Lymphedema refers to subcutaneous fibrous connective tissue hyperplasia and liposclerosis caused by the obstruction of lymphatic reflux in some parts of the body after repeated infection of soft tissue fluid on the body surface. If it is a limb, it will thicken, and the skin will thicken in the later stage, rough and tough as elephant skin, also called "elephant skin swelling".

diagnose

1. Have a history of recurrent filariasis infection or erysipelas, or have a history of axillary and inguinal lymph node dissection and radiotherapy.

2. 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.

3. microfilaria can be found in the peripheral blood of patients with filariasis. Lymphangiography can determine the development or obstruction of lymphatic vessels.

Treatment measures

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.

(a) acute lymphedema, mainly non-surgical treatment.

1. Postural drainage makes lymphatic retention in the interstitial space more serious. 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, pressure bandaging is performed on the fingers of the affected limb with elastic socks or elastic bandages to squeeze the tissue space and assist lymphatic reflux. The elasticity of the elastic bandage should be appropriate. Intermittent compression pump can also be used for many times and for a long time, which has a certain effect on improving edema. Literature reports that lymha-press is an advanced and effective inflator abroad. The inflatable device is divided into 9 ~ 12 pieces, each piece can be inflated and pressurized independently, and the pressurization is gradually carried out from the distal end to the proximal end of the limb, and the cycle is no more than 25 weeks. Compared with other simple compression devices, the inflation time of seed lymph is greatly shortened (the cycle period of simple compression device is about 100 second), and at the same time, it can generate higher pressure as high as15.6 ~ 20.8 kPa (120 ~160 mmhg), which is more effective than surgery and simple elastic socks to reduce swelling. However, its use is complicated, and the protein component in the gap cannot be reduced. It is only suitable for short-term treatment such as acute phase and preoperative preparation.

3. Limit sodium intake and use diuretics to appropriately limit sodium chloride intake in acute phase, generally 1 ~ 2g/d, so as 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. Dihydrochlorothiazide 25mg each time, 3 times a day, and appropriate potassium supplementation. Stop taking it when the condition is stable.

4. Preventing infection, choosing antifungal ointment and powder and keeping toes dry are the most effective ways 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. 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 includes 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. The temperature is controlled at 80 ~ 100℃, 1 time every day, 1 hour every time, and 20 times is a course of treatment. The interval of each course of treatment is 1 ~ 2 weeks. Elastic bandages should be added after each treatment. According to clinical observation, after 1 ~ 2 courses of treatment, it can be seen that the affected limb tissue becomes soft and the limb gradually shrinks, especially the number of erysipelas-like attacks is greatly reduced or stopped.

2. Most lymphedema can be treated by surgery without surgery. 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.

Indications for operation: ① Limb function damage: due to heavy limbs, fatigue and limited joint activity. ② Excessive swelling with pain. ③ Recurrent cellulitis and lymphangitis were ineffective after medical treatment. ④ Lymphangiosarcoma: the cause of death of long-term lymphedema malignant tumor. ⑤ Beauty: Primary lymphedema is mostly young women. For those with obvious swelling and cosmetic requirements, surgery can be considered, but the function improvement should be the main one, and the beauty should be supplemented by the state, otherwise the curative effect may not be ideal.

Preoperative preparation and postoperative treatment:

⑵ Preoperative preparation plays an important role in the surgical effect. They include: ① bed rest to raise the affected limb: reduce limb edema to a minimum. There are lower limb pads, lower limb suspension, bone traction and other methods, and the lower limb is raised by 60? Suitable. ② Infection control: For recurrent acute cellulitis and acute lymphangitis, sensitive drugs should be injected intravenously or intramuscularly before and during operation to reduce the chance of skin flap infection after operation. ③ Cleaning skin: to achieve the purpose of ulcer healing or control of local infection. ④ Keep postoperative drainage unobstructed; The separated rough surface may have continuous capillary exudate. Negative pressure drainage must be placed to keep no hematocele and effusion under the flap, reduce the factors affecting the blood supply of the flap, prevent necrosis and infection of the flap, and reduce the failure rate of the operation. ⑤ Continue to raise the affected limb after operation to reduce the edema of the affected limb, which is beneficial to venous and lymphatic reflux.

