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C.M. GORST, D.A. MUNNOCH and K. HANCOCK, Laser Suite, Alder Hey Children’s Hospital, Eaton Road, Liverpool, UK
Rapidly proliferating haemangiomas of the face may obscure vision with the development of deprivation amblyopia. Early intervention is required to prevent complications. We present a case successfully treated with a combination of pulsed dye laser and intralesional steroid injection. The current management of haemangiomas is reviewed.
Key words: deprivation amblyopia, haemangioma, intralesional steroids pulsed dye, laser
J.R.Coll.Surg.Edinb., 46, August 2001, 000-000
Haemangiomas are the commonest cutaneous congenital vascular abnormality.1 These lesions have previously been classified as strawberry, capillary, cavernous, juvenile or cellular. Classification was simplified by Mulliken and Glowacki (1982) who proposed that lesions showing endothelial cell proliferation be termed haemangiomas, and that other vascular lesions with a normal endothelial cell cycle be termed malformations.2 Haemangiomas present either at birth or in the first few weeks of life, often preceded by a herald patch. 3-5 They occur more frequently in females with the head being the commonest site.3 They are dynamic cellular lesions which proliferate rapidly over a few weeks, then remain static for some months before gradually regressing over a number of years. They become raised, bulky, compressible lesions, which vary in size from a few millimetres to several centimetres. Some are predominately superficial in appearance, with others demonstrating a much deeper component, often described as compound.
The majority do not cause any problems and simply require to be observed until regression takes place. Ninety per cent of these lesions will regress by the age of 9 years, although this often leaves an area of atrophic skin that requires subsequent surgical excision. However, 10-20% of haemangiomas (described as “alarming” by Enjolras et al (1990)) may threaten function and will require some form of intervention.6 Treatment may be with a laser, steroids (systemic or intralesional), surgery, or a combination of these. 4, 6-10 Interferon a-2a has also been described in the treatment of life-threatening haemangiomas. 11 Indications for early treatment include visual or airway obstruction, ulceration, severe bleeding, or rapid growth. Haemangiomas involving the eyelids and orbit threaten visual function by causing amblyopia, anisometropia, strabismus, proptosis and sensory deprivation.12-14 Deprivation amblyopia will occur in up to 60% of eyelid haemangiomas if there is substantial impairment of the visual field for longer than a week in the first 6 months of life. 7,12,14,15 Proper development of the visual system requires clear retinal stimulation from sharply focused images during this critical period. Early treatment of eyelid haemangiomas has been shown to result in a marked resolution of the deformity with some improvement in visual outcome. 16
An 8-week-old girl was referred to our unit with a large haemangioma involving her right cheek and eyelids (Figure 1). She had been born prematurely at 31 weeks gestation, and was initially treated in the special care baby unit. Although there were no visible marks at birth, a small “pink spot” was observed by the mother after one week. She was reassured by staff that this was nothing serious and would eventually disappear!
In view of the significant size of the lesion with obvious ocular obstruction, combination treatment was commenced within 7 days of referral. Under general anaesthesia, the lesion was treated using a tuneable dye laser (Coherent-Artisan) with a wavelength of 585-nanometers. In addition, the lesion was injected widely with 10 mg of Triamcinolone. Three treatments were given at 4-week intervals with a final treatment 8 weeks later. After two treatments, there was significant regression and the child was able to open her eye fully, thus reducing the risk of deprivation amblyopia (Figure 2).
When reviewed at 18 months, she had normal vision with marked regression of the haemangioma (Figure 3). She will require surgery when older to improve the cosmetic appearance.
More than 50 years ago, it was observed by Lister (1938) that 53% of 92 haemangiomas in 77 patients spontaneously 17 Of the remaining, 41% had begun to regress with 4% being lost to follow up.
The benign history of most ‘strawberry’ haemangiomas has led the majority of clinicians to adopt a policy of non-intervention for virtually all lesions. Although we do observe some of the haemangiomas referred, it is the policy of this unit to treat any lesion which is causing a significant problem, such as rapid growth, bleeding or ulceration. Any facial lesion is also treated.
