8.14 Hemangiomas, Vascular Malformations, and Lymphatic Malformations of the Head and Neck
Key Features
Hemangiomas, vascular malformations, and lymphatic malformations of the head and neck are common congenital and neonatal abnormalities.
Vascular tumors are classified into hemangiomas and vascular malformations.
Accurate diagnosis is the key to prognosis and treatment plan.
Accurate diagnosis is based on natural history and key clinical features.
Vascular tumors are common pediatric anomalies and may develop throughout the head and neck. They are divided broadly into hemangiomas and vascular malformations, each with a distinct natural history. Lymphatic malformations (or lymphangiomas) are defects in the lymphatic system that present as swellings or nodules in the skin or mucous membranes of the head and neck. The malformation of lymphatic tissue causes an accumulation of fluid, which accounts for the clinical presentation.
Epidemiology
Infantile hemangiomas occur in up to 12% of all children. They are more common in females than males, at a ratio of 3:1, and more common in European Americans. Sixty percent of hemangiomas are located in the head and neck, including the upper aerodigestive tract, and 80% occur as single lesions.
Vascular malformations are divided into lymphatic malformations, capillary malformations or portwine stains, venous malformations, and high-flow arteriovenous malformations (AVMs). Lymphatic malformations are further divided into microcystic lymphatic malformations and macrocystic lymphatic malformations involving the soft tissue of the neck. Vascular malformations have no gender or racial predilection and most commonly occur in the head and neck. Lymphatic malformations represent 6% of benign cervicofacial tumors of childhood; they are seen in fewer than 2.8 per 1000 people. Patients with Down′s, Turner′s, or fetal alcohol syndrome have a higher incidence of lymphatic malformations.
Clinical
Signs and Symptoms
Hemangiomas
Infantile hemangiomas are generally not present at birth and first appear during the first 6 weeks of life. Hemangiomas go through predictable phases of growth. The initial proliferative phase occurs over the first year, is characterized by rapid growth, and is followed by the involution phase with subsequent regression. Complete involution occurs in 50% of children by 5 years of age, 70% of children by 7 years of age, and 90% of children by 9 years of age.
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