SPR Unknown #82 -- FINAL
1549 PHACES Facial Hemangiomas Ocular hemangiomas Intracranial Vascular Malformations
Publication Date: 2009-08-20
Case #1-2 month old male with ocular and facial hemangiomas
Case #2-3 year old female with lip, eye and nose hemangiomas
Facial Hemangiomas associated with PHACES syndrome
Differental Diagnosis of Facial Hemangiomas:
Hemangiomas are the most common benign tumor of infancy, diagnosed in 4-10% of population. Segmental facial hemangiomas may be part of a more complex syndrome of multiple malformations known as PHACES. This syndrome has a broad definition that is currently undergoing revision, but refers to associated Posterior fossa, Hemangioma, Arterial, Cardiac, Eye, and Sternal
The most commonly affected dermatome in PHACES syndrome is cranial nerve V. Associated structural brain anomalies include Dandy-Walker Malformation, agenesis or hypoplasia of the corpus callosum and the cerebellum. Associated intracranial vessel abnormalities include stenosis, absence, hypoplasia or occlusion of the internal carotid arteries, intracranial aneurysms, and aberrant vasculature course. Coarctation of the aorta is the most common cardiac anomaly. Ocular manifestations include Horner's syndrome and increased retinal vascularity.
There are two phases of evolution of the cutaneous hemangiomas, proliferative and involution. The proliferative phase involves increase in size of soft tissue mass, reddish cutaneous discoloration of lesions, overlying bluish coloration of skin due to draining veins, possible ulceration of lesions, and proptosis of orbital lesions. The involution phase involves spontaneous involution of lesions over several years. Cutaneous lesions are usually not treated unless symptomatic or for cosmetic reasons.
- Antaya, RJ MD. Infantile Hemangioma. E Medicine article 3/29/2007.
- Chuch, DG MD., Lowe, LH MD. PHACE Syndrome. December 2006 Radiology, 241, 939-942.
- Heyer, GL et. Al. The Cerebral Vasculopathy of PHACES Syndrome. Stroke. 2008;39:308-316
- Judd, CD, Chapman PR, Koch, B, Shea, CJ. Intracranial Infantile Hemangiomas Associated With PHACE Syndrome. American Journal of Neuroradiology 28:25-29, January 2007