Lymphostasis

Lymphostasis, which is used interchangeably with lymphedema, is a chronic condition characterized by an increase in the lymphatic fluid collection in the body, resulting in swelling and skin and tissue abnormalities. The lymphatic system's capacity to move protein-rich fluids inside the interstitial space and fibro-adipose tissue has been exceeded by a chronic, increasing accumulation of fluid within the interstitium and fibro-adipose tissue. Lymphedema can cause swelling in any area of the body, such as the arms, legs, genital area, cheeks, neck, chest wall, and mouth. A diagnosis of lymphostasis comes with a slew of psychological, physical, and social consequences. Lymphedema is divided into two types: primary lymphedema (inherited) and secondary lymphedema (acquired).

Lymph is transported through lymphatic vessels. White blood cells, lipids, bacteria, cell detritus, water, and protein make up lymph. It has a strong resemblance to blood plasma. Initial lymphatics (lymph capillaries), pre-collectors, collectors, lymphatic trunks, and lymph nodes make up the lymph drainage system. The lymph system is divided into two types: superficial (subcutaneous) and deep (intramuscular or subfascial). The skin and subcutaneous tissue are drained through the superficial system. Muscles, joints, tendon sheaths, and nerves are all drained by the deep system. The perforating vessels, which carry lymph fluid from the subfascial regions to the surface, connect the two systems.

Distal swelling in the extremities, such as the arms, hands, and feet; proximal swelling in the breast, chest, shoulder, pelvis, perineum, genital area, and face tissues; limited range of motion in the joints due to swelling and tissue changes; skin discoloration; pain and altered sensation; limb heaviness; and difficulty fitting into clothing are all physical signs and symptoms of lymphedema.

What is the Lymph System?

The lymphatic system is a component of both the immunological and circulatory systems.

The lymphatic system is composed of a series of small vessels that flow throughout the body and eventually merge to become larger vessels. Little lymphatic vessels can be seen in the dermis of the skin, but not in the subcutaneous fat. A transparent fluid called lymph travels through these vessels. The extra protein, fluids, bacteria, and waste materials that the body doesn't need are collected in lymph, which moves through the lymphatic system till it enters the lymph nodes (glands).

The lymph nodes, which are found in the neck, armpits, groins, and deep within the chest and abdominal cavities, filter and clean the lymph to eliminate any foreign materials (such as bacteria) and initiate any appropriate immune responses. Lymph eventually drains into big veins in the lower neck after passing via lymph nodes. The lymph slowly moved along the lymphatic system by the movement of neighboring muscles. Furthermore, the sidewalls of lymphatic vessels compress, and valves within the channels keep the system pumping.

Epidemiology

Primary lymphedema is an uncommon condition that affects 1 in 100,000 children. The most frequent cause of lymphedema is secondary lymphedema, which affects about 1 in 1000 American children.

The incidence rates of lymphedema are difficult to determine. Lymphedema is quite common, although the consequences of lymphatic malfunction in the general population have not been thoroughly investigated. Despite the fact that lymphedema prevalence estimates are very high, the diseases' frequency is likely underestimated. There are not enough prospectively designed and thoroughly conducted appropriate epidemiologic investigations to determine the disease's actual prevalence.

The occurrence of lymphedema is studied the most among patients with advanced cancer. Lymphatic and soft tissue complications can occur in children with head and neck malignancies over the first 17 months after treatment, with more than 91% of patients developing internal, external, or mixed lymphedema. More than 50% of the children will have fibrosis. More comprehensive lymph node dissection, radiation and chemotherapy, an increase in body mass index, inadequate physical activity, and the appearance of pre-treatment lymphedema were recognized as possible risk factors for lymphedema progression in cancer children.

Lymphostasis Pathophysiology

Disease-related Changes in Lymphatic System

The lymphatic transport capacity is impaired in a pathological condition. As a result, the typical amount of interstitial fluid production outpaces the rate of lymphatic drainage, causing high-molecular-weight proteins to accumulate in the interstitial space. This normally happens when the flow has been lowered by at least 80 percent. High-protein fluid, or lymphedema, is the consequence, as opposed to forms of edema with significantly lower protein levels. Protein concentrations range from 1.0 to 5 g/mL. The interstitial’s strong oncotic pressure encourages the retention of more water.