High dose intralesional or systemic steroid therapy has been reported to arrest growth and to precipitate involution of rapidly enlarging haemangiomas and has become the treatment of choice for lesions threatening respiration, feeding or vision. 6,18,19 The systemic use of steroids requires careful monitoring of the child’s growth, metabolic status and exposure to infective agents. 6,10 Thirty to 60 per cent of haemangiomas are reported to respond well to oral steroids, with 30% showing no response at all. 6,10 A favourable response is most likely during the early proliferative phase, and in superficial lesions. Regrowth of the haemangioma has also been noted after the reduction or termination of therapy.8 Systemic steroids are not routinely used in this unit except in cases where surgical excision is required. High dose steroids (4mg/kg) are given orally over a 6-week period prior to surgery, and the child is carefully monitored throughout.
Intralesional steroid injection is an effective treatment of haemangiomas, with a volume reduction of over 50% being reported in 45-80%, without the problems associated with systemic treatment. 10,16,19,20 Kushner (1985) reported an occular complication (amblyopia or strabismus) rate of 16% following intralesional steroid treatment of peri-orbital haemangiomas. 20 Care must be taken to ensure that retrobulbar injection or haemorrhage does not occur as damage to the optic nerve has been reported. 21 The drug of choice in our unit is Triamcinolone, with a maximum of 20 mgs being given at each treatment. This is injected directly into the lesion and repeated at 4-weekly intervals for a maximum of three treatments. Recent research into the mechanism of action of intralesional triamcinolone has demonstrated an increase in mast cell numbers within the haemangioma, with decreased transcription of the growth factors which play a role in cell proliferation and angiogenesis (platelet-derived growth factor-A and -B, interleukin-6, transforming growth factor-ß1 and -ß3). This research also demonstrated increased expression of the mitochondrial cytochrome b gene, which may have a role in promoting steroid -induced involution of a haemangioma. 22
Laser treatment of haemangiomas has been studied for a number of years, with early intervention being advocated by several authors. 8,9,23 Pulsed dye lasers produce laser light in the 585-nanometer wavelength, which is absorbed by haemoglobin. The heat exchange results in destruction of the red cells and surrounding blood vessels, without significant damage to the overlying skin. Unfortunately, the penetration depth is very small, making treatment of deeper lesions much more difficult. 8,23 It has previously been reported that treatment of periorbital haemangiomas with a dye laser does not prevent visual obstruction, with intralesional steroids producing a more dramatic response. 23 Intralesional laser photocoagulation using a neodymium:yttrium-aluminum-garnet (Nd:YAG) bare fibre with adjuvant steroid therapy and/or selective embolization has also been described. This involves inserting the bare laser fibre deeply into the haemangioma, creating several areas of coagulation necrosis, producing up to 50% shrinkage of the lesion. 24
In our unit, laser treatment (using an Argon-pumped timeable dye laser (Coherent-Artisan, Coherent (UK) Ltd)) is carried out under a general anaesthetic every 4 weeks, with intra-lesional steroids being injected simultaneously. We believe this deals effectively with both the superficial and deeper elements of the haemangioma, thus, resulting in more rapid involution and better cosmesis.
Our experience in this case and others demonstrates that future treatment of large compound haemangiomas could involve combination therapy with both intralesional steroids and lasers. Early referral will ensure effective treatment before these proliferative lesions become disfiguring. Prevention of amblyopia is imperative and we are pleased to report the development of normal vision in this case.
Figure 1: The initial appearance of the haemangioma
Figure 2: The appearance after two treatments
Figure 3: The appearance at 18 months regressed within 5 years.
