Infantile Hemangioma Follow-up
- Author: Richard J Antaya, MD; Chief Editor: William D James, MD more...
Complications
No demographic, prenatal, and perinatal factors have been shown to be associated with higher rates of complications.[7]
Ulceration
Ulceration occurs in 10-15% of infantile hemangiomas, especially combined superficial and deep lesions. The cause of ulceration is not clear but may be a result of outstripped blood supply to the overlying skin or secondary to the action of certain cytokines.
Ulceration usually occurs in tense, rapidly proliferating hemangiomas and occurs more commonly in the anogenital region, lip, and chest, although any site may develop an ulcer. The ulcerations are extremely painful and result in scar formation upon healing, which may take months. A white discoloration seen early in the course (usually within the first 3 months of life), mimicking that seen in early involution, may herald an impending ulceration, particularly in segmental infantile hemangiomas.[73]
Secondary infection can occur, but cellulitis, abscess, and bacteremia are rare.
While intermittent bleeding is common, serious hemorrhage appears to be rare. Life-threatening arterial hemorrhage has been reported in at least 7 infants, mostly complicating segmental hemangiomas of the head and neck. Closer observation and imaging studies to assess underlying vasculature may be helpful in very high-risk cases.[74]
Treatment for ulcerated hemangiomas includes topical or oral antibiotics, bio-occlusive dressings (especially hydrocolloid dressings), pulsed-dye laser surgery, becaplermin gel (human recombinant platelet-derived growth factor),[75] and external compression therapy (especially helpful for limb lesions).[76] Pulsed-dye laser surgery has been reported to be effective for ulcerated superficial hemangiomas and often decreases pain, even before the ulcer has reepithelialized.[49] Medical therapy to hasten hemangioma involution with glucocorticosteroids or beta-blockers can also be helpful for recalcitrant ulcers.
Airway obstruction
Airway obstruction is a rare complication of hemangiomas; upper lip lesions very seldom obstruct both nasal passages. This can be a problem for young infants who are obligate nose breathers. Cervical parapharyngeal or palatal hemangiomas can cause acute or subacute obstruction.
Insidious signs and symptoms, such as sleep apnea, cor pulmonale, or even failure to thrive, can be associated with hemangiomas in the upper aerodigestive tract. Laryngeal (often referred to as subglottic) hemangiomas present early (6-8 wk) with symptoms of inspiratory or biphasic stridor, especially with feeding or crying. Cough, cyanosis, or hoarseness may be associated findings. The diagnosis is confirmed by direct laryngoscopy, MRI, soft tissue anteroposterior neck radiographs, or esophagography.
Prompt consultation with a pediatric otolaryngologist should be sought for all suspected cases. Treatment includes systemic corticosteroids or interferon alfa, as well as excisional or laser surgery. Tracheostomy is sometimes necessary until the hemangioma involutes.
Upper airway hemangiomas appear to be associated more commonly with superficial cutaneous hemangiomas involving the mandibular branch of the trigeminal nerve (beard area hemangiomas).[77] They can occur without cutaneous involvement.
Visual obstruction
Visual obstruction should be considered whenever a hemangioma involves the eyelids or periorbital tissues.
Hemangiomas can lead to visual deprivation amblyopia by 3 separate mechanisms: physical obstruction of the visual axis, astigmatism from direct pressure on the anterior segment from eyelid involvement (upper eyelid is more common than lower eyelid), and unilateral myopia. Strabismus can result either secondary to amblyopia or from paralysis of the extraocular muscles infiltrated by an orbital hemangioma.
A pediatric ophthalmologist should evaluate all children with periorbital hemangiomas using refraction with retinoscopy, with upper eyelid lesions requiring the most frequent observation. A thorough discussion of periocular hemangiomas is beyond the scope of this chapter; however, a review by Ceisler and colleagues describes the appropriate evaluation and potential treatments.[78]
Also see Hemangioma, Capillary in the eMedicine Ophthalmology section.
Other complications
Diffuse neonatal hemangiomatosis is a potentially life-threatening condition characterized by numerous cutaneous hemangiomas accompanied by visceral hemangiomas. When more than 10 cutaneous hemangiomas are present, the risk of visceral lesions rises. The liver and gastrointestinal tract are affected most often, although any organ can be involved. Congestive heart failure is a cause of early mortality because of increased vascular volume. Evaluation should include an imaging study of the liver (eg, liver scanning, MRI, ultrasonography) and stool guaiac tests to rule out intestinal bleeding from gut hemangiomas. Systemic corticosteroids and/or interferon alfa and conventional surgery are possible treatments.
