Description, Causes and Risk Factors:
Lymphangioma is a benign slow growing tumour of lymphatic vessels. It may arise in any organ or soft tissue. The most frequent occurrence is in the head and neck region and involves the axilla and mediastinum. This tumour is mainly confined to the pediatric age group, but occurrences have been reported in adults. It manifests at birth in up to 65% and presents by the age of 2 years in 80-90% of cases. The incidence of lymphangioma is reported to be from 1.5-2.8 per 1000, and it has no predilection for either sex or any race.
They are fluid filled sacs that result from blockage of the lymphatic system. They are often associated with karyotype abnormalities such as Turner syndrome.
There is no known cause for these lymphatic abnormalities. Genetic syndromes that are associated with cystic hygroma include Turner syndrome and some trisomies e.g. trisomy 13, 18 and 21.
Types may include:
Oral mucosal lymphangioma almost always becomes apparent prior to the third year of life; half of all cases are congenital. There is no gender predilection. Oral lesions are most frequently found on the tongue, where they may produce considerable macroglossia and dysfunction. Any oral or pharyngeal site may, however, be affected and the most common head and neck location is the lateral neck, where this lesion typically contains large cystic spaces and is commonly called cystic lymphangioma or cystic hygroma, discussed elsewhere in the present text.
The cervical lymphangioma occurs most frequently in the posterior triangle, but lesions of the anterior triangle tend to be more problematic, interfering with the patient's ability to breath or swallow and extending upward into the oral cavity, or downward into the mediastinum. Torticollis or wry neck may develop from cervical involvement and cervical lesions tend to be much larger than oral or pharyngeal lesions, sometimes larger than the patient's head at birth.
Superficial oral mucosal lymphangiomas often demonstrate a pebbled or botryoid appearance, once referred to as chronic "clustered blisters" because of the translucent appearance of the lymphatic channels, which may have only sparse fibrovascular tissue separating their endothelial walls from the surface epithelium. Secondary hemorrhage into the lymphatic vessels may cause some of the surface "vesicles" to appear red or blue. Satellite lesions several millimeters from the main lesional mass may be seen.
Occasional lesions show only widely scattered clear "vesicles" interspersed with blood-filled papules or blebs. Such lesions are, presumably, a combination of lymphangioma and hemangioma, and are termed hemangiolymphangioma. They behave more like lymphatic than vascular tumors.
A unique congenital alveolar lymphangioma is seen on the alveolar mucosa of African-American neonates. This lesion is seldom greater than a centimeter in size and is often bilateral on the alveolar ridge (Figure 5). The mandible is more often affected than the maxilla and the lesion typically disappears during the months after birth.
Deeper lymphangiomas present with an irregular surface nodularity and are quite soft and painless. They may feel like a "ball of worms" on palpation, but are usually rather nonspecific and ill-defined.
The most common symptom of lymphangiomas are the appearance of a soft, sometimes discolored, mass on the skin of the face or neck. The mass can range in color from a light pint or yellow to a dark red or even blue and is usually painless. Depending on the location of the mass, other symptoms can include difficulty breathing or eating.
The diagnosis of lymphangioma in these patientswas made on the basis of clinical examination andimaging findings, mainly ultrasound (USG) andcomputed tomography (CT), and in some cases magneticresonance imaging (MRI). Ultrasonographic assessmentwas performed on all patients.
Because of the nonencapsulated and "infiltrating" nature of the lymphangioma, complete removal is often inadvisable and may be impossible without excessive removal of surrounding normal structures. Surgical debulking of the tumor is, therefore, the typical treatment provided, with the understanding that additional debulking procedures will most likely be required as the affected child grows. Most patients will need two to four procedures before full growth and development have been achieved. Recurrence is possible but unlikely for those lesions able to be removed completely via excisional surgery. Radiotherapy and chemical cauteries are much less effective with the lymphangioma than they are with the hemangioma.
Intralesional sclerotherapy has become an acceptable method of treatment for lymphangiomas in children. It involves the use of a sclerosing agent that causes irritation of the endothelial lining of the lymphangioma, which leads to inflammation, fibrosis and involution. Other modes of treatment such as surgical resection, incision and drainage, and radiation therapy have produced unsatisfactory results. Surgical resection for complete removal in many cases is impossible due to the nature of the lesion, which has a propensity to infiltrate tissue planes and encircle important neurovascular structures. Tumour recurrences and nerve injuries are common complications following surgery.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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