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Hemangiomas in Infancy and Childhood

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Vol. 13 •Issue 11 • Page 41
Hemangiomas in Infancy and Childhood

Psychosocial Issues Require Close Attention

Hemangiomas occur in 2.6% of all neonates and 10% of infants by 2 months of age.1 Although these benign tumors usually regress after a period of growth in early infancy, they are a major source of concern for parents.

Background

Hemangiomas are distinguishable from vascular malformations using established classifications for vascular birthmarks. Hemangiomas have a growth phase involving endothelial cell proliferation, followed by an involutional phase.2 The involutional phase is characterized by apoptosis, a process of preprogrammed cell death.

Although the pathogenesis of hemangiomas is not known, investigators have implicated vascular dysgenesis and hormonal, immune-mediated, iatrogenic, infectious and genetic factors. Increased levels of heparin and mast cells may also play a role.1 High serum estradiol has been the subject of study as well, since 17B levels have been found in hemangiomas.3 Researchers have also noted an association between vessels in the placenta and hemangiomas involving a shared immunoreactivity.4 An increased risk has been reported in women who undergo chorionic villus sampling and in some women with human papillomavirus infection.5,6

Cellular markers of angiogenesis (development of new vessels from existing vasculature) have been identified by immunochemical analysis. These include proliferating-cell nuclear antigen, type IV collagenase, basic fibroblastic growth factor, vascular endothelial growth factor, urokinase, and E-selectin. Basic fibroblastic growth factor is elevated in infants with hemangiomas.7 In rare cases, hemangiomas may occur as an autosomal dominant trait instead of sporadically.8

Early clinical findings of superficial hemangiomas include a small pale or blanched macule, a telangiectasias surrounded by a blanched halo, or a red macule.9 Deeper hemangiomas have a bluish cast, since they are within the dermis and the telangiectasias or erythema may not be as visible. During the rapid growth stage, a bright plaque forms. It may range from 5 mm to 2 cm in diameter or larger.

During the involutional phase, lesions soften and the colors shift from red to purple to gray. Eventually, they lighten to skin color. Between 20% and 40% of children have some residual changes in the skin, such as telangiectasias, hypopigmentation or epidermal atrophy. Subcutaneous hemangiomas may leave fibrofatty residuum. Areas that take longer to recede are the tip of the nose, lip and parotid.10

Vascular Malformations

A brief discussion of vascular malformations assists in clinically distinguishing hemangiomas. Vascular malformations have normal endothelial cell turnover. They derive mainly from the capillary system, although they can develop from venous, arterial or lymphatic vessels.1 Most vascular malformations are present at birth and clinically stable. The most common vascular malformations include the salmon patch (also known as stork bite, angel's kiss, nevus simplex and erythema nuchae) and the port wine stain.11 A salmon patch is present at birth in more than 75% of infants. It appears as a pink to red macule on the nape of the neck, glabella, forehead, upper eyelids, nasolabial region or lower back. Facial salmon patches tend to be located on the central portion of the face and do not follow dermatomal distribution. These fade by age 1 or 2 years, but lesions on the back of the neck often persist into adulthood. Another form of the salmon patch, which disappears more slowly, occurs on the sacral region and is known as the butterfly mark.12

A port wine stain, also called nevus flammeus, is a congenital reddish purple patch. In childhood, port wine stains tend to be stable and increase proportionately in size. They are present at birth and are usually unilateral and segmental. Although 50% of port wine stains occur on the head and neck, any body part can be affected. The port wine stain initially is pink but darkens and becomes more irregular, thickened and nodular with advancing age. Although most port wine stains are isolated findings, they can sometimes be associated with malformation syndromes. Pyogenic granulomas and basal cell carcinomas can occur within a port wine stain.12

Klippel-Trenaunay Weber syndrome is characterized by a large port wine stain. It is associated with venous varicosities and progressive hypertrophy of underlying soft tissues or bone. It usually involves an arm or leg, but multiple extremities or the trunk can be involved. Lesions can be extensive.13

In Cobb syndrome, a port wine stain occurs in a dermatonal pattern on the posterior trunk. This syndrome is associated with an underlying vascular malformation of the spine. The severity of neurological problems depends on the amount of underlying spinal cord involvement.14

Epidemiology of Hemangiomas

Hemangiomas occur in 10% to 12% of children younger than 1 year. Hemangiomas can progress rapidly within a few weeks, with coloring ranging from subtle to pronounced.15,16 Fewer than 30% of hemangiomas are evident at birth, but 90% are visible within the first 4 weeks of life.17 They are three times more common in girls than boys and are also more common in preterm infants. Some investigators believe that black and Japanese infants have a decreased incidence of hemangiomas compared with white infants.13

