Infantile hemangioma - External and Internal Eye
Usually, infantile hemangiomas are noticed at approximately 2-3 weeks of life. The initial proliferative phase usually lasts for about 1 year, with rapid growth during the first 4 months. This is followed by gradual involution over several years, with > 90% completely involuting by age 10. Although infantile hemangiomas involute with time, residual skin changes, such as telangiectasia, scarring, atrophy and fibrosis, persist in up to 30% of lesions after involution is complete.
Children with eyelid and/or orbital hemangiomas are at high risk (greater than 50%) of developing visual impairment. When around the eye, the exact location and extension should be determined with either Doppler ultrasonography, MRI, and/or CT. Extraconal (posterior to the anterior margins of the bony orbit but outside the extraocular muscle cone), or intraconal (extending within the extraocular muscle cone) hemangiomas are more likely to be associated with visual impairment. Infants with palpebral hemangiomas (anterior to the ocular globe) are less likely to develop visual impairment; however, amblyopia and/or astigmatism can occur in about 30% of these infants.
In adults, hemangiomas occur most often in the fourth and fifth decades of life with a strong preference for females. The most common symptom is painless proptosis, although pain, eyelid swelling, and diplopia have been reported.
D18.01 – Hemangioma of skin and subcutaneous tissue
83343001 – Infantile hemangioma
- Congenital hemangioma (CH) – Fully formed vascular tumors at birth; two main types are: rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH), and variants have been reported. The proliferative phase in these tumors occurs in utero, and tumors typically do not proliferate postnatally as seen with infantile hemangiomas. CHs are characterized by hemispheric pink to purple vascular plaques with overlying telangiectasia and the characteristic peripheral rim of pallor. Both RICH and NICH may look similar; however, NICH is less impressive and flatter. Histologically, they are GLUT-1 negative, which helps to differentiate them from other hemangiomas.
- Capillary malformation or "port-wine stain" – Early superficial or segmental infantile hemangiomas, or their precursor lesions, may be confused with port-wine stains, which are capillary malformations present at birth. They are pink or red patches that persist throughout life and do not have the typical growth phase of hemangiomas.
- Sometimes tufted angioma and kaposiform hemangioendothelioma may mimic hemangiomas. They present as extensive brownish vascular plaques with deep fibrotic consistency.
- Infantile myofibromatosis – Fibrohistiocytic proliferations characterized by skin-colored to purple, firm to hard or rubbery nodules.
- Rhabdomyosarcoma – Soft tissue sarcoma of skeletal muscle origin typically seen in the first and second decades of life. In children, this presents as an asymptomatic, rapidly growing, red, firm cutaneous mass.
- Deep infantile hemangiomas sometimes look similar to venous malformations, which do not go through a phase of rapid proliferation. Phleboliths are often seen in venous malformations. Bedside Doppler study or ultrasound imaging can help to differentiate these two entities.
- Arteriovenous malformation
- Congenital hydrops of the nasolacrimal sac
- Dermoid cyst
- Orbital cellulitis