Lymphangioma

Summary about Disease


Lymphangiomas are rare, benign malformations of the lymphatic system, characterized by fluid-filled cysts or channels arising from abnormal lymphatic vessels. They can occur anywhere in the body, but are most commonly found in the head and neck region in children. While usually benign, they can cause complications due to their size and location, impacting surrounding tissues and organs.

Symptoms


Symptoms vary depending on the size and location of the lymphangioma. Common signs include:

Painless, soft, slow-growing lump or swelling

Skin discoloration or texture changes over the affected area

Difficulty breathing or swallowing (if in the neck or chest)

Recurrent infections in the affected area

Lymphorrhea (leakage of lymphatic fluid)

Pain (less common, but can occur if the lymphangioma compresses nerves or other structures)

Causes


The exact cause of lymphangiomas is not fully understood, but they are believed to be caused by:

Congenital malformations: Problems with the lymphatic system development during embryonic stages.

Blockage of lymphatic vessels: Obstruction can lead to lymphatic fluid accumulation and cyst formation.

Genetic factors: Rarely, some cases may have a genetic component.

Medicine Used


There is no specific medicine that cures lymphangiomas. Medical management focuses on reducing symptoms and preventing complications. The following may be used:

Sclerosing agents: Substances injected into the lymphangioma to cause it to shrink by scarring the inner lining of the cysts. (e.g., Bleomycin, Doxycycline, OK-432 (Picibanil))

Sirolimus: An mTOR inhibitor used to manage the growth of lymphangiomas, particularly in complex or diffuse cases.

Antibiotics: For treating secondary infections.

Pain relievers: For pain management.

Is Communicable


No, lymphangiomas are not communicable. They are not caused by infectious agents and cannot be spread from person to person.

Precautions


There are no specific precautions to prevent lymphangiomas, as they are often congenital. However, people with lymphangiomas can take steps to manage their condition and prevent complications:

Protect the affected area from injury to prevent bleeding or infection.

Maintain good hygiene to prevent infections.

Follow up regularly with a healthcare provider for monitoring and management.

Seek prompt medical attention for any signs of infection or complications.

How long does an outbreak last?


Lymphangiomas are not an "outbreak" in the infectious disease sense. They are chronic conditions. They can grow slowly over time, or remain stable in size. Interventions may provide long term relief or be needed over and over.

How is it diagnosed?


Diagnosis typically involves a combination of:

Physical examination: Evaluating the size, location, and characteristics of the lump.

Imaging studies:

Ultrasound: to visualize fluid-filled cysts.

MRI (Magnetic Resonance Imaging): to define the extent of the lymphangioma and its relationship to surrounding structures.

CT (Computed Tomography) scan: Provides detailed images, but less commonly used than MRI.

Lymphangiography: An x-ray procedure involving injection of dye into lymph vessels to visualize the lymphatic system (less commonly used).

Biopsy: In some cases, a tissue sample may be taken to confirm the diagnosis and rule out other conditions.

Timeline of Symptoms


Lymphangiomas are often present at birth or develop in early childhood (typically before age 2). The timeline can vary:

Congenital: Present at birth.

Early Childhood: Develops in the first few years of life.

Gradual Onset: Swelling may start small and slowly increase in size over time.

Stable: In some cases, the lymphangioma may remain relatively stable in size and not cause significant symptoms.

Complications: Symptoms can worsen if the lymphangioma grows rapidly, becomes infected, or compresses surrounding structures.

Important Considerations


Lymphangiomas can affect the quality of life, especially if they cause pain, disfigurement, or functional impairment.

Treatment decisions should be individualized based on the size, location, and symptoms of the lymphangioma, as well as the patient's age and overall health.

Surgical removal of lymphangiomas can be challenging due to their complex nature and proximity to vital structures. Recurrence is possible after surgery.

Multidisciplinary care involving specialists such as surgeons, radiologists, and hematologists may be needed to manage complex cases.

Ongoing research is exploring new and improved treatment options for lymphangiomas.