Although the majority of affected infants have a single lesion, in rare cases a baby can have hundreds of hemangiomas. They can appear at any site, including internal organs, but they most commonly occur on the head and neck. Hemangiomas can be located in the superficial dermis and extend into the deep soft tissue. Maximum size is typically reached by age 4 months to 6 months, but some may continue to grow for up to 12 months.18 In a case review of 510 patients with hemangiomas, less than 80% to 90% doubled in size, less than 5% tripled in size, and only about 2% quadrupled in size or grew dramatically.19 A hospital-based study showed that 50% of hemangiomas resolved by age 5 years, 75% by age 7 years, and 90% by age 10 to 12 years.20

Management

Proper diagnosis of hemangioma can usually be made with a thorough history and physical alone. But when the presentation is atypical — an increase in size or number, or systemic symptoms — studies such as ultrasound, magnetic resonance imaging and angiography may be warranted.

Since most hemangiomas resolve completely on their own, treatment consists of observation and education of families about the usual course. Regular follow-up, more frequent during the growth phase, is important to reassure parents and manage complications. Images of children with hemangiomas demonstrating the usual course of spontaneous regression can also be reassuring, particularly during the early period of rapid growth. Many references and Web sites are available for parents, including http://www.dermatlas.org, http://www.birthmarks.com and http://www.novanews.org.

Take photographs semiannually or annually to monitor the course of the hemangioma. Parents may benefit from support groups and, when necessary, mental health counseling. The cosmetic concern or disfiguring nature of especially large facial hemangiomas can create significant stress for families.

Primary care nurse practitioners are in an ideal position to incorporate careful follow-up, teaching and reassurance to patients with hemangiomas and their families. Because these lesions can last 5 to 10 years or more, families require ongoing monitoring and support.

Complications of Hemangiomas

Hemangiomas are occasionally complicated by high-output cardiac failure, necrosis and ulceration (Figure 4), compression of vital structures, or associated malformation syndromes. These problems may require special monitoring, imaging studies, laboratory evaluation, and specific medical or surgical intervention.

High-output cardiac failure is a complication of visceral hemangiomas typically associated with involvement of the liver. Multiple hepatic hemangiomas may be difficult to manage and carry a high mortality rate. Be aware that infants with or without cutaneous hemangiomas can present with heart failure from occult hepatic hemangiomas. Hepatomegaly and a bruit over the liver suggest this diagnosis.7

In diffuse neonatal hemangiomatosis, multiple cutaneous hemangiomas may be associated with visceral involvement of one or more organs, usually the liver, lung, gastrointestinal system or central nervous system. Hepatic involvement with heart failure is a common complication of diffuse neonatal hemangiomatosis.16 Affected infants develop multiple rapidly growing 1- to 3-mm hemangiomas shortly after birth. The physical examination should include palpation for cranial and abdominal bruits. Further studies (magnetic resonance imaging, cardiac or abdominal ultrasound and laboratory evaluation) should be directed by individual clinical findings and symptoms. Healthy infants with normal growth and development and no evidence of complications are diagnosed with benign cutaneous hemangiomatosis.12

During the rapid growth period, hemangiomas in certain locations are prone to complications. In areas that receive friction, such as the diaper area, neck crease, flexural creases of arms and legs, or occipital scalp, hemangiomas may develop painful ulcerations. This is also true of massive lesions at any site. Bleeding and secondary infection can occur. Anogenital hemangiomas are particularly prone to ulceration and can interfere with urination or defecation. Infected hemangiomas (a rare occurrence) can lead to cellulitis, arthritis, osteomyelitis or septicemia.7

Close proximity to vital organs can result in occlusion or obstruction. The most common sites of concern are the eye, airway and auditory canal. Periocular hemangiomas require close evaluation and early evaluation by an ophthalmologist to prevent permanent visual impairment. Amblyopia, significant refractive errors and strabismus have been noted in 80% of patients with untreated periocular hemangiomas.1 Hemangiomas involving the nose or mouth can cause partial or complete airway obstruction and eating difficulties. Complete airway obstruction can also result from circumferential laryngeal hemangiomas. Hemangiomas involving the ear and external auditory canal can cause scarring and conduction hearing deficits. Oral steroids may be used to treat these complications.1,7,12

Large facial hemangiomas may be associated with PHACES syndrome. This is a malformation syndrome with posterior fossa brain abnormalities, hemangiomas, arterial anomalies or intracranial hemangioma, cardiac anomalies (coarctation of the aorta), eye anomalies, and sternal or other midline deformities. PHACES syndrome should be considered a possible diagnosis in children with large, segmental, plaque-like facial hemangiomas including one or more dermatomes. These patients require eye examination, imaging studies of the brain with contrast, careful cardiac examination, and ultrasound of the heart and major vessels.1,21

