(1) Congenital simple inheritance.

(2) Early-onset primary lymphedema can be divided into: ① Lymphatic hypoplasia with hypodermic lymphopenia; ② Lymphatic dysplasia with small lymph nodes and lymphatic vessels; ③ Lymphatic hyperplasia, with large and numerous lymph nodes and lymphatic vessels, sometimes twisted and varicose.

Lymphatic hypoplasia is very rare and common in congenital lymphedema. Underdevelopment is the most common type. Both simple and reactive lymphedema are congenital. Early onset lymphedema is more common in adolescent women or young women, and menstrual symptoms are aggravated, so it is speculated that the cause may be related to endocrine disorders, accounting for 85% ~ 90% of primary lymphedema. It is called delayed lymphedema after the age of 35.

2. Secondary lymphedema

(1) Infectious parasites, bacteria, fungi, etc.

(2) Traumatic surgery, radiotherapy, burns, etc.

(3) Malignant primary tumor and secondary tumor.

(4) Other systemic diseases, pregnancy, etc.

clinical picture

Skin and subcutaneous tissue proliferate, skin wrinkles deepen, skin thickens, becomes hard and rough, and there may be thorns and verrucous protrusions that look like elephant skin. The early swelling of the affected limb can be relieved after elevation. In the late stage, the affected limb is obviously swollen, with rough surface keratinization and rubbery swelling. A few may have skin cracks, ulcers or warty vegetation.

cheque

1. Diagnostic puncture tissue fluid analysis

The analysis of subcutaneous edema tissue fluid is helpful to the differential diagnosis of difficult cases. Examination is usually used for chronic massive swelling of limbs, and can only be operated with syringes and fine needles. This method is simple and convenient. But we can't understand the lesion site and function of lymphatic vessels. This is a rough diagnosis method.

2. Lymphangiography

Contrast agent is injected into lymphatic vessels, and the photos show the morphology of lymphatic system, which is a specific auxiliary examination of lymphedema.

3. Isotopic lymphangiography

X-ray photos of lymphatic system can't provide quantitative dynamic data of lymphatic system function, nor can they provide simple information of lymphatic drainage in different limbs. At present, valuable static endolymphatic photos (radionuclide imaging) are taken, and 0.25ml(75MBq) of 99m sulfide colloid is injected into the subcutaneous tissue of the second webbed toes of both feet. The patient's lower abdomen and inguinal region were scanned with R camera at 1/2, 1, 2 and 3 hours respectively, and then the isotope uptake of ilioinguinal lymph nodes was calculated respectively. Using isotope imaging to study the lymphatic function of chronic lymphedema, it is suggested that the reduction of lymphatic reflux of the affected limb is related to the severity of lymphedema. In severe lymphedema, the isotope uptake rate is almost zero, while in venous edema, the absorption percentage of lymphatic reflux increases significantly.

4. Other inspections

In addition, the newly developed non-invasive vascular detection technology is also helpful to distinguish venous edema from lymphatic edema, which is simple and convenient as an outpatient screening method.

diagnose

Have a history of filariasis infection or recurrent erysipelas, or have a history of axillary and inguinal lymph node dissection and radiotherapy. The early swelling of the affected limb can be relieved after elevation. In the late stage, the affected limb is obviously swollen, with rough surface keratinization and rubbery swelling. A few may have skin cracks, ulcers or warty vegetation. Filariasis can be found in the blood around filariasis patients. Lymphangiography can determine the development or obstruction of lymphatic vessels. If necessary, lymph node biopsy can make a definite diagnosis.

treat cordially

According to the course of lymphedema, the treatment principle is different. In the early stage, the purpose is to eliminate stagnant lymph and prevent the regeneration of hydrolymph. In the later stage, the purpose is to surgically remove irrecoverable diseased tissue or treat localized lymphatic obstruction through shunt.

1. Acute lymphedema

Non-surgical treatment is the main method.

(1) Postural drainage aggravated lymphatic retention in the space. Lifting the affected limb by 30 ~ 40 cm can promote lymphatic reflux and reduce edema caused by gravity. This is not simple and effective, but the effect is not lasting, and the edema of the affected limb is aggravated again.

(2) Pressure bandaging On the basis of postural drainage, when the affected limb is raised, pressure bandaging is performed with elastic socks or elastic bandages to squeeze the tissue space and assist lymphatic reflux. The elasticity of the elastic bandage should be appropriate. It can also be used repeatedly for a long time, which has a certain effect on improving edema.

(3) Limit sodium intake. In acute phase, limit sodium chloride intake with diuretics, generally 1 ~ 2g/ day, to reduce tissue sodium and water retention. At the same time, use a proper amount of diuretics to speed up the discharge of water and sodium. Dichlorothiazide can be supplemented with potassium three times a day. Stop taking it when the condition is stable.

(4) Preventing infection, using antifungal ointment and powder and keeping toes dry are the most effective methods to prevent fungal infection; Bacterial infections under toenail beds are also more common. Qin Ying cut toenails to remove dirt and reduce the way of bacterial invasion. When streptococcal infection presents systemic symptoms, drugs such as penicillin should be selected to actively control the infection and stay in bed. Late lymphedema complicated with chapped skin can be protected and lubricated with ointment.

In addition, various vaccines, milk and foreign protein injections are long-standing anti-infection therapies. The various defense mechanisms of the human body have been improved. Foreign scholars have proved that when typhoid triple vaccine is injected, the number of lymphocytes exporting lymphatic vessels increases, and gamma globulin in blood also increases, which can prevent the occurrence of permanent lymphatic obstruction. Some authors speculate that foreign proteins may act through pituitary and adrenal glands.

2. Chronic lymphedema

Including non-surgical treatment and various surgical treatments.

(1) baking and stretching therapy is a therapeutic method to explore the medical heritage of the motherland. The principle of treatment is to use continuous radiant heat to dilate blood vessels in the skin of the affected limb, sweat a lot, and the liquid in the local tissue space returns to the blood to improve lymphatic circulation. For lymphedema patients who have not experienced severe limb skin hyperplasia, baking and stretching therapy can be used. There are two methods: electric radiation hyperthermia and oven heating.

(2) Surgery is not needed to treat most lymphedema. About 15% of primary lymphedema eventually needs lower limb plastic surgery. The existing surgical methods can not cure lymphedema radically except amputation, but can obviously improve the symptoms.