The diagnosis of hemangioma of infancy is made by clinical observation along with a careful history. Pertinent points range from the location of the lesion as well as other comorbid conditions. Occasionally, an imaging study may be used. Although computed tomography (CT) can be utilized, probably the most useful imaging study remains magnetic resonance imaging (MRI).
A benefit of MRI is the delineation of flow characteristics, tissue involvement and penetration with excellent resolution of involved adjacent structures, with no risk of ionizing radiation. These advantages are most useful for imaging of hemangiomas from the neck and head, as well as visceral hemangiomas. The reader is referred to radiologic reviews for an overview of imaging characteristics specific to those lesions.
A vessel density in excess of 5/cm2 and a peak arterial shift more than 2 kHz give a positive predictive value more than 97% for any proliferative hemangioma. Other lesions that can be included in the differential diagnosis in an infant include sarcomas (rhabdomyosarcoma, fibrosarcoma, neurofibroma) as well as other vascular malformations.
Occasionally, the need for definitive distinction from a proliferating as well as an involuting hemangioma exists. Some markers of proliferating hemangiomas include VEGF, bFGF and PCNA. Involuting hemangiomas may have fewer levels of PCNA, more TIMP, endostatin, angiostatin and IL-12 along with an increased number of mast cells.
There are several levels of urgency for treatment. At one end from the spectrum of surgical urgency lie those hemangiomas that mandate immediate intervention. These include airway-obstructing oro-tracheal-laryngeal hemangiomas, hemangiomas from the periorbit that can produce visual disturbances, including blindness, lesions that exhibit severe hemorrhage and ulceration, and the occasional liver hemangioma that can result in cardiac failure.
Most hemangiomas, however, can be managed by observation, because they will eventually involute spontaneously. About half of hemangiomas of infancy will involute in a manner that leaves minimal or no disfigurement. However, the rest will leave a conspicuous scar along with a residuum of fibro-fatty tissue that can be aesthetically disfiguring. These will need reconstructive interventions following involution to obtain a pleasing cosmetic result.
Recently, a fourth tier of lesions has been understood to be those that would be best prophylactically removed by surgery before resolution of the involution phase, most often to prevent the social-psychological trauma in affected children, or in order to prevent further distortion of structures and avert a more complex reconstruction.
Including hemangiomas of the nasal tip, where the delicate nasal cartilages are often involved. Even though management of hemangiomas requiring urgent treatment is clear, the controversy lies in management of the nonurgent lesions. Unfortunately, there has been few prospective studies to steer the management of those patients. Problems in managing these patients is the entrenched belief of numerous referring physicians, unfortunately communicated towards the parents, that hemangiomas shouldn't be treated until involution is complete.
One recent useful tool that may assist in therapeutic making decisions stratifies patients into groups that are high or low risk. Those hemangiomas having a significant dermal component will likely leave greater residual cutaneous deformations with post-involution changes and scarring. The location of hemangiomas is a significant risk factor, with the ones from the face having high likelihood of surgical intervention due not just to the involvement of eyes, ears and nose, but additionally because those in a beard-like pattern have a likelihood of airway involvement. Future research should further define subgroups that will best respond to specific interventions, whether medical or surgical.
Those hemangiomas that need to be urgently treated are first given high doses of systemic steroids and intra-lesional steroid injections. Second-level therapeutics for nonresponders or poorly tolerant patients include interferon-a or occasionally vincristine. Interferon-a is less popular than in the past because of its unwanted effects, the most serious of which is spastic diplegia. Orbital and airway lesions may be treatable with laser cauterization and surgical debulking like a second choice.
The treatment of less catastrophic lesions starts with supportive or hygienic measures; many of the important for ulcerated lesions which may benefit from topical analgesics and dressings. Pharmacologic treatment is brought into play for the above-mentioned absolute and relative indications. Small lesions may be treatable with direct intralesional infiltration of the corticosteroid. Multiple injections (4-7) are usually needed. For more extensive hemangiomas, prednisone given at 2-3 mg/kg daily is initiated; for life-threatening lesions, intravenous steroids receive, as stated above.
80 % of hemangiomas of infancy will respond to corticosteroid therapy. Responses should be seen within 2 weeks, and the steroids should then be tapered gradually during a period of many months. The clinician should be on guard for nonresponsiveness or rebound growth.
Laser therapy will often prove useful for superficial hemangiomas and is perhaps most useful for telangiectasias remaining in the residual tissue following involution from the hemangioma. Mixed and deep lesions will have a larger tendency to result in distorting fibro-fatty sequelae, which might require excision or reconstruction.
Surgery, as stated before, is definitively indicated for removal of a symptomatic hemangioma, as in the nasal tip. Uncomplicated hemangiomas are rarely resected; involuting hemangiomas might be resected for psychosocial reasons before the kid reaches school age or, if in the opinion from the surgeon, the residual scar would be persistent and would eventually require surgery anyway.
The other vascular tumors are treated differently. Pyogenic granulomas will typically require curettage and cauterization to prevent bleeding. A NICH will require surgical excision, since it won't involute. Kasabach-Merrit syndrome is a consumptive coagulopathy in the setting of an enlarging soft tissue mass, usually a kaposiform hemangioendothelioma (KHE); the individual displays a thrombocytopenia and bleeding diathesis that may be unresponsive to platelet transfusions. These patients require admittance to some hospital, steroid infusions and antiplatelet/antifibrinolytic medications, with or without embolization from the lesion.
Interferon-alpha is not as effective as other antineoplastics for example vincristine and cyclophosphamide in the treatment of a KHE involved in the Kasabach-Merritt syndrome. The KHE lesion should be surgically resected when possible; otherwise, these lesions take time and effort to manage medically. As stated before, this syndrome does not occur in hemangioma of infancy; it's found only in tufted hemangiomas or kaposiform hemangioendotheliomas.
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