1. Jacobs AH, Walton RG. The incidence of birthmarks in the neonate. Pediatrics 1976;
58: 218-22
2. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and
children: a classification based on endothelial characteristics. Plast Reconstr
Surg1982; 69: 412-20
3. Simpson JR. Natural history of cavernous haemangiomata. Lancet 1959; 2: 1057-9
4. Achauer BM, Chang C-J, Vander Kam VM. Management of hemangioma of infancy: review of
245 patients. Plast Reconstr Surg 1997; 99: 1301-8
5. Hidano A, Nakajima S. Earliest features of the strawberry mark in the newborn. Br J
Derm 1972; 87: 138-44
6. Enjolras O, Riche MC, Merland JJ, Escande JP. Management of alarming hemangiomas in
infancy: a review of 25 cases. Pediatrics 1990; 85: 491-8
7. Thomson HG, Ward CM, Crawford JS, Stigmar G. Hemangiomas of the eyelid: visual
complications and prophylactic concepts. Plast Reconstr Surg 1979; 63: 641-7
8. Waner M, Suen JY, Dinehart S. Treatment of hemangiomas of the head and neck. Laryngoscope
1992; 102: 1123-32
9. Landthaler M, Hohenleutner U, Abd El-Raheem T. Laser therapy of childhood haemangiomas.
Br J Derm 1995; 133: 275-81
10. Gangopadhyay AN, Sinha CK, Gopal SC, Gupta DK, Sahoo SP, Ahmad M. Role of steroid in
childhood haemangioma: a 10 years review. Int Surg 1997; 82: 49-5
11. Ezekowitz RAB, Mulliken JB, Folkma J. Interferon alfa-2a therapy for life-threatening
hemangiomas of infancy. N Engl J Med 1992; 326: 1456-63
12. Stigmar G, Crawford JS, Ward CM, Thomson HG. Ophthalmic sequelae of infantile
hemangiomas of the eyelids and orbit. Am J Ophthalmol 1978; 85: 806-13
13. Boyd MJ, Collin JRO. Capillary haemangiomas: an approach to their management. Br J
Opthalmol 1991; 75: 298-300
14. Haik GR, Jakobiak FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lids and
orbit: an analysis of the clinical features and therapeutic results in 101 cases. Ophthalmology
1979; 86: 760-89
15. Robb RM. Refractive errors associated with hemangiomas of the eyelids in infancy. Am
J Ophthalmol 1977; 83: 52-8
16. Morrell AJ, Willshaw HE. Normalisation of refractive error after steroid injection for
adnexal haemangiomas. Br J Ophthalmol 1991; 75: 301-5
17. ListerWA.Naturalhistoryofstrawberrynaevi.Lancet1938;1:1429-34
18. Zarem HA, Edgerton MT. Induced resolution of cavernous hemangiomas following
prednisolone therapy. Plast Reconstr Surg 1967; 39: 76-83
19. Sloan GM, Reinisch JF, Nichter LS, Saber WL, Lew K, Morwood DT. Intralesional
corticosteroid therapy for infantile hemangiomas. Plast Reconstr Surg 1989; 83: 459-66
20. Kushner BJ. The treatment of periorbital infantile hemangioma with intralesional
corticosteroid. Plast Reconstr Surg 1985; 76: 517-24
21. Ellis PP. Occlusion of the central retinal artery after retrobulbar corticosteroid
injection. Am J Ophthalmol 1978; 85: 352-6
22. Hasan Q, Tan ST, Gush J, Peters SG, Davis PF. Steroid therapy of a proliferating
hemangioma: histochemical and molecular changes. Pediatrics 2000; 105: 117-21
23. Scheepers JH, Quaba AA. Does the pulsed tunable dye laser have a role in the
management of infantile hemangiomas? Observations based on 3 years’ experience. Plast
Reconstr Surg 1995; 95: 305-12
24. Apfelberg DB. Intralesional laser photocoagulation-steroids as an adjunct to surgery
for massive hemangiomas and vascular malformations. Ann Plast Surg 1995; 35: 144-9
Copyright date: 21st February 2001
Correspondence: Ms CM Gorst, Laser Suite, Alder Hey Children’s Hospital, Eaton
Road, Liverpool L12 2AP, UK
©2001 The Royal College of Surgeons of Edinburgh, J.R.Coll.Surg.Edinb.