When a cutaneous hemangioma involves the anogenital area or the lower back, rule out midline malformations of the spinal cord (tethered spinal cord) and anorectal or urogenital defects by ultrasound examination of the lumbosacral spine. This should be performed before 6 months of age.22,23 In older infants, magnetic resonance imaging is necessary.22,23

Kasabach-Merritt syndrome, which occurs rarely in newborns and infants, presents as a large, rapidly growing, deep-seated vascular lesion. The three components of this syndrome are severe thrombocytopenia, hemolytic anemia and a consumption coagulopathy. Although the vascular lesions associated with this disorder may demonstrate clinical features of a hemangioma, biopsies usually reveal kaposiform hemangioendothelioma or tufted angioma. Screening tests should include a complete blood count, blood smear, prothrombin time, partial thromboplastin time, platelet count and fibrinogen level. It is also necessary to determine soluble fibrin, platelet and fibrinogen survival. Systemic corticosteroids and alpha-interferon have been used to stop the progression of rapidly growing tumors and correct the potentially life-threatening thrombocytopenia and consumptive coagulopathy.12

Treatment of Complications

Ulcerated hemangiomas can be treated conservatively with topical antibiotics (mupirocin [Bactroban], bacitracin), gentle daily cleansing and nonstick dressings. Recalcitrant lesions may improve with pulsed dye laser therapy, and when all else fails, oral corticosteroids.7,18

Systemic or intralesional corticosteroids are used when the hemangiomas impinge on vital organs and act to shrink them or stop the rapid growth phase. Oral steroids may be safer and more effective. Most prescribers choose oral corticosteroids for complicated hemangiomas at a dose of 3 mg/kg/day to 5 mg/kg/day until the lesions respond, and then taper the dose over 2 to 6 months. Interferon is usually reserved for life-threatening or eye-threatening complications that do not respond to systemic steroids.1 Risks of oral steroids include colic, hypertension, decreased growth, fluid retention and predisposition to viral and bacterial infection. These children need to be monitored closely and the corticosteroid tapered as soon as possible.

The pulsed-dye laser (PDL) causes selective thermal destruction of the abnormally dilated blood vessels but has little effect on normal surrounding cutaneous structures. For this reason, some experts advocated early treatment of hemangiomas with PDL to stop the development of hemangiomas and trigger early regression. However, a prospective, randomized controlled study demonstrated no value to laser treatment for hemangiomas during the first year of life.18 In this study, 121 patients were randomized to an observation or a treatment group. The clearing of hemangiomas occurred similarly in the observation and treatment groups, but PDL significantly increased the risk of skin atrophy and hypopigmentation. Other studies have shown that PDL is an effective treatment for residual telangiectasias after the hemangiomas have involuted without therapy.24

Recent reports of spastic paresis have discouraged the use of interferon for hemangiomas except in life-threatening circumstances when other measures fail.1

In select cases, there may be a role for surgical excision or interventional radiologic sclerotherapy.1

Psychosocial Issues

Fifty percent of hemangiomas occur on the head and neck, often in a highly visible location. Understandably, the period of rapid growth is a concern for parents and families.

Upon entering school, residual lesions become a social concern for the child. Research shows that parents' emotional reactions include feelings of loss, grief, guilt, self-blame and aloneness.24 Reaction from the general public is a major stress and prevents some parents from taking their children on outings. A common concern is how the child's self-image and development will be affected over time. Parents recognize their child's awareness of the lesion by age 12 to 24 months, and social sensitivity starting after age 4 years.

Children with craniofacial anomalies receive less attention and are provided with less social involvements than unaffected children, shortfalls that lead to signs of aggression and antisocial behaviors.25 Research has documented few psychological differences between 3- to 5-year-old children with and without facial hemangiomas.26 The differences begin to surface as the children enter school.

To improve the psychological outlook for families, provide ongoing support to parents. Discuss the natural history of hemangiomas, and give families the opportunity to ask questions and express concerns. Explain that you are following the progression of the lesion, and that you will be available to discuss treatment options and follow their child's social and behavioral development.

Parents can help their children by modeling how to react appropriately to the lesion. Parents have found it helpful to teach a simple response to the inevitable questions about the hemangioma. "It's my birthmark and will go away" is an appropriate response. Regular playgroups or preschool environments provide support for the child, as well as a "rehearsal" for the school years.24

In most cases, sequential photographs demonstrate regression of the lesion and are a source of comfort to parents. Direct all parents to educational Web sites where they can see images of how other children's hemangiomas have regressed. These, too, can be a source of comfort.

