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LYMPHANGIOMA - Lymphatic Malformation

This page has been updated. For current information, please see the following pages:

 Lymphangiomas 

http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiomas.htm

 Cystic Hygroma 

http://www.lymphedemapeople.com/thesite/lymphedema_cystic_hygroma.htm 

Lymphatic Malformations 

http://www.lymphedemapeople.com/thesite/lymphedema_lymphatic_malformations

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LYMPHANGIOMA - Lymphatic Malformation

Related Terms:  

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

Classification:  

Lymphatic Malformation

Clinical:

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.

Diagnosis:

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.

Treatment:

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.

Complications:

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.

Definition:

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)

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                               Lymphangioma

  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.  

Localization

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.  

Complications  

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).  

Treatment  

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).
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For further information:

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Lymphatic Malformations

http://www.childrenshospital.org/az/Site1256/mainpageS1256P0.html

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Lymphangioma

http://www.maxillofacialcenter.com/BondBook/softtissue/lymphangioma.html

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Lymphangioma

eMedicine

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

http://emedicine.medscape.com/article/1086806-overview

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Cystic Hygroma Support Group

http://www.cafamily.org.uk/Direct/c95.html

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CysticHygroma.co.UK

http://www.cystichygroma.co.uk/

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Cystic Hygroma

AllRef Health.com

http://health.allrefer.com/health/cystic-hygroma-info.html

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Cystic Hygroma

PubMed Health

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001203/

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Cystic Hygroma 

Emory Univrtsity School of Health

http://genetics.emory.edu/pdf/Emory_Human_Genetics_Cystic_Hygroma.PDF

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Cystic Hygroma Imaging

http://emedicine.medscape.com/article/402757-overview
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Lymphedema People - Support Groups

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Children with Lymphedema

The time has come for families, parents, caregivers to have a support group of their own. Support group for parents, families and caregivers of chilren with lymphedema. Sharing information on coping, diagnosis, treatment and prognosis. Sponsored by Lymphedema People.

http://health.groups.yahoo.com/group/childrenwithlymphedema/

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Lipedema Lipodema Lipoedema

No matter how you spell it, this is another very little understood and totally frustrating conditions out there. This will be a support group for those suffering with lipedema/lipodema. A place for information, sharing experiences, exploring treatment options and coping.

Come join, be a part of the family!

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MEN WITH LYMPHEDEMA

If you are a man with lymphedema; a man with a loved one with lymphedema who you are trying to help and understand come join us and discover what it is to be the master instead of the sufferer of lymphedema.

http://health.groups.yahoo.com/group/menwithlymphedema/

Subscribe: menwithlymphedema-subscribe@yahoogroups.com

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All About Lymphangiectasia

Support group for parents, patients, children who suffer from all forms of lymphangiectasia. This condition is caused by dilation of the lymphatics. It can affect the intestinal tract, lungs and other critical body areas.

http://health.groups.yahoo.com/group/allaboutlymphangiectasia/

Subscribe: allaboutlymphangiectasia-subscribe@yahoogroups.com

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Lymphatic Disorders Support Group @ Yahoo Groups

While we have a number of support groups for lymphedema... there is nothing out there for other lymphatic disorders. Because we have one of the most comprehensive information sites on all lymphatic disorders, I thought perhaps, it is time that one be offered.

DISCRIPTION

Information and support for rare and unusual disorders affecting the lymph system. Includes lymphangiomas, lymphatic malformations, telangiectasia, hennekam's syndrome, distichiasis, Figueroa
syndrome, ptosis syndrome, plus many more. Extensive database of information available through sister site Lymphedema People.

http://health.groups.yahoo.com/group/lymphaticdisorders/

Subscribe: lymphaticdisorders-subscribe@yahoogroups.com

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Lymphedema Glossary 

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Lymphedema 

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The Lymphedema Diet 

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Exercises for Lymphedema  

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Diuretics are not for Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=diuretics_are_not_for_lymphedema 

Lymphedema People Online Support Groups 

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Lipedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=lipedema 

Treatment 

http://www.lymphedemapeople.com/wiki/doku.php?id=treatment 

Lymphedema and Pain Management 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_and_pain_management 

Manual Lymphatic Drainage (MLD) and Complex Decongestive Therapy (CDT)

http://www.lymphedemapeople.com/wiki/doku.php?id=manual_lymphatic_drainage_mld_complex_decongestive_therapy_cdt 

Infections Associated with Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=infections_associated_with_lymphedema 

How to Treat a Lymphedema Wound 

http://www.lymphedemapeople.com/wiki/doku.php?id=how_to_treat_a_lymphedema_wound 

Fungal Infections Associated with Lymphedema 

http://www.lymphedemapeople.com/wiki/doku.php?id=fungal_infections_associated_with_lymphedema 

Lymphedema in Children 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_in_children 

Lymphoscintigraphy 

http://www.lymphedemapeople.com/wiki/doku.php?id=lymphoscintigraphy 

Magnetic Resonance Imaging 

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Extraperitoneal para-aortic lymph node dissection (EPLND) 

http://www.lymphedemapeople.com/wiki/doku.php?id=extraperitoneal_para-aortic_lymph_node_dissection_eplnd 

Axillary node biopsy 

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Magnetic Resonance Imaging 

http://www.lymphedemapeople.com/wiki/doku.php?id=magnetic_resonance_imaging 

Lymphedema Gene FOXC2

 http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_foxc2

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http://www.lymphedemapeople.com/wiki/doku.php?id=lymphedema_gene_vegfc

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Page Updated: Jan. 8, 2012