Lymphatic malformations are abnormal channels and cysts filled with clear lymphatic fluid. Processing this fluid thru the vascular system is very slow resulting in swelling to the affected area. More than 70% of the time, these lesions are in the neck area or axilla. However, the malformations can form in any area of the body. Cause is unknown but it is believed that the error occurs during fetal development. Recent findings by Harvard Medical School and other researchers have shown hope for further genetic testing. Most LM’s are discovered before the age of 2. MRI (magnetic resonance imaging) or CT (computed tomography) is typically used to make a diagnosis.
The lesions can be defined as macrocystic, a single large cyst, or microcystic, a collection of tiny spaces and cysts. The LM’s are also described as localized, diffused, deep, or superficial. Depending on location, macrocystic are the easiest to remove. Microcystic are more difficult since they are typically a collection of small cysts which tend to be diffused decreasing the chance of complete removal and increasing the chances of regrowth. If LM’s can be completely removed, outcomes are very successful. However, if LM cannot be completely removed, vitality to the surrounding anatomical structures is critical. Recently, neck/mouth/facial (cervicofacial) LM’s have began to be categorized as Stage I, II, III, IV based on area and severity. This staging has allowed surgeons to collect data on the best treatment and review outcomes.
Since many of LM’s are in the neck area, airway issues are the highest priority. LM’s can cause macroglossia, thickening of the tongue, which can block airway and cause speech/eating complications. Viral infections or sudden trauma can cause rapid but temporary swelling. Many patients require a tracheostomy to provide a patent airway and a feeding tube for nutrition. However, once LM’s are treated and stable, many of the tracheostomy and feeding tubes can be removed. Bone overgrowth can develop causing facial deformity. The overgrowth of the jaw line can be cosmetically repaired with surgery when the patient is in late teens and have reached their maximum growth. Dental and oral hygiene can also be areas if concern.
There are currently several types of treatments for LM’s. However, there is no one “best practice” which outlines the optimal approach as each LM patient presents and responds differently to treatment. Surgical removal still remains the best among surgeons surveyed. However, there are other treatments which can be used alone or with surgery. Sclerotherapy is an injection of an agent that shrinks the cyst. Macrocystic lesions respond the best to sclerotherapy. Some agents used are alcohol, doxycycline, or Bleomycin. OK-432 is another sclerosing agent that remains in a non-FDA approved clinical study. Laser therapy is typically used in conjunction to other procedures with good results and may require several treatments. Radio frequency ablation uses high-energy radio frequency sound waves to destroy lesions. Chemotherapy agents have also been used.
In 2005, Harvard Medical along with Boston Children’s Hospital made an important finding in patients dealing with LM and other related vascular anomalies that have opened the door to current treatment of infantile hemangiomas and new clinical trials for LM and other vascular anomalies.
LM patients can go through several surgeries and treatments during their youth. Therefore, it is strongly encouraged that they seek professional counseling to deal with any emotional and physical challenges.