Finally, counsel parents that residual hemangiomas, scarring, persistent vessels and loose skin can usually be repaired before children enter school. Intervention at that time may be safer and more effective than aggressive treatment in early infancy.18

References

1. Ceisler EJ, Santos L, Blei F. Periocular hemangiomas: what every physician should know. Pediatric Dermatology. 2004;21:1-9.

2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69:412-420.

3. Sasaki GH, Pang CY, Wittliff JL. Pathogenesis and treatment of infant skin strawberry hemangiomas: clinical and in vitro studies of hormonal effects. Plast Reconstr Surg. 1984;73:359-370.

4. North P, et al. A unique microvascular phenotype shared by juvenile hemangiomas and human placenta. Arch Dermatol. 2001;137:559-570.

5. Burton BK, Schulz CJ, Angle B, Burd LI. An increased incidence of hemangioma in infants born following chorionic villus sampling (CVS). Prenatal Diagnosis. 1995;15:209-214.

6. Cannistra C, Standoli L. Viral implication in immature angiomas. Etiopathogenic hypothesis and immunohistopathologic study of eleven cases. Pathol Biol. (Paris) 1994;42:150-155.

7. Drolet BA, Esterly NB, Frieden IJ. Hemangiomas in children. NEJM. 1999;341:173-181.

8. Blei F, Walter J, Orlow SJ, Marchuk DA. Familial segregation of hemangiomas and vascular malformations as an autosomal dominant trait. Arch Dermatol. 1998;134:718-772.

9. Hidano A, Nakajima S. Earliest features of the strawberry mark in the newborn. Br J Dermatol. 1972;87:138-144.

10. Nakayama H. Clinical and histological studies of the classification and the natural course of the strawberry mark. J Dermatol. 1981;2:277-291.

11. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics. 1976;58:218-222.

12. Rabinowitz LG, Esterly NB. Vascular birthmarks and other abnormalities of blood vessels and lymphatics. In: Schachner LA, Hansen RC, eds. Pediatric Dermatology. 2nd ed. New York, N.Y.: Churchill Livingstone; 1996: 953-989.

13. Young A, Cohen BA, Siegfried E. Cutaneous congenital defects. In: Taeusch HW, Ballard RA, Gleason CA, eds. Avery's Diseases of the Newborn. Philadelphia, Pa.: Elsevier Saunders; 2004:1521-1538.

14. Enjolras O, Garzon MC. Vascular stains, malformations, and tumors. In: Eichenfield LF, Friedman IJ, Esterly NB, eds. Textbook of Neonatal Dermatology. Philadelphia, Pa.: W.B. Saunders Co.; 2001: 324-352.

15. Jacobs AH. Strawberry hemangiomas: the natural history of the untreated lesion. Calif Med. 1957;86:8-10.

16. Holmdahl K. Cutaneous haemangiomas in premature and mature infants. Acta Paediatr. 1955;44:370-79.

17. Finn MC, Glowacki J, Mulliken JB. Congenital vascular lesions: clinical application of a new classification. J Pediatr Surg. 1983;18:894-900.

18. Betta K, et al. Randomized controlled study of early pulsed dye laser treatment of uncomplicated childhood haemangiomas: results of a 1-year analysis. Lancet. 2002;360:521-27.

19. Bowers RE, Graham EA, Tomlinson KM. The natural history of the strawberry naevus. Arch Dermatol. 1960;82:667-680.

20. Ezekowitz RA, et al. Interferon alfa-2a therapy for life threatening hemangiomas of infancy. New England Journal of Medicine. 1992;326(22):1456-1463.

21. Poetke M, Frommeld T, Berlien HP. PHACE syndrome: new views on diagnostic criteria. Eur J Pediatr Surg. 2002;12:366-374.

22. Albright AL, Gartneer JC, Wiener ES. Lumbar cutaneous hemangiomas as indicators of tethered spinal cords. Pediatrics. 1998;83:977-80.

23. Goldberg NS, Herbert AA, Esterly NB. Sacral hemangiomas and multiple congenital anomalies. Arch Dermatol. 1986;122:684-687.

24. Tanner JL, Dechert MP, Frieden IJ. Growing up with a facial hemangioma: parent and child coping and adaptation. Pediatrics. 1998;101:3:446-451.

25. Dieterich-Miller CA, Safford PL. Psychosocial development of children with hemangiomas: home, school, health care collaboration. Children's Health Care. 1992;21:84-89.

26. Dieterich-Miller C, et al. Behavioral adjustment and self concept of young children with hemangiomas. Pediatric Dermatology. 1992;9(3):241-245.

Sherry Guralnick Cohen is a family nurse practitioner and psychiatric clinical nurse specialist who practices dermatology at Johns Hopkins University Medical Center in Baltimore.




     

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