Lymphangioma (patient information)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Badria Munir M.B.B.S.[2]

Lymphangioma is abnormality of lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream. The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels. Lymphangiomas most commonly develop at the head and neck regions.

• The majority of patients with lymphangioma are asymptomatic.
• As the mass increases in size, lymphangioma patients may develop site-specific symptoms, such as:

• Dysphagia
• Difficulty breathing
• Constipation
• Failure to thrive

• There are no known direct causes for lymphangioma.
• Cystic hygroma usually presents in syndromes such as Turner syndrome and Down syndrome.
• Therefore it is thought that the lymphatic malformations can arise from gene mutations, occuring as missing or deleted part of chromosomes.
• Acquired lymphangioma circumscriptum occur as a sequele of chronic lymphatic obstruction such as after surgery, infections or radiotherapy

The most potent risk factor in the development of lymphangioma is the presence of genetic disorders such as Turner syndrome, Down syndrome, and Noonan syndrome.

• Biopsy is used to analyze the structure of tissue.
• It shows:
  • Dilated lymph channels ultimately causes the papillary dermis to expand.
  • These channels extend to subcutis.
  • Deeper vessels have larger lumen and muscular coat.
  • The lumen has lymphatic fluid, but it often contains red blood cells, lymphocytes, macrophages, and neutrophils.
  • These channels are lined by endothelial cells.
  • Numerous lymphoid cells are also seen.

• Histochemical staining is recommended to document lymphangioma.

• MRI is primarily used for the diagnosis of lymphangioma.
• MRI can mar the degree of involvement and extent of lesion.
• MRI can prevent extensive, incomplete surgical resection, because poorly removed lesion can lead to recurrence.

In cases of severe complications of disease, such as

• Difficulty breathing
• Difficulty swallowing

There is no medical treatment for lymphangioma; the mainstay of therapy is surgery.

The mainstay of therapy for lymphangioma is surgery which involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Most patients need at least two procedures done for the removal process to be achieved.

Directions to Hospitals Treating Lymphangioma

There are no established measures for the primary prevention of lymphangioma.

Prognosis of lymphangioma is generally good after surgical intervention.

• Common complications of lymphangiomas are primarily associated to the location of lesion.
• Growth of lesion causes compressive effects on surrounding tissues. Which include following:
  • Dyspnea: Trachea
  • Dysphagia: Esophagous
  • Constipation: Gastrointestinal tract
  • Diplopia and proptosis: Retro-orbital tissue of eye
  • Wheeze: Lower Respiratory tract
  • Chest Pain: Mediastinum
  • Shortness of breadth: Lungs
  • Bladder Obstruction: Urinary tract
  • Bone loss or overgrowth: Skeletal tissue
  • Inflammation or bleeding into the lesion
  • Recurrent Cellulitis:Visible deformity

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