Infantile hemangioma - External and Internal Eye
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, though 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 types are described: rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH). The proliferative phase in these tumors occurs in utero, and typically they 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 than RICH. Histologically, they are GLUT-1 negative, which helps to differentiate them from other hemangiomas.
- Port-wine stain – Early lesions of hemangioma 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 that are usually present in the head and neck region.
- Rhabdomyosarcoma – Soft tissue sarcoma of skeletal muscle origin typically seen in the first and second decades of life. In children, the head and neck areas are affected with an asymptomatic, rapidly growing, reddish cutaneous mass.
- Deep hemangiomas sometimes look similar to venous malformations, which do not go through a phase of rapid proliferation. Phleboliths are often seen in venous malformations. Magnetic resonance (MR) imaging will help to differentiate these two entities.
- Congenital hydrops of the nasolacrimal sac
- Dermoid cyst
- Orbital cellulitis