Tessa's Lymphatic Malformation before treatment

with OK-432

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

       Lymphatic Malformations  are congenital malformations that result in dilation of the lymphatic vessels and lymphatic channels responsible for lymphatic fluid drainage in the body.

      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 smaller 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.The prevalence of LM is 1.2-2.8/1000 live births.  Approximately 50% of lymphatic malformations are present at birth.  90% of this type of malformation appear before age 2.  There is an equal distribution present in males and females.

     When a malforamtion is present 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.  LM can develop into an anomly where there is a resulting severe anatomical deformity of the surrounding structures. 

      Lymphatic Malformations grow at a steady pace, although some grow more rapidly then others. 

     There are two types of lymphatic malformations.  Macrocystic malformations are large, soft, smooth masses that have either a normal or bluish tint under the skin.    Microcystic malformations are small, raised, lesions.  They contain a clear fluid. Both types of anomalies  may result in significan enlargment of the surrounding structures. 

Diagnosis: 

     Physical examination by a physician experienced in the managment of vascular anomalies may be all that is needed to diagnose a lymphatic malformation.  However, since these lesions can be confused with other types of vascualr anomalies MRI (magnecti resonacne imaging) or CT (computer tomography) is often used to both diagnose and determine the extent of the malformation.

Management:

      Surgery: Lesions that are localized and well defined  or macorcystic are generally treated by surgically removing the malformation. Surgical excision may also be sued for larger, microcystic lesions  as part of a managment / treatment  plan involving the other methods listed below.  Lesions that involve vital organ structures may be difficult to manage with surgical excision.

     Sclerotherapy:  Macrocystic LM can be successfully managed using sclerotherapy with alcohol, doxycycline or other drugs that cause a shrinkage fo the lesion in about 80% of the cases.  Unfortunately this method is not effective for microcystic lesions.  OK-432 is a chemical agent that has been used to successfully treat Macrocysitc LM.  This agent has been considered "experimental" and is only available on a limited use. 

Link to OK-432 Article

Tessa after treatment with OK-432

     Laser:  Laser therapy may be used on selected LM with good results.  Laser is often combined with other methods of treatment for best results. 

     Radio Frequency Ablation:  RFA is a relatively new technique that emits high energy radio frequency sound waves to selectively destroy superficial lymphatic malformations.  It is often combined with other methods of treatment.

     Chemotherapy Agents:  Large, life threatening lymphatic malformations that are not able to be surgically resected are sometimes treated with chemotherapy agents. 

     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 will typically enlarge during and upper respiratory illness.  Large Malformations in the neck can compromise the airway and result in difficulting breathing.

References: 

Hemangiomas and Vascular Malformations: Conditions and Diagnoses/Lymphatic Malformations. On line May 2007  http://www.cincinnatichildrens.org/health/info/vascular/diagnose/lymphatic.htm

Waner, Milton, Suen, James, Hemangiomas and Vascular Malformations of the Head and Neck. (1999) Wiley-Liss, pp64-79

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