Strawberry naevus

Synonym: infantile haemangioma

What is strawberry naevus?

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

Natural history¹

• The strawberry naevus may be present at birth, or may develop within the first weeks after birth.

• They may begin as a small flat red area but usually develop into a raised dimpled (strawberry-like) lesion.

• The strawberry naevus typically grow in size as the child grows, until the age of approximately 9 months, when they start to regress spontaneously.

• Involution is usually comlete by 10-12 years of age.

Epidemiology^{2 3}

• Strawberry naevi are the most common vascular tumours of infancy, usually appearing in early infancy.

• Strawberry naevi are very common, occurring in about 5-10% of Caucasian babies.

• Strawberry naevi are rather less common in children of Afro-Caribbean origin and even more uncommon in those of Asian extraction.

• There is a 3:1 female preponderance.

• They can be associated with a low birth weight, prematurity, multiple gestation and chorionic villus sampling.

Presentation⁴

• One third of strawberry naevi will be evident at birth, the remainder developing in the first 4-6 weeks after birth.

• In the initial stages, the strawberry naevus may be bluish or red spots or patches and the superficial lesions may remain flat. Deeper lesions have a bluish appearance. Compound lesions may occur, having both superficial and deep areas.

• The vast majority (around 60%) of strawberry naevi occur on the head and neck but, occasionally, these lesions may occur at other sites throughout the body.
  

  Capillary haemangioma or strawberry birthmark

  

  By Zeimusu, Public domain, via Wikimedia Commons
  

• Around 20% are multiple. If more than five are present, this can indicate internal haemangiomatosis. The liver is the most common site.

• They classically undergo a proliferative period lasting months during young infancy, followed by a longer period of gradual involution throughout childhood.

• The vast majority of strawberry naevi cause no symptoms at all; however, visceral haemangiomas or large superficial haemangiomas may cause problems.

Differential diagnosis

• Angiosarcoma.

• Arteriovenous (AV) malformation.

• Congenital haemangioma (that does not involute).

• Teratoma.

• Venous malformation.

• Diffuse neonatal haemangiomatosis.

Investigations^{1 5}

• Usually, no investigations will be required. The diagnosis is usually made clinically.

• If there is uncertainty about the lesion, MRI and Doppler ultrasound may be used. Doppler may be useful to assess the persistence, increase or resolution of AV fistulae, as this can influence the decision to observe or to treat.

• Imaging with ultrasound or MRI is recommended for those patients with complicated strawberry naevi.

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

• If more than five strawberry naevi are found then an ultrasound of the liver should be undertaken.

Associated diseases

• In approximately 10% of patients strawberry naevi can be complicated, particularly those involving the airway, the oral cavity or the eye⁶ .

• Periorbital strawberry naevi particularly with involvement of the upper eyelid, require refraction with retinoscopy to check for visual disturbances, especially astigmatism, and to prevent visual deprivation amblyopia.

• Rapidly growing strawberry naevi can impinge on vital structures of the head and neck, particularly the airway or auditory canals. Large strawberry naevi of the beard area have a higher incidence of upper airway haemangiomas. This may present as noisy breathing or stridor.

• A strawberry naevus over the midline of the back may be a sign of an underlying occult spinal dysraphism, such as a tethered cord.

• Very rarely, they can be associated with systemic conditions, such as Kassabach-Merritt syndrome (thrombocytopenia) and neurocutaneous PHACE syndrome (a disorder in which a child is born with posterior fossa malformation, cervico-facial haemangiomas, arterial anomalies, cardiac defects and various eye anomalies)⁷ .

Management

• The majority of these strawberry naevus will involute spontaneously over time and will require no treatment² . This is especially true of those in premature babies.

• Some strawberry naevi of the head and neck cause disturbance of the visual axis or airway compromise due to mass effect and therefore require early intervention.

• Currently, propranolol is the preferred treatment for problematic proliferating strawberry naevi. A Cochrane review found that oral propranolol and topical timolol maleate are more beneficial than placebo in terms of clearance or other measures of resolution, or both, without an increase in harms⁸

  • Beta-blockers, usually oral propranolol, can be very effective in treating these lesions⁹ .

  • Treatment with topical beta-blockers, such as topical timolol, have been shown to be an effective treatment¹⁰ .

  • The rapid action of propranolol has been shown to be especially dramatic in strawberry naevi involving dyspnoea, haemodynamic compromise, palpebral occlusion or ulceration¹¹ .

  • The mechanism of effect of beta-blockers is not fully understood.

• Intralesional corticosteroids slow proliferation but are less commonly used now¹² .

• Interferon alfa (IFN-α) is used in some cases¹³ .

• Surgical excision of proliferating strawberry naevi 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 strawberry naevi may cause problems with sight, hearing, breathing, swallowing and speaking. 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.

• Hepatic haemangiomas are associated with high-output cardiac failure that can be life threatening¹⁴ .

• Occasionally, they may impinge on vital structures, ulcerate, bleed, and induce consumptive coagulopathy or significant structural abnormalities. Those on the face are of greatest concern.

Prognosis

• Strawberry naevus are the most common tumours in infancy and usually resolve without any scarring.

• Up to 90% resolve completely by the age of 9 years² .