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Lymphatic Malformations

Abstract

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NORD is very grateful to Jonathan A. Perkins, DO, Professor of Otolaryngology/Head and Neck Surgery, University of Washington, Attending Pediatric Otolaryngologist, Seattle Childrens Hospital, for assistance in the preparation of this report.

Synonyms of Lymphatic Malformations

  • cavernous lymphangioma
  • cystic hygroma
  • cystic lymphangioma
  • lymphangioma
  • lymphangioma circumscriptum
  • lymphangiomatosis
  • macrocystic lymphatic malformation
  • microcystic lymphatic malformation

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Lymphatic malformations are rare non-malignant masses consisting of fluid-filled channels or spaces thought to be caused by the abnormal development of the lymphatic system. These malformations are usually apparent at birth or by two years of age. Lymphatic malformations can affect any area of the body (except the brain), but most commonly affect the head and neck. When evident at birth (congenital), lymphatic malformations tend to be soft, spongy, non-tender masses. The specific symptoms and severity of lymphatic malformations varies based upon the size and specific location of the malformation. Some lymphatic malformations can be massive. Lymphatic malformations regardless of size can potentially cause functional impairment of nearby structures or organs and disfigurement of affected areas.

The lymphatic system functions as part of the immune system and helps to protect the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that transport a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes. As lymph moves through the lymphatic system, it passes through a network of lymph nodes that help the body to deactivate sources of infection (e.g., viruses, bacteria, etc.) and inflammation (e.g. antigens). Groups of lymph nodes are located throughout the body, including in the neck, under the arms (axillae), at the elbows, and in the chest, abdomen, and groin. The lymphatic system includes the spleen, which filters worn-out red blood cells and produces lymphocytes, and bone marrow, which is the spongy tissue inside the cavities of bones that manufactures blood cells.

Several different terms were once used to describe conditions now grouped under the umbrella term "lymphatic malformations". Such terms include cystic hygroma, lymphangioma, cavernous lymphangioma, cystic lymphangioma, and lymphangioma circumscriptum. These terms have been abandoned because some of the outdated terms imply a relationship to cancer. Lymphatic malformations are not cancerous and there is no known risk of malignant transformation.

Lymphatic Malformations Resources

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