⑶ Classification of operations: lymphedema operations can be divided into two categories: ① extensive resection of diseased tissues. ② Reconstruction of lymphatic reflux. According to experimental and clinical evidence, some or most of the good results of the latter were actually obtained on the basis of extensive resection of diseased tissues. Simple reconstruction of lymphatic reflux, the operation is very elaborate, but the effect is very small. Because the function of lymphatic system near and far from the lymphatic obstruction point of secondary lymphedema is intact, surgical reconstruction of regional lymphatic reflux should achieve good results. On the contrary, the proximal and distal lymphatic vessels of most primary lymphedema are underdeveloped, so it is impossible to expect the reconstruction of lymphatic reflux surgery to improve the symptoms.

etiology

The etiology of lymphedema can be divided into two categories: primary and secondary. Primary lymphedema is mostly caused by congenital dysplasia, such as lymphatic dilatation, valve insufficiency or loss. According to lymphangiography, primary lymphedema can be divided into: ① hypoplasia of lymph 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 the 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. Secondary lymphedema is mostly caused by lymphatic obstruction. Filariasis lymphedema and streptococcal lymphedema are the most common in China. Lymphedema of upper limbs after radical mastectomy is not uncommon.

Although Herophilos and Aristotle observed the lymphatic system as early as the third and fourth centuries and did a lot of experimental research in modern times, the exact pathogenesis of lymphedema is still unclear.

Classification:

(i) Primary lymphedema

1. Congenital: simple

Hereditary (Milroy's disease)

2. Early onset

(ii) Secondary lymphedema

1. Infectious: parasites, bacteria, fungi, etc.

2. Injury: surgery, radiotherapy, burns, etc.

3. Malignant tumor: primary tumor and secondary tumor.

4. Others: systemic diseases, pregnancy, etc.

pathological change

Lymph is tissue fluid in intercellular space, which flows into vein through lymphatic reflux. Lymphatic circulation is also a physiological functional physical circulation of human body. When the lymphatic system is congenital dysplasia or blocked or destroyed for some reason, the reflux of distal lymph will be hindered and the number of lymph in interstitial will increase abnormally. If it happens in the limb, the uniformity of the affected limb becomes thicker. At first, the skin was still smooth and soft, and the edema of the affected limb could be obviously alleviated by raising it. Because the accumulated lymph is rich in protein, which can be as high as 5.8g/dl, [normal 0.72 g/dl] long-term stimulation causes abnormal proliferation of connective tissue, and adipose tissue is replaced by a large number of fibrous tissues. The skin and subcutaneous tissue are extremely thickened, the skin surface is keratinized and rough, there is no indentation after finger pressing, and warty growth appears, forming a typical "elephantiasis". Infection increases inflammatory exudate, stimulates a lot of connective tissue proliferation, destroys more lymphatic vessels, aggravates lymphatic retention, increases the chance of secondary infection, forms a vicious circle, and leads to lymphedema getting worse day by day.

clinical picture

According to the above etiological classification, their respective clinical features are described as follows:

(a) congenital lymphedema is divided into two categories:

1. Simple onset has no family or genetic factors. The incidence of primary lymphedema was 65438 02%. After birth, there is localized or diffuse swelling of one limb, which is painless and ulcer-free, and rarely complicated with infection. Generally in good condition, more common in lower limbs.

2. Hereditary Milroy's disease is rare. Many people in the same family get sick, that is, they get sick after birth, and most of them involve one lower limb.

(2) Early onset lymphedema is more common in women, with the ratio of male to female being 65,438+0 ∶ 3. The onset age is 9-35 years old, and 70% of them are unilateral. Generally, there is slight swelling of the ankle without obvious inducement, which is aggravated when standing, moving and menstruating, and edema of the affected limb can be temporarily relieved when the climate is warm. The lesion gradually aggravates and spreads to the calf, but generally does not exceed the knee joint. In the later stage, it can be a typical "elephant skin leg", but it is rarely complicated with ulcers and secondary infections.

