Disorders of lymphatic system

Lymphadenopathy

Lymphadenopathy is the enlargement of one or more lymph nodes as a result of normal reactive effects or a pathologic occurrence.

Etiology:

An immune response to infective agents (eg, bacteria, virus, fungus) Inflammatory cells in infections involving the lymph node Infiltration of neoplastic cells carried to the node by lymphatic or blood circulation (metastasis) Localized neoplastic proliferation of lymphocytes or macrophages (eg, leukemia, lymphoma) Infiltration of macrophages filled with metabolite deposits (eg, storage disorders)

A localized lymphadenopathy usually results from abnormalities of the area in which the lymph node drains, although it cannot be excluded as the first sign of a precocious clinical manifestation in the course of a progressive systemic process The appearance of a generalized lymphadenopathy orients the clinician more directly toward serologic and hematologic testing.

Regional lymphadenopathies

Of the regional lymphadenopathies, occipital and preauricular locations are rarely malignant; The occipital are often related to scalp and outer ear infections, exanthematous diseases, and toxoplasmosis, The preauricular are associated with infections of superficial tissue of the orbit, the middle ear, and the parotid glands.
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Submental lymphadenopathy requires a search for disorders in the anterior portion of the mouth and the lower lip, submental salivary gland. The submandibular lymphadenopathy with disorders in submandibular portion of the face, the nose, the maxillary sinus, the mucosa of the oral cavity, the floor of the mouth, and the submandibular salivary gland

Laterocervical lymphadenopathy in the upper portion of the neck can be associated with inflammatory or neoplastic disorders of the hypopharynx, the larynx, or the thyroid gland, Those in the lower part of the neck are related to disorders of the hypoglottic larynx, the thyroid, and the upper portion of the esophagus.

Supraclavicular and epitrochlear enlargement must be considered as

red flags
for the potential of malignancy.

Axillary lymphadenopathy is seen with infections of the upper extremity, chest wall, breast tissue, and intrathoracic lesions. Inguinal lymphadenopathies are caused by sexually transmitted diseases of the genitalia and other infections of the perineum and pelvis. Enlarged popliteal lymph nodes are generally associated with infections of the foot and leg.
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Lymphadenopathies of the mediastinum, retroperitoneum, and mesentery are usually not detected at the time of physical examination but are sometimes suspected by compression of the surrounding structures.

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General lymphadenopathy

Any of the common viral illnesses may produce generalized lymphadenopathy (eg, Epstein-Barr virus [EBV], cytomegalovirus [CMV], human immunodeficiency virus [HIV]). Hematogenous malignancies (eg, leukemia, lymphomas) Oethr malignancies (eg, neuroblastoma, rhabdomyosarcoma). Some rare causes of generalized lymphadenopathy include autoimmune connective tissue diseases and use of certain drugs, particularly phenytoin and carbamazepine.

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In most instances, lymph nodes up to 1 cm can still be considered normal. The two exceptions to this rule include the epitrochlear node in which up to 0.5 cm is allowed and the inguinal nodes in which up to 1.5 cm is allowed.

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A complete blood cell count with a manual differentiation provides useful information. leukemias are often accompanied by pancytopenia. A predominantly lymphocytic elevation (>1 X 109 cells/L) is practically diagnostic of mononucleosis; when the proportion of these cells is less elevated but still predominant, CMV and toxoplasmosis must be considered. Finding medium-to-large lymphocytes that can be classified as in transformation or activated is useful to indicate a viral infection.

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Serum lactate dehydrogenase (LDH) may be used to determine the turnover rate of cells in the case of leukemia or lymphoma. Other tests, such as tuberculin skin test; monospot; and titers for EBV, CMV, catscratch disease, or toxoplasmosis, may be performed to evaluate for specific etiologies

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Imaging Studies

Chest radiography may be useful to assess for potential sources of infection, such as bacterial pneumonias or tuberculosis, and hilar adenopathy in the case of malignancy. Indeed, because numerous reports describe airway collapse with anesthetics in the case of a large anterior mediastinal mass, chest radiography should be considered before any general anesthetic is administered.

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Ultrasonography may be performed to distinguish the lymph nodal nature if palpation is not sufficient. Furthermore, it may be used to distinguish the abnormality from other potential anatomic structures (eg, dermoid cysts, thyroglossal duct cysts, branchial cleft cysts, inguinal hernias, undescended testicles). Ultrasonography may reveal relationships to contiguous structures Offer information about the content of the enlarged lymph node or nodes (ie, solid, liquid, gas, homogeneous or nonhomogenous). Finally, ultrasonography has been used in an effort to establish etiology based on ultrasonographic characteristics
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(CT) scanning is useful to depict deep lymph nodes, especially in the thoracic and abdominal cavities. This may be the only noninvasive technique available to evaluate these areas and determine a potential source of malignancy (eg, neuroblastoma, Burkitt lymphoma, rhabdomyosarcoma). Furthermore, chest CT scanning depict an anterior mediastinal mass as well as the extent of tracheal or bronchial airway compression

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18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) has been used in adult patients with lymphoma and more recently in children to assist in diagnosis and to monitor disease during therapy with promising findings.

