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Strawberry NaevusSynonym: infantile haemangioma

Strawberry naevus is the name given to an haemangioma which occurs in infancy, usually on the face.

Natural history

The lesions may be present at birth, or may develop in the first few weeks after birth.They may begin as a small flat red area, but usually develop into a raised dimpled (strawberry-like)lesion.The lesions typically grow in size as the child grows, until the age of approximately 3-4 years, whenthey start to regress spontaneously.This process can take up to 10 years. [1]

Epidemiology

There are currently no recent published prospective studies examining the incidence ofhaemangiomas. Olderstudies were performed before the discrimination of 'haemangiomas' from other vascular birthmarks was well-established.[2]

Strawberry naevi are very common, occurring in about 3-5% of babies.They are rather less common in children of Afro-Caribbean origin and even more uncommon in thoseof Asian extraction.There is a 3:1 female preponderance. [3]

In children with a birthweight of less than 1 kg, there is a 1 in 4 chance that they will develop a

haemangioma. [4] In this group, the sex ratio is equal. [3]

Presentation

One third of strawberry naevi will be evident at birth, the remainder developing in the first 4 weeks afterbirth.In the initial stages, the lesions may be bluish or red spots or patches, and the superficial lesions mayremain flat. Deeper lesions have a bluish appearance. Compound lesions may occur, having bothsuperficial and deep areas.The vast majority of strawberry naevi occur on the head and neck but, occasionally, these lesions mayoccur at other sites throughout the body.

The vast majority of strawberry naevi cause no symptoms at all, but visceral haemangiomas, or largesuperficial haemangiomas may cause problems.

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Differential diagnosis

Angiosarcoma.Arteriovenous (AV) malformation.Congenital haemangioma (that does not involute).Teratoma.Venous malformation.

Diffuse neonatal haemangiomatosis.

Investigations

Usually, no investigations will be required but, if an infant has 3 or more visible strawberry naevi, awhole body MRI scan should be performed to rule out internal lesionsIf there is uncertainty about the lesion, MRI and Doppler ultrasound may be used. Doppler may beuseful to assess the persistence, increase or resolution of AV fistulae as this can influence thedecision to observe or to treat. [5]

Biopsy, if required, should be undertaken with caution, as these lesions are very vascular.

Associated diseases

Periorbital haemangiomas, particularly with involvement of the upper eyelid, require refraction withretinoscopy to check for visual disturbances, especially astigmatism, and to prevent visual deprivationamblyopia.

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Rapidly growing haemangiomas can impinge on vital structures of the head and neck, particularly theairway or auditory canals. Large haemangiomas of the beard area have a higher incidence of upperairway haemangiomas. This may present as noisy breathing orstridor.An infantile haemangioma over the midline of the back may be a sign of an underlying occult spinaldysraphism, such as a tethered cord. MRI is indicated, or ultrasound, if the infant is younger than 5months.

Management

The majority of these lesions will involute spontaneously over time and will require no treatment. Thisis especially true of those in premature babies. [4]

Occasionally, plastic surgery will be required following involution of the lesion, in order to removeredundant folds of floppy skin.Pulsed dye laser may be valuable if they are not resolving well, or are in places that cause particularembarrassment:

It is used to treat ulcerating haemangiomas.Laser treatment gives a better outcome than observation. [3] Sometimes treatment causespain.Not all children need active treatment. [6]

A number of drugs have been used to treat proliferation of these tumours when the lesion is severe orcausing medical problems:Intralesional corticosteroids slow proliferation.Those who fail to respond to steroids may respond to interferon-alfa 2a, but it can havetoxic effects in some and spastic diplegia has been described.[7] [8]

Imiquimod, an immune response modifier, has also been used. Such treatment is generallyreserved for dangerous and proliferating lesions. [9] It has been shown to be moreefficacious in superficial lesions. [10]

Propranolol has recently been found to be an effective treatment. Its mechanism of actionfor haemangiomas remains unclear. Vasoconstriction, down-regulation of angiogenicfactors such as VEGF and bFGF and up-regulation of apoptosis of capillary endothelialcells may be responsible. [11] [12]

Surgical excision of proliferating haemangiomas needs care as they are very vascular.

