Congenital Hemangioma

Unlike IHs, congenital hemangiomas are present at birth and do not exhibit postnatal growth (Image 9.3).

Two different lesions are recognized:

§ rapidly involuting congenital hemangioma (RICH)

§ noninvoluting congenital hemangioma (NICH)

Image 9.3 Congenital hemangioma. Most lesions are solitary. There is no gender bias. These lesions are further distinguished from IH by staining negatively for GLUT-1.

RICHs undergo involution early, often beginning before birth. Involution is complete between 6 and 14 months of age. RICHs commonly affect the head/neck and extremities. On physical examination, the lesions are raised, firm, pink to violaceous with central depression or ulceration and a surrounding pale rim. After involution, the residual area often appears deflated and is not accompanied by the usual fatty residuum of IH. Prenatal detection is possible because RICHs arise in utero.

NICHs present as bossed, round-to-ovoid shape lesions in shades of pink to purple. The average diameter is 5 cm. There may be overlying coarse telangiectasia. NICHs most commonly affect the head/neck (43%) followed by the limbs (38%) and trunk (19%). As the name implies, NICHs do not undergo involution and persist essentially unchanged. Although they are tumors histologically, the static behavior of NICHs resembles that of a malformation.

RICHs and NICHs are fast-flow by Doppler evaluation. RICHs exhibit large flow voids near the surface and have areas that enhance inhomogenously on MRI. Arterial aneurysms and direct arteriovenous shunts can be seen on angiography. NICHs and IHs are indistinguishable radiographically. The differential diagnosis of congenital hemangioma includes infantile fibrosarcoma.

Medical treatment is similar to that of infantile hemangiomas.