Lymphangioma natural history

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Badria Munir M.B.B.S.[2] Haytham Allaham, M.D. [3]

Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after surgical interventions. Common complications of lymphangioma include esophageal obstruction, upper respiratory tract obstruction, infections, and compression of adjacent tissues such as nerves and blood vessels. Prognosis of lymphangioma is generally excellent.

Lymphangiomas have slower rate of progression. These malformations continue to grow as with the growth of patient.^[1]

• These are present on birth and may not be evident until the age of 5 years.
• Initially they grow as soft, cystic, translucent swelling in head and neck region, but it is commonly found in extremities. Some cases are reported in the internal organs such as pancreas and stomach as well.^[2]
• The presence of lymphangiomas and soft tissues and bone is not unusual and this form of disease has been termed as lymphangiomatosis.
• If left untreated lymphangiomas can either progress into large swellings or they can resolve spontaneously.^[3]
• If the continue to progress, they cause overgrowth and swelling of tissue involved, such as tongue, jaws, cheeks, neck, arms, fingers and toes.^[4]
• Clinical picture may vary from person to person because certain events such as puberty, infection, trauma and bleeding into lymphatic malformation can trigger rapid growth.
• Overgrowth can subsequently cause compressive effects on surrounding tissues, such as dyspnea due to compression on trachea, or dysphagia due to compression on esophagus and diplopia due if eye socket is involved.^[5][6]
• Rarely do cutaneous lymphangiomas interfere with the well-being of patients. Patients are expected to live a full healthy life, and they usually seek medical intervention because of cosmetic reason.
• The signs and symptoms of lymphangioma vary depending on the type, size and location of the mass.^[7]

• Cystic hygroma, which is also known as cystic lymphangioma or macrocystic lymphatic malformation, is a red or bluish swelling of the skin the neck, axilla or groin region that consists of large, fluid-filled spaces. Some say it is a type of cavernous lymphangioma.
• It consists of tiny, fluid-filled spaces and ranges in color from clear to pink, dark red, brown or black.

Common complications of lymphangiomas are primarily associated to the location of lesion.^[8]

Growth of lesion causes compressive effects on surrounding tissues. Which include following:^[9]

• Dyspnea: Trachea^[10]
• Dysphagia: Esophagous^[11]
• Constipation: Gastrointestinal tract^[12]
• Diplopia and proptosis: Retro-orbital tissue of eye^[13]
• Wheeze: Lower Respiratory tract^[14]
• Chest Pain: Mediastinum
• Shortness of breadth: Lungs^[10]
• Bladder Obstruction: Urinary tract
• Bone loss or overgrowth: Skeletal tissue^[15]
• Inflammation or bleeding into the lesion
• Recurrent Cellulitis:Visible deformity^[16]

Prognosis of lymphangioma is generally excellent after surgical intervention.

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