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Diagnostic Procedures

(FNA) biopsy has been used extensively in adults and is being described in children The cited advantages of FNA biopsy are that it can be performed in the outpatient department, is simple and rapid, does not require a general anesthetic, has low morbidity, is cost effective, and produces minimal scarring The sensitivity and specificity of FNA biopsy in determining the etiology of lymphadenopathy are more than 90%

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The limits include center dependence on pathologists, the potential risk of seeding a tract with malignancy, and the continued need for at least conscious sedation in most children. Most oncology protocols now require special studies to be performed on the nodal tissue, including cytogenetics, flow cytometry, electron microscopy, and special stains that FNA does not allow. To obtain more tissue, some investigators have used core needle techniques
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Excisional biopsy

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Contraindications:

An absolute contraindication to lymph node biopsy exists if the etiology is clear and the lymphadenopathy is expected to improve with no further management. A relative contraindication exists if the suspected etiology can be treated expectantly, eg, in cases of bacterial infection of the node in which the use of antibiotics is expected to improve the clinical scenario. Another relative contraindication exists if an anterior mediastinal mass is noted on chest radiography and considered to be a high anesthetic risk.

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The complications

The injury of surrounding structures around the nodethe soft tissue, blood vessels, and nerves. With malignancy the spread of tumor cells in the area of the biopsy, Production of a draining sinus in the case of atypical Mycobacterium if the entire node is not excised, The risks associated with general anesthetics, especially if the patient has an anterior mediastinal mass.
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TB
In the United States, atypical Mycobacterium account for most cases of adenitis due to Mycobacterium infection. Present with cervical node enlargement, most often around the paratracheal nodes or the supraclavicular nodes.

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Nodal enlargement is usually painless; nodes are likely to suppurate and form sinuses. Clinical features are not helpful in distinguishing atypical from tuberculous mycobacterial infections. Performing a tuberculin test is usually helpful. Abnormal findings are observed on chest radiography in most cases.

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Treatment involves administration of rifampin and isoniazid Treatment involves complete excision of the involved node because incision and drainage may lead to a chronically draining sinus

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Malignancies
The nodes are usually painless and continue to enlarge. Inflammatory signs or focuses are usually absent. Associated B symptoms of HD may be present, including fever, night sweats, weight loss, and malaise. If malignancy is suspected, biopsy is needed to establish the diagnosis

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The diagnosis of lymph node disorders will improve as molecular tools become more available,which will allow clinicians to diagnose the etiology with more exact science and less invasive means. The use of FNA in children will become more frequent

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Lymphedema

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Lymphedema is a notoriously debilitating progressive condition with no known cure. The underlying problem is lymphatic dysfunction, resulting in an abnormal accumulation of interstitial fluid containing high molecular weight proteins.

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Primary lymphedema represents a developmental abnormality of the lymphatic system.

Primary lymphedema has been further subdivided into 3 forms, including congenital lymphedema, lymphedema praecox, and lymphedema tarda, depending on age at presentation. These conditions are most often sporadic, with no family history, and involve the lower extremity almost exclusively.

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In the United States, the highest incidence of lymphedema is observed following breast cancer surgery, particularly among those who undergo radiation therapy following axillary lymphadenectomy. Among this population, 10-40% develop some degree of ipsilateral upper extremity lymphedema. Worldwide, 140-250 million cases of lymphedema are estimated to exist, with filariasis being the most common cause.
o Other causes include vein stripping, peripheral vascular surgery, lipectomy, burns, burn scar excision, and insect bites.

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Clinically

Patients present with varying degrees of severity, from mild swelling to severe disabling enlargement with potentially life-threatening complications. Asymmetry or increased circumference of an extremity. Difficulty fitting into clothing. may cause fatigue related to the size and weight of the extremity, and severe impairment of daily activities. Recurrent bacterial or fungal infections are also common

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Patients with chronic lymphedema for 10 years have a 10% risk of developing lymphangiosarcoma, commonly present with a reddish purple discoloration or nodule that tends to form satellite lesions. It may be confused with Kaposi sarcoma or traumatic ecchymosis. The 5-year survival rate is less than 10%, and average survival following diagnosis is 19 months. This malignant degeneration is most commonly observed in patients with postmastectomy lymphedema (Stewart-Treves syndrome), where incidence is estimated to be 0.5%.
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D.D.

Other causes of edema, such as edema secondary to congestive heart failure, renal insufficiency, hepatic insufficiency, or venous stasis disease, must be excluded. Malignancy must always be considered, particularly when patients report sudden onset, rapid progression, or associated pain. These symptoms may indicate direct tumor growth or metastatic disease in the regional lymph node basin.

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Lymphoscintigraphy has replaced lymphangiography An indication for CT scan or MRI is suspicion of malignancy, Doppler ultrasonography is also used by some to evaluate flow in the lymphatic and venous systems.

The presence of a deep vein thrombosis is in the differential diagnosis of unilateral extremity swelling, and it may also occur concomitantly with lymphedema.
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Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy. Moreover, it is rarely indicated as the primary treatment modality. Rather, reserve surgical treatment for those who do not improve with conservative measures or in cases where the extremity is so large that it impairs daily activities and prevents successful conservative management.

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Physiologic procedures attempt to improve lymphatic drainage. omental transposition, enteromesenteric bridging, buried dermal flaps, lymphangioplasty, microvascular lympholymphatic or lymphovenous anastomoses.

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Excisional techniques remove the affected tissues, thus reducing the lymphedema load. The Charles procedure is quite radical excisional technique. This procedure involves the total excision of all skin and subcutaneous tissue from the affected extremity. The underlying fascia is then grafted, using the skin that has been excised

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Staged excision has become the option of choice for many authors and is described in greater detail. This procedure involves removing only a portion of skin and subcutaneous tissue, followed by primary closure. After approximately 3 months, the procedure is repeated on a different area of the extremity. This procedure is safe, reliable, and demonstrates the most consistent improvement with the lowest incidence of complications.
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