The association of extracranial and intracranial vascular malformations in children.

This review is an attempt to relate the types of angiomas and other cutaneous vascular lesions appearing in the face with abnormalities in the formation of cerebral vessels including arteries at their origin, veins and venous sinuses, and with other less constant alterations that affect other organs, particularly the heart and cerebellum. After angiographic study of a series of patients exhibiting diverse types of vascular nevi (capillary and cavernous hemangiomas, nevus flammeus, and lymphangiomas), a correlation was established for each type of dermal nevus with a corresponding group of intracranial vascular anomalies. An explanation of these malformations based on the embryological development of the principal cerebral vessels during the 7th and 8th weeks of gestation is offered. Involvement of the facial and scalp dermatome innervated by the first sensory trigeminal branch correlates most consistently with the presence of abnormal cerebral vessels. The capillary hemangioma occurs preferentially in girls and frequently is associated with developmental anomalies of major cerebral arteries characterised by the absence of some adult vessels and the persistence of others that should have disappeared during embryogenesis such as the fetal trigeminal artery. The complete angiographic exploration of the cerebral vessels from their origin in the aortic arch to the phase of intracranial venous evacuation may reveal unexpected important findings, and is recommended for children with major facial vascular nevi, even if they are neurologically asymptomatic.