Lymphatic Malformations

Lymphatic Malformations are classified as simple vascular malformations.  

Traditionally vascular malformations involving the lymphatic vessels were called cystic hygroma, lymphangioma or hemangiolymphangioma.  Recently these vascular anomalies have been called Lymphatic Malformations.

The vessels of the lymphatic system serve as a fluid transfer and fluid collecting system for excess fluids in the tissues of the body.  The lymphatic vessels collect this excess fluid from the tissue and transport it back into the venous system through a series of small vessels.  If this process is slowed excess fluid will accumulate within the vessels and dilate them; the result is swelling in the involved tissue.

Lymphatic Malformations occur during fetal development for unknown reasons.  Fluid transfer through the malformation is slowed resulting in fluid accumulation and swelling of the affected area.  Lymphatic malformations may occur anywhere in the body, although most are found in the head and neck area.

Lymphatic Malformations grow at a steady pace, although some grow more rapidly then others.  Large lesions are known as macrocystic lesions and small lesions are called microcystic.  Unfortunately most lesions of the head and neck are macrocystic.  In addition to size, lymphatic malformations can be superficial, deep, localized or diffuse.  There are some conditions that can result in sudden but temporary “growth” of lymphatic lesions.  Infection and trauma are two known conditions resulting in the expansion of the tissue of the malformation called lymphatic edema. 

Lymphatic Malformations of the head and neck may enlarge during and upper respiratory illness.

MRI may be used to Diagnose a Lymphatic Malformation. 

Lymphatic Malformations are often difficult to treat.

 

Treatment options include embolization or surgical excision of laser vaporization of the lesion.  Lymphatic abnormalities of the main structures are often connected to the smaller deep vessels.  If laser vaporization is selected care must be taken to reach the internal deep vessels without causing thermal damage to the surrounding tissues.  Recurrence is frequent; several laser treatments may be necessary to reach the deep vessels.  If it is diagnosed as a deep lesion surgical excision is the treatment of choice for many physicians.  Since bleeding is not a concern the focus is to completely remove the lesion.   The MRI will define the boundaries of the lesion from the good tissue, care must be taken to not leave abnormal tissue or re-growth will likely occur.

Many physicians prefer embolization procedures to surgical excision.  This procedure works best and with few complications in the extremities.  It does have risk in the head and neck area.  As with surgical excision sclerosing the entire lesion is key to success.

Many treating facilities are using the multi-disciplinary approach to the diagnosis and treatment of vascular malformations.  This allows all specialties to examine the patient in one visit and confer with each other to derive the best treatment plan for the patient.  Although the team of physicians entering the room overwhelms some children, it may prove easier then several single visits to the different physicians.

 

Although there is no cure for lymphatic there are several clinical treatments that may be used to lessen the symptoms of edema in affected areas.  Compression garments or compression wraps, elevation, massage and lymphatic drainage pumps are known to reduce lymphatic edema and discomfort.  These garments should be fitted by a person trained in use for lymphatic malformations.

References:

Andrews, Diseases of the Skin-clinical Dermatology, 8 edition, W.B. Saunders, Philadelphia, 1990 pp682-699.

Arndt, Kenneth, Cutaneous Medical Surgery, Vol. 2,  W. B. Saunders Co. Philadelphia, 1996 pp1500-1510.

Burrows, Patricia, Thomson, Hugh, Vascular Malformations, Mastery of Plastic and Reconstructive

Surgery,  Vol. 1, edited by M. Cohen,  Little and Brown co.,  Boston, 1994 pp352-373

Crabb, Smith, Plastic Surgery, 4th edition, Little and Brown, Boston, 1991 pp.813-816.

 THE NATIONAL ORGANIZATION OF VASCULAR ANOMALIES PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE.  IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN.  NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE.  NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCOL.  IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS.

Revised 04/05/2014

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