Microcystic lymphatic malformation

Alternate names

Superficial lymphangioma; Capillary lymphangioma; Capillary lymphatic malformation; Cutaneous lymphangioma circumscriptum; Microcystic infiltrating lymphatic malformation; Microcystic lymphangioma; Superficial lymphatic malformation

Definition

Microcystic lymphatic malformation is one subtype of lymphatic malformation (LM), a congenital malformation of the lymphatic vessels in soft tissues, including the skin.

Cause

The exact cause is unknown but is likely related to a malformation of the lymphatic system at six to ten weeks of gestation, when some lymphatic tissue fails to communicate with the lymphatic and venous system.

Types

• LM is classified into the macrocystic type, cysts larger than 2 cm with clear margins (previously known as cystic hygromas), and the microcystic type, consisting of cysts smaller than 2 cm, that appear diffuse, and grow without clear borders (previously known as lymphangioma circumscriptum).
• When the two types concur it is called the combined type.

Signs and symptoms

• Microcystic lesions are commonly found inside the mouth, throat, and in the tongue, parotid gland and submandibular gland.
• Symptoms include deformity, and problems with breathing and feeding.

Treatment

• Treatment depends upon the clinical presentation, the size of the lesion, the location, and resulting complications.
• Superficial mucosal microcystic lesions may be treated with laser therapy.

NIH genetic and rare disease info

• NIH info on Microcystic lymphatic malformation

Microcystic lymphatic malformation is a rare disease.

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