Chiari-frommel syndrome.

Chiari-Frommel syndrome is a chronic galactorrhea, amenorrhea and increasing hypotrophy of the reproductive organs. This pathology was long attributed to postpartum consequences. However, later the disease was also discovered in nulliparous patients: the syndrome developed as a result of severe stress, malignant pituitary tumors, and after prolonged use of hormonal drugs.

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Causes Chiari-frommel syndrome

The exact cause of the syndrome is not fully understood. One of the causes of Chiari-Frommel syndrome is considered to be increased release of prolactin by cells of microscopic tumors of the pituitary gland (microadenomas). However, the disease can be caused by any damage to the hypothalamus or pituitary gland that affects the production of prolactin.

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Pathogenesis

The pathogenesis of the syndrome is explained by a functional failure of prolactin production. Such a failure can occur as a result of pregnancy (due to a natural increase in pituitary lactotrophs), as a result of pituitary tumors. The following factors accelerate the development of the disease:

• prolonged lactation period;
• complicated pregnancy (with risk of miscarriage, with gestosis);
• long-term use of hormonal contraceptives.

Most scientists tend to consider Chiari-Frommel syndrome as damage to the hypothalamus with subsequent endocrinological disorders. The possibility of pressure on the hypothalamus by a tumor process is also not excluded.

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Symptoms Chiari-frommel syndrome

The first signs of Chiari-Frommel syndrome may appear at the age of 17-35. Most often, this occurs after the end of breastfeeding, when a woman discovers lactorea (abnormal secretion of milk) and amenorrhea (absence of menstruation). The syndrome may also occur without any connection to pregnancy.

In most cases, patients develop symptoms that indicate damage to the diencephalic zone:

• change in body weight (in one direction or another);
• headaches;
• dizziness, fatigue;
• hypopigmentation of the areola;
• increased hair growth;
• cessation of menstruation;
• sleep disorders;
• mood instability, capriciousness;
• hyperhidrosis;
• decreased sexual desire, etc.

During a medical examination, atrophy of the reproductive organs of varying degrees is detected, including thinning of the mucous tissues and endometrium. In some patients, visual changes are absent.

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Complications and consequences

Chiari-Frommel syndrome develops as a result of a breakdown in the main regulatory link - the hypothalamus. Those cellular structures that are responsible for the production of regulatory hormones are destroyed. This occurs against the background of the absence of a logical decrease in the function of the pituitary gland. As a result, the uncontrolled pituitary gland excessively produces prolactin - a hormone that activates lactation. What can be the consequences of these disorders?

• Simultaneous decrease in estrogen production.
• Failure of FSH and LH production.
• Irregular menstrual cycle with absence of ovulation.
• Atrophic processes in the ovaries and external genitalia.
• Changes in behavior, psyche and neurovegetative system.

All of the listed consequences are interconnected and sooner or later lead to such a complication as infertility – the inability to conceive a child.

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Diagnostics Chiari-frommel syndrome

Diagnosis of a disease such as Chiari-Frommel syndrome is based on several factors:

• based on information obtained during the patient interview (for example, recent pregnancy or abortion);
• on the characteristic signs of the disease;
• based on the results of additional research.

Instrumental diagnostics involves conducting an X-ray examination or MRI of the sella turcica (location of the pituitary gland and hypothalamus). An ultrasound of the pelvic organs and mammary glands may also be prescribed.

Laboratory tests include cytological and histological analyses of scrapings from the walls of the vagina and cervix. In addition, patients give blood from a vein for the amount of hormones FSH, LH, prolactin, estradiol, progesterone, releasing hormones.

The diagnosis of Chiari-Frommel syndrome is made if an increase in prolactin levels is detected simultaneously with a general decrease in hormonal levels in the body. Additional signs should include:

• atrophy of mucous tissues;
• absence of ovulation;
• enlargement of the sella turcica, or the presence of tumors in the nearby area.

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Differential diagnosis

Differential diagnostics are carried out with such a disease as fibrocystic mastopathy. With prolonged lactation against the background of deterioration of visual functions, a tumor process in the brain (for example, pituitary adenoma) can be suspected.

