Hemangiomas and vascular malformations (VMs) are not cancers, but they occur frequently in the head and neck region and commonly present to the head and neck surgeon. These can be very destructive and even life threatening. The head and neck surgeon needs to know how to identify and manage these uncommon but complex benign vascular lesions. Furthermore, there is increasing evidence that many vascular malformations exhibit behavior similar to low-grade malignancies.

The treatment of vascular anomalies should be performed by an experienced team of specialists, consisting of surgeons, diagnostic radiologists, interventional radiologists, dermatologists, pediatricians, medical oncologists, and other specialties. Because of the psychological impact on these patients, it is important to understand and address this issue as well. Vascular anomalies may cause aesthetic complications, functional disorders, physical disabilities, and pain. These patients can become quite withdrawn and depressed, and these aspects should be similarly addressed by the multidisciplinary team, including psychologists, to improve quality of life and patient satisfaction.

Many of the VMs cannot be cured but can only be controlled. It is important to convey full understanding of this concept, when counseling patients and their families at the onset of diagnosis in order to set appropriate levels of expectation from treatment. Frequently multiple treatments are required to control vascular anomalies, which may require lifelong medical and psychological care.

IHs are composed of proliferating immature endothelial cells that express histologic markers found on placental blood vessels as reported by North et al.¹ These are the most common tumor of infancy and are present in ˜5% of the population. They occur more frequently in infants from mothers with early trimester bleeding, preeclampsia, and placental anomalies.² IHs are rarely noted at birth, but commonly a macular erythema area of the skin is noted in the first 6 weeks of life and then a rapid growth is usually noted. These lesions present in the head and neck region in over 60% of cases. Most IHs grow within the first 3 months of life and continue to grow up to 1 year of age and may be very extensive. After 1 year of age, IHs will go into a quiescent phase and began to involute and eventually resolve. Most will involute completely by 7 years of age. This natural history is important to differentiate IHs from other congenital vascular lesions and will help to guide management decisions.

IHs can present as focal or segmental lesions involving multiple areas. They are also characterized as superficial, deep, or compound. It is rare for a focal IH to involve muscle or penetrate beyond subcutaneous fat. However, an IH involving the parotid gland can involve the gland itself.

When IHs involve the lip, eyelids, orbit, and nose, or subglottic region, they may lead to significant functional and/or aesthetic problems during the rapid proliferation phase (Fig. 37.1A-C).

Infants with five or more focal IHs may also have hepatic involvement and should undergo abdominal ultrasound. Segmental IHs are usually more complex and in the head and neck will follow a trigeminal nerve distribution. They are diffuse and compound and maintain irregular borders. It is common to see more than one facial subunit involved (Fig. 37.2).
They commonly penetrate into deep fascial planes of the head and neck. IHs involving the beard distribution are those most commonly described. These usually involve the lower lip, chin, neck, and preauricular areas, and ulcerations are frequently present. As many as 60% of IHs with a segmental beard distribution will involve the subglottis and require airway endoscopy.³ Patients with segmentally distributed IHs should be evaluated for PHACES syndrome (posterior fossa malformations, hemangiomas, arterial lesions, cardiac abnormalities, eye abnormalities, and sternal clefts).

The diagnosis of IHs is primarily clinical. They are not usually noticed at birth, and when the child is between 1 and 3 months old, these lesions will begin growing and after 1 year, they will begin to involute. Biopsies are not usually necessary and scans are not usually indicated unless there is a concern for the PHACES syndrome.

The management of IHs has improved significantly in recent years. Because of their natural involution, IHs were historically managed with observation only. Even though they resolve spontaneously, they can cause significant functional and disfiguring sequelae. Most problematic events from IHs will occur during the proliferative phase and may include ulceration, bleeding, pain, disturbance of vision, airway compromise, and feeding difficulties. The late sequelae will include scarring, telangiectasias, and fibrofatty residuum.

The treatment of IHs may involve surgical excision, laser therapy, topical therapy, intralesional corticosteroids, systemic corticosteroids, systemic β-blockers, and vincristine chemotherapy. If an infant has a focal hemangioma, which is primarily deep, the lesion can typically be observed unless ulceration or bleeding occurs. For the superficial focal lesions, laser therapy or surgery can be used to control the IH. For the problematic hemangiomas, β-blockers have been found to be effective in over 95% of the cases with rapid involution. The most common dose of β-blockers has been 2 mg/kg/day in divided doses two to three times daily.⁴ During the involution phase, surgical excision can give excellent results and prevent late sequelae.

As mentioned previously, VMs are very different from IHs. They do not involute and will continue to progress throughout life. They may have rapid growth spurts at different times of life. Our research has shown that many VMs have progesterone receptors⁵ and this is associated with growth spurts during puberty, use of oral contraceptives, and pregnancy. We commonly see patients
who did not notice their malformation until these events occurred. There are also unknown causes for growth spurts, which have not been identified yet. The destructive and life-threatening events that occur from many VMs will be illustrated.