Segmental Neurovascular Syndromes in Children
Section snippets
General concepts: neural crest, cephalic migration, and the arterial-veno-lymphatic tree
The neural crest and neural plate share a common lineage. Cells of the lateral border of the developing neural plate (ie, the neural folds), under the inductive influence of the adjacent epithelium and possibly the mesoderm, develop into crest cells; hence, their common metameric origin with the cells of the hindbrain [9], [10]. Moreover, it appears that all neural plate cells can become crest cells given the appropriate signals, and vice versa, and even epithelial cells can, in vitro,
Cerebrofacial and spinal arteriovenous metameric syndromes
The association of AVMs of the brain, the orbit (retinal or retrobulbar lesions), and the maxillofacial region was named originally after Bonnet-Dechaume-Blanc and Wyburn-Mason. Because of the previously mentioned metameric concept of neural crest development, the authors have proposed a rational classification reflecting the putative underlying disorder, and have coined the acronym CAMS. Depending on the involved structures, several CAMS can be differentiated: CAMS 1 as a midline
Cerebrofacial venous metameric syndromes
Encephalotrigeminal angiomatosis or Sturge-Weber syndrome (SWS) is a nonfamilial disease with a skin discoloration (port wine stain) in the V1 territory, associated with a calcified leptomeningeal venous malformation of the ipsilateral supratentorial hemisphere [30]. Symptoms appear before the second year of age and include cosmetic and neurologic problems related to subjacent cerebral atrophy, leading to epilepsy, deficits, and mental retardation. Port wine stains that represent localized
PHACES
PHACES is a rare, congenital, syndromal pediatric disorder with less than 150 cases reported in the literature and a broad spectrum of clinical manifestations [8], [21], [37], [38], [39], [40], [41], [42], [43], [44], [45]. As mentioned previously, its characteristics are posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormalities, and sternum/abdominal raphe defects. Recently, arterial intracranial stenoses have been added as an
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