Treatment Chiari-frommel syndrome

Chiari-Frommel syndrome is relatively successfully treated. The treatment regimen directly depends on the factor that provoked the development of the disease.

If the pathology appeared as a result of damage to hypothalamus cells, treatment is carried out with drugs containing hormones. With a long-term course of the syndrome with pronounced tissue atrophy, treatment becomes more complex and combined.

Infertility resulting from Chiari-Frommel syndrome is not treated separately, since after normalization of the pituitary-hypothalamus function, reproductive function will be restored on its own.

In advanced cases, lifelong use of certain medications may be required.

Patients diagnosed with the disease use the following types of therapy.

Medicines that inhibit prolactin production:

• Bromocriptine is a dopamine receptor stimulant. The drug is prescribed from 5 to 10 mg per day, the duration of therapy is up to 8 months. Bromocriptine cannot be used with low blood pressure, with arrhythmia. The drug is incompatible with alcohol.

Hormonal agents:

• Puregon is a recombinant follicle-stimulating hormone. The drug is used in the form of subcutaneous and intramuscular injections, according to an individual scheme. Puregon can only be used under the supervision of a doctor, since there is a high risk of developing ovarian hyperstimulation;
• Menogon is a human menopausal gonadotropin containing FSH and LH. Menogon is not used for estrogen-dependent tumors. The average dosage of the drug is 1-2 ampoules per day. The dosage is prescribed by a doctor, observing the ovaries' response to treatment.

Preparations containing ovarian hormones:

• Estrogel is a gel with estradiol. The drug is applied to the skin in the abdominal area, in the amount of 2.5 g once a day. Do not use in the presence of estrogen-dependent neoplasms;
• Crinone is a vaginal preparation containing progesterone. The standard dosage is 1 dose of Crinone on a certain day of the cycle, daily, according to an individual regimen.

Vitamins and complex preparations to support reproductive function:

• Aevit - contains large doses of vitamins A and E. Take 1 capsule once a day, immediately after meals. The duration of treatment is determined by the doctor. The drug is not prescribed for thyrotoxicosis, as well as for patients with an increased risk of thromboembolism.
• Vitamin E Zentiva is an antioxidant drug that regulates the functioning of the sexual sphere. Usually prescribed 1 capsule of 400 mg per day. Long-term use of vitamin E in large doses can disrupt the metabolism of thyroid hormones.

Physical therapy treatment for Chiari-Frommel syndrome may include:

• endonasal electrophoresis with a complex of B vitamins is an effective procedure that facilitates the delivery of drugs to the brain and the back of the eyeball;
• transcerebral therapy is a physiotherapeutic method often used to relieve functional disorders of the central nervous system. It may include procedures such as electrosleep, transcranial electroanalgesia, amplipulse therapy, transcerebral ionization;
• galvanic collar – use of low voltage direct electric current.

Physiotherapy is not used in cases of proven tumor processes in the brain.

Homeopathy is an effective treatment and support of sexual function without pronounced side effects. At the discretion of the doctor, the following homeopathic preparations may be prescribed:

• Cyclodinone is a combined herbal remedy that normalizes hormonal levels in the body. The standard dose is 40 drops or 1 tablet once a day in the morning before breakfast. The minimum continuous course of therapy is 90 days. Before using the drug, you must make sure that you are not allergic to its components.
• Ovariamin is a cytamin with an estrogen-like effect. The drug is taken as prescribed by a doctor, from 1 to 9 tablets per day. The duration of treatment is 2 weeks. There are no side effects when taking Ovariamin.
• Ovarium compositum is a homeopathic injection solution that is actively used for problems with the pituitary-ovarian relationship. The drug is administered intramuscularly, 1 ampoule, from one to three times a week.
• Klimakt Hel - sublingual tablets that activate the function of the appendages and normalize the work of the anterior part of the hypothalamus and pituitary gland. Take 1 tablet three times a day. The drug has virtually no contraindications (the only contraindication is allergy);
• Conium Plus is a homeopathic multi-component granule that is used sublingually (under the tongue) 8 pcs. up to 5 times a day. The course of therapy is up to 2 months. Contraindications: age under 18, tendency to allergies.

