Lymphatic Pathologies: Therapeutic strategies under consideration of regional aspects

Lymphatic pathologies in primary lymphedema: The clinical symptoms are diverse and can happen in the arm, leg, or face. The pathogenesis is complicated, underlying the following symptoms, it can happen in the lymphatic vessels or the lymph nodes. It can also be an anatomical problem or a functional problem.

Ningfei Liu

Dr. Ningfei presented some images from Lympho angiography.  

We can see the distinct types of lymphatic malformations in primary lymphedema. It can be a malformation in the superficial lymphatic systems and in the deep lymphatic systems.  

The lymph nodes, too, can be the primary cause of primary lymphedema; it can be the high preparation of the inguinal lymph nodes or the problem with the structure. Deformity of the lymph nodes and the vascularized lymph nodes, so both infecting vessels and lymph nodes can be the problem of primary lymphedema.  

According to results tested on 5500 patients, the lymphatic system pathology is divided into four types:  

1- Lymph node affected  

2- Lymphatic affected  

3- Lymphatic & lymph node affected  

4- Lymphatic dysfunction  

 Lymphatic malformations can happen at diverse levels of the lymphatic system and in the initial lymphatics or plaque collectors. Dr. Ningfei showed a picture of a primary lymphedema patient presenting his bilateral lower extremity. There are a lot of dilated lymphatic vessels, but the lymph nodes look normal.  

In another patient in his lymph angiography, it is seen that on her left lower extremity, there is a prominent dilated network, an abnormality of the lymphatic vessels, and the superficial lymphatic system. There is a big vein, but in the inguinal region, the lymph nodes cannot be seen, so this patient has hyper lymphatic pressure and lymphomas are pressure.  

Lymphatic hyperplasia and lymph node aplasia are common diseases of primary lymphedema; it is called Milroy's disease; this patient has FLT4 mutation. On the ICG lymphogram, there are some lymphatics in the fold, which means that the lymphatic existed in his leg, but it cannot work well to transport the lymph flow, so this is the lymphatic dysfunction.  

In another case of Milroy's disease in the icing Lymphogram, the lymph vessels cannot be seen, so a skin biopsy is taken to make the staining; there are no initial lymphatics can be identified in the skin, so this patient also has FLT4 mutation and the mutation of VEGFC, so the conclusion is this patient has the lymphatic up the pressure, so the lymphatic malformations is complicated.  

  • There is no targeted treatment for the malformation of the lymphatic system in primary lymphedema.  
  • Lymphatic pathology in secondary lymph edema  
  • It can happen in the collecting lymph vessels, initial lymphatics, or the lymph nodes  
  • Lymphatic dilation, disruption, and lymphorrhea  
  • Damage to lymph node: Fibrosis, atrophy  
  • Lymphatic sclerosis or obliteration  

Picture of lymph angiogram for breast cancer-related lymphedema; in the picture, there are dilated lymphatic collectors with disruption and leakage of the length of flow, which is after the regeneration of the damaged lymphatic vessels, so this happens in the secondary arm lymphedema.  

Another picture shows secondary lymphedema. The lymphatic vessels in the lower leg are dilated; with the disruption of the lymphatic vessels in the thigh, there are highly dilated lymphatic vessels.  

In a patient with cervical cancer, the lymph nodes are without standard structure after the surgical treatment under radiotherapy.  

Dr. Ningfei showed an image illustrating dermal initial lymphatic sclerosis. Whether it is secondary or primarily lymphedema, it is common to see damage to the initial lymphatic vessels. Dermal initial lymphatic sclerosis may be why treating lymphedema is tricky because the initial lymphatics are sclerosis and fibrosis.  

For surgical and physiological treatment, lymph venous shunt procedures, vascularized lymph nodes transplantation, or lymphatic transplantation can be performed, but you should be careful in the selection of the patients before surgery; an imaging test to see if the lymphatic vessels are essential because if the lymphatic is damaged the results badly are not good.  

Another picture shows the various stages of lymphedema; in the early stage, there is some water in the lower leg, but as the disease develops, there is more edema fluid in the subcutaneous tissues. In the advanced stage, there is a lot of fibrosis and fat tissue deposition in the skin, so this is the most challenging work with this pathologist. Examples of Ablative surgical treatment are liposuction and debunking surgery.  

The most common use for conservative treatment is complex physical decongestion therapy (CDT); it is used to cause the level to regress to a clinically edema-free stage or decrease the stage of lymphedema. Other treatments are the far- infrared radiation therapy and the IPC, but they should be worked under the doctor's or physician's instruction.  

A picture shows how MLD works, and the picture shows how it works; it can stimulate lymph nodes and vessels to speed up the lymph flow and the bandage.   

Far-infrared radiation therapy is a specially designed device for the treatment of the lymphedema of the lower legs; this is only for the treatment of chronic lymphedema of the extremity of the arm or the leg. A figure shows that it can reduce inflammatory reaction complications.  IPC is also combined with the CDT or surgery as in other areas or countries.  

Knowledge is the most crucial thing in individual self-treatment because lymphedema is a long lifetime treatment, so every patient should be educated on how to do after the treatment in the hospital; if the patients follow the instruction of the surgeons, satisfactory results can be seen. 

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