Introduction

 Growing a human is no small task. During pregnancy, the body undergoes dramatic changes: periods stop, breasts swell, and the body quite literally stretches to its limits to accommodate the new life. So, these changes may linger in the months after giving birth (the postpartum period)1 - but what if those changes persist for months or even years? This article aims to explore Chiari-Frommel Syndrome as a cause for these symptoms and shed light on its diagnosis and treatment. 

What is chiari-frommel syndrome?

Chiari-Frommel (key-AR-ee–FROHM-el) Syndrome is a rare hormonal disorder that can develop after childbirth.² It is also known as Galactorrhea-Amenorrhea Syndrome, named after its two main symptoms:³

• Galactorrhea (guh-lak-tuh-REE-uh) - the abnormal production of breast milk
• Amenorrhoea (uh-me-nuh-REE-uh) - the absence of menstrual periods.

Other possible symptoms include:³

• Anovulation - when ovulation is absent⁴
• Headache
• Back or abdominal pain
• Vision impairment
• Weight gain
• Decreased libido - when someone experiences less sexual desire than is normal for them⁵
• Uterine atrophy – shrinking of the womb^3,7

Some people with Chiari-Frommel Syndrome experience all these symptoms, while others may have only a few, or even just one. Either way, it is important to speak to a health professional if something doesn’t feel right. 

Understanding postpartum galactorrhea

Everyone has nipples, asall embryos initially start out as a female, before specific hormones tell the embryo to develop as either a boy or a girl.⁸ As a result, everyone has the basic plumbing necessary to make breast milk. So, galactorrhea - the production of milky discharge from the nipples - can occur in anyone regardless of sex or gender.⁹ But in the context of Chiari-Frommel Syndrome, we’re talking about postpartum galactorrhea, which is related to giving birth.

Think of the body as having a switch. Giving birth flips the body’s ‘milk switch’ on, allowing a person to feed their baby. Typically, it turns off after weaning, but sometimes it stays stuck on, causing the nipples to keep secreting milk when it is no longer needed. This is postpartum galactorrhea.³ For some, this can last months after stopping breastfeeding, or in some cases, even years. For people who decide not to breastfeed and choose alternatives like baby formula, the release of breast milk any more than six months after giving birth would also be postpartum galactorrhoea.¹⁰

Recognising postpartum galactorrhoea

The key signs of postpartum galactorrhoea include:

• Nipple discharge when not breastfeeding

This discharge is likely postpartum galactorrhoea if:

• You gave birth more than six months ago and are no longer breastfeeding¹⁰
• The discharge looks milky (white, cream, or slightly green or yellow)¹¹
• It is bilateral (leaking from both nipples)¹⁰
• It is coming out of multiple nipple ducts (appearing at multiple spots, like water spraying from a shower head)⁹
• It appears randomly, or when the nipple is rubbed or squeezed¹⁰

Everyone’s body is different, so if you’re experiencing any of these symptoms or something just feels off, it’s worth having a chat with a healthcare professional. They can help determine if it’s postpartum galactorrhea - or Chiari-Frommel Syndrome if other symptoms are present - and talk you through your treatment options (more on that below).

Importantly, if you have nipple discharge that is:

• Bloody¹⁰
• Unilateral (leaking out of one nipple only)¹⁰
• Coming out of a single duct¹¹

This could be a sign of a more serious problem.^12,13 If you notice any of these symptoms, see your healthcare provider as soon as possible. 

Understanding amenorrhoea 

Periods might not be the most welcome monthly visitor, but they are a valuable sign that our hormones are doing their jobs properly. 

Every cycle, rising levels of a hormone called progesterone cause the endometrium lining to thicken in case of pregnancy.¹² If no pregnancy occurs, progesterone levels drop, and the lining sheds, resulting in  a period. But when a person is  pregnant, the lining remains intact to nourish the growing foetus, and periods stop altogether. This is called amenorrhoea.¹³ In this context, it is called secondary amenorrhoea – where periods stop for more than 3-6 months after they have already started.¹⁴ Meanwhile, primary amenorrhoea is a diagnosis given to someone who has never had periods to begin with, usually by the age of 15.¹³

Amenorrhoea is the new norm during pregnancy. But it is a drastic change from the previous pattern So, after nine months of no periods, it can take a while for our bodies to get back into its original rhythm. Accordingly, amenorrhoea is perfectly normal in the postpartum period, especially when breastfeeding. But when amenorrhoea persists for a long time after giving birth, this may become a problem, especially if you want to get pregnant again.

