Management Of Haemangioma
BULLETIN FOR MEDICAL PRACTITIONERS
Dr. Chan Yuin Chew
Associate Consultant Dermatologist, National Skin Centre
Definition
Haemangiomas are benign tumours of the vascular endothelium. They are extremely heterogenous clinically, with size, location and rate of proliferation having a significant effect on the risk of complications.
Hemangioma of infancy (HOI)
These are the most common type of hemangiomas. They appear within the first few weeks of life and have a predilection for the head and neck. They demonstrate rapid growth in early infancy, usually from 3-6 months of age, and subsequently, slower growth to their maximum size at approximately 9-12 months of age. They then enter the involution stage, which may begin anytime between a few months to18 months of age.
The superficial component undergoes a colour change from bright red to dull red, then to gray; the deep component becomes less blue and less warm. Hemangiomas complete involution at the rate of 10% per year and 50% at complete resolution have no clinical trace. However 50% of hemangiomas resolve with minor residual changes like telangiectasia, atrophic wrinkling and discolouration or more cosmetically significant changes like redundant skin with underlying fibrofatty tissue or scarring.
Worrisome presentations and their associated complications are summarised in Table 1.
Multifocal hemangiomas with or without extracutaneous involvement
Synonym: benign neonatal hemangiomatosis (cutaneouslimited), disseminated neonatal hemangiomatosis (visceral involvement)
Multiple small cutaneous lesions, varying in size from a few millimetres to several centimetres, are present in the neonatal period. There is an associated risk of visceral involvement, especially in the liver and gastrointestinal tract, which may in turn lead to congestive cardiac failure and haemorrhage. Findings suggestive of hepatic involvement include hepatomegaly and a hepatic bruit. Tachypnoea and respiratory crepitations may suggest cardiac failure. Useful investigations include FBC, LFT, stool occult blood and ultrasound of the liver and abdomen.
PHACES syndrome 1
In the presence of a large facial hemangioma, the possibility of this rare syndrome should be kept in mind: Posterior fossa malformation, Hemangioma (large facial), Arterial anomalies, Cardiac anomalies, Eye anomalies, Sternal cleft +/- supraumbilical raphe.
Investigations
In the vast majority of cases, the diagnosis can be established on the basis of history and physical examination alone. The differential diagnosis include but are not limited to: tufted angioma, port-wine stain, venous / lymphatic / arteriovenous malformation, kaposiform hemangioendothelioma, pyogenic granuloma, nasal glioma, myofibromatosis, spindle and Spitz nevus, dermoid cyst, etc.
The Kasabach-Merritt syndrome does not occur in haemangiomas. Instead it is a complication of 2 other much less common vascular tumours, namely kaposiform hemangioendothelioma and tufted angioma.
The following diagnostic tests may be considered in clinically atypical cases:
Skin biopsy
The increased risk of bleeding makes this a relatively risky way to make a diagnosis. However it is occasionally necessary to differentiate atypical cases from other soft tissue tumours such as kaposiform hemangioendothelioma, myofibromatosis and rhabdomyosarcoma.
Ultrasound
This is helpful for the diagnosis of hepatic lesions as well as to evaluate infants (less than 3-6 months of age, before closure of posterior fontanelle) with large facial haemangiomas for possible structural brain abnormalities.
MRI
This is useful for differentiating hemangiomas from vascular malformations. It helps to delineate the extent of the tumour in danger areas like the "beard area" (lower face/ neck) where airway compromise may occur. It can be used to evaluate infants with large facial haemangiomas for possible structural brain abnormalities.
CT scan
It has the same uses as the MRI, but is not as specific in differentiating haemangiomas from vascular malformations.
Thyroid function test2
Thyroid function screening of infants with either very large haemangiomas or hepatic lesions should be considered. Huang et al reported a 3-month-old infant with a massive hepatic hemangioma and primary hypothyroidism. High levels of type 3 iodothyronine deiodinase activity were found in the haemangioma tissue. This enzyme is involved in the inactivation of thyroxine.
Management
Clinical heterogeneity and the difficulty in predicting the course during early infancy make the management of haemangiomas a potentially challenging one.
An uncomplicated hemangioma, which refers to a cutaneous hemangioma that is asymptomatic, nonulcerated and not impairing a vital function, can be observed. A complicated hemangioma or one that may lead to cosmetic disfigurement and subsequent social stigmatisation should be treated. In such cases, comanagement with the paediatrican should be considered.
It is often difficult to predict the progress and prognosis of the hemangioma during the first few months of life. One should monitor haemangiomas carefully for the first few weeks to months. The parents should be advised to return if unusually rapid growth or complications occur. Both the physician and parents can afford to worry less after 6 months and much less after 1 year.
First Line Therapy
Intralesional steroids4
The mechanism of action of steroids is not well-known, but vasoconstriction, hormonal influences and inhibition of angiogenesis have been proposed. Intralesional steroids can be used for well-defined hemangiomas during the proliferative phase. Two to 5 injections at 4 to 8 weekly intervals. Maximum: 3 mg/kg triamcinolone (10-20mg/ml) per treatment session.
Not recommended for periocular hemangioma as eyelid necrosis and central retinal artery occlusion are potential serious complications5.
