Trenone syndrome

Trenaunay syndrome (also known as Klippel-Trenaunay syndrome, or Klippel-Weber-Trenaunay syndrome, or varicose osteohypertrophic nevus) is an abnormal development of deep-lying main venous vessels of the legs against the background of deterioration of their patency. The disease received its name from the French doctor P. Trenaunay, who conducted research on this pathology.

Epidemiology

Trenaunay syndrome is most often discovered by chance and is recorded as isolated sporadic cases.

The frequency of occurrence of the syndrome in the population is unknown. It is believed that the disease affects mainly boys, although this statement has no real and substantiated evidence.

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Risk factors

Risk factors leading to disruption of embryogenesis in early pregnancy:

• chemical poisoning;
• taking prohibited drugs;
• stress loads;
• exposure to radioactive radiation;
• Infectious diseases – viral and microbial infections.

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Pathogenesis

The pathogenesis of Trenaunay syndrome is believed to be a disturbance of embryogenesis with dysplasia of the deep venous vessels.

The possibility of mechanical damage to the embryo at the time of the formation of the vascular system is also not excluded.

However, Trenaunay syndrome has been found in some cases in patients from one family line, which is represented mainly by women.

There is no reliable and definitive information about the variants and possibility of genetic inheritance of the disease yet. However, scientists insist that the degree of danger of inheriting Trenaunay syndrome is reduced to a minimum.

It is generally accepted that increased venous pressure develops as a result of deterioration of patency in deep veins. Blood from deep venous vessels passes into subcutaneous veins. A hypoxic condition develops, trophism of limb tissues worsens, fluid accumulation in tissues and lymphostasis occur. Additionally, venous-arterial anastomoses open, which leads to worsening of circulatory disorders.

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Symptoms of Trenaunay syndrome

The first signs of Trenaunay syndrome can be detected already in a newborn child. Less often, such signs proceed unnoticed and appear after 2 years.

The characteristic symptoms of the disease are considered to be:

• Vascular hemangiomas are peculiar spots, quite large in area and located in most cases on one limb. The spots can be light or dark purple. The skin above them is very vulnerable: it is damaged and bleeds with the slightest physical impact.
• Varicose veins and dilated subcutaneous venous vessels of the lower limb: the veins thicken in places, twist, and hurt when touched.
• The affected lower limb is usually larger in volume than the healthy leg. The size of the foot, the volume of the shin, and the length of the affected leg may be increased. Such changes occur due to pathology of the deep venous vessels.

The patient may have all three signs, or only two of them. Depending on this, the following types of Trenaunay syndrome are distinguished:

• without vascular hemangiomas;
• without limb enlargement;
• without varicose veins.

It is also possible to develop “cross” types of syndrome, when vascular spots appear on one leg, and hypertrophic signs on the other limb.

Sometimes the syndrome develops slowly, and sometimes rapidly: in the second case, the pathology spreads from the affected leg to the lower, and then to the upper part of the body.

Additional, but optional, symptoms may include:

• cataracts;
• glaucoma;
• colobomas;
• heterochromia of the iris;
• gastrointestinal bleeding;
• blood in urine;
• visceromegaly;
• lipodystrophy;
• convulsions;
• additional large vessels.

Stages

• Stage 0 – no visible symptoms of venous disease.
• Stage 1 – the presence of spider veins, visible network of veins, areas of reddened skin.
• Stage 2 – presence of varicose veins.
• Stage 3 – presence of limb hypertrophy without skin changes.
• Stage 4 – the presence of visible skin changes in the form of pigmentation, venous eczema, dermatosclerosis.
• Stage 5 – presence of visible skin changes with a healed ulcer.
• Stage 6 – the presence of visible skin changes with an ulcerative process in the active phase.

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Complications and consequences

Trenaunay syndrome can lead to the development of other pathological conditions that will affect not only the vascular network, but also the internal organs of the patient.

In most cases, changes in the vessels provoke the development of "elephantiasis" and lymphangiomas. External signs of the disease are gigantism, dislocation of the hip joint (as a result of a violation of the formation of the musculoskeletal system), non-closure of the palate or lip.

If the pathology spreads to the organs of the digestive system, internal bleeding and rectal bleeding (if the veins of the rectum are affected) may occur.

If timely assistance is not provided, extremely negative consequences may develop, one of which is death.

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Diagnostics of Trenaunay syndrome

The diagnosis of Trenaunay syndrome is established based on the collected information about the anamnesis and the results of the physical examination. Additional examination methods may include:

• tests – general blood and urine tests, biochemical blood tests, blood clotting tests, cholesterol blood tests, blood glucose tests;
• instrumental diagnostics – ultrasound examination with Doppler, magnetic resonance imaging, angiography (venography).