Interstitial fluid buildup produces significant dilation of the remaining outflow pathways and valvular insufficiency, causing flow from subcutaneous tissues to the dermal plexus to reverse. Formed fibrinoid thrombi aggregate within the lumen of the lymphatic vessels, obliterating much of the residual lymph routes. Lymphovenous shunts can arise on their own. The typical design of lymph nodes is lost as they stiffen and shrivel.

Protein and fluid buildup in the interstitial space triggers a significant inflammatory response. Increased macrophage activity leads to the breakdown of elastic fibers and the formation of fibrosclerotic tissue. Fibroblasts produce collagen in the interstitial space as they migrate. As a result of the inflammatory response, the pitting edema transforms into the beefy nonpitting edema that is distinctive from lymphedema. As a result, local immunologic surveillance is reduced, and persistent infections, as well as neoplastic degeneration leading to lymphangiosarcoma, are possible outcomes.

Dermatologic Changes

The overlying skin thickens and takes on the characteristic orange skin appearance of clogged dermal lymphatic vessels.

Chronic lymphostasis produces fissuring and deterioration of the epidermis, permitting bacteria to enter and thrive, as well as lymphorrhea, or lymph leaks onto the skin's surface. Elephantiasis nostra verrucosa is a disorder caused by persistent lymphostasis that results in the formation of verrucous, cobblestone patches.

Protein Content in Lymphostasis

Chronic lymphostasis may alter the protein content of lymph in affected locations, according to one hypothesis. There has been a drop in alpha-2 globulin levels and an increase in the albumin-to-globulin proportion. This alteration in proteins, together with the resulting slowdown of transport to lymph vessels, has been hypothesized as a factor in reducing immune surveillance efficiency and preventing the early diagnosis of tumor-specific antigens. Repeated episodes of chronic ulcers and healing may also increase keratinocyte growth, which can lead to malignant transformation.

Lymphostasis Causes

Primary lymphedema is a hereditary or congenital disorder in which the lymphatic system is malformed, usually due to a genetic defect. Primary lymphedema is classified into three types:  

1. Congenital lymphedema, which appears at birth or within two years of birth.
2. Lymphedema praecox, which appears during adolescence or the third decade.
3. Lymphedema tarda, which appears after the age of 34.

Secondary lymphedema occurs when the lymphatic system is damaged, injured, or obstructed. While filariasis caused by infection with Wuchereria bancrofti is the most prevalent cause of lymphedema globally, the majority of secondary lymphedema cases in developed countries are caused by cancer or associated with cancer therapy. Surgical removal of lymph nodes, local radiation treatment, and pharmacological treatment are all options. In developed countries, breast cancer is the most prevalent malignancy linked to secondary lymphedema.

Lymphostasis Symptoms

Because primary and secondary lymphostasis (lymphedema) are so identical, a comprehensive history and physical examination are essential in distinguishing the two. Primary lymphedema is present at birth, whereas secondary lymphedema develops as a result of a lesion, damage, or blockage. It is vital to have a complete history of length, location, infections, international travel, malignancy, liver failure, heart failure, and past surgery, particularly with lymph node involvement.

If primary lymphedema is considered, thorough family history is required. It's crucial to determine whether other family members, typically from a previous generation, have or have had swollen ankles, feet, and legs due to an unknown reason. Cancer, injuries, and burns that are really painful are all causes of secondary lymphedema.

The following are common manifestations of lymphostasis:

• Edema, particularly in the extremities
• Hyperkeratosis is a condition in which the skin becomes scaly and thickens.
• Lymphangioma is a skin condition that causes tiny blisters and lumps.
• Lymphorrhea is a condition in which lymph fluid leaks from the skin.

Lymphostasis Diagnosis

Lymphostasis (lymphedema) is frequently confused with other sources of edema and swelling of the extremities. Recognizing the risk factors and physical exam indications of lymphedema allows doctors to correctly identify the patients most of the time. It's critical to get the right diagnosis so that patients can be treated properly. The history and physical examination are used to make a diagnosis. The diagnosis is confirmed by lymphoscintigraphy.

Physical Examination

1. Skin

• Lymphedema symptoms include dryness, increased thickness, hyperpigmentation, lymphangiomas (lymph fluid-filled blisters), abnormally warm or hot skin, abnormally dark skin, and any lumps.
• Papillomatosis, a cobblestone pattern of the skin caused by dilated and distended lymph veins encased in fibrous tissue, may also be evident.
• Positive Stemmer's sign: the difficulty to pinch a fold of skin near the second toe's root indicates lymphedema.

