Benign proliferative lesions of vascular origin, commonly presenting at birth and may become more apparent later in life.
- M/C benign orbital neoplasms in children
History:
Initially, these vascular anomalies were classified as angiomas by John Mulliken and Julie Glowacki, as early as 1982: they were thought to be proliferating lesions with an independent life cycle. However, these classifications have undergone several revisions. Capillary hemangiomas have been reclassified as benign vascular neoplasms as per the revised International Society for the study of vascular anomalies (ISSVA).
Aetiology
Risk factors:
- Prematurity
- Low birth weight
Syndromic assocaitions:
- Kasabach – Merritt syndrome
- PHACES syndrome (Posterior fossa anomalies, Hemangiomas, Arterial anomalies, Cardiac anomalies, and Eye anomalies)
Pathophysiology
Proliferative phase (3-12 months of age):
Endothelial cells undergo rapid proliferation causing new vessel growth, and the lesions become nodular with a scarlet-colored hue: thus the name ‘strawberry nevus.’
- Strawberry nevus
Involutional phase (3 years of age):
Spontaneous involution of the lesions
- Endothelial proliferation comes to a halt, and the lesions are replaced by fibrous tissue
Clinical features
Superficial lesions
Bright red, nodular masses typically involving the eyelids. Lesions below the dermis tend to have a deep blue to purple hue.
- Skin discoloration or eyelid mass
- Mechanical ptosis obscuring the visual axis and producing astigmatism and amblyopia.
Deep lesions:
Invisible to the naked eye
- Gradually progressive proptosis
- Strabismus
- Decreased visual acuity (due to optic nerve compression)
Mixed lesions:
Consist of both superficial and deep components.
Characteristic clinical feature of capillary hemangiomas is an increase in size or change in color to dark blue when the child cries or strains which are often noted by the parents. This is due to the increased accumulation of deoxygenated blood.
Diagnosis
Ultrasound:
- Irregular lesions in the orbit demonstrating high internal reflectivity with irregular acoustic structures
Computed Tomography (CT) scan:
- Well-defined to irregular pre/post-septal, intra/extra-conal heterogenous soft tissue masses which enhance with contrast
Magnetic resonance imaging (MRI):
- Well/ill-defined lesions hypointense on T1 weighted images and hyperintense on T2 weighted images.
- Fflow voids within the lesion (characteristic)
Differential diagnosis:
- Lymphangioma
- Arterio-venous malformations
- Mucocele or meningocele
- Rhabdomyosarcoma
Management
β-blockers:
Mainstay of treatment: Suppress vascular endothelial growth factors (VEGF) and fibroblast growth factors (FGF) which are responsible for proliferation
- Oral Propranolol
- Topical Timolol (superficial lesions only)
Corticosteroids:
effective only during the early proliferative phase and are associated with significant side effects
Immunomodulators:
Steroid-resistant cases
- Interferon α
- Vincristine
- Cyclophosphamide
Pulsed – dye lasers:
Treatment of superficial lesions
Surgery:
Reserved only for large vision-threatening lesions
- Debulking of the lesion