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Differential diagnosis

Differential diagnosis of Trenaunay syndrome is carried out with such diseases and conditions as:

• varicose symptom complex (occurs without hemangiomas and hypertrophic changes in the skeletal system);
• Servelli syndrome (characterized by the appearance of enlarged varicose veins with the formation of calcifications in them, against the background of osteoporosis and shortening of the limbs);
• Maffucci syndrome (characterized by the fact that, in addition to vascular spots, there is bone dyschondroplasia);
• Bean's syndrome (occurs with the formation of cavernous hemangiomas and the development of gastrointestinal bleeding).

In some cases, Trenaunay syndrome must be differentiated from lymphedema and Sturge-Weber syndrome.

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Treatment of Trenaunay syndrome

Treatment of Trenaunay syndrome presents certain difficulties and largely depends on the results of the examination and the general condition of the patient.

The main types of treatment for the syndrome usually include:

• use of compression garments and clothing;
• laser treatment;
• sclerotherapy;
• surgical treatment;
• drug therapy.

Treatment of Trenaunay syndrome is carried out with several types of medications, which are prescribed in combination or depending on the existing symptoms.

It is imperative to prescribe medications that improve the tone of the vascular wall: Detralex, Aescusan, Troxevasin, etc.

In the presence of an inflammatory process, anti-inflammatory drugs are prescribed, which are used both in the form of tablets and in the form of external preparations: Ketoprofen, Voltaren, Ibuprofen.

If there is a high probability of thrombus formation, Heparin, Curantil, and Fraxeparin are used.

	Method of administration and dosage	Side effects	Special instructions
Detralex	Take 2 tablets daily, after meals.	Dyspepsia, headaches, skin rashes.	Detralex combines well with other drugs.

	Method of administration and dosage	Side effects	Special instructions
Troxevasin	Take after meals, 1 capsule three times a day. The duration of treatment can be 3-4 weeks or more.	Nausea, heartburn, headaches, stomach ulcers.	The use of the drug to treat children under 15 years of age has not been studied.

	Method of administration and dosage	Side effects	Special instructions
Ibuprofen	For pain, use 200 mg three times a day.	Abdominal pain, dyspepsia, liver pain, shortness of breath, increased blood pressure, anemia.	Do not use to treat children under 12 years of age.

	Method of administration and dosage	Side effects	Special instructions
Curantil	Take on an empty stomach, from 75 mg per day, depending on the doctor’s prescription.	Tachycardia, abdominal pain, headache, feeling of weakness, nasal congestion.	Not used to treat children under 12 years of age.

	Method of administration and dosage	Side effects	Special instructions
Fraxiparin	It is used in the form of subcutaneous injections. The dosage is selected individually.	Allergy, thrombocytopenia, local hypersensitivity reactions.	Prescribed only to patients over 18 years of age.

To improve tissue restoration and reparation, complex vitamins are used:

• Actovegin is a medicinal product created on the basis of blood components – deproteinized hemoderivative;
• Ascorutin is a drug that replenishes the deficiency of vitamins C and P;
• Ascorbic acid is a well-known vitamin that takes part in the production of collagen and strengthens vascular walls;
• Aevit – prevents the development of atherosclerotic changes in blood vessels.

In addition, it is necessary to adhere to a diet with a high content of ascorbic acid and calcium. A natural source of vitamin D is dosed exposure to the sun. If such exposure is impossible for some reason, the doctor can prescribe 400 to 800 IU of vitamin D.

Walking, light jogging and aerobic exercise are encouraged.

Physiotherapy treatment

Complex treatment of Trenaunay syndrome is often supplemented by periodic courses of physiotherapy. The following procedures are considered the most common:

• UHF, or the use of infrared radiation, is based on the effect of heat, which helps to increase blood circulation;
• darsonvalization – exposure to high-frequency pulsed currents;
• Diadynamic currents are the use of direct electric current with low voltage and low power;
• treatment with leeches – hirudotherapy;
• Sollux is a light therapy using a special incandescent lamp.

Folk remedies

In case of Trenaunay syndrome, one can actively use traditional medicine recipes, which involve both the use of infusions and decoctions internally, and the external application of various agents to the affected limb.

Patients with Trenaunay syndrome should pay special attention to the following recipes:

• At night, it is useful to apply several burdock leaves to the sore limb, with the inner side of the leaf to the leg. The leaves are bandaged and removed only in the morning, using this treatment every other day. If burdock leaves are not available, they can be easily replaced with horseradish leaves.
• Prepare an infusion of 100 g of grated nutmeg and 500 ml of vodka (infuse for 2 weeks, in the dark, stirring occasionally). Strain the infusion and drink 20 drops in the morning before breakfast, before lunch and before dinner.
• Prepare a tincture of Kalanchoe: for 200 g of plant leaves take 500 ml of vodka, leave for a week, filter. The tincture can be used to rub the sore limb.
• Take 500 g of peeled garlic cloves, grind them in a meat grinder, combine with 700 ml of honey, mix and leave in the refrigerator for a week. Then take the remedy 1 tbsp. three times a day immediately before meals. Duration of treatment is at least 2 months.