2. Extremity

• Size: A discrepancy of less than 20% in the affected limb is termed mild or moderate, while a discrepancy of more than 20% is regarded as severe.

Laboratory Testing

To establish the diagnosis, no blood, urine, or tissue tests are required. When the etiology of lower extremities edema is unknown, these tests may be useful in determining the underlying reasons. Liver function, blood urea nitrogen (BUN)/creatinine ratios, and urinalysis findings should all be evaluated if a renal or hepatic etiology is suspected. If neoplastic signs are detected, they can be tested. If an infection is suspected, a complete blood count (CBC) with differential should be performed.

Imaging Studies

Imaging is not required to make the diagnosis, but it can be used to establish it, estimate the level of involvement, and aid in the decision-making process.

A little amount of radioactive protein dye is introduced into the web space between the first and second fingers of the diseased limb during lymphoscintigraphy. A digital image is used to scan the limb in order to track the dye as it passes through the lymphatic system. Images that reveal dye outside of the lymphatic system indicate lymphedema.

three-dimensional magnetic resonance imaging (MRI), computed tomography (CT), ultrasonography, and bioelectrical impedance assessment are some of the more recent technologies. Ultrasonography can be used to rule out other causes such as DVT and venous incompetence, as well as to detect tissue changes and masses that could be causing lymphatic entrapment. Soft tissue edema can be investigated with high sensitivity and specificity using CT and MRI, but they are pricey.

Lymphostasis Stages

Zero Stage (Latency stage)

1. Due to injuries to the lymph system, the patient is considered at-risk for lymphedema formation but does not show any visible signs of edema.
2. Patients, who have had a sentinel lymph node excision and/ or radiotherapy for breast cancer but have not yet experienced edema, are included.
3. The patient's lymphatic transport capacity has been diminished, putting him at risk of lymphatic overflow and edema.

First Stage (Spontaneous)

1. Reversible
2. You may have pitting edema
3. Swelling is gentle at this time and maybe responsive to elevation

Second Stage (Spontaneously irreversible)

1. Fibrosis or induration in the tissue
2. Elevation has no effect on edema.
3. As the volume of the limb expands, the skin and tissue thicken.
4. Pitting may be evident, however, due to tissue and/or skin stiffness, it may be difficult to detect 

Third Stage (Lymphostatic elephantiasis)

Display the following details:

1. Pitting edema is a type of edema that occurs when the
2. Changes in the skin caused by fibrosis
3. Papilloma may form, infections/cellulitis may occur, and the skin may become dry during this period.

Differential Diagnosis

The following are among the possible causes that produce a similar picture of lymphedema:

• Congestive heart failure
• Glomerulonephritis
• Nephrotic syndrome
• Hypoproteinemia
• Drug reactions
• Cirrhosis of the liver
• Pretibial myxedema
• Constrictive pericarditis
• Lower limb dependency syndrome
• Lipedema
• Bilateral chronic venous insufficiency
• Malignancy
• Deep vein thrombosis (DVT)
• Malignant lymphedema
• Postoperative complications after surgery
• Cellulitis

Lymphostasis Treatment

Lymphostasis (lymphedema) is a chronic condition that requires prompt diagnosis and treatment. To prevent the progression of this chronic and frequently disabling disorder, it is vital to accurately diagnose and treat both mild and early-onset patients. Patients must be directed to a lymphedema treatment and management professional with certification in order to strengthen their knowledge and understanding and acquire beneficial evidence-based treatment and coping methods. A clinician, occupational therapist, or physiotherapist could be this specialist.

Therapy Choices

1. Decongestive lymphedema therapy mobilizes lymph and dissolves fibrosclerotic tissue and is the main treatment for moderate-to-severe lymphedema.
2. Manual lymph drainage increases lymph flow with a little lymph massage.
3. Compression Aids drainage, although the inappropriate application can irritate the skin and raise the risk of infection.
4. Skincare is used to avoid subsequent skin infections; meticulous skincare is required.
5. Light exercise increases lymph drainage and protein intake by contracting muscles.
6. Medications are usually used as an adjunct to treat pain or subsequent infection.

