What is Lymphangioma & How is it Treated?

Lymphangiomas are congenital abnormalities of the lymphatic system with cysts or pockets of lymphatic fluid collection, they belong to a wide range of malformations in the vascular system. They are seen as lymphatic analogue of heamangiomas and are consequently benign. Lymphangioma is located majorly around the head and neck regions, axilla, chest, retroperitoneum and scrotum and are not compressible, but may be found in any part of the body with lymphatic drainage.

There are different classification of Lymphangioma namely; Lymphangioma Macrocystic (formerly cystic hygroma), Microcystic (formerly cavernous lymphangioma) and Mixed. These classifications are based on the lesions type, size or location.

Lymphangiomas is a noncancerous rare tumors of the lymphatic system.

Lymphangioma is mainly congenital. Chromosomal abnormalities like Turner syndrome are linked with congenital lymphangioma. Another cause of lymphangioma is acquired. These acquired lymphangiomas can be caused because of inflammation, lymphatic obstruction or trauma.

Generally lymphangiomas are asymptomatic, especially if outside the head and neck region but common presentation are due to pressure symptoms which will depend on the site. Patient will complain of one or more of the following:

• Disfigurement (aesthetic).
• Pressure effect (respiratory difficulty, difficulty in swallowing etc…).
• Pain.
• Ulceration.
• interstinal obstruction (internal lymphangioma).

Diagnosis of lymphangioma is mainly clinical, but some investigations could be done.

• Ultrasongraphy.
• Plain X-ray.
• Computer tomography scan.
• Magnetic resonance imaging.
• Needle aspiration and culture.

When the diagnosis has been established, observation during the first years of life is adequate unless the lesion causes severe deformity or a complication occurs. But the main stay of treatment for lymphangioma is slcerotherapy and surgery.

1. Percutaneous Sclerotherapy for Treating Lymphangioma

   Percutaneous sclerotherapy for treating lymphangioma is performed with a sterile technique, usually under sedation or general anaesthesia depending on the age of patient. The sclerosing material is then introduced into the cysts. Most of sclerosing agents often destroy the simple squamous (endothelial) layer of the cysts and they produce an inflammatory reaction, which is usually marked. The use of some prophylactic antibiotics, systemic steroid, or nonsteroidal anti-inflammatory drugs should be used to reduce swelling and also to reduce the risk of infection.

2. Surgery for Lymphangioma

   Surgical resection for treating lymphangioma should follow the precise documentation of the lesion with MRI or CT scan and if any lesion found it should be removed entirely. One complete surgery is more easier and efficient than repeated surgical excisions of a particular area. In cases where the lesion is too large for complete resection in one procedure, anatomic areas are resected in staged procedures.

With continuous treatment for 6-36 months, patient will be able to have recovered fully from lymphangiomas except further complications which may vary from patient to patient as the case may be, some patients may recover faster that others especially when detected at the early and quickly managed, but for some rural area due to poor medical facilities and the people been ignorant of the infection it often gets to serious cases before they are attended to, thereby increasing the period of longer and the chances of recovery becomes very small.

There are no known preventive measures for lymphangioma as it is an infection that victims or patients are born with as such cannot be prevented, but can only be managed when discovered on time.

These lesions are apparent in 40 to 60% children at birth or prenatally and 70 to 80% present within 5 years. However, presentation may occur late during adolescent stage or in adulthood, but there are no identifiable risk factors in the diagnosis of lymphangioma.

The following are the complications of lymphangioma:

• Respiratory obstruction is a complication of head and neck lymphangiomas, especially in late presentations and can be due to the infiltration of the malformation in the tongue, pharynx, or larynx, or blockage of a normal airway by large cysts.
• A sudden increase in the size of the lymphangiomas can occur due to a bacterial infection that might have the tendency to affect anywhere in the body, but it occurs most around the upper airway as this area is verily prone to such infections. This is as a result of a rapid increase in the lymphatic flow. A sudden increase in size may also be due to intralesional haemorrhage.
• Another complication is ulceration and infection. Ulceration is due to pressure necrosis. The ulcerated area often rapidly becomes infected, and the infection may extend into the cyst, which induce general malaise of the patient.
• Speech impairment is also seen in lymphangioma. Speech difficulties can be present in lesions of the suprahyoid area with tongue involvement or can occur sporadically during episodes of rapid increase of the size of the lesion.
• Feeding difficulties like speech impairment may be due to suprahyoid lesions or sudden increase in size of the lesion.
• Death may occur as a result of multiple complications, late presentation and poor management, especially in neonates and in regions with poor health facilities, example in some rural African settings.

The prognosis for lymphangioma is excellent, but the outcome also depends on the size, type and the location of lymphangioma in the patient, some are as follows:

• Macrocystic lesions have the good prognosis for successful treatment with sclerotherapy or surgery. The large cysts are more easily removed completely, because of less infiltration of the adjacent structures as such it is still small and has spread wide.
• Microcystic lesions are penetrating lesions and that is why they are further difficult to treat both with sclerotherapy and surgically. There is little space available for the sclerosing material and surgery is limited by the solubility of the major and important structures of the lesions. Other malformations that are residual is often becomes complicated in majority of cases unless the lesion is well delineated and completely excised. For cervicofacial lymphangiomas, recurrence and complications depend on the early stage or initial extent of the lesion.

Due to the defects that comes with it, lymphangiomas affected individuals find it difficult to cope with the challenges which involves difficulty in breathing, eating, speech and even association. The sufferers should not lose hope and continue with the treatment. Joining a support group for lymphangioma is a good way to cope with lymphangioma.