Recognising amenorrhoea

So, what are the symptoms of amenorrhoea?

• Lack of a period for more than 3-6 months after giving birth - the timeline here depends on how frequently you breastfeed your baby. If you decide not to or cannot breastfeed, your period may return as soon as three months postpartum, while breastfeeding may delay the return of your period for as long as six months¹⁵

This is the hallmark feature of amenorrhoea, but some other signs to look out for include:¹⁶

• Pelvic pain
• Vaginal dryness
• Hair loss or excessive hair growth
• Headaches

Finally, while not a symptom of amenorrhoea itself, many people with amenorrhoea also experience postpartum galactorrhea.¹⁷ As previously stated, when these two symptoms co-occur, this is a sign of Chiari-Frommel Syndrome.

What causes chiari-frommel syndrome?

There is no one cause of Chiari-Frommel Syndrome – it varies from person to person. But one of the most common causes is hyperprolactinemia.

Hyperprolactinemia is a condition where the levels of prolactin – a hormone that stimulates breast milk production and regulates the menstrual cycle – in the blood are abnormally high.¹⁸ This can make the body think it should continue or start producing breast milk and stop having periods even when it does not need to.

There are several causes of hyperprolactinaemia:

• A tiny tumour in the pituitary gland – a structure in the brain that controls the release of prolactin. This sounds scary, but these tumours are usually non-cancerous and can be treated¹⁹
• Side effects of medications, e.g. oestrogen-based hormone therapies, antipsychotics, or antidepressants²⁰
• Hypothyroidism – underactivity of the thyroid¹⁸

Please read one of our previous articles for more information regarding the potential causes of Chiari-Frommel Syndrome.

Diagnosing chiari-frommel syndrome

So, how is Chiari-Frommel Syndrome diagnosed?

Firstly, if you suspect you may have the condition, it is essential to monitor any symptoms you experience (see above for what to look for) and your menstrual cycle. This can help your healthcare provider understand what’s going on and know what follow-up questions to ask.

After discussing your symptoms and medical history, your doctor may suggest a few tests, such as:

• Blood tests to check hormone levels (especially prolactin). They will usually take a sample from you around mid-morning or later to avoid the natural spike in prolactin associated with sleep²¹
• Breast examination – to check the source and appearance of any nipple discharge¹⁷
• Brain scan – usually an MRI (magnetic resonance imaging) of the pituitary gland if a tumour is suspected¹⁸

 Treating chiari-frommel syndrome

While Chiari-Frommel Syndrome can be distressing, the good news is that treatment is straightforward and usually successful. Here are some of the options available:

• Medication – bromocriptine (administered once daily) or cabergoline (administered once or twice a week) can be taken to lower prolactin levels in the blood.²¹ Research suggests these medications are effective at normalising prolactin levels in up to 95% of patients, and improve postpartum galactorrhoea (78% of cases) and amenorrhoea (86% of patients).²² These medications are typically taken for 2 years or more²¹
• Stopping or changing medication – if Chiari-Frommel Syndrome is related to hyperprolactinemia induced by a specific drug, adjusting your dose or medication type might relieve the symptoms· You should not do this without consulting a doctor first
• Surgical therapy – this is rare, and usually only considered if an especially large pituitary tumour is present, or medication has been unsuccessful¹⁸

In a nutshell, with early diagnosis, the right treatment and continued support, the majority of people with Chiari-Frommel Syndrome can recover or are able to manage the condition.

Summary

• Chiari-Frommel Syndrome is a rare but treatable postpartum disorder
• Its main symptoms are postpartum galactorrhea (milk production from the nipples after stopping breastfeeding) and amenorrhoea (a lack of menstrual periods)
• It is usually caused by persistently high levels of prolactin following pregnancy, often due to hormonal imbalances, certain medications or small non-cancerous growths on the pituitary gland
• Diagnosis usually involves a simple blood test and physical examination, taking a patient's history, and brain scans, where necessary
• Most importantly, people recover with the right treatment – usually medication, or in some rare cases, surgery
• While the symptoms of Chiari-Frommel Syndrome can be worrying, support is available. If something doesn’t feel right in the months after you give birth, remember this: you know your body best, so don’t be afraid to ask for help