(3) Infectious lymphedema includes infections such as bacteria, fungi and filariasis. Cracks or blisters on toe skin are the most common route of pathogen invasion, followed by local injuries or infections such as varicose veins of lower limbs complicated with ulcer secondary infection. In addition, it has also been reported that pelvic lymphadenitis caused by female pelvic inflammatory disease can hinder the lymphatic reflux of lower limbs, resulting in the lymph of the affected limb not being enlarged. Streptococcus is the most common pathogen of secondary infection. Clinical manifestations are recurrent acute cellulitis and acute lymphangitis, with serious systemic symptoms, including chills, high fever with nausea and vomiting, local groove lymph node enlargement with tenderness. After anti-inflammatory symptomatic treatment, the systemic symptoms subsided quickly, but the local lesions eased slowly and were easy to repeat. After each attack, the swelling of the lower limbs is aggravated, and finally the skin is rough and warty, and a few can be secondary to chronic ulcers.

Tinea pedis itself or secondary infection can also cause lymphedema, which is generally limited to the foot and instep. Severe fungal infection is usually a precursor to acute cellulitis and acute lymphangitis. Controlling fungal infection is one of the effective measures to prevent lymphedema.

Filariasis is a common cause of lymphedema of lower limbs in the southeast coastal areas of China. The incidence rate is 4 ~ 7%, which is more common in men. At the initial stage of filariasis infection, there are different degrees of fever and local swelling and pain. Repeated filariasis infection narrows, blocks and destroys the local lymphatic vessels of lower limbs, and the lymphatic reflux of distal skin and subcutaneous tissue is blocked, resulting in lymphedema. Local lesions such as tinea pedis or repeated attacks of erysipelas make lymphatic drainage blocked and infection mutually causal, forming a vicious circle and eventually becoming a typical "elephant skin leg". Flash and scrotal lymphedema are not uncommon, which can cause extremely enlarged scrotum in the late stage. This is also a major feature of filarial infectious lymphedema.

(4) Traumatic lymphedema mainly includes lymphedema after breakup and lymphedema after radiotherapy.

1. Postoperative lymphedema often occurs after lymph node dissection, especially after radical mastectomy. After extensive lymph node dissection, the distal lymph was blocked, which stimulated tissue fibrosis and aggravated the swelling. The time of lymphedema after operation varies greatly. Generally, the proximal limb is slightly swollen when the limb starts to move after operation, but it can also appear weeks or even months after operation.

2. Deep X-ray and radium ingot treatment of lymphedema after radiotherapy cause local tissue fibrosis, and lymphatic occlusion causes lymphedema.

(5) Malignant tumor lymphedema Both primary and secondary malignant tumors of the lymphatic system can block lymphatic vessels and produce lymphedema. The former is found in Hodgkin's disease, lymphosarcoma, Kaposi's multiple hemorrhagic sarcoma and lymphangiosarcoma. Lymphangiosarcoma is rare, but it is the result of long-term malignant transformation of lymphedema. Most of them occur in patients with limb lymphedema after radical mastectomy. It usually occurs 10 years after operation, and the skin appears red or purple spots, which are multiple, and then it is combined with ulcerative mass. Limb lymphedema is more serious after onset. Biopsy should be done in time. You need to amputate after diagnosis.

Secondary lymphatic diseases are metastatic cases of breast, cervix, labia, prostate, bladder, testis, skin, internal bones and other cancers. Sometimes the primary focus is small and difficult to find, and the clinical manifestations are chronic recurrent, painless and progressive lymphedema. Therefore, for lymphedema of unknown cause, we should be alert to the possibility of tumor and make a clear diagnosis through lymph node biopsy if necessary.

In addition, pregnancy and many systemic diseases, such as pneumonia, influenza and typhoid fever, can also lead to recurrent cellulitis and lymphangitis, while venous thrombosis and lymphatic obstruction can cause lymphedema.

accessory examination

(1) Diagnostic puncture tissue fluid analysis The analysis of subcutaneous edema tissue fluid is helpful for the differential diagnosis of difficult cases. The protein content of lymphedema fluid is usually very high, generally 1.0 ~ 5.5g/dl, while the protein content of edema tissue fluid with simple venous stasis, heart failure or hypoproteinemia is 0. 1 ~ 0.9g/dl. 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: A specific auxiliary examination of lymphedema is to show the morphology of lymphatic system by injecting contrast agent into lymphatic puncture.

1. instruction

(1) distinguish lymphedema from venous edema.

⑵ Distinguish primary lymphedema from secondary lymphedema.

(3) Those who intend to undergo lymphatic-venous anastomosis.

