gargoyle face
gargoyle face
The characteristic facies of gargoylism, formally, mucopolysaccharidoses–MPS; the classic gargoyle face is seen in MPS type.I-H/Hurler syndrome and MPS type IV/Morquio syndrome and characterized by thickening and coarsening of facial features due to subcutaneous deposition of MPSs, most commonly seen after the first yr of age; the head is large and dolichocephalic, with frontal bossing and prominent sagittal and metopic sutures, with mid-face hypoplasia, depressed nasal bridge, flared nares, and a prominent lower1⁄3 of face, thickened facies, widely spaced teeth and attenuated dental enamel, gingival hyperplasia.