Systemic steroids
Response is variable: 1/3 dramatic shrinkage, 1/3 stabilisation, 1/3 minimal or no response. Cessation of growth or the onset of involution is usually seen within 2 weeks if the tumour is steroid-responsive. As a rough guide, the following dosing regimen can be used and should be tailored to the patient’s response: 2 to 4 mg/kg/ day for 2 to 4 weeks, followed by 0.5 to 1.5 mg/kg/day for 4 to 8 weeks, then taper off over the next 4 to 8 weeks.
Potential side effects include adrenal axis suppression, Cushingoid facies, personality changes, delayed skeletal growth, gastric upset, hypertension and immunosuppression. Aseptic necrosis of femoral head, osteoporosis, cataracts are uncommon complications if oral steroids are limited to less than 6 months. For several months after cessation of therapy, there is a need for "stress" doses of oral steroids if the child falls sick. Live attenuated vaccines are contraindicated during the period of treatment.
Topical corticosteroids
Mid-potency to potent topical steroids can be used.
Vascular-specific pulse dye laser3
Due to the limited depth of penetration, this laser works better for thin superficial hemangiomas than for those with a deep component. It can improve residual telangiectasia after involution and may be effective in treating ulcerated hemangiomas.
Second Line Therapy
Interferon alpha6-8
This inhibits endothelial cell proliferation. It is given subcutaneously at a dose of 1-3 million U/m2 body surface area daily for life-threatening haemangiomas that failed to respond to systemic steroid therapy. Treatment is usually given for 2 to 12 months. Side effects include transient neutropenia, fever, elevated liver enzymes and flu-like symptoms. Neurotoxicity, specifically spastic diplegia, is the most feared complication that may develop in 5-10% of patients.
Vincristine9,10
Several anecdoctal reports of its efficacy in treating large, life-threatening hemangiomas. The usual dosage is 0.05mg/kg in infants less than 10 kg, or 1.5mg/m2 in infants greater than 10 kg, given intravenously on a weekly basis via a central venous line.
Surgical excision
This can be considered for well localised lifethreatening or function-impairing hemangiomas not responding to medical treatment, lesions complicated by persistent bleeding/ulceration and lesions at risk of significant scarring (lips, nose, ears, glabella).
Haemangiomas may involute completely but leave cosmetically significant changes like telangiectasia, atrophic wrinkling, discolouration, redundant skin with underlying fibrofatty tissue or scarring. Surgical excision can be offered to such patients for cosmetic improvement.
Special cases
I. Ulcerated hemangioma
Management focuses on pain relief, local wound care, prevention and treatment of secondary skin sepsis. In addition, systemic or intralesional steroids and vascularspecific pulsed dye laser should be started early.
II. Function-impairing periorbital hemangioma
Animal studies have shown that a few days of abnormal visual input in the first few months of life may result in permanent visual defects. For a periorbital hemangioma that obstructs the visual axis or exerts pressure on the eye, immediate intervention is required.
The patient should be referred to and be seen by a paediatric ophthalmologist within 1 to 2 days. Should there be a delay, the unaffected eye should be patched to prevent amblyopia. Systemic steroids should be considered early as first line therapy.
References
- Frieden IJ, Reese V, Cohen D. PHACE syndrome. The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. Arch Dermatol 1996; 132:307-11.
- Huang SA, Tu HM, Harney JW, Venihaki M, Butte AJ, Kozakewich HP, Fishman SJ, Larsen PR. Severe hypothyroidism caused by type 3 iodothyronine deiodinase in infantile hemangiomas. N Engl J Med 2000; 343:185-9.
- Batta K, Goodyear HM, Moss C, Williams HC, Hiller L, Waters R. Randomised controlled study of early pulsed dye laser treatment of uncomplicated childhood haemangiomas: results of a 1-year analysis. Lancet 2002; 360:521-7.
- Sloan GM, Reinisch JF, Nichter LS, Saber WL, Lew K, Morwood DT. Intralesional corticosteroid therapy for infantile hemangiomas. Plast Reconstr Surg 1989; 83:459-67
- Egbert JE, Paul S, Engel WK, Summers CG. High injection pressure during intralesional injection of corticosteroids into capillary hemangiomas. Arch Ophthalmol 2001; 119:677- 83.
- Ezekowitz RA, Mulliken JB, Folkman J. Interferon alfa-2a therapy for life-threatening hemangiomas of infancy. N Engl J Med 1992; 326:1456-63.
- Bauman NM, Burke DK, Smith RJ. Treatment of massive or life-threatening hemangiomas with recombinant alpha(2a)- interferon. Otolaryngol Head Neck Surg 1997; 117:99-110.
- Chang E, Boyd A, Nelson CC, Crowley D, Law T, Keough KM, Folkman J, Ezekowitz RA, Castle VP. Successful treatment of infantile hemangiomas with interferon-alpha- 2b. J Pediatr Hematol Oncol 1997; 19:237-44.
- Boehm DK, Kobrinsky NL. Treatment of cavernous hemangioma with vincristine. Ann Pharmacother 1993; 27:981.
- Enjolras O, Breviere GM, Roger G, Tovi M, Pellegrino B, Varotti E, Soupre V, Picard A, Leverger G. [Vincristine treatment for function- and life-threatening infantile hemangioma] Arch Pediatr 2004; 11:99-107.
DEDICATED TO EXCELLENCE IN DERMATOLOGY
By National Skin Centre (Singapore)
Copyright (C) 1995 - National Skin Centre (Singapore)
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