2. At present, most of the methods of lymphangiography are direct injection lymphangiography. Evans blue was injected subcutaneously at the metatarsal bone 1 ~ 4 level of the dorsum of the foot. After 3 ~ 5 minutes, blue thin strips of superficial lymphatic vessels can be seen. Under local anesthesia, the superficial lymphatic vessels were separated by cutting the skin, and filament wires were wound at the proximal end and the distal end respectively, so that the proximal end was temporarily closed, thus retaining the lymph. Puncture the lymphatic vessel with No.27 ~ No.30 needle, and then inject a little 1% procaine to confirm that it is in the cavity and does not leak. The needle was fixed, connected with the syringe through a plastic pipe, and injected at a constant speed of 0. 1 ~ 0.2 ml/min. After 2ml injection, photos were taken in ankle joint and pelvic cavity to identify whether the contrast agent was extravasated or not, and to exclude accidental injection into vein. After injection, the needle was pulled out, lymphatic vessels were ligated to prevent lymphatic leakage and the skin was sutured. The contrast photos include: anteroposterior position of calf, anteroposterior position of thigh, anteroposterior position of groin to the first lumbar vertebra, oblique position or lateral position.

3. Abnormal manifestations of lymphangiography

(1) Primary lymphedema: absence or insufficiency of lymphatic valve, dilated and tortuous lymphatic vessels.

⑵ Secondary lymphedema: The middle and distal lymphatic vessels are dilated and tortuous, and the number is increased and irregular. There are filling defects and wormlike edges in metastatic lymph nodes.

4. Complications

(1) Incision infection, horse leakage.

⑵ Systemic reaction: allergic to contrast media, and some cases may have fever, nausea and vomiting and peripheral circulation failure.

⑶ Local lymphoreactive inflammation aggravates lymphedema.

⑷ Pulmonary embolism: The pressure of contrast agent may increase and enter the vein through the anastomotic collateral, causing pulmonary embolism, the incidence rate is 2 ~ 10%, and there are reports of death due to pulmonary embolism in the literature.

(3) Isotopic lymphangiography can't provide quantitative dynamic data of lymphatic system function and simple information of lymphatic drainage in different limb parts. At present, a valuable static lymphangiography (radionuclide imaging) is performed, and 0.25ml(75MBq) of 99m sulfide gel 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. Therefore, it can be used to distinguish lymphedema from venous edema, and its sensitivity and specificity in diagnosing lymphedema are 97% and 100% respectively. Compared with lymphangiography, radionuclide imaging is simple and definite. But it can't locate lymphatic vessels and lymph nodes anatomically. If lymphatic surgery is considered, lymphangiography is still the best.

In addition, the newly developed non-invasive detection technology of blood vessels is also helpful to distinguish venous edema from lymphatic edema. As an outpatient screening method, it is simple and convenient.

differential diagnosis

In the early stage, there are slight changes in skin and subcutaneous tissue, so it should be differentiated from other diseases:

1. Venous edema is more common in deep venous thrombosis of lower limbs, which is characterized by sudden swelling of one limb, obvious tenderness of gastrocnemius and femoral triangle, exposure of superficial veins, but not obvious edema of dorsum of foot. The onset of lymphedema is slow, and swelling of the instep and ankle is more common.

2. Angioneurotic edema occurs under the stimulation of external allergic factors, characterized by rapid onset and rapid regression, and intermittent onset. Lymphedema is getting worse.

3. Systemic diseases such as hypoproteinemia, heart failure, nephropathy, liver cirrhosis and myxedema can all produce edema of lower limbs. Generally bilateral symmetry, accompanied by clinical manifestations of its own primary disease. It can usually be identified by detailed medical history inquiry, careful physical examination and necessary laboratory examination.

4. Congenital arteriovenous fistula Congenital arteriovenous fistula can be manifested as limb edema, but generally the length and circumference of the affected limb are longer than that of the healthy side, the skin temperature increases, superficial varicose veins are heard, vascular murmurs can be heard in some areas, and the oxygen content of peripheral venous blood is close to that of arterial oxygen. All the above are its unique characteristics.

5. Lipoma A small amount of lipoma or adipose tissue hyperplasia has a wide range of lesions and can be confused with lymphedema. However, most lipomas show local growth, slow course of disease, soft subcutaneous tissue and no edema. When necessary, mammography of soft tissue is helpful for diagnosis.