use of oral contraceptive pills is also associated with Chiari-Frommel syndrome.

How Is Chiari Frommel Syndrome Diagnosed?

The diagnosis of Chiari-Frommel syndrome involves:

• Symptoms: The patient’s medical history is recorded along with a thorough physical and clinical examination. The presence of all the hallmark features (excessive lactation, amenorrhea, anovulation, and uterus atrophy) suggests a diagnosis of Chiari Frommel syndrome.

• Blood Examinations: Blood samples are collected to detect any variations in the levels of several hormones. Blood prolactin levels in some Chiari-Frommel Syndrome patients may be very high. However, these levels of prolactin in other women may be normal. Unusual low levels of gonadotropins and other hormones (estrogen) in the urine may be additional laboratory results.

• Imaging Tests: Patients should be evaluated with a CT (computed tomography) scan or MRI (magnetic resonance imaging) for the presence of a pituitary tumor if the symptoms last for a long time.

How Is Chiari Frommel Syndrome Treated?

The treatment of Chiari-Frommel syndrome includes:

• Some cases of Chiari-Frommel syndrome subside spontaneously without any treatment within five years, while others may require therapy.

• To reduce the prolactin levels, doctors may prescribe the drug Bromocriptine. Normal ovulation cycles and regular menstrual cycles might be restored when normal hormone levels are restored.

• Hormone replacement therapy may also be advised in the case of Chiari Frommel syndrome to restore the levels of hormones that may be reduced (estrogen).

• In the case of abnormalities associated with the hypothalamus or the pituitary gland, specific treatment for that particular issue is done.

• In the case of pituitary microadenomas (microscopic tumors of the pituitary gland), surgery may not be done. However, larger pituitary tumors can be easily removed with the help of surgical excision.

What Are the Disorders Similar to Chiari Frommel Syndrome?

Symptoms of several disorders can be similar to that of the Chiari-Frommel syndrome; these include:

• Forbes Albright Syndrome: Forbes-Albright Syndrome is one of the few uncommon endocrine conditions marked by unusually high prolactin levels caused by a pituitary tumor. The production and release of milk from the breasts without an accompanying pregnancy or nursing period, as well as the absence of a regular menstrual cycle, are the characteristic symptoms of the condition. Although the body hair pattern and the sexual urge may be reduced, women with Forbes-Albright Syndrome typically have breasts and nipples that are normal in size and appearance.

• Ahumada-del Castillo Syndrome: Ahumada-del Castillo Syndrome is an endocrine condition marked by impaired hypothalamic and pituitary gland function that affects hormone secretion. Women are affected by this condition, and it is unrelated to pregnancy. The production and expression of milk from the breasts that is not related to childbirth or nursing, as well as irregular menstruation, are the two primary manifestations of this condition. The emergence of secondary sexual traits is normal.

Conclusion:

Chiari-Frommel syndrome is an uncommon endocrine disorder characterized by a group of symptoms. These symptoms may be due to small or large pituitary gland tumors, hypothalamus abnormalities, use of oral contraceptive pills, etc. The syndrome usually has a very good prognosis, and about 40% of the cases do not even require any treatment, and they resolve spontaneously within a period of five years. However, other cases may require hormone replacement therapy. Drug therapy or surgical intervention to treat the condition. The prognosis after treatment is also excellent.