Techniques of microsurgery

1. Transfer of Vascularized Lymph Nodes
2. Lymphaticovenous Anastomosis (LVA): vascularized lymph node transfer and lymphaticovenous anastomosis are microsurgical treatments that enhance the patient's physiological lymphatic drainage and, in some cases, reduce the need for compression stockings. When a patient's lymph vessels are less damaged, these operations lead to better outcomes.
3. Suction-Assisted Protein Lipectomy is more successful in later stages of lymphedema and allows for the excision of lymphatic solids and fatty deposits that are ineligible for conservative lymphedema therapy, vascularized lymph node transfer, or lymphaticovenous anastomosis procedures.

Lymphostasis Prognosis

The prognosis for people with lymphostasis is determined by the severity of the disease, the consequences that arise, and the disease condition that produced the lymphedema. Primary lymphedema normally does not worsen, and after several years of exercise, the condition stabilizes.

Patients with persistent lymphedema for ten years have a 10 percent chance of developing lymphangiosarcoma, the disease's most dangerous consequence. A reddish-purple discoloration or nodule that tends to produce satellite lesions is prevalent in patients with this tumor. It's possible that it's mistaken for Kaposi sarcoma or traumatic petechiae. This tumor is extremely aggressive, necessitating amputation of the affected limb, and has a dismal prognosis.

Lymphangiosarcoma has a 5-year survival rate of fewer than 9%, with a median survival rate of 20 months after diagnosis. This malignant degeneration is most typically seen in patients with postmastectomy lymphedema (Stewart-Treves syndrome), with a 0.6 percent incidence rate. Squamous cell carcinoma, Kaposi sarcoma, and B-cell lymphoma have all been seen in sites of persistent lymphedema.

Frequent episodes of cellulitis and/or lymphangitis, bacterial and fungal infectious diseases, lymph-angio-adenitis, deep venous thrombosis (DVT), severe functional disability, cosmetic humiliation, and obligatory amputation are all complications of lymphedema. Protein-losing enteropathy and visceral dysfunction may occur in some cases. Chylous ascites and chylothorax can occur, however, they are uncommon. Amyloidosis is a consequence of primary lymphedema.

Partially separated wounds, seroma, hematoma, and worsening of the foot or hand edema are all typical consequences after surgery.

Lymphostasis complications

Complications of lymphostasis also include:

• Cellulitis that is usually recurrent
• Lymphangitis
• Superficial bacterial and fungal infections
• Lymph-angio-adenitis
• Deep vein thrombosis (DVT)
• Severe functional impairment
• Psychosocial dysfunction
• Cosmetic embarrassment
• Amputation

Enhancing Lymphostasis Outcomes

Lymphostasis (lymphedema) is a prevalent disease, but it can be difficult to diagnose and treat. Most lymphedema sufferers go to their primary care physician initially. These practitioners should be aware of the symptoms and causes of lymphedema. While the majority of cases are benign, the condition could be produced by a tumor.

To get the greatest lymphedema diagnosis and management, an interdisciplinary team is required. The first measure is early detection and diagnosis, which is usually performed by a primary care doctor or registered nurse. Because the majority of cases are associated with neoplasms and post-surgical complications, surgeons and oncologists with a strong understanding of lymphedema identification, prevention, and therapy are important. The team is now comprehensive with the inclusion of a licensed lymphedema specialist.

Compression stockings or garments should be explained to the patient by the pharmacist. Compression garments, regardless of treatment, make a significant effect in reducing edema. Because many patients get worried or sad as a result of poor cosmetics, they should be counseled by a mental health worker.

Lymphedema has no cure and requires lifetime treatment. The condition can affect health and quality of life depending on which extremities are affected. Compression garments must be used consistently in order to provide relief from pain and edema. Furthermore, skin dryness and itching must be treated. If there is tissue disintegration, all patients should be evaluated by a wound care nurse. The possibilities of healing are minimal at this stage, hence regular wound dressings are required.

Conclusion

Lymphostasis is a chronic disorder caused by inadequate lymph flow, resulting in swelling of the limbs or genital area. It's critical to diagnose lymphedema correctly in order to determine the extent and intensity of extra fluid and customize treatment as needed. There are various diagnostic methods available, each with its own implementation method. Lymphedema is separated into two types: primary and secondary. Full decongestive therapy, surgery, pneumatic compression, and complementary and alternative medicine are all used to manage lymphedema.