Larger, visceral or life-threatening lesions may be treated with a combination of surgery and embolisation therapy.

Complications

Large facial haemangiomas may cause problems with sight, hearing, breathing, swallowing andspeaking. They should be treated immediately to prevent further problems.Bleeding or ulceration may occur.Visceral haemangiomas may produce symptoms due to bleeding or mass effect. The symptoms will

vary according to site.Occasionally, they may impinge on vital structures, ulcerate, bleed, induce consumptive coagulopathy,or cause high-output cardiac failure or significant structural abnormalities. Those on the face are ofgreatest concern.

Prognosis

They are the most common tumours in infancy and usually resolve without problem.

Further reading & references

Antaya RJ; Infantile Hemangioma, eMedicine, Sep 2010

1. Kane WJ, Morris S, Jackson IT, et al; Significant hemangiomas and vascular malformations of the head and neck: clinicalmanagement and treatment outcomes. Ann Plast Surg. 1995 Aug;35(2):133-43.

2. Kilcline C, Frieden IJ; Infantile hemangiomas: how common are they? Asystematic review of the medical literature. PediatrDermatol. 2008 Mar-Apr;25(2):168-73.

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http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=9105356http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=18429772http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Abstract&list_uids=7486734http://www.emedicine.com/derm/topic201.htmhttp://www.patient.co.uk/search.asp?searchterm=DISSEMINATED+INTRAVASCULAR+COAGULATION&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=IMIQUIMOD&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=SPASTIC+PARAPLEGIA&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=INTERFERONS&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=CORTICOSTEROIDS&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=PLASTIC+SURGERY&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=SPINA+BIFIDA&collections=PPsearchhttp://www.patient.co.uk/search.asp?searchterm=STRIDOR&collections=PPsearch

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3. Achauer BM, Chang CJ, Vander Kam VM; Management of hemangioma of infancy: review of 245 patients. Plast ReconstrSurg. 1997 Apr;99(5):1301-8.

4. Amir J, Metzker A, Krikler R, et al; Strawberry hemangioma in preterm infants. Pediatr Dermatol. 1986 Sep;3(4):331-2.5. Bingham HG; Predicting the course of a congenital hemangioma. Plast Reconstr Surg. 1979 Feb;63(2):161-6.6. Michel JL; Treatment of hemangiomas with 595 nm pulsed dye laser dermobeam. Eur J Dermatol. 2003 Mar-

Apr;13(2):136-41.7. Dubois J, Hershon L, Carmant L, et al;Toxicity profile of interferon alfa-2b in children: Aprospective evaluation. J Pediatr.

1999 Dec;135(6):782-5.8. Michaud AP, Bauman NM, Burke DK, et al ; Spastic diplegia and other motor disturbances in infants receiving interferon-

alpha. Laryngoscope. 2004 Jul;114(7):1231-6.9. Welsh O, Olazaran Z, Gomez M, et al; Treatment of infantile hemangiomas with short-term application of imiquimod 5%

cream. J Am Acad Dermatol. 2004 Oct;51(4):639-42.10. Ho NT, Lansang P, Pope E; Topical imiquimod in the treatment of infantile hemangiomas: a retrospective study. J Am Acad

Dermatol. 2007 Jan;56(1):63-8.11. Leaute-Labreze C, Dumas de la Roque E, Hubiche T, et al; Propranolol for severe hemangiomas of infancy. N Engl J Med.

2008 Jun 12;358(24):2649-51.12. Zimmermann AP, Wiegand S, Werner JA, et al ; Propranolol therapy for infantile haemangiomas: review of the literature. Int

J Pediatr Otorhinolaryngol. 2010 Apr;74(4):338-42. Epub 2010 Feb 1.

Original Author: Dr Hayley Willacy Current Version: Dr Gurvinder Rull Peer Reviewer: Dr Hannah Gronow

Last Checked: 19/07/2012 Document ID: 1580 Version: 23 EMIS

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