If tumor processes are detected in the pituitary gland, surgical treatment is prescribed. Surgical intervention can be supplemented by radiation therapy, the introduction of radioactive iodine, cobalt, etc.

In the postoperative period, hormone replacement therapy is indicated, and, if necessary, restoration of metabolic processes.

Traditional medicine, as an independent type of therapy, is not used for Chiari-Frommel syndrome. However, it can be successfully combined with the main treatment prescribed by the doctor, provided that all his instructions are followed. It is necessary to take into account that some medicinal herbs can affect the hormonal background, so their use must be agreed with the doctor.

• At the very beginning of the disease, an equal mixture of peeled pumpkin seeds, natural honey and sesame seeds, ground ginger root and primrose plant can help. The mixture is taken 4 times a day, 1 teaspoon.
• In case of malfunctions of the pituitary system, a 10% tincture of bedbug, which can be purchased at a pharmacy, helps well. The drug is diluted in water in the proportion: 10 drops of medicine per 100 ml of water.
• In Chiari-Frommel syndrome, hemlock infusion in olive oil helps, which is used for nasal drops. There are also good reviews of 10% alcohol hemlock tincture, which is taken orally, starting with 1 drop and gradually increasing the dosage to 40 drops, adding 1 drop every day. After 40 days, they begin to reduce by 1 drop, gradually reducing the dose to the minimum.
• An infusion of rowan, sage, valerian, plantain and lemon balm is beneficial for Chiari-Frommel syndrome. Take 1 tbsp of each component, pour boiling water over it and drink 100 ml before each meal.
• It is also recommended to add chamomile, marigold, oregano, adonis and immortelle flowers to the tea. This drink should be consumed half an hour before meals.

Herbal treatment involves the regular use of the following plants in the preparation of drinks:

• Motherwort is a herb that has a pronounced cardiotonic and regulating effect;
• valerian - rhizomes with medicinal neuroregulatory and vasodilating properties;
• Melissa - leaves that have capillary-stimulating, antispasmodic properties. Reduces smooth muscle tone;
• St. John's wort is a herb with a multifaceted healing effect. It serves as a catalyst for intracellular processes and regulates metabolism;
• hop cones – a plant with neurotropic action. Regulates metabolic processes;
• hawthorn fruits are a plant rich in flavonoids, organic acids, carotenoids, fatty oils, glycosides, etc.;
• elderberry – reduces the permeability of vascular walls, relieves inflammation and swelling, restores the function of vital organs;
• Passionflower is a tropical plant that is successfully used for climacteric and menstrual disorders.

The production of prolactin is inextricably linked with the amount of progesterone in the blood. Therefore, with the permission of the doctor, it is possible to take preparations from plants with a progestogenic effect. These are herbs such as raspberry leaves, medicinal lady's mantle, sacred vitex, meadow pasqueflower, goose cinquefoil.

Prevention

There is no specific prevention of Chiari-Frommel syndrome, since the pathogenesis of the disease has not yet been fully studied. General preventive measures may include possible influence on the development of primary disorders that provoke secondary hyperfunction of the pituitary-hypothalamus system.

In this regard, preventive measures include:

• prevention of complications during pregnancy and childbirth;
• preventing stress on the body, creating a positive psychological environment at home and at work;
• protection against all kinds of traumatic brain injuries;
• prevention of hormonal imbalances.

Unfortunately, there are no measures yet to prevent primary excess prolactin production.

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Forecast

The prognosis of the disease depends on the cause of the pathological synthesis of the hormone prolactin, as well as on the chosen method of treatment. Thus, with successful correction of hormonal disorders, the syndrome passes without a trace, and the woman can easily become pregnant and give birth to a child. In some cases, taking hormonal drugs can continue for life.

When tumor processes are detected in the pituitary gland, the prognosis depends only on the effectiveness of the surgical and restorative treatment.

The above-described methods of therapy, which combine the lactation-blocking effect of estrogens and physiotherapy procedures that regulate the pituitary-hypothalamic relationship, lead to a lasting improvement in the condition of patients with a disease such as Chiari-Frommel syndrome.

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