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LYMPHANGIOMA - Lymphatic Malformation

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 Cystic Hygroma 

Lymphatic Malformations


LYMPHANGIOMA - Lymphatic Malformation

Related Terms:  

Cutaneous lymphangioma, lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, abnormal lymph vessel, lymph vessel (1)


Lymphatic Malformation


A lymphangioma is a benign tumor composed of newly formed lymph-containing vascular channels and spaces. They involve the skin and subcutaneous tissues.

There are three main types of lymphagiomas:

Lymphangioma circumscriptum - Also referred to as cutaneous this is a "kind" of birthmark generally occurring in clusters. They resemble small blisters and range in color from pink to dark red. They are benign and usually require no medical treatment. For cosmetic reasons, some patients may choose to have them surgically removed. Lymphangiomas Simplex is often included in this type.

Cavernous lymphangioma - This is an uncommon form generally arising during infancy. These are deep seated underneath the dermis and the external skin is not involved. Because they are deep seated, they form a bulging mass, painless mass.

Cystic Hygroma - These are soft lymph filled masses within a thin-walled "sac" generally appearing on the neck (75%), axilla (20%), or trunk or extremity (5%). They usually present a swollen bulges underneath the skin. While they generally present at birth, children up to three years old may experience them. There is also disagreement on whether this type is distinctly different enough from cavernous lymphangioma to be classified separately.


Confirmed through use of x-ray, ultra-sound, MRI, CT scan for cavernous and cystic hygromas. Lymphangioma circumscriptum may be diagnoses by observation and external evaluation.


There is no recognized or effective non-surgical treatment for the condition. Antibiotics may be prescribed to cellulitis infections.

The accepted therapy is total surgical removal, however incidence of
recurrence is high. However, if there has been infiltration of surrounding tissues treatments may include sclerosings agents, steroids, chemotherapy or even radiation. Also, even with removal, there is a high incidence of reoccurrence.


Cellulitis is the most common and most serious complication of all hygromas. With cavernous and cystic hygromas another life threatening complication may be the obstruction of breathing passages.


Old term for a mass of anomalous lymphatic vessels or channels that vary in size, are usually greatly dilated, and are lined with normal endothelial cells; lymphoid tissue is usually present in the peripheral portions of the lesions, which are present at birth, or shortly thereafter, and probably represent maldevelopment of lymphatic vessels (rather than true neoplasms); they occur most frequently in the neck and axilla, but may also develop in the arm, mesentery, retroperitoneum, and other sites. Etymology: lymphangio- + G. [-oma,] tumor (Stedman's Medical Dictionary)



  Lymphangiomas, or lymphangiomata, are embryologic malformations of the lymphatic system consisting of benign lymphatic tumors, usually present at birth. Most (70-90%) of these vascular malformations are usually identified by the end of the 1st year of postpartum life. 

These lymph channel or cystic lymph spaces lined by endothelium are probably the result of abnormalities in lymphangiogenesis (lymphatic development). Lymphangiomata or lymphatic endothelial “cysts” usually grow slowly and may gradually compress surrounding structures, but they do not undergo malignant changes.  


Lymphangiomata can arise almost anywhere in the skin, subcutaneous tissue, intermuscular septa and mucous membranes. The most common sites are the head and neck (including the tongue), the proximal extremities, trunk and buttocks. Rarely they can be found in the abdominal viscera (liver, spleen, intestines, heart, pancreas).  

Clinical manifestations:  

There are four classical types of lymphangioma:  

            1- Lymphangioma simplex  

These are single, well-circumscribed, usually smooth, subcutaneous tumors.  

            2- Lymphangioma circumscriptum  

A very common form of cutaneous lymphangioma consisting of multiple clusters of clear, pink or red vesicles. Pink or red appearance indicates the presence of blood mixed in with the lymph; in this case, mixed vascular malformations are present.  

            3- Cavernous lymphangioma  

A large soft tissue tumor composed of cavernous lymphatic spaces. This condition is rare.  

            4- Cystic lymphangioma or cystic hygroma  

These benign lymphatic tumors are unilocular or multilocular masses composed of a collection of thin-walled vesicles resembling a bunch of grapes, filled with clear or yellowish lymph fluid.

These cysts are usually soft, translucent and painless.

They are most commonly located in the neck (hygroma colli), head, intraoral (tongue, floor of the mouth, salivary glands), mediastinum, axilla, groin, and popliteal fossa.  


Lymphangiomata may swell. On rare occasion they become infected or hemorrhage. Their extension may lead to respiratory or digestive problems, chylothorax or chylopericardium (accumulation of chyle or lymph in the thorax or pericardium, respectively).  


These conditions usually require surgery, laser therapy or sclerotherapy.

Some lesions (especially lymphangiomata circumscripta and cavernous lymphangiomata) have high recurrence rates after excision.

Information about Dr. Chikly's book:
In his definitive text, "Silent Waves: Theory and Practice of LymphDrainage
Therapy," Dr. Chikly addresses the applications for lymphedema, chronic pain
and inflammation. "Silent Waves" is carried by Stanford University Medical
Library and is the first comprehensive book on the lymphatic system and
lymphedema in North America. (ISBN: 0-9700530-5-3, Hardcover).

For further information:


Lymphatic Malformations






Author: Geover Fernandez, MD, Staff Physician, Department of Dermatology, UMDNJ-New Jersey Medical School


Cystic Hygroma Support Group



Cystic Hygroma



Cystic Hygroma

PubMed Health


Cystic Hygroma 

Emory Univrtsity School of Health


Cystic Hygroma Imaging

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Page Updated: Jan. 8, 2012