1.Authors    
2.Abstract      
3.Epidemiology  
4.Classification 
5.Natural History
6.Complications  
7.Evaluation   
8.Treatment     
9. Table 1     
10.Figures       
11.References
12.Quiz       

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Complications

While the majority of hemangiomas are associated with a benign course, some tumors create health problems for an affected child. Spontaneous bleeding is a rare complication, most often seen in patients with multiple hemangiomas or the Kasabach-Merritt phenomenon. Occasionally an ulcerative hemangioma will be associated with a minimal amount of bleeding. Many a mother has expressed fears of spontaneous rupture of a hemangioma with the resultant demise of the child. In our experience with thousands of patients this is not a likely possibility. Some parents limit their child’s activities and playtime with other children so that the hemangioma can be protected from minor trauma. It is important to uncover these fears and spend time counseling and addressing parental concerns.32,33

Ulceration is the most common complication seen. The incidence of ulceration ranges from fewer than 5% to as many as 10% of patients with hemangiomas.12,34 Ulcerated hemangiomas occasionally will be associated with infection or a minimal amount of hemorrhage. Hemangiomas that ulcerate invariably leave a scar. The likelihood of a scar should be explained to parents early so that there is not a misconceptionfig6.jpg (11607 bytes) that treatment has caused the scar.

Ophthalmologic evaluation is suggested for any periorbital hemangioma.

Ocular complications include amblyopia, astigmatism, strabismus, proptosis, and optic atrophy. Even small lesions on the upper lid should be taken seriously because they often affect visual acuity (Figure 6). In rare cases, hemangiomas located at or near a tear duct will block the lacrimal drainage system resulting in matting and conjunctivitis.

Infants with laryngeal hemangiomas usually present between 6 and 12 weeks of age with stridor. Patients with hemangiomas located in the subglottic region of the larynx are particularly prone to have airway symptoms because of the narrowness of the airway in this region. More than half of patients with laryngeal hemangiomas also have cervicofacial cutaneous hemangiomas. A strong association between a "beard" distribution of the facial hemangiomas and laryngeal involvement has been reported.35 The "beard" distribution includes the preauricular areas, chin, anterior portion of the neck and lower lip. It has been our experience that the more extensive the "beard-like" cutaneous hemangioma, the more likely it is that the child will have airway symptoms.

Hemangiomas that block or cover body orifices are associated with a variety of difficulties. It is rare that hemangiomas occlude the nasal passages but a significant narrowing or occlusion is indication for treatment. Since children are obligate "nose breathers" during the first few months of life they generally do not easily tolerate nasal constriction or obstruction. Large tongue hemangiomas can make breathing and eating difficult and may require intervention. If a hemangioma occludes the external auditory canal, hearing and speech will be impaired. Partial occlusion of the canal can promote otitis externa. In a similar fashion, perirectal hemangiomas can impair defecation and make hygiene difficult.

fig7.jpg (11901 bytes)Infants with lumbosacral cutaneous hemangiomas (Figure 7) may have associated spinal and genitourinary abnormalities.36-38 These abnormalities may be asymptomatic and have no neurologic symptoms. If not identified early, the hemangioma can involute and the underlying anatomic abnormalities may not be apparent until permanent neurologic deficits have occurred. Conditions associated with lumbosacral hemangiomas include the tethered cord syndrome, spinal dysraphism, and lipomeningocele.

The Kasabach-Merritt phenomenon identifies a subset of patients with a large vascular tumor and an associated thrombocytopenic coagulopathy. Studies have revealed that other vascular tumors, not common hemangiomas, are responsible for Kasabach-Merritt coagulopathy.10 Most often these tumors are Kaposiform hemangioendothelioma (KHE), but other vascular tumors, such as tufted angioma, have been reported to cause this condition. In contrast to hemangiomas, Kaposiform hemangioendotheliomas occur equally in males and females, can occur in older children and adolescents, tend to proliferate for a longer duration, and frequently leave significant residual lesions after the resolution of the coagulopathy.10,39-41 The histologic of KHE are distinctive.

Tumors associated with the Kasabach-Merritt phenomenon undergo rapid enlargement and are associated with petechiae and purpura. The overlying skin of these lesions is often tense and shiny. The thrombocytopenia seen in the KM phenomenon is generally profound. Spontaneous bleeding is rare with common hemangiomas but acute hemorrhage is a paramount concern in patients with KM phenomenon. Despite aggressive treatment the KM phenomenon is associated with a high mortality, especially in those patients with retroperitoneal tumors.10

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