Chapter 29
Hemangioma and
Lymphangioma
Roly Squire
Key Points
››The vast majority of hemangiomas involute spontaneously and do not need treatment.
››Early treatment for hemangiomas can be required for: obstruction of the airway, oropharynx, vision, hearing; ulceration causing infection; pain; persistent bleeding. Initial treatment is with oral propranalol or prednisalone.
››Lymphatic malformations are generally only of cosmetic importance, but their natural history is unpredictable, and they seldom resolve spontaneously.
››Venous malformations generally only need treatment for symptoms, mostly pain with activity but occasionally phlebitis.
››Vascular malformations are best treated in collaboration with an interventional vascular radiologist
29.1 Introduction
Vascular anomalies are common, and the majority present in childhood. The terminology has been confusing, but there is now a consensus classification, which distinguishes vascular
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tumors, such as hemangiomas, from vascular malformations, which can be capillary, lymphatic, venous or arterial. Most vascular anomalies are of cosmetic importance only, but symptoms can be both distressing and dangerous, and choice of treatment depends upon accurate diagnosis and careful assessment of the extent of the abnormality. This is often not apparent on simple clinical examination.
29.2 Types of Vascular Anomalies
1.Hemangioma: this is a common congenital abnormality, occurring in up to 5% of infants. It may only show as a small red mark at birth, but proliferates over the first two weeks of life to produce a firm, red, raised benign tumor (Fig. 29.1), often referred to as a strawberry naevus.
FIGURE 29.1. A large hemangioma on the back, showing early signs of involution, but also some scarring from healed ulceration.
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Proliferation continues variably during the first year of life, but generally a hemangioma remains asymptomatic, simply a cosmetic abnormality, with a favorable natural history: 50% will involute by age 5, and in more than 90% involution will mean that there is no need for intervention, though in up to 40% a faint blemish will always be visible.
Early treatment can be required for hemangiomas that occur at sites that interfere with normal bodily functions, such as peri-orbital or oropharyngeal lesions. Complex lesions around the mouth can extend into the pharynx or airway. Treatment can also be required for complications such as ulceration and infection, which lead to pain and scarring. Bleeding can be a nuisance, but is not dangerous, and can be controlled by simple pressure. Some large hemangiomas can have prominent arteriolar feeding vessels, but are still likely to spontaneously involute. The high flow component is only important in the very rare situation where there are signs of a hyperdynamic circulation causing heart failure or venticulomegaly.
Rarely hemangiomas are fully developed at the time of birth (congenital hemangioma), and only some of these will show the same tendency to involute as the classic infantile hemangioma.
2.Lymphangioma: a benign soft tissue tumor which can occur practically anywhere in the body.Typical sites are the neck, axilla or groin, but lymphatic malformations of the trunk or limbs are also regularly encountered. Lymphangioma can be present at birth, and 70% have presented by 1 year of age. However an occult lymphangioma can suddenly present at any age, even in an adult, as new soft tissue swelling appearing over a few hours in association with increased lymphatic flow. This would usually be as a result of an intercurrent infection, or an acute inflammatory process in the same lymphatic drainage area as the anomaly. Lymphangiomas can be macrocystic, often soft and flaccid making the diagnosis quite straightforward, or microcystic, in which case they can be firm and ill defined. Microcystic or mixed lesions can be difficult to distinguish clinically
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from other soft tissue tumors, including malignant tumors as well as other vascular anomalies. The history of rapid onset and/or fluctuation in size in association with viral infections is often diagnostic. The natural history of lymphatic malformations is unpredictable; although spontaneous resolution is rare some become less prominent with time, yet others enlarge unexpectedly.
3.Lymphangioma circumscriptum: the cutaneous form of lymphangioma, presenting as small blisters in the skin overlying a subcutaneous lymphatic swelling (Fig. 29.2), nearly always on the trunk. The blisters are often painful and can sometimes leak clear fluid. They can occur as part of the initial presentation of a lymphatic anomaly, but are more commonly a complication of attempted resection.
4.Venous (low flow) malformations: these may present as a visible varicosity or a soft tissue mass. Reduction of the swelling with pressure can be diagnostic. Unlike hemangiomas they do not tend to proliferate in the first few months, but also do not involute with time. Some venous malformations may not have a significant cosmetic impact, but may have an occult component that causes symptoms
FIGURE 29.2. Lymphangioma circumscriptum.
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as the child becomes older. Common symptoms are pain or swelling with activity,and rarely phlebitis.Malformations extending into joints can be a particular problem. Venous malformations can also be complicated by phleboliths, calcified thrombi sitting within the abnormal veins, presumably caused by turbulent flow.
5.Arterial (high flow) malformations: often called arteriovenous malformations (AVMs) these are rare in children, and are more likely to be congenital than post-traumatic. Heart failure and tissue overgrowth are the main complications in childhood, whereas adult AVMs tend to cause pain.
6.Kaposiform hemangioendothelioma: this rare form of vascular tumor is distinct from congenital or infant hemangioma because of its characteristic brawniness and infiltration (Fig. 29.3). Recognition is important because it can be complicated by platelet sequestration, resulting in profound and life-threatening thrombocytopenia, known as Kasabach Merritt Syndrome (KMS).
FIGURE 29.3. Kaposiform hemangioendothelioma (complicated by profound thrombocytopenia).