Lymphatic malformation

What are some conditions related to lymphatic malformation (LMs)?

• Gorham syndrome (Gorham-Stout syndrome) - A type of LM that occurs in bones and partially destroys them
• Milroy disease - an inherited lymphedema (generalized swelling typically occurring in the legs) that is present at birth
• Lymphangioma circumscriptum - an abnormal collection of tiny lymphatic cysts. It’s often more widespread than it first appears
• Angiokeratoma - a group of skin lesions that are raised, dark red to black in color, and about 1 to 10 mm in size. Because these lesions often bleed spontaneously or following abrasion, treatment may be required to control bleeding.

What do lymphatic malformations look like?

• They typically lie just beneath the skin in the neck and the armpit, although can also occur in other organs and in bones.
• LMs appear as a swelling and sometimes more extensive enlargement of soft tissues and bones.
• An LM in the skin looks like tiny clear bubbles that often become dark red due to bleeding.
• LMs can cause the enlargement of any part of the body, including the lip, cheek, ear, tongue, limb, finger or toe.

What are the possible complications of LMs?

• The two major complications are infection and bleeding.
• A long-term complication is overgrowth of involved tissues and bones.

Can LMs be prevented?

No, but complications can be diminished.

• LM often swells when the patient has a viral or bacterial infection anywhere in the body.
• Infection in a LM of the head/neck region is less likely if your child brushes his teeth regularly and gets frequent cleanings by a dental hygienist.
• Treat your child promptly for middle-ear infections.

If repeated infection in an LM occurs, preventive antibiotic therapy may be indicated and your doctor may consider more aggressive treatment options, which are discussed in the Treatment and care section.