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Herbal treatment

To alleviate the condition of Trenaunay syndrome, it is useful to use medicinal plants such as:

• Sweet clover - has a positive effect on intravascular blood coagulation processes, improves blood circulation in the extremities;
• peony, raspberry - they contain salicylic acids, which prevent the formation of blood clots;
• hop cones – improve venous tone;
• buckthorn, alder – eliminate signs of inflammation;
• Horse chestnut – strengthens vascular walls, reduces permeability.

It is useful to do foot baths daily for 15 minutes using mint and chamomile infusion.

Homeopathy

Homeopathic remedies will support and improve the effect of traditional drug treatment for Trenaunay syndrome. Among homeopathic remedies, the following are most suitable:

• Carbo vegetabilis – natural charcoal (from beech or birch) improves blood circulation and hematopoiesis, eliminates congestion in the venous network (in particular, the functioning of the inferior vena cava system is improved).
• Pulsatilla is a drug that improves venous circulation, which is especially relevant for congestive processes in the portal and vena cava system.
• Silicea is a preparation based on silicic acid, which improves the rheological properties of blood, increases venous tone and facilitates intra-tissue microcirculation.

The dosage of the listed drugs is prescribed individually, depending on the characteristics of the disease and the age of the patient.

Side effects when taking homeopathic remedies are usually kept to a minimum: only rarely is it possible to develop an allergic reaction to the components of the drug.

Among complex homeopathic remedies, the drug Aesculus compositum is especially popular. The drug is taken orally, 10 drops, previously dissolved in 30 ml of clean water. Dosage: three times a day half an hour before meals, or 1 hour after meals. Duration of therapy is at least 4-5 weeks. Aesculus compositum is not prescribed during pregnancy, as well as in childhood (under 18 years). Before using the drug, you should definitely consult a doctor.

Surgical treatment

Surgical treatment is one of the main treatments for Trenaunay syndrome. However, not all patients decide to undergo surgery, as the likelihood of developing unfavorable postoperative consequences is very high.

For Trenaunay syndrome, your doctor may recommend the following surgical options:

• If the narrowing of the vessels is considered critical, then bypass surgery may be performed – the creation of an additional vessel that bypasses the narrowed area and restores normal blood flow.
• In case of significant damage to the main vein, a section of the vessel is replaced with an artificial analogue. This operation is called vascular transplantation.
• To “turn off” narrowed small venous vessels, sclerotherapy can be used – the introduction of a specific sclerosing drug into the lumen of the vein, which causes the vascular walls to connect and the subsequent resorption of the no longer used vessel.
• Laser surgery, or laser angioplasty, can be used to remove small spider veins or isolated superficial venous networks.

Before prescribing an operation, the doctor is obliged to inform the patient and his relatives about the possible negative consequences of the surgical intervention. The final decision on the operation is made by the patient (or his relatives, if the patient is a child).

It is generally accepted that the most optimal results after surgical treatment can be expected if the operation is performed at the age of 6-7 years. If there are indications, the intervention can be performed even in the neonatal and infancy period.

Prevention

Prevention of further progression of Trenaunay syndrome consists of following the following recommendations:

1. It is necessary to undergo regular treatment courses with thinning and anti-inflammatory agents, strengthen blood vessels and take medications that improve blood circulation. Preventive treatment should be aimed at preventing thrombosis and narrowing of the vascular walls.
2. Prevention should also include proper nutrition. The diet for Trenaunay syndrome should limit the amount of salt, sugar, baked goods, smoked and fatty foods, sausages and semi-finished products. The emphasis in nutrition should be on vegetable and fruit dishes, greens, berries, cereals and vegetable oils.
3. Drink plenty of fluids every day. Water naturally thins the blood, improving circulation and preventing blood clots.
4. It is not recommended to use too often: rose hips, nettle, valerian, St. John's wort. Decoctions and infusions of the listed plants can lead to the opposite effect and worsen the condition of a patient with Trenaunay syndrome.
5. You should “forget” about alcohol, smoking, taking diuretics and hormonal contraceptive pills.
6. It is necessary to exclude hypodynamia from your life. Dosed physical activity is recommended. Swimming, walking, yoga, dancing are especially welcome.

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Forecast

Trenaunay syndrome may be accompanied by the development of adverse health complications. The condition may worsen due to cardiovascular failure. Hemangiomas may form in the brain, causing the onset of neurovegetative disorders.

Children usually survive and become adults, but the prognosis for the syndrome remains serious. If surgery is successful, Trenaunay syndrome goes into a long-term stable remission.

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