Kasabach-Merritt phenomenon (KMP) is marked by platelet sequestration and severe thrombocytopenia associated with a rapidly proliferating vascular neoplasm. This often is accompanied by a potentially fatal, generalized bleeding disorder. KMP is heralded by rapid enlargement, edema of the surrounding tissues, and accompanying purpura.[79] Evidence suggests that most cases of KMP are associated with kaposiform hemangioendothelioma or tufted angioma and not infantile hemangiomas, as previously believed.[8] The early reports of KMP were described in lesions in which a clinical, not histological, diagnosis was made. Systemic corticosteroids are not usually effective, but vincristine has been effective in several cases of KMP caused by kaposiform hemangioendotheliomas and tufted angiomas.
Patients with PHACE syndrome present with hemangiomas and one or more extracutaneous congenital anomalies. Reports have also described intracranial invasion of associated infantile hemangiomas.[80] PHACE syndrome is an acronym denoting posterior fossa abnormalities (most characteristically Dandy-Walker malformation and other forms of brain hypoplasia), hemangiomas (cervicofacial, segmental/> 5 cm in diameter), arterial anomalies (especially carotid, cerebral, and vertebral), cardiac anomalies (especially coarctation of the aorta), eye abnormalities, and, rarely, midline ventral abnormalities (sternal clefting or supraumbilical raphe). See Mortality/Morbidity.
Segmental infantile hemangiomas involving the perineal area may be associated with other underlying congenital anomalies as delineated in the PELVIS or SACRAL syndromes.[12] The acronymic PELVIS syndrome describes the association of a perineal hemangioma with any of the following: external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, or skin tag. SACRAL syndrome is spinal dysraphism with anogenital, cutaneous, renal, and urologic anomalies, associated with an angioma of lumbosacral localization.
An isolated midline lumbosacral hemangioma may be a cutaneous marker for underlying occult spinal dysraphism, the most common being an intraspinal lipoma with resultant tethered cord. Spinal imaging should be performed in these cases. Symptoms may not occur for several years and which infants require corrective surgery has been debated, because as many as 10% of the population have asymptomatic tethering of the spinal cord. The decision should be left to a neurosurgeon with experience with this condition.
Rarely, infantile hemangiomas have been implicated in cases of consumptive hypothyroidism.[81] This was initially reported with hepatic hemangiomas; however, this has also been reported with bulky cutaneous infantile hemangiomas.[82] This phenomenon appears be secondary to high activity of the type 3 iodothyronine deiodinase enzyme in hemangioma tissue, which is responsible for degradation of T4 to reverse T3 (rT3). One case reported also demonstrated increased production of a thyrotropinlike hormone from a hepatic hemangioma.[83] Thyroid function tests should be ordered in the appropriate clinical setting.
Psychosocial problems associated with disfiguring facial hemangiomas can be significant.[84] During infancy and early childhood, parents often have reactions of loss and grief. Parental feelings of disbelief, panic, or fear often are associated with the rapid growth of these lesions. The variability in the natural course, in regard to timing and completeness of resolution, adds to parental anxiety. Parental stress is heightened by strangers who stare, startle, or raise questions about causality, such as trauma (especially implied or suspected child abuse), infection, or cancer. Psychosocial stigmatization can be problematic for both parents and patients with disfiguring facial hemangiomas. Lesions that result in significant facial or obvious disfigurement should be addressed before the child starts school.
Prognosis
The prognosis for most uncomplicated infantile hemangiomas is very good, with complete involution of 50% by age 5 years, 70% by age 7 years, and 90% by age 9 years. Despite resolution of the vascular component, residual skin changes are observed in roughly 50% of cases. Of hemangiomas that have involuted by age 6 years, 38% still have residual evidence with scar formation, telangiectasia, or redundant or anetodermic skin. Hemangiomas that take longer to involute have a higher incidence of permanent cutaneous residua. Eighty percent of lesions that complete involution after age 6 years may exhibit significant cosmetic deformities. An increased incidence of permanent residua exists when the lip, nasal tip, eyelid, and ear are involved.
Patient Education
Educating parents about the variable natural history, prognosis, risks, and benefits of potential treatments and possible complications is essential.[84] Emotional support should be offered for parents of children with severe or complicated hemangiomas. Birthmarks: A Guide to Hemangiomas and Vascular Malformations by Milton Waner, MD, a book for parents and caregivers of children with vascular lesions, can be quite helpful. The Vascular Birthmarks Foundation is a useful source for accurate information for patients and family members.
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