Chiari–Frommel syndrome

Overview

Chiari–Frommel syndrome is a rare endocrine disorder that occurs in women after childbirth and is characterized by prolonged lactation (galactorrhea), absence of menstruation (amenorrhea), and infertility. It is a form of hyperprolactinemia, where the body produces abnormally high levels of prolactin—the hormone responsible for stimulating milk production. This syndrome is considered part of the broader category of postpartum pituitary dysfunction and is most often linked to persistent hormonal imbalance following delivery.

Causes

Chiari–Frommel syndrome is primarily caused by an abnormal increase in prolactin secretion from the anterior pituitary gland. Common causes and contributing factors include:

• Pituitary adenoma (prolactinoma): A benign tumor that secretes prolactin

• Postpartum pituitary dysfunction: Following complicated or stressful deliveries, including Sheehan’s syndrome (postpartum pituitary infarction)

• Hypothalamic dysfunction: Interruption in the inhibitory control of prolactin secretion

• Medications: Certain antipsychotics, antidepressants, and antihypertensives can raise prolactin levels

• Idiopathic cases: Where no specific cause can be identified

Symptoms

The hallmark symptoms of Chiari–Frommel syndrome typically develop in the months following childbirth and may persist for extended periods. These include:

• Galactorrhea: Persistent milk production unrelated to breastfeeding needs

• Amenorrhea: Absence of menstrual periods beyond six months postpartum

• Infertility: Due to suppression of ovulation and gonadotropin release

• Breast tenderness or engorgement

• Decreased libido and vaginal dryness

• Headaches or visual disturbances: In cases caused by pituitary tumors

Diagnosis

Diagnosis of Chiari–Frommel syndrome involves a combination of clinical history, physical examination, and hormonal testing. Key diagnostic steps include:

• Serum prolactin levels: Elevated levels confirm hyperprolactinemia

• Thyroid function tests: To exclude hypothyroidism as a cause of high prolactin

• Gonadotropin levels (LH, FSH): Often suppressed in hyperprolactinemia

• Pituitary MRI: To detect pituitary adenomas or other abnormalities

• Gynecological evaluation: To assess menstrual and reproductive status

Treatment

The goal of treatment is to normalize prolactin levels, restore menstrual function, and resolve galactorrhea. Treatment options include:

• Dopamine agonists: Such as bromocriptine or cabergoline, which suppress prolactin secretion and are first-line therapies

• Hormonal therapy: Estrogen-progestin replacement in women with persistent amenorrhea and low gonadotropins

• Surgical intervention: Rarely needed, but may be considered if a prolactinoma does not respond to medication

• Treatment of underlying conditions: Such as thyroid disorders or discontinuation of causative medications

• Fertility support: Ovulation induction may be necessary for women trying to conceive

Prognosis

With appropriate diagnosis and medical treatment, the prognosis for Chiari–Frommel syndrome is generally good. Most women respond well to dopamine agonist therapy, with resolution of galactorrhea, return of normal menstrual cycles, and restored fertility. If untreated, persistent hyperprolactinemia can lead to chronic infertility, bone loss (osteopenia), and psychological distress. Long-term follow-up is often recommended to monitor hormonal levels and prevent recurrence.