Refining the clinicopathological pattern of cerebral proliferative glomeruloid vasculopathy (Fowler syndrome): report of 16 fetal cases
B Bessieres-Grattagliano, B Foliguet, L Devisme… - European journal of …, 2009 - Elsevier
Cerebral proliferative glomeruloid vasculopathy (PGV) is a severe disorder of brain
angiogenesis, resulting in abnormally thickened and aberrant perforating vessels, forming
glomeruloids with inclusion-bearing endothelial cells. This peculiar vascular malformation
was delineated by Fowler in 1972 as a stereotyped lethal fetal phenotype associating
hydranencephaly–hydrocephaly with limb deformities, called Fowler syndrome (FS) or
“proliferative vasculopathy and hydranencephaly-hydrocephaly” or “encephaloclastic …
angiogenesis, resulting in abnormally thickened and aberrant perforating vessels, forming
glomeruloids with inclusion-bearing endothelial cells. This peculiar vascular malformation
was delineated by Fowler in 1972 as a stereotyped lethal fetal phenotype associating
hydranencephaly–hydrocephaly with limb deformities, called Fowler syndrome (FS) or
“proliferative vasculopathy and hydranencephaly-hydrocephaly” or “encephaloclastic …
