Evaluation

"More mistakes are made…by not looking rather than not knowing"
Hiram E. Miller

In most instances the diagnosis of common hemangioma can be established on the basis of the history and physical examination. A history of early onset, followed by rapid proliferation in early infancy is characteristic of hemangiomas. At times it will be difficult to distinguish a "field" hemangioma from a portwine stain. Careful observation over several weeks will demonstrate that the hemangioma is proliferating. Subcutaneous hemangiomas are sometimes difficult to separate from lymphatic malformations of the cystic type. While lymphangiomas will not involute, sometimes the physician or parents would like to know prior to the age of resolution. Ultrasound, CT scans and MRI are helpful in separating these two entities.^42-44 In the proliferative phase, CT and MR imaging demonstrate hemangiomas as well-circumscribed, densely lobulated, uniformly enhancing lesions with dilated feeding and draining vessels in the center or at the periphery.(Figure 8a,b) The measurement of urinary levels of basic fibroblast growth factor may be helpful in distinguishing hemangiomas from structural malformations.⁴⁵ Measurement of this growth factor, which is elevated in hemangiomas, can also be used to monitor involution in select circumstances.

It has been mentioned that periorbital hemangiomas, particularly those involving the upper lid, need complete ophthalmologic examination and careful observation. Likewise, hemangiomas that threaten the airway or ear canal should be evaluated with consideration for treatment (Figure 9). Plain radiographs can be helpful in the initial evaluation of patients with possible laryngeal hemangiomas. The shadow of a mass in the airway can often be detected using plain films. Confirmation and visualization of the hemangioma is often desirable and can be accomplished using laryngoscopy. If it is suspected that a hemangioma is infected then appropriate cultures prior to antibiotics should be considered. Patients suspected of having the Kasabach-Merritt phenomenon should have a hematologic workup that includes platelet and complete blood count as well as coagulation studies. Proper evaluation of midline lumbosacral hemangiomas includes magnetic resonance imaging.³⁷ Plain films or ultrasound are not useful screening tools for patients with midline spinal hemangiomas.³⁷

There is considerable controversy in determining how infants with multiple cutaneous hemangiomas (>3) should be evaluated so that the possibility of significant visceral hemangiomas can be excluded. In addition to a history and physical examination, an abdominal ultrasound should be requested to identify possible hepatic hemangiomas. Liver hemangiomas correlate directly with prognosis in this condition and it is important to identify them early, prior to the time of proliferation. The presence of hepatomegaly is suggestive of hemangiomas however it is not recommended that one rely upon this physical finding alone. If a screening ultrasound is positive then CT scans and MRI can be used to more clearly delineate the extent of the involvement (Figure 10). Some authors have suggested a plain film of the abdomen, but these are generally of little use and have the additional disadvantage of radiation exposure. If hepatic hemangiomas are discovered or there is additional evidence of visceral hemangiomas then we commonly request a total body MRI to help identify any other internal lesions. CT scans will provide a similar documentation but they do involve the use of radiation. When there is doubt concerning the best imaging technique for a specific patient, it is useful to consult with an experienced radiologist. Blood counts, urinalysis, stool examination for blood, EKG, ophthalmologic or neurologic consultation may be appropriate for the patient with multiple hemangiomas if specific organ involvement is suspected.

Extensive facial hemangiomas (Figure 11) have been associated with arterial, central nervous system and ophthalmologic anomalies.⁴⁶ The acronym, PHACE, has been proposed for these patients to emphasize the characteristic findings: posterior fossa malformations, hemangiomas, arterial anomalies, coartation of the aorta and cardiac defects and eye abnormalities.⁴⁶ Patients with large facial hemangiomas should have a complete neurologic and ophthalmologic examination, including the measurement of head circumference. If neurologically normal, then an ultrasound examination is indicated in children less than 6 months old, an age at which the fontenelles have yet to close. If neurologic abnormalities exist then MRI should be performed.

Histologic examination is rarely necessary when evaluating patients with routine hemangiomas but it can be useful in select circumstances when the diagnosis of hemangioma is uncertain. A biopsy can help differentiate hemangiomas from other conditions. Cutaneous rhabdomyosarcoma, nasal glioma, hemangiopericytoma, tufted angioma, Kaposiform hemangioendothelioma, dermoid cysts, and some types of histeocytosis can all mimic the clinical appearance of common hemangiomas. Each of these conditions has a distinctive histologic appearance. In the future it may be possible to distinguish infantile hemangiomas from vascular malformation based